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CO LO R ATLAS & SYNO PSIS OF

CLINICAL OPHTHALMOLOGY
W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s
S ECON D EDITION
EDITOR
Robert B. Penne, MD
Director and Attending Surgeon,
Oculoplastics Service Co-Director,
Ocular Cicatricial Pemphigoid Clinic
Wills Eye Institute
Philadelphia, Pennsylvania

SERIES EDITOR
Christopher J. Rapuano, MD
Director and Attending Surgeon, Cornea Service
Co-Director, Refractive Surgery Department
Wills Eye Institute
Professor of Ophthalmology
Jefferson Medical College of Thomas Jefferson University
Philadelphia, Pennsylvania
CO LO R ATLAS & SYNO PSIS OF
CLINICAL OPHTHALMOLOGY
W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s
S ECON D EDITION
Senior Executive Editor: Jona han W. Pine, Jr.
Senior Product Managers: Emilie Moyer and Grace Capu o
Senior Manufacturing Coordinator: Benjamin Rivera
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Creative Director: Doug Smock
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10 9 8 7 6 5 4 3 2 1
To Devany, Daniel, and Mara—
the source of pride and balance in my life
Abou he Series
he beau y o he a las/ synopsis concep is T e goal o he series is o provide an up- o-
he power ul combina ion o illus ra ive da e clinical overview o he major areas o
pho ographs and a summary approach o he oph halmology or s uden s, residen s, and
ex . Oph halmology is a very visual discipline prac i ioners in all he heal h care pro essions.
ha lends i sel nicely o clinical pho ographs. T e abundance o large, excellen -quali y pho-
While he seven oph halmic subspecial ies in ographs and concise, ou line- orm ex will
his series—cornea, re ina, glaucoma, ocu- help achieve ha objec ive.
loplas ics, neurooph halmology, uvei is, and
pedia rics—employ varying levels o visual
recogni ion, a rela ively s andard orma or Chris opher J. Rapuano, MD
he ex is used or all volumes. Series Editor

vi
Pre ace
his ex is aimed a assis ing physicians condi ions. Once hese condi ions are recog-
(oph halmologis s and nonoph halmolo- nized, he ex describes o her es s ha may
gis s) in recognizing he mos common oculo- be needed and he di eren ial diagnoses ha
plas ic condi ions. Many oculoplas ic condi ions should be considered. T e managemen op ions
can be diagnosed on simple visual examina ion, or hese condi ions are also described.
which makes his a las an ideal resource o have
in emergency depar men s and in he of ce.
I provides a solid basis o pho ographic and Rober B. Penne, MD
descrip ive in orma ion o diagnose oculoplas ic Editor

vii
Acknowledgmen s
S
pecial hank o my colleagues who provided assis ance: Edward Bedrossian, MD; Jurij
Bilyk, MD; Richard Her le, MD; Ka e Lane, MD; and Mary A S e anyszyn, MD.

viii
Con en s
Abou he Series vi
Pre ace vii
Acknowledgmen s viii

SECT IO N I: EYELIDS
Ch a pt er 1 Benign Eyelid Lesions 2
Papilloma 2
Seborrheic Kera osis 4
Cu aneous Horn 6
Epidermal Inclusion Cys 8
Molluscum Con agiosum 10
Xan helasma 12
Syringoma 14
Apocrine Hydrocys oma 16
richoepi helioma 18
Nevi (Nevocellular Nevi) 20
Hemangioma o he Eyelid (Cherry Angioma) 22

Ch a pt er 2 Eyelid Inf ammation 24


Chalazion 24
Hordeolum 26
Floppy Eyelid Syndrome 28

Ch a pt er 3 Eyelid Neoplasms 30
Kera oacan homa 30
Ac inic Kera osis 32
Len igo Maligna 34
Basal Cell Carcinoma 36
Squamous Cell Carcinoma 40
Sebaceous Adenocarcinoma 42
Malignan Melanoma 44
Kaposi’s Sarcoma 46

Ch a pt er 4 Eyelid rauma 48
Marginal Eyelid Lacera ion 48
Canalicular Eyelid Lacera ion 50
Dog Bi es 52
Eyelid Burns 54

ix
x CO NT ENTS

Ch a pt er 5 Eyelid Malpositions 56
En ropion 56
Acu e Spas ic En ropion 56
Involu ional En ropion 58
Cica ricial En ropion 60
Ec ropion 62
Involu ional Ec ropion 62
Paraly ic Ec ropion 64
Cica ricial Ec ropion 66
Mechanical Ec ropion 68
Symblepharon 70
richiasis 72
P osis 74
Congeni al Myogenic P osis 74
Acquired Myogenic P osis 76
Aponeuro ic P osis 78
Neurogenic P osis 80
T ird Nerve Palsy 80
Myas henia Gravis 82
Marcus Gunn Jaw-Winking Syndrome 84
Horner’s Syndrome 88
Mechanical P osis 90
rauma ic P osis 92
Pseudop osis 94
Brow P osis 96
Derma ochalasis 98
Blepharochalasis 100
Eyelid Re rac ion 102
Eyelid Dyskinesis 104
Benign Essen ial Blepharospasm 104
Hemi acial Spasm 106

Ch a pt er 6 Congenital Eyelid Anomalies 108


Blepharophimosis 108
Epican hus 110
Epiblepharon 112
Congeni al En ropion 114
Congeni al Coloboma 116
Congeni al Dis ichiasis 118
Ankyloblepharon 120

Ch a pt er 7 Miscellaneous Eyelid Conditions 122


Ocular Cica ricial Pemphigoid 122
CO NTENTS xi

SECT
T IO N II: L
LACRIMAL
ACR
R I M AL
LAAPPARAT
PP
PAR
R AT
T US
S
Ch a pt er 8 Lacrimal Obstructions 126
Congeni al Obs ruc ions 126
Congeni al Nasolacrimal Duc Obs ruc ion 126
Dacryocys ocele 128
Lacrimal Fis ula 129
Acquired Obs ruc ions 130
Acquired Nasolacrimal Duc Obs ruc ion 130
Canalicular Obs ruc ion 132

Ch a pt er 9 Lacrimal In ections 134


Dacryocys i is 134
Canaliculi is 137

Ch a pt er 10 Lacrimal Sac umors 140

SECT
S ECT
T IO N III:
III I : T H E O R
RBIT
BIT
T
Ch a pt er 11 Orbital In ections 142
Orbi al Celluli is 142
Orbi al Abscess 146
Phycomycosis (Mucormycosis) 150
Aspergillosis 152

Ch a pt er 12 Orbital Inf ammation 154


T yroid-Rela ed Oph halmopa hy 154
Idiopa hic Orbi al In amma ion (Orbi al Pseudo umor) 160
Sarcoidosis 164
Wegener’s Granuloma osis 168

Ch a pt er 13 Congenital Orbital Anomalies 170


Microph halmos 170

Ch a pt er 14 Orbital Neoplasms 174


Congeni al Orbi al umors 174
Dermoid Cys s 174
Lipodermoids 178
Vascular Orbi al umors 180
Capillary Hemangiomas 180
Cavernous Hemangiomas 184
Lymphangiomas 188
xii CO NTENTS

Hemangiopericy oma 192


Orbi al Varices 196
Ar eriovenous Mal orma ions 200
Neural umors 204
Op ic Nerve Gliomas 204
Neuro bromas 208
Meningiomas 210
Schwannomas 218
Mesenchymal umors 222
Rhabdomyosarcoma 222
Fibrous His iocy oma 226
Lymphoproli era ive umors 228
Lymphoid Hyperplasia and Lymphomas 228
Plasmacy oma 232
His iocy ic Disorders 236
Lacrimal Gland umors 240
Epi helial umors o he Lacrimal Gland 240
Miscellaneous Orbi al umors 246
Secondary Orbi al umors 246
Me as a ic Orbi al umors 252

Ch a pt er 15 Orbital rauma 258


Orbi al Frac ures 258
Orbi al Floor Frac ure 258
Medial Wall Frac ure 262
Orbi al Roo Frac ure 266
Zygoma ic Frac ure 268
Miscellaneous rauma 272
Orbi al Hemorrhage 272
Orbi al Foreign Bodies 276
Mucocele 282

Index 285
CO LO R ATLAS & SYNO PSIS OF
CLINICAL OPHTHALMOLOGY
W i l l s Ey e I n s t i t u t e

Oc u lo p la s tic s
S ECON D EDITION
C H AP T ER

Benign Eyelid Lesions

PAPILLOMA papillomas ( Fig. 1-1A & B). T e lesion is


of en cons ric ed a he base.

A papilloma is a common benign, of en


asymp oma ic, skin lesion ha occurs
mos commonly in he in er riginous areas
Size ranges rom less han 1 mm o 10 mm.

Special Considerations
(axillae, in ramammary, and groin) bu is also May grow or become more numerous
commonly seen on he neck and eyelids. during pregnancy
Papillomas are of en numerous on he eye-
More common in obese pa ien s
lids when presen , and he number ends o
increase wi h age. Dif erential Diagnosis
Synonyms: skin ag, acrochordon. Peduncula ed seborrheic kera osis
Epidemiology and Etiology Dermal nevus
Age: More common in middle-aged and Soli ary neuro broma
elderly people Molluscum con agiosum
Gender: More common in emales Conjunc ival papillomas ( Fig. 1-1C) can
E iology: Unknown appear on he eyelid margin bu have a di er-
en appearance and he base o he lesion is
History rom he conjunc ival sur ace.
Mos commonly asymp oma ic bu may
become ender af er rauma. Treatment
Wi h ime, lesions may become crus ed or Excision by simply snipping he lesion a
hemorrhagic. he base

Examination Prognosis
Lesions are sof ; skin-colored, an, Excellen . Pa ien s may develop o her pap-
or brown; round or oval, peduncula ed illomas wi h ime.

2
Papilloma 3

FIGURE 1-1. Papilloma. A. Multiple small papillomas o the upper eyelid. B. Larger papilloma o the right
lower eyelid. Conjunctival papilloma. C. Papillomatous lesions may grow rom the conjunctival sur ace and
protrude onto the eyelid margin. T ese papillomas are f esh colored and more riable than cutaneous papillomas.
Conjunctival papillomas can be associated with a viral origin.
4 1 BENIGN EYELID LESIO NS

SEBORRHEIC KERATOSIS Lesions vary in size rom 1 mm o 6 cm.

T he seborrheic kera osis is one o he mos


common benign epi helial umors. T ese
lesions are heredi ary, are rarely seen be ore he
Special Considerations
Mos common on lower lids
age o 30 years, and will con inue o increase over
a li e ime. Some pa ien s will only have a ew, and Dif erential Diagnosis
o hers can have hundreds over heir body. Pigmen ed ac inic kera osis
Epidemiology and Etiology Verruca vulgaris
Age: More common as pa ien s age. Rare Pigmen ed basal cell carcinoma
be ore age 30 years.
Gender: More common and more ex en- Pathophysiology
sive in males Epidermal lesion. Benign proli era ion o
E iology: Unknown kera inocy es, melanocy es, and orma ion
o horn cys s.
Inheri ance: Probably au osomal dominan
History Treatment
Lesions are of en presen or mon hs o Excision-ligh elec rocau ery or cryo her-
years and are of en asymp oma ic. apy will permi he lesion o be easily rubbed
T ey are mos common on he ace, runk, or curet ed o . T e underlying base can hen
and upper ex remi ies. be re rea ed wi h cau ery.
Examination
Prognosis
Lesion s ar s as a a , ligh an lesion. Wi h
ime, he lesion becomes more pigmen ed Excellen wi h rare recurrence
and will become eleva ed ( Fig. 1-2A). As Pa ien s will of en have many lesions
hey age, he lesion’s sur ace becomes “war y” and will develop addi ional lesions over
( Fig. 1-2B). ime.
Seborrheic Keratosis 5

FIGURE 1-2. Seborrheic keratosis (A&B). Seborrheic keratosis are common lesions o the eyelids. T ey tend to
get darker as they have been present longer, as seen in B.
6 1 BENIGN EYELID LESIO NS

CUTANEOUS HORN Special Considerations


Biopsy o hese lesions is required o rule

C u aneous horn is a clinically descrip ive


erm or lesions wi h exuberan hyper-
kera osis. T e e iology o his hyperkera osis
ou malignan lesion a he base o he lesion
such as basal cell carcinoma or squamous cell
carcinoma.
can be variable and biopsy o de ermine he
cause is required. Dif erential Diagnosis
T is is a descrip ive erm and no a pa ho-
Epidemiology and Etiology logic, diagnos ic erm.
Age: Older adul s T e base o his lesion may be a seborrheic
Gender: Equal in males and emales kera osis, verruca vulgaris, basal cell carci-
noma, or squamous cell carcinoma.
E iology: Hyperkera osis is associa ed
wi h a varie y o underlying lesions Laboratory Tests
History Pa hologic evalua ion
Lesion may grow slowly or rapidly. Treatment
Examination Excisional biopsy wi h pa hologic
evalua ion
Raised lesion, of en on a s alk, usually
whi e in color. Prognosis
T e sur ace is hyperkera o ic ( Fig. 1-3). Good
Cutaneous Horn 7

FIGURE 1-3. Cutaneous horn. A cutaneous horn has a hard, rough sur ace that is white in color. A. T is lesion is less
pointed, but some end in a point, giving them their name o cutaneous “horn.” B. Cutaneous horn o the lower eyelid.
8 1 BENIGN EYELID LESIO NS

EPIDERMAL INCLUSION T e underlying cys is whi e and can of en


CYST be visualized hrough he hin eyelid skin
( Fig. 1-4).

C ommon whi e o yellow cys seen around


he eyes and elsewhere on he ace. Easily
rea ed wi h excision.
Special Considerations
T ese cys s may become secondarily
in ec ed and cause a celluli is.
Epidemiology and Etiology
Age: Any Dif erential Diagnosis
Gender: Equal in males and emales Molluscum con agiosum
E iology: Arises spon aneously rom he Chalazion
in undibulum o he hair ollicle or ollowing Syringoma
rauma ic implan a ion o epidermal issue
in o he dermis. Treatment
History Excision; at emp should be made o
May have his ory o rauma o he area ei her excise he en ire cys wall or i lef
a he base, i should be des royed wi h
Lesions usually grow slowly or a period o
cau ery.
ime and hen remain s able.
Examination Prognosis
Smoo h, round, eleva ed cys Excellen . Recurrence is rare.
Epidermal Inclusion Cyst 9

FIGURE 1-4. Inclusion cyst. A. Inclusion cyst o the le upper eyelid. B. A smaller cyst o the le lower lid.
Patients with inclusion cysts on the eyelids o en seek treatment be ore they become very large.
10 1 BENIGN EYELID LESIO NS

MOLLUSCUM CONTAGIOSUM Special Considerations


I hese lesions are loca ed on he eyelid

M olluscum con agiosum is a sel -limi ed


viral in ec ion charac erized by skin-
colored papules ha are of en umbilica ed
margin, hey may cause a mild o severe,
chronic ollicular conjunc ivi is.
In immunocompromised pa ien s, his
a he cen er. In immunocompromised indi- viral in ec ion may no be sel -limi ed and can
viduals, his may no be sel -limi ed and lead o large, cosme ically dis guring lesions,
can lead o large cosme ically dis guring especially on he ace.
lesions. I hese lesions are loca ed on he
eyelid margin, hey may cause a ollicular Dif erential Diagnosis
conjunc ivi is. Epidermal inclusion cys
Syringoma
Epidemiology and Etiology
Kera oacan homa
Age: Children and young adul s
Gender: More common in males han Laboratory Tests
emales Direc microscopy o he kera in plug wi h
E iology: Viral lesions spread by skin- o- Giemsa s ain shows “molluscum bodies.”
skin con ac Treatment
T ese lesions will regress spon aneously
History
over ime excep in immunocompromised
Spon aneously occurring lesions pa ien s.
Known con ac wi h o her person wi h I removal is desired, small lesions can
lesions is no usual be rozen or he core can be rea ed wi h
elec rodesicca ion.
Examination
Curet age or direc excision is also e ec ive.
Single or mul iple small 1- o 2-mm
papules ( Fig. 1-5). Rarely, hese lesions can Prognosis
become larger. Good in heal hy people
T ey are pearly whi e or skin colored wi h T e chance o in ec ing o her people is
a cen ral kera in plug ha gives hem heir low when he lesions are presen bu in ec ed
cen ral umbilica ion. pa ien s should avoid skin- o-skin con ac .
Molluscum Contagiosum 11

FIGURE 1-5. Molluscum contagiosum. A. T ere are three lesions on the upper eyelid. I the lesions are on the
eyelid margin, the eye itsel may be injected with a ollicular conjunctivitis. T is patient also had similar lesions
on her leg. B. Multiple lesions o the eyelid margin. T ere was a mild ollicular reaction in the in erior ornix.
(Courtesy o Jurij Bilyk, MD.)
12 1 BENIGN EYELID LESIO NS

XANTHELASMA Special Considerations


I LDL is eleva ed in he lipid pro le, i is a

X an helasma are yellowish plaques ha


occur medially on he upper or lower
lids and are classic in appearance. T ey end
sign o a amilial lipopro ein disorder.

Dif erential Diagnosis


o enlarge wi h ime and may or may no be I he xan helasmas are presen bila erally,
associa ed wi h hyperlipidemia. no o her lesions look like his.
Synonyms: xan homa. Early, a xan helasma can look like an inclu-
sion cys or syringoma.
Epidemiology and Etiology Laboratory Tests
Age: More han 50 years o age. I Labora ory evalua ion o lipid pro le
younger, mus consider a amilial lipopro-
ein disorder. Pathophysiology
Gender: Ei her Macrophages con aining drople s o lipids
orm xan homa cells.
E iology: May or may no be associa ed
wi h hyperlipopro einemia T ese xan homa cells hen accumula e
orming he xan helasma.
History Treatment
T e lesions are no ed or mon hs o years Excision is mos common.
wi h slow enlargemen .
Elec rodesicca ion, laser, and applica ion
o richloroace ic acid are o her rea men s.
Examination
Sof , yellow-orange plaques loca ed medi- Prognosis
ally on he upper and/ or lower eyelids Good, bu wi h ime addi ional deposi ion
( Fig. 1-6) may occur and he lesions reappear.
Xanthelasma 13

FIGURE 1-6. Xanthelasma. T ese lesions are in the classic area o the upper eyelids. T ey are still relatively
small but with time, the lipid deposition will continue and they will enlarge. Less commonly, they can occur in a
similar position on the lower eyelids.
14 1 BENIGN EYELID LESIO NS

SYRINGOMA Lesions occur commonly on he lower


eyelids bu may occur elsewhere on he ace,

S yringoma presen as mul iple lesions on


he lower lids o women. T e onse is usu-
ally insidious. Pa ien s ypically presen wi h
axillae, umbilicus, upper ches , and vulva.

Dif erential Diagnosis


cosme ic concerns because o he numer- Very ew o her lesions look similar or
ous “bumps” on he lower eyelids. T e chal- presen wi h numerous lesions on he
lenge can be excision o he large number o lower eyelids. A single lesion can look like
lesions presen wi hou causing scarring or an an inclusion cys , basal cell carcinoma, or
ec ropion. richoepi helioma.

Epidemiology and Etiology Pathophysiology


Age: Begins in puber y Benign adenoma o he in raepi helial
Gender: Occurs in women and may be eccrine duc s
amilial Pa hology shows many small duc s in he
E iology: An adenoma o he in raepider- dermis wi h comma-like ails wi h he appear-
mal eccrine duc s ance o adpoles.

History Treatment
Lesions no ed on he lower eyelids wi h Pa ien s of en reques removal on a cos-
insidious onse . me ic basis.
Lesions may be presen elsewhere on Removal is by elec rosurgery or direc
he ace, axillae, umbilicus, upper ches , and excision.
vulva.
Prognosis
Examination A large number on he ace can be di cul
Lesions are 1 o 2 mm, skin colored or o remove.
yellowish, and usually appear in mul iples Addi ional lesions may grow af er
( Fig. 1-7). excision.
Syringoma 15

FIGURE 1-7. Syringoma. Multiple lesions in the classic area o the lower eyelids. T ere can be just a ew lesions
or even more than in this patient.
16 1 BENIGN EYELID LESIO NS

APO CRINE HYDRO CYSTOMA Lesions are ranslucen or bluish and


ransillumina e.

A pocrine hydrocys oma is a very common


lesion arising along he eyelid margin. I
is a clear, cys ic lesion ha ransillumina es,
T ere may be mul iple lesions.

Dif erential Diagnosis


al hough he overlying skin may give i a blu- Cys ic basal cell carcinoma
ish color. Eccrine hydrocys oma (re en ion cys o
Epidemiology and Etiology eccrine glands)
Age: Adul s Pathophysiology
Gender: Equal T is lesion is an adenoma o he secre ory
E iology: Cys orma ion rom he glands cells o Moll and no a re en ion cys .
o Moll along he eyelid margin
Treatment
History Marsupializa ion o he cys may be
Cys no ed and may slowly enlarge adequa e or super icial lesions, bu deeper
lesions require comple e cys wall excision.
Examination
Cys ic lesion near or on he eyelid margin Prognosis
( Fig. 1-8). Excellen . Rare recurrence af er excision.
Apocrine Hydrocystoma 17

FIGURE 1-8. Apocrine hydrocystoma. A. T is lesion on the upper eyelid transilluminates with the slit beam.
On excision there will be a gush o clear f uid. B. Multiple lesions. Lesions are o en smaller than these and are
di cult to photograph.
18 1 BENIGN EYELID LESIO NS

TRICHOEPITHELIOMA Examination
Small pink or skin-colored papules ha

T richoepi helioma is a benign lesh-


colored papule ha arises rom an imma-
ure hair ollicle. I can occur on he eyelid
can increase in size and become qui e large
( Fig. 1-9).

margin bu more commonly elsewhere on he Special Considerations


ace, scalp, neck, and upper runk. May be con used wi h a basal cell carci-
noma, especially i i appears as a soli ary umor.
Epidemiology and Etiology
Dif erential Diagnosis
Age: Firs appears a puber y
Epidermal inclusion cys
Gender: More common in males
Basal cell carcinoma
E iology: Benign appendage umor wi h
hair di eren ia ion Syringoma
Treatment
History
Excision wi h pa hologic evalua ion
Lesions o he eyelid and orehead appear
a puber y and can slowly increase in size and Prognosis
number. Excellen
Trichoepithelioma 19

FIGURE 1-9. Trichoepithelioma. T ese pink or skin-colored lesions can occur on the skin or eyelid margin. T ey
can enlarge and be con used with a basal cell carcinoma. (From Fitzpatrick B, Johnson R , Wol K, et al. Color
Atlas and Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.)
20 1 BENIGN EYELID LESIO NS

NEVI (NEVOCELLULAR NEVI) becomes mot led as he lesion evolves in o


a dermal nevus; of en has hairs growing

N evocellular nevi are small (less han


1 cm), circumscribed, acquired pig-
men ed lesions ha are made up o melano-
ou o he lesion.
Dermal nevi: Round, dome-shaped,
eleva ed nodule, skin-colored, an, or
cy ic nevus cells loca ed in he epidermis and brown wi h elangiec asias. T ese do no
dermis bu rarely deeper. disappear wi h age and may become more
peduncula ed.
Epidemiology and Etiology
Age: Appear in early childhood and reach Special Considerations
a maximum size in young adul hood. T ese Any enlarging lesions, hose changing
lesions gradually involu e and disappear by color, or becoming irri a ed in any way af er
age 60 years. T e excep ion is he dermal age 20 years need o be biopsied o rule ou
nevus, which does no involu e. malignan change.
Gender: Equal
Dif erential Diagnosis
E iology: Groups o melanocy ic nevus
Seborrheic kera osis
cells loca ed in he epidermis, dermis, or,
rarely, in he subcu aneous issue. Malignan melanoma
Derma o broma
History Basal cell carcinoma
Pigmen ed lesion ha is s able or involu ing
Laboratory Tests
Lesions are asymp oma ic.
His ologic examina ion i biopsied
Examination
Treatment
Nevi can be grouped as ollows ( Fig. 1-10):
Observa ion, unless he lesion changes
Junctional nevi: Round or oval, a , or color, i s borders become irregular, or he
very sligh ly raised lesion, less han 1 cm lesion begins o i ch, hur , or bleed. Any o
in diame er. an or brown in color wi h hese are indica ions or excisional biopsy
smoo h regular borders. wi h his ologic evalua ion.
Compound nevi: Round, eleva ed,
dome-shaped lesion wi h smoo h or papil- Prognosis
loma ous sur ace. Dark brown in color bu Rare chance o malignan rans orma ion
Nevi (Nevocellular Nevi) 21

FIGURE 1-10. Nevi. A.T is small nevus o the lower eyelid is amelanotic except or a ew pigmented spots.
Nevi on the eyelid margin will o en mold against the eyeball, as in this picture, but cause no discom ort or
corneal changes. B. A split nevus where nevi cells were split congenitally as the eyelid ssure ormed. T is nevus
is very dark and shows the variation that can occur in the color o these lesions.
22 1 BENIGN EYELID LESIO NS

HEMANGIOMA OF THE Examination


EYELID (CHERRY ANGIOMA) Raised, brigh red, blood- lled lesions ha
can occur anywhere on he body ( Fig. 1-11A)

H emangiomas o he eyelid are raised, red,


benign lesions ha appear on he eyelids
in adul hood and can increase in size bu usu-
Lesions may be single or mul iple.

Dif erential Diagnosis


ally remain less han 3 o 5 mm. Pyogenic granuloma ( Fig. 1-11B)
Melanoma
Epidemiology and Etiology
Age: Adul hood Laboratory Tests
Gender: Equal Pa hologic evalua ion af er excision
E iology: Unknown Treatment
Excision usually or cosme ic reasons,
History
less commonly o have he lesion evalua ed
Usually appear spon aneously and can pa hologically.
increase in size over a shor ime.
Pa ien s may have o her similar lesions Prognosis
elsewhere on heir body. Excellen
Hemangioma of the Eyelid (Cherry Angioma) 23

FIGURE 1-11. Eyelid hemangioma. A. Very red, blood- lled lesion that may be just slightly raised or very elevated
as in this picture. On excision, there is usually a small gush o blood, but these lesions usually do not bleed excessively.
arely, these can bleed actively so the surgeon must be prepared. Pyogenic granuloma. B. A pyogenic granuloma can
look similar to a hemangioma, but it usually is solid, not blood lled, and o en somewhat papillomatous.
C H AP T ER

Eyelid Inf amma ion

CH ALAZION he involved area ( Fig. 2-1A). here may


be poin ing over he blocked meibomian

A ype o ocal inf amma ion o he eyelid;


i is a common lesion o he eyelid. T e
mos common cause is blockage o a meibo-
gland.
As he inf amma ion resolves, he resul ing
lesion is a rm mass in he arsal pla e wi h or
mian gland o he eyelid. Chalazia can presen wi hou residual inf amma ion ( Fig. 2-1B).
wi h an inf amed, ender, red eyelid or as a dis-
cre e non ender lump in he eyelid. Special Considerations
Chronic, nonresolving chalazion needs o
Epidemiology and Etiology
be biopsied o rule ou a carcinoma.
Age: Any
Gender: Equal Dif erential Diagnosis
E iology: Focal inf amma ion o he eyelid Sebaceous adenocarcinoma
resul ing rom he obs ruc ion o he meibo- Squamous cell carcinoma
mian glands Basal cell carcinoma
History
Pathophysiology
O en presen wi h acu e onse o ocal eye-
lid inf amma ion. T e inf amma ion will resolve Blockage o he eyelid glands resul s in
bu may urn in o a chronic cys -like lesion. release o he gland con en s in o he arsus
and eyelid, resul ing in an inf amma ory
T e onse may be more insidious wi h process.
appearance o he cys -like lesion wi h mini-
mal inf amma ion. he in lamma ory process is hen walled
o wi h ime, resul ing in he cys -like
Examination lesion.
In he acu e process, he eyelid may be T e exac role o bac eria in his process is
di usely in lamed wi h pain ocally over unclear.

24
Chalazion 25

Treatment o be used cau iously in pa ien s wi h


When in he inf amma ory phase, ini ial darkly pigmen ed skin, as hey can cause
rea men is warm compresses and s eroid depigmen a ion.
an ibio ic drops or oin men .
As he lesion becomes cys ic, rea men is Prognosis
hen excision via a conjunc iva incision. Good
Injec ion o s eroid in o he lesion may T ese lesions can be mul iple and are
also be e ec ive. S eroid injec ions need rarely resis an o rea men .

FIGURE 2-1. Chalazion. A. A f rm, ormed lump o the le lower eyelid. T ere is still some in ammation o the
chalazion. T e eye is red rom blepharoconjunctivitis, which o en is part o a chalazion. Most o the time, the
eye is white and quiet. B. A chronic chalazion o the le upper eyelid with some crusting over the chalazion rom
external drainage.
26 2 EYELID INFLAMMATIO N

HORDEOLUM Examination
Red, swollen, ender eyelid, o en wi h a

A n acu e in ec ion o he glands o Zeis


(ex ernal hordeolum) or meibomian
glands (in ernal hordeolum). I presen s as a
ocal area o in ec ion around a gland o he
eyelid ( Fig. 2-2)

red, inf amed, ender eyelid. In prac ice, he Dif erential Diagnosis
erms chalazion, hordeolum, and stye are o en Presep al celluli is
used in erchangeably (and incorrec ly). Eyelid abscess
Synonym: s ye. Pathophysiology
Epidemiology and Etiology Eyelid gland becomes in ec ed probably
associa ed wi h blockage o he gland.
Age: Any
Gender: Equal Treatment
E iology: Acu e bac erial in ec ion o Warm compresses and opical s eroid/
he glands o Zeis or he meibomian an ibio ic drops or oin men
glands Rarely, his can evolve in o an abscess,
which needs drainage, or a celluli is ha
History requires sys emic an ibio ics.
Sudden onse o ocal inf amma ion o
he eyelid cen ered around a gland o he Prognosis
eyelid Excellen
Hordeolum 27

FIGURE 2-2. Hordeolum. A. Acute in ammation o the le lower eyelid caused by blockage and in ection/
in ammation o a meibomian gland. T is lesion may resolve as the acute in ammation resolves or evolve into a
chalazion. B. Blockage with in ection/ in ammation o the glands o Zeis is involved in this eyelid lesion. T is
lesion is on the external eyelid in the area o the eyelash ollicles. Hordeola usually resolve without sequelae but
can sometimes become chronic and take many weeks to resolve.
28 2 EYELID INFLAMMATIO N

FLOPPY EYELID SYNDROME T ere is chronic papillary conjunc ivi is


wi h a kera i is o en wi h di use super cial

F loppy eyelid syndrome is seen in obese


pa ien s, many o whom have sleep apnea.
T e eyelids become very loose and f oppy
punc a e kera i is.
ypically, he palpebral conjunc iva has a
velve y appearance.
ei her as a primary process or secondary o T ere is a high incidence o obesi y in
chronic rubbing o he eyelids a nigh . T is hese pa ien s.
syndrome mus be considered as a cause or
chronic, papillary conjunc ivi is. Special Considerations
Epidemiology and Etiology T ere is a signi can incidence o sleep
apnea in pa ien s wi h f oppy eyelid syn-
Age: Adul s
drome. All pa ien s need o have sleep s udies.
Gender: Males more commonly a ec ed
E iology: Unknown. T e eyelid laxi y and Dif erential Diagnosis
loss o s ruc ure may be rela ed o chronic O her orms o conjunc ivi is in a pa ien
mechanical eyelid rubbing or may be due wi h eyelid laxi y
o some inna e abnormali y o he pa ien ’s
eyelids. Pathophysiology
T ere is loss o elas in bers in he arsus,
History bu he cause remains specula ive.
Pa ien presen s wi h chronic papillary
conjunc ivi is ha is usually bila eral and may Treatment
give he his ory o his eyelids spon aneously Pa ching or a shield over he eye can be
ever ing a nigh . at emp ed bu is usually no help ul in he
T e pa ien may complain o chronic non- long erm.
speci c irri a ion. Horizon al eyelid igh ening is usually
T e symp oms are o en worse on he side required.
he pa ien sleeps on.
Prognosis
Examination T e laxi y will recur wi h ime.
Eyelids are f accid and easily ever ed Horizon al igh ening will relieve symp-
( Fig. 2-3). oms or a ime.
Floppy Eyelid Syndrome 29

FIGURE 2-3. Floppy eyelid syndrome. A. Patient with mild ptosis but complains o chronic irritation o the eyes.
His eyes are white but he has moderate corneal superf cial punctate keratitis. B. Upper eyelids are easily everted
and the undersides o the eyelids are red with a di use papillary reaction. C. T e eyelids are very loose and, once
everted, will o en remain everted even with blinking.
C H AP T ER

Eyelid Neoplasms

KERATOACANTHOMA Examination
Single, dome-shaped nodule with a central

K eratoacanthoma presents as an iso-


lated lesion on the ace with a very
unique appearance. he lesion is dome
keratotic plug
T e lesion is rm and is slightly red to light
brown in color ( Fig. 3-1).
shaped with a central keratin- illed crater. It
grows rapidly over weeks and may undergo Special Considerations
spontaneous regression over months. Once Once considered a benign lesion, there
considered benign, most pathologists may still be some con usion in the literature
now consider this a low-grade squamous about whether this is a squamous carcinoma.
carcinoma. T e lesion must be treated as a low-grade
squamous carcinoma.
Epidemiology and Etiology
Age: Most of en older than 50 years o age; Dif erential Diagnosis
rare younger than 20 Basal cell carcinoma
Gender: More common in males than Hyperkeratotic actinic keratosis
emales by a ratio o 2 to 1 Squamous carcinoma
Etiology: Unknown; ultraviolet radiation Laboratory Tests
and chemical carcinogens may have a causative
role. Histopathology o the excised lesion
Should be excised with rozen section
History guidance or with Mohs surgery
Rapid onset o growth over a ew weeks Treatment
T e lesion is of en asymptomatic except Excision with pathologic evaluation
or cosmetic changes. T ese lesions will sometimes spontane-
T ere may be occasional tenderness. ously regress over a ew months to a year.

30
Keratoacanthoma 31

T e need to rule out a squamous cell Prognosis


carcinoma and the cosmetic appearance Good
should lead to biopsy and excision be ore
spontaneous regression, especially around Depending on the size o the lesion, recon-
the eyelids. struction o the de ect may leave some eyelid
changes.

FIGURE 3-1. Keratoacanthoma. A. Lesion o the lef upper eyelid that grew over 2 to 3 weeks. It was excised
without recurrence. B. Large lesion o the lef lower eyelid in a 40-year-old patient. T e appearance could be that
o a squamous cell carcinoma; however, the history o growth over 4 weeks and the patient’s younger age point to
a keratoacanthoma. T is lesion was excised without recurrence.
32 3 EYELID NEO PLASMS

ACTINIC KERATOSIS Laboratory Tests


Pathologic evaluation i biopsied

T hese lesions may be single or multiple


on chronically sun-exposed skin. T ey
appear as dry, rough, scaly lesions that are
Pathophysiology
Repeated solar exposure results in damage
stable but can rarely disappear spontaneously. to the keratinocytes by the cumulative e ects
Synonym: solar keratosis. o ultraviolet radiation.

Epidemiology and Etiology Treatment


Age: Older than age 40; rare younger than Prevention through early and li elong use
30 years o sunscreen
Gender: Higher incidence in males Excise nodular lesions and submit or
pathologic evaluation.
Etiology: Sun exposure over time in a air-
skinned white population results in actinic Most at lesions respond to liquid nitro-
keratosis. gen or topical application o 5% 5- uorouracil
cream over a ew days to weeks.
History opical imiquimod cream has also
Extensive sun exposure in youth been approved or treatment o actinic
Lesions present or months. keratosis.
T ese three treatments (liquid nitrogen,
Examination 5- uorouracil, and imiquimod) must be used
Rough, slightly elevated, skin-colored or with caution around the eye and should be
light brown lesions with hyperkeratotic scale avoided in lesions at or near the lid margin.
( Fig. 3-2)
Prognosis
Special Considerations Some actinic keratoses may disappear
It is estimated that one squamous cell spontaneously but others remain or years
carcinoma will develop per 1000 actinic unless treated.
keratoses. Incidence o squamous cell carcinoma
developing in these lesions is unknown
Dif erential Diagnosis
but has been estimated to be one squa-
Squamous cell carcinoma mous cell carcinoma in every 1000 actinic
Discoid lupus keratoses.
Actinic Keratosis 33

FIGURE 3-2. Actinic keratosis. A. Multiple actinic keratoses on the cheek and brow with signs o chronic sun
damage. B. Lesion involving the lower eyelid. (Courtesy o Jurij Bilyk, MD.)
34 3 EYELID NEO PLASMS

LENTIGO MALIGNA Of en appears like a dark “stain” on the skin


Special Considerations
L entigo maligna is a at intraepidermal
neoplasm and the precursor lesion o len-
tigo maligna melanoma. T e lesion has strik-
T is is a premalignant lesion and should
be excised because o the chance o develop-
ing variations o brown and black ( Fig. 3-3), ment into a lentigo maligna melanoma.
of en described as a “stain.” Dif erential Diagnosis
Seborrheic keratosis
Epidemiology and Etiology
Actinic keratosis
Age: Median age is 65 years
Malignant melanoma
Gender: Equal incidence in males and
emales Laboratory Tests
Etiology: Sun exposure is a de nite actor. Histopathologic evaluation
History Treatment
History is usually not help ul, as exact Excision with margins sent or pathologic
onset o lesion is unclear. evaluation
Examination Prognosis
Flat, dark brown or black color, sharply Excellent i excised be ore developing into
de ned edges a melanoma
Lentigo Maligna 35

FIGURE 3-3. Lentigo maligna. A. A large macule with irregular borders and di erent shades o brown.
(From Fitzpatrick B et al. Color Atlas & Synopsis of Clinical Dermatology, 4th ed. New York, McGraw-Hill, 2001.)
B. Recurrent lentigo maligna o lef brow.
36 3 EYELID NEO PLASMS

BASAL CELL CARCINOMA Basal cell carcinomas almost never


metastasize.

B asal cell carcinoma is the most common


type o skin cancer. It is locally invasive
and aggressive but has very limited capacity to
Sclerosing basal cell carcinomas have
poorly de ned margins and may recur.
Basal cell nevus syndrome is an autoso-
metastasize. I neglected, it can invade the orbit, mal dominate syndrome in which patients
especially i located in the medial canthal area. develop multiple basal cells at a very young
Most commonly, it occurs on the lower eyelid age ( Fig. 3-4E and F).
and is treated by complete surgical excision.
Dif erential Diagnosis
Epidemiology and Etiology
Squamous cell carcinoma
Age: More than 40 years o age. Rare cases
do occur in the 20s and 30s richoepithelioma
Gender: Males more than emales Laboratory Tests
Etiology: Sun exposure and air skin with Lesions are sent or pathologic evaluation.
poor ability to tan are risk actors. reatment
with x-ray ( or acne) increases the risk. Treatment
Incidence: 500 to 1000 per 100,000 people Complete surgical excision with patho-
History logic evaluation
Slowly enlarging lesions in sun-exposed areas Frozen sections or Mohs surgery is needed
to assure complete excision.
T e lesions may be associated with bleeding.
Reconstruction o the de ect is then com-
Examination pleted at the same time.
Round or oval, rm lesions with depressed reatment with radiation should not be
center used or lesions around the eye unless the
T e lesions are pink or red with ne patient is not a surgical candidate.
thread-like telangiectasia. Imiquimod cream can be used as a non-
T e center may be ulcerated. Basal cell car- surgical option i the lesion is not close to the
cinoma may also appear scar-like or be cystic eyelid margin.
( Fig. 3-4A–D).
Prognosis
Special Considerations Good when promptly and completely
Aggressive treatment o basal cell carci- excised
noma o the medial canthal area is indicated Neglected cases can invade the orbit and
because o the risk o orbital extension rom brain and have the potential, in rare cases, to
the medial canthal area. be atal.
Basal Cell Carcinoma 37

FIGURE 3-4. Basal cell carcinoma. A. Classic appearance o a basal cell carcinoma. T is lesion does not involve
the eyelid margin, but large lesions such as this one are a challenge or reconstruction because o the chance o
lower eyelid ectropion. B. Notching o the eyelid margin is a sign o an eyelid neoplasm. T is basal cell carcinoma
has caused a notch and demonstrates the smooth pearly borders o a basal cell carcinoma.
( continued)
38 3 EYELID NEO PLASMS

FIGURE 3-4. (Continued) Basal cell carcinoma. C. Basal cell carcinoma may present as pigmented lesions,
especially in patients with darker pigmented skin. Note the pearly edges on the in erior part o the lesion. D.
A cystic lesion can be a basal cell carcinoma. T is lesion is larger than most hydrocystomas and has a slightly
violaceous hue. T is cystic basal cell carcinoma was lled with a thick clear gel-like material, which is classic.
(continued)
Basal Cell Carcinoma 39

FIGURE 3-4. (Continued) Basal cell carcinoma. E. Basal cell nevus syndrome with many basal cell carcinomas
all over the ace occurring at a young age. F. Pits o the palms o the hands that are of en seen in basal cell nevus
syndrome.
40 3 EYELID NEO PLASMS

SQUAMOUS CELL Undi erentiated lesions are eshy,


CARCINOMA granulomatous, and sof .
T ese can present rom very small to large.

S quamous cell carcinoma is a malignant


tumor o epithelial keratinocytes. It is of en
the result o exogenous carcinogens (ultravio-
T ey may be crusted with bleeding or
smooth ( Fig. 3-5).

let exposure, exposure to ionizing radiation, Dif erential Diagnosis


arsenic). T ese lesions are much less common
Actinic keratosis
than basal cell carcinoma on the eyelids and
are usually success ully treated with excision. Basal cell carcinoma
Keratoacanthoma
Epidemiology and Etiology
Age: Older than age 55 years Laboratory Tests
Gender: Males more commonly involved Pathologic evaluation
than emales
Etiology: Sun exposure and air skin with Treatment
poor ability to tan are risk actors. reatment Complete surgical excision with con-
with x-ray ( or acne) increases the risk. trolled margins
Incidence: 12 per 100,000 white males; 7 per Frozen sections and Mohs surgery are
100,000 white emales; 1 per 100,000 blacks both appropriate options.
History Imiquimod cream can be used as a non-
surgical option i not close to the eyelid
Persistent keratotic lesion or plaque that
margin.
does not resolve af er 1 month must be consid-
ered a potential carcinoma, especially in sun-
Prognosis
exposed areas.
Excellent unless the lesion is
Examination neglected
wo types o lesions: Squamous cell carcinoma rarely spreads
Di erentiated lesions are keratinized, via lymphatics, blood vessels, or along
rm, and hard. nerves.
Squamous Cell Carcinoma 41

FIGURE 3-5. Squamous cell carcinoma. A.T is is a very large squamous cell carcinoma that was neglected. It
now in ltrates the entire lower eyelid. Note the crusting on the lesion, which is usually present with squamous
cell carcinoma and is less common with basal cell carcinoma. B. Smaller lesion o the lower eyelid that shows
crusting and an irregular, erosive central area.
42 3 EYELID NEO PLASMS

SEBACEOUS Special Considerations


ADENO CARCINOMA T is lesion is the great masquerader. Delay
in diagnosis as a carcinoma and subsequent

S ebaceous adenocarcinoma is a highly malig-


nant and potentially atal tumor that arises
rom the sebaceous glands o the eyelid. Early,
growth o the lesion add to the poor progno-
sis or this tumor.

this tumor can be di cult to recognize and Dif erential Diagnosis


once it grows, it is di cult to contain, as it can Basal cell carcinoma
have skip areas. Early recognition and aggressive Squamous cell carcinoma
excision are the keys to success ul treatment.
Chronic blepharitis
Epidemiology and Etiology Chronic chalazion
Age: Usually greater than 50 years o age
Pathology
Gender: More common in emales than
It is important to alert your patholo-
males
gist i this sebaceous adenocarcinoma is
Etiology: Arises rom the meibomian suspected.
glands, glands o Zeis, or rom the sebaceous
Special stains/ studies are per ormed on
glands o the caruncle, eyebrow, or acial skin.
lesions that are suspected to be sebaceous
History adenocarcinoma.
Of en starts as a chronic blepharitis or Without these stains, the lesion may be
nonresolving chalazion misdiagnosed.
Patients may have a chronic red, irritated Treatment
eye or months to years.
Diagnosing the lesion is of en the biggest
Examination challenge.
Multiple potential presentations: Biopsy o any suspicious lesion is the key.
Nodular lesion simulating a chalazion Once diagnosed, complete excision with
wide, controlled margins is the treatment o
Unilateral chronic blepharitis
choice.
Cellular membrane growing over the
T ere can be skip areas, so care ul ollow-
conjunctiva
up or recurrence is needed.
Destructive, of en ulcerated, lesion on
the eyelid margin ( Fig. 3-6) Prognosis
Occurrence in the upper eyelid is twice T is is a potentially lethal tumor that must
as common as the lower eyelid. be treated aggressively.
Sebaceous Adenocarcinoma 43

FIGURE 3-6. Sebaceous adenocarcinoma. A.T e eyelid margin is red and in amed with notching. B. When the
eyelid is everted, there is an in ltrative lesion o the tarsal conjunctiva.
44 3 EYELID NEO PLASMS

MALIGNANT MELANOMA Dif erential Diagnosis


Nevus

M alignant melanoma is a rare but very


dangerous malignant lesion o the eye-
lids. Sun exposure as a child is an etiologic ac-
Pigmented basal cell carcinoma

Laboratory Tests
tor. Despite aggressive surgical excision, this Specimens are sent or pathologic evaluation.
can still be a atal tumor.
Treatment
Epidemiology and Etiology Complete excision with aggressive, con-
Age: T ird decade and beyond trolled surgical margins
Gender: Equal between males and emales T e deeper the lesion, the wider the mar-
Etiology: Sun exposure and genetic gins required.
predisposition Sentinel lymph node biopsy is usually
required.
History Melanomas are best excised via a team
Pigmented lesion with recent growth or approach at centers experienced with mela-
change in appearance noma treatment.

Examination Prognosis
Pigmented lesion with irregular pigment Dependent on the depth o the tumor
deposition, irregular margins, or just increase Eight-year survival rate is 33% to 93%,
in size ( Fig. 3-7) depending on depth o melanoma invasion
T ere may be ulceration and bleeding. ( Table 3-1).

TABLE 3-1. Survival in Relation to


umor Depth

Depth (mm) 8-Year Survival (%)


<0.76 93.2
0.76–1.69 85.6
1.70–3.60 59.8
>3.60 33.3
Malignant Melanoma 45

FIGURE 3-7. Malignant melanoma. A. Lesion o the right eyebrow that has grown over a ew months. T e
lesion has irregular areas o lighter and darker pigmentation. B. Malignant melanoma o the lower eyelid.
46 3 EYELID NEO PLASMS

KAPOSI’S SARCOMA Dif erential Diagnosis


Pyogenic granuloma

K aposi’s sarcoma is a vascular neoplasia


that can involve multiple systems. It
is a rare lesion o the eyelids but when pres-
Chalazion
Hemangioma
ent, is usually associated with a compromised Melanocytic nevus
immune system, most commonly HIV disease.
Laboratory Tests
Epidemiology and Etiology Pathologic evaluation i biopsied
Age: Any Evaluation o immune system i indicated
Gender: More common in males Treatment
Etiology: Vascular neoplasia is of en Excision with pathologic evaluation
associated with immune compromise in the
United States. Cryotherapy or intralesional chemothera-
peutic agents may be used or local control o
History the lesions.
Rapid growth o lesion may occur. Radiation treatment or some large lesions
Patients most commonly are HIV-positive, Prognosis
although other orms o immune compromise
may predispose patients to these lesions. Patients who develop lesions associated
with HIV of en have a short survival and die
Examination rom advancement o the HIV disease.
Elevated dermal lesions that are red or Patients with primary Kaposi’s sarcoma
purple ( Fig. 3-8) may survive or years.
Kaposi’s Sarcoma 47

FIGURE 3-8. Kaposi’s sarcoma. Lesion o the lower lid in a patient with AIDS.
C H AP T ER

Eyelid rauma

MARGINAL EYELID he amoun o orce causing he injury


LACERATION will help de ermine he likelihood o
more signi ican injuries o he orbi and

M arginal eyelid lacera ions are mos globe.


commonly associa ed wi h rauma
Examination
o he en ire orbi al area, and of en here are
o her associa ed injuries. T e ex en o lacera- Mus evalua e globe and orbi or injuries.
ion can vary grea ly. Promp , me iculous clo- Evalua e he ex en o he injury o he eye-
sure is he rea men o choice. lid and be sure ha he lacrimal sys em is no
injured ( Fig. 4-1).
Epidemiology and Etiology
C scanning may be required i o her inju-
Age: Any age. Second hrough our h
ries or oreign bodies are suspec ed.
decades mos common.
Gender: Males are more commonly a ec ed. Special Considerations
E iology: Blun rauma (e.g., s ), direc cu Dog bi es (also ca s and humans) require
(e.g., glass, kni e), or dog bi e mos commonly copious irriga ion o he wound and special
care because o he grea er risk o in ec ion.
History
e anus immuniza ion mus be up o
rauma his ory is variable rom minor o da e.
major injuries.
I is impor an o de ermine he cause o Treatment
he rauma o know whe her o suspec or- Me iculous closure o he wound wi hin
eign bodies. 24 o 48 hours

48
Marginal Eyelid Laceration 49

Surgery can be done in he o ce or emer- Prognosis


gency room set ing unless he lacera ions are Good. T e more complex he wound, he
complex or in children, where an opera ing grea er he chance o scarring, which may
room set ing wi h general anes hesia is required. hen require secondary repair a a la er da e.

FIGURE 4-1. Marginal eyelid laceration. Cen ral eyelid lacera ion rom an umbrella ca ching under he eyelid.
Isola ed marginal lacera ions wi hou canalicular involvemen are more likely due o some objec direc ly cut ing
he eyelid. Canalicular lacera ions are more of en because o earing and s re ching, as he medial lid is he
weakes area and he rs o ear.
50 4 EYELID TRAUMA

CANALICULAR EYELID Probing o he canalicular sys em may be


LACERATION necessary i a lacera ion is suspec ed ( Fig. 4-2).

Special Considerations
T he medial eyelid is he weakes area o
he eyelid, so any horizon al rac ion on
he eyelid is more likely o resul in damage
T e ar her he lacera ion is medially rom
he lacrimal punc a, he more di cul i is o
o he medial eyelid and he canaliculus. nd he dis al cu end.
Eyelid rauma requires care ul inspec ion o
Treatment
he medial can hal area o recognize he lacer-
a ed canaliculus. Repair wi h silicone in uba- Surgical repair wi h anas omosis o he
ion is he rea men o choice. canalicular ends and in uba ion o he lacri-
mal sys em
Epidemiology and Etiology
Loca ing he dis al end o he canaliculus
Age: Any age. Second hrough our h may be di cul and of en requires loupes or
decades mos common. an opera ing microscope.
Gender: Males are more commonly Depending on he severi y o he injury
a ec ed. and he pa ien ’s coopera ion, surgical repair
E iology: Usually a earing injury, as he is usually per ormed in he opera ing room
medial eyelid is he weakes area o he eyelid. set ing wi h local or general anes hesia.
T e ubing is lef in he lacrimal sys em or
History
6 weeks o 6 mon hs, depending on severi y
rauma his ory is variable, including blun and he individual prac i ioner.
orce, dog bi es, and, rarely, sharp objec s.
Prognosis
Examination
Good. Even he injuries ha resul in
Evalua e eye and orbi or injuries. a scarred canaliculus usually do well, as
Any cu medial o he lacrimal punc a mos pa ien s do well wi h one unc ioning
mus be evalua ed or a canalicular lacera ion. canaliculus.
Canalicular Eyelid Laceration 51

FIGURE 4-2. Canalicular laceration. A. Any cu or ear medial o he punc um, no mat er how super cial i
appears, needs o be explored or involvemen o he canaliculus. T is lacera ion involved bo h he upper and
lower canaliculi. B. T e punc um and cu canaliculus can be seen a he medial edge o he cu eyelid. T is eyelid
was nearly comple ely avulsed.
52 4 EYELID TRAUMA

DO G BITES T ere is mos commonly a single bi e wi h


mul iple areas o injury.

D og bi es are highly variable in heir ex en


bu generally do no involve injury o he
globe i sel . Promp repair wi h copious irriga-
T ere may be punc ure wounds or larger
ears and gashes ( Fig. 4-3).

ion of en gives airly good resul s, depending Special Considerations


on he ex en o he original injury. T e bi e mus be repor ed o he heal h
depar men or ollow up o be sure he dog is
Epidemiology and Etiology properly vaccina ed or rabies.
Age: Mos commonly children; adul s less e anus immuniza ion mus be up o da e.
common.
Gender: Equal Treatment
E iology: In children, he dog may be T e eyelid lacera ion mus be rea ed as
rying o bi e he child on he nose o show ou lined previously.
domina ion, which is wha happens be ween T e risk o in ec ion in animal and human
dogs, and usually does no represen an bi es is high because o he bac eria in he
at ack. T e eyelid lacera ion is he resul o mou h.
he dog’s canine oo h ca ching he eyelid and Copious irriga ion o he wounds is he
earing he medial lid ra her han a rue bi e o only rea men o proven bene o decrease
he eyelid. he risk o in ec ion.
History T e use o broad-spec rum, sys emic
an ibio ics has no been proven o lessen he
Of en, he child knows he dog and here chance o in ec ion bu should be considered.
is a single bi e and no a vicious at ack.
Prognosis
Examination
Good. T e more severe he injury, he
Evalua ion o he eye and orbi or o her grea er he risk o pos opera ive de ormi y.
injuries
Dog Bites 53

FIGURE 4-3. Dog bite with eyelid lacerations. Dog bi es are usually a single bi e, bu he amoun o damage can
be highly variable rom mild o severe. T e eyelid damage is usually rela ed o earing as he dog pulls away and he
ee h ge caugh on he eyelid. Bo h he medial and la eral can hi are orn and mul iple punc ure wounds are seen.
54 4 EYELID TRAUMA

EYELID BURNS T e concern is o pro ec he cornea wi h


lubrica ion.

E yelid burns are usually associa ed wi h sig-


ni can burns o he res o he ace and
body unless hey are elec rical or chemical. All
Wi h ime, he eyelids will scar, resul ing
in poor closure and more corneal exposure
( Fig. 4-4).
burns ake days o weeks or he ull issue dea h
and necrosis o mani es . Recons ruc ion can be
very di cul because o poor vasculariza ion. Treatment
An ibio ic oin men and copious corneal
Epidemiology and Etiology
lubrica ion
Age: Any
Sys emic an ibio ics are usually par o he
Gender: Males more commonly a ec ed sys emic care.
E iology: Burns ha involve he eyelids As he burns heal, cica ricial changes
are usually associa ed wi h burns over a large become more prominen and he use o skin
percen age o he body. graf s is required.
History
Generally associa ed wi h o her acial burns Prognosis
unless he e iology is elec rical or chemical. Dependen on he severi y o he burns
Examination Severe burns may require mul iple
Burns o he eyelid vary in dep h and surgeries and skin graf s o pro ec he
severi y. cornea.
Eyelid Burns 55

FIGURE 4-4. Eyelid burn. A. Elec rical burn wi h necrosis o he upper eyelid and underlying scleral necrosis.
Elec rical burns can ake weeks or he o al amoun o issue necrosis o become apparen . B. Mol en lead was
splashed on o he eyelid and in o he eye. Wi h hermal burns, he ex en o damage is eviden more quickly.
No e he rela ive lack o vasculariza ion along he lower eyelid margin rom he burn. T ere was par ial necrosis
and loss o par o he eyelid margin over ime.
C H AP T ER

Eyelid Malposi ions

ENTROPION History
ecen surgery on he eye or recen onse
ACUTE SPASTIC ENT OPION o ocular irri a ion

A cu e spas ic en ropion is he resul o eye-


lid swelling along wi h orbicularis spasm
ha resul s in a emporary in- urning o he
Examination
Lower eyelid en ropion ( Fig. 5-1) wi h
eyelid. A cycle o corneal irri a ion rom he associa ed involu ional ac ors such as
en ropion, causing more eyelid spasm, causing horizon al laxi y and orbicularis override.
more irri a ion, mus be broken so he eyelid
In addi ion, here is a separa e iden if able
can re urn o normal. Some o hese pa ien s
irri an o he eye.
will have underlying involu ional changes (lax-
i y) ha may resul in a recurren en ropion. his irri an may be kera i is, or-
eign body, su ure, or jus in lamma ion
Epidemiology and Etiology pos opera ively.
Age: More common in older pa ien
popula ion Dif erential Diagnosis
Gender: Equal occurrence in males and Involu ional en ropion
emales Cica ricial en ropion
E iology: Ocular irri a ion or in amma-
ion causes con inued orced blinking and Pathophysiology
closure o he eye. T is will lead o in- urning Involu ional changes o he eyelid allow
o he lower eyelid in eyelids ha have invo- he orced closure o he eyelid orbicularis
lu ional changes predisposing hem o en ro- muscle o override he arsus and drive he
pion (see “Involu ional En ropion”). eyelid margin inward oward he eye.

56
Entropion 57

Treatment Some cases will hen resolve; o hers will


rea men o he underlying ocular irri a- become an involu ional en ropion and need
ion or in amma ion will resolve some cases. more ex ensive surgery.
T is involves rea ing he ocular irri a ion Prognosis
and s abilizing he eyelid o hal he addi- Excellen . ecurrence in pa ien s wi h
ional irri a ion he eyelid is causing. signif can involu ional ac ors o he eyelid
S abilizing he eyelid may involve aping may develop an involu ional en ropion a a
he eyelid ou or Quicker su ures. la er ime.

FIGURE 5-1. Acute spastic entropion. Pa ien wi h a corneal abrasion. Con inued irri a ion and blinking leads
o an en ropion.
58 5 EYELID MALPO SITIO NS

INVOLUTIONAL ENT OPION Orbicularis muscle override is o en no ed

E yelid laxi y bo h horizon ally and ver i-


cally predisposes o he ins abili y o he
lower eyelid. T e addi ional ac or required is
as ullness over he arsal pla e when he lid is
en ropic.
T e en ropion can be in ermit en and no
he abili y o he pa ien ’s orbicularis muscle o always presen on examina ion.
override he arsus and drive he eyelid inward. Placing opical anes he ic drops in he eye,
Pa ien s presen wi h red, irri a ed eyes rom having he pa ien close he eyes orce ully,
he eyelid margin and he eyelashes in con ac and look downward will usually bring ou he
wi h he eye i sel . en ropion.
Epidemiology and Etiology Dif erential Diagnosis
Age: More common in older pa ien Cica ricial en ropion
popula ion
Acu e spas ic en ropion
Gender: Equal occurrence in males and
emales Pathophysiology
E iology: Horizon al laxi y and orbicularis Aging o eyelid issues resul s in laxi y and
override resul in inversion o he eyelid. s re ching o suppor ing s ruc ures.
History
Treatment
Acu e onse o eye irri a ion. T is irri a-
ion is some imes in ermit en in na ure and Surgical correc ion is based on correc ing
becomes more cons an . he ac ors con ribu ing o he en ropion,
usually horizon al shor ening o he eyelid
Examination and igh ening he eyelid re rac ors in any o
Inver ed lower eyelid wi h in erior corneal mul iple ways.
superf cial punc a e kera i is (SPK) or corneal
abrasion ( Fig. 5-2) Prognosis
En ropion is usually associa ed wi h hori- Excellen . T ere is a 5% o 10% chance o
zon al eyelid laxi y. recurrence over 5 o 10 years.
Entropion 59

FIGURE 5-2. Involutional entropion. Lef lower eyelid en ropion wi h involu ional changes. T e rolled in
orbicularis muscle can be seen driving he eyelid margin inward on he lef .
60 5 EYELID MALPO SITIO NS

CICAT ICIAL ENT OPION T is may include evalua ion o he

C ica ricial en ropion is caused by conjunc-


ival scarring pulling he eyelid inward.
Generally, rea men is surgical, bu def ning
o her eyelids o de ermine i he en ropion
is isola ed or involving all our eyelids,
which may help de ermine he e iology
and rea ing he cause o he conjunc ival scar- ( Fig. 5-3).
ring mus be done f rs or mos cases will recur.
Special Considerations
Occurs in he upper or lower eyelid.
Mus de ermine he e iology o he
Epidemiology and Etiology conjunc ival scarring be ore rea ing.
Age: Any age Any progressive disease mus be quie ed
be ore surgery can be done on he eyelids.
Gender: Equal occurrence in males and
emales Dif erential Diagnosis
E iology: Scar issue on he conjunc ival Acu e spas ic en ropion
sur aces resul s in shor ening o he pos erior
lamella, physically pulling he eyelid inward. Involu ional en ropion
Fac ors include: Laboratory Tests
Surgery Conjunc ival biopsy wi h immuno uores-
Conjunc ival scarring diseases cence es ing i ocular cica ricial pemphigoid
(e.g., ocular cica ricial pemphigoid, is suspec ed.
S evens-Johnson syndrome, rachoma)
Treatment
rauma
De ermine he e iology o he conjunc ival
Conjunc ival burns (e.g., chemical)
scarring.
An iglaucoma drops
Quie any ac ive disease.
History Surgical correc ion o he en ropion wi h
Chronic low-grade in amma ion over a marginal ro a ion or buccal mucosal gra is
mon hs o years resul s in he en ropion ha hen he rea men o choice.
hen causes more irri a ion.
Prognosis
T e o her scenario is a his ory o rauma or
Variable depending on he e iology
surgery resul ing in an en ropion and associ-
a ed irri a ion. En ropion secondary o rauma and sur-
gery usually do very well.
Examination Progressive disease processes, such as ocu-
Care ul evalua ion o he conjunc iva or lar cica ricial pemphigoid, can make i much
signs o scarring causing inversion o he more di cul o preven recurrence o he
eyelid. en ropion.
Entropion 61

FIGURE 5-3. Cicatricial entropion. Ex ernally (A), i is di cul o di eren ia e his cica ricial en ropion rom
an involu ional en ropion un il he eyelid is ever ed (B), and he cica ricial changes are no ed pulling he eyelid
inward.
62 5 EYELID MALPO SITIO NS

ECTROPION Mus look or he amoun o horizon al


laxi y, corneal exposure, and s enosis o he
INVOLUTIONAL ECT OPION lacrimal punc a.

I nvolu ional ec ropion has he same involu-


ional ac ors as in an involu ional en ropion
(e.g., horizon al laxi y, ver ical ins abili y).
Special Considerations
arsal ec ropion is comple e eversion o
T ese pa ien s do no have hyper rophic, spas- he eyelid and indica es de achmen o he
ic orbicularis muscle o override, so he uns a- lower eyelid re rac ors.
ble eyelid sags ou ward ins ead o being driven T is condi ion mus be recognized, as i
inward. Symp oms are less acu e and no as requires bo h horizon al igh ening and rea -
severe as in involu ional en ropion. Many achmen o he re rac ors.
pa ien s will have mild involu ional ec ropions
and may be asymp oma ic. Dif erential Diagnosis
Cica ricial ec ropion
Epidemiology and Etiology Paraly ic ec ropion
Age: Incidence increases as age increases.
Gender: Equal occurrence in males and Treatment
emales Mild ec ropion wi h only mild exposure
E iology: Eyelid issue laxi y, especially symp oms can some imes be rea ed wi h
horizon al laxi y ocular lubrica ion.
De ini ive rea men involves hori-
History zon al eyelid shor ening and possible
Insidious onse o ocular irri a ion and/ or punc oplas y.
earing
Prognosis
Pa ien may no e redness and in amma-
ion o he eyelid margin. Excellen . ecurrence a er surgery is
es ima ed a 5% o 10%, bu is higher he
Examination longer he ollow-up is done and he more
Eyelid sagging in eriorly and away rom severe he ec ropion was a he ime o he
he globe sur ace ( Fig. 5-4) repair.
Ectropion 63

FIGURE 5-4. Involutional ectropion. Bila eral ec ropions wi h very lax eyelids. No e he red palpebral
conjunc iva rom chronic exposure.
64 5 EYELID MALPO SITIO NS

PAR LYTIC ECT OPION Evalua e severi y o acial palsy, degree

P araly ic ec ropion is he resul o emporary


or permanen seven h cranial nerve palsy.
T e lower eyelid sags away rom he globe, resul -
o ec ropion, amoun o corneal exposure,
amoun o lagoph halmos, and presence o an
in ac Bell’s phenomenon.
ing in loss o pro ec ion o he eye and inabili y
Special Considerations
o he lacrimal sys em o collec ears. Pa ien s
wi h less severe palsy and o her eye pro ec ive Mus check or corneal sensa ion as loss
mechanisms in ac presen wi h earing. Pa ien s o corneal sensa ion will make all exposure
wi h more severe palsies and poor eye pro ec ion symp oms much worse.
mechanisms presen wi h corneal breakdown. Any unexplained acial palsy mus be
worked up.
Epidemiology and Etiology
Age: Any age Dif erential Diagnosis
Gender: Equal occurrence in males and Bell’s palsy versus nonresolving acial
emales palsy
E iology: Facial palsy e iologies include: Treatment
Bell’s palsy rea men depends on he an icipa ed
Surgery: in racranial or acial dura ion o he paralysis.
S roke I spon aneous improvemen is an ici-
umor pa ed, hen rea men wi h lubrica ion and
a emporary arsorrhaphy i severe corneal
History problems are presen is indica ed.
Previous onse o acial palsy I corneal exposure is s ill a problem wi h
Depending on he severi y o he acial lubrica ion use and he paralysis is long erm,
palsy, he ec ropion may have onse a he same hen horizon al eyelid igh ening is used o
ime or he eyelid may slowly sag wi h ime. rea he paraly ic ec ropion.
T e severi y o he condi ion depends on Placing a gold weigh in he upper eyelid
he severi y o he paralysis, corneal sensa ion, may also be required. arely, a permanen
and ocular lubrica ion. arsorrhaphy may be needed.

Examination Prognosis
T e lower eyelid is ound o be sagging Variable. T e ec ropion ends o recur over
away rom he globe ( Fig. 5-5). ime i he paralysis is permanen .
Ectropion 65

FIGURE 5-5. Paralytic ectropion. Righ lower eyelid ec ropion as he resul o a acial palsy.
66 5 EYELID MALPO SITIO NS

CICAT ICIAL ECT OPION T is scarring resul s in shor ening o he

C ica ricial ec ropion is caused by mechani-


cal shor ening o he an erior lamellae o
he eyelid pulling he eyelid down and ou -
eyelid skin and ou - urning o he eyelid
margin ( Fig. 5-6A).
Special Considerations
ward. T is resul s in earing and corneal expo-
Mus always consider a skin carcinoma as
sure. More common in he lower eyelid bu
he possible cause o scarring o he skin. I
can occur in he upper eyelid.
he cause is unclear, a biopsy is needed.
Epidemiology and Etiology Dif erential Diagnosis
Age: Any age Impor an o di eren ia e involu ional
Gender: More common in males because ec ropion rom hose wi h cica ricial changes.
o higher incidence o rauma ic even s. Treatment
E iology: Scarring o he an erior lamellae rea men o any underlying derma ologic
o he eyelid pulls he eyelid ou ward. T e condi ion is impor an .
e iologies include:
In rauma ic or pos surgical cases, he
rauma scarring should be le or 6 mon hs or longer
Surgery unless exposure or o her problems necessi a e
Derma i is earlier rea men .
Skin carcinoma rea men involves lysis o any deep scar
issue wi h horizon al igh ening.
History I he skin shor ening is severe, ull- hick-
May include a specif c his ory such as ness skin gra s will be required.
rauma or surgery Skin gra s have he po en ial or scarring
I a chronic derma ologic condi ion is he and a cosme ically no iceable area a he gra
cause o he scarring, his may be a known or si e ( Fig. 5-6B).
a previously unrecognized condi ion.
Prognosis
Examination rauma or surgically induced cases do well
Ex ernal scarring or skin changes are no ed wi h repair.
on he upper or more commonly he lower Chronic condi ions o he skin end o
eyelid. resul in recurrences.
Ectropion 67

FIGURE 5-6. Cicatricial ectropion. A. rauma o he lef lower eyelid resul s in scarring o he skin wi h ver ical
shor ening as well as scarring in ernally wi hin he eyelid. B.A f er repair, using a skin graf .
68 5 EYELID MALPO SITIO NS

MECHANICAL ECT OPION History

M echanical ec ropion is a rare cause


o ec ropion in which a mass
o some ype pushes he eyelid ou ward.
Pa ien may be asymp oma ic, have
symp oms o corneal irri a ion, or have red-
ness and irri a ion o he eyelid.
here are usually associa ed involu ional
Examination
changes ha allow he eyelid o be pushed
ou ward. Mus de ermine he degree o involu ional
changes o he eyelid as well as he e iology o
Epidemiology and Etiology he mass dis or ing he eyelid.
Age: Older pa ien s T e amoun o corneal exposure and any
Gender: Equal occurrence in males and corneal scarring should also be no ed ( Fig. 5-7).
emales Dif erential Diagnosis
E iology: Gravi y pulls he eyelid away Involu ional ec ropion
rom he eye or pushes he eyelid away rom Cica ricial ec ropion
he eye secondary o a mass. Causes o he
mass e ec include: Paraly ic ec ropion
Derma ochalasis Treatment
Edema Excision o he mass and correc ion o he
involu ional ac ors o he eyelid
Chalazion
Eyelid umor (e.g., hemangioma, inclusion Prognosis
cys ) Good i he mass can be elimina ed
Ectropion 69

FIGURE 5-7. Mechanical ectropion. A. Chemosis rom an in amma ory process mechanically pushes he
lower eyelid ou ward. T ere are usually some involu ional changes presen o allow he eyelid o be pushed ou .
Resolu ion o he chemosis allowed he eyelid o re urn o a normal posi ion. B. Mechanical ec ropion rom a
chalazion. Even wi h chalazion resolu ion, he ec ropion may remain.
70 5 EYELID MALPO SITIO NS

SYMBLEPH ARON Mus be sure o examine under he upper


lid or conjunc ival scarring, as early signs are

S ymblepharon is scarring be ween he bul-


bar and palpebral conjunc iva. T is may
be associa ed wi h ac ive in amma ion or
some imes more obvious here.

Special Considerations
here may be no in amma ory signs. I is impor an o de ermine he cause o
he symblepharon.
Epidemiology and Etiology I asymp oma ic, he symblepharon may
Age: Any age require no rea men excep looking or he
cause o he scarring.
Gender: More requen in women
uling ou a progressive conjunc ival
E iology: T e ollowing can resul in scar-
scarring disease, such as ocular cica ricial
ring o wo conjunc ival sur aces:
pemphigoid, is impor an .
Chronic blephari is
Previous rauma Dif erential Diagnosis
Conjunc ival scarring diseases (e.g., T e di eren ial diagnosis involves de er-
ocular cica ricial pemphigoid, S evens- mining he cause o he symblepharon, no
Johnson syndrome) whe her he process is a symblepharon.
A opic disease Laboratory Tests
Eyelid surgery Conjunc ival scarring o unknown e iology
Conjunc ival burns requires a conjunc ival biopsy wi h immuno-
Chronic glaucoma drops, especially uorescence es ing o rule ou ocular cica ri-
mio ics cial pemphigoid.
In rare cases, squamous cell carcinoma may
History cause symblepharon; here ore, pa hologic
T ere may be no his ory, jus asymp om- evalua ion should be considered in selec cases.
a ic symblepharon no ed on examina ion.
Treatment
Pa ien s wi h his ory o eye or eyelid
None or mild symblepharon
rauma or in amma ion may also have
symblepharon. Moni oring or progression is impor an .
Signif can symblepharon may cause ri-
Examination chiasis and cica ricial en ropion ha hen may
Scarring o he conjunc ival sur aces may require rea men .
be very sub le wi h sligh in erior ornix
shor ening or i may be very obvious wi h Prognosis
large conjunc ival bands be ween he eye and Variable depending on he cause o he
eyelid ( Fig. 5-8). symblepharon
Symblepharon 71

FIGURE 5-8. Symblepharon. A. Scarring is seen be ween he eyelid and he in erior cornea. B.E arly
symblepharon may be no ed only as shor ening o he ornix.
72 5 EYELID MALPO SITIO NS

TRICHIASIS Dif erential Diagnosis


Spas ic en ropion
richiasis is an acquired misdirec ion o eye- Involu ional en ropion
lashes. richiasis may be ocal, as is seen
a er eyelid rauma in he area o he lacera ion. Cica ricial en ropion
T e process may be di use wi h eyelid scarring Congeni al dis ichiasis
and lashes along he en ire eyelid margin.
Laboratory Tests
Epidemiology and Etiology Conjunc ival scarring o unknown
Age: Any age. Non rauma ic causes are e iology requires a conjunc ival biopsy wi h
rare in childhood. More common wi h immuno uorescence es ing o rule ou ocu-
increasing age. lar cica ricial pemphigoid.
Gender: More common in emales Treatment
E iology: Lash ollicles are dis or ed and Lashes can be epila ed or emporary relie
become misdirec ed wi h scarring o he eye- bu hey always grow back.
lid. Chronic eyelid in amma ion may resul in
grow h o misdirec ed lashes. Chronic bleph- Elec rolysis or cryo herapy will abla e
ari is, eyelid rauma, and conjunc ival scarring lashes on a more “permanen ” basis.
diseases can all cause richiasis. A bes , 50% o he lashes will no regrow
so mul iple rea men s are required.
History
In cases o severe scarring, eyelid surgery
Pa ien s will o en have a his ory o chronic will be needed o correc he problem. T is
eye irri a ion and in amma ion. T ey may also may include marginal ro a ion, excision
have a long his ory o eyelash problems. T ere o he abnormal lashes, and a buccal mucosal
may be a his ory o eyelid rauma or surgery. gra .
Examination
Prognosis
Eyelashes are seen rubbing on he eyelid
sur ace ( Fig. 5-9). Dependen on he cause o he
richiasis
T e amoun o corneal changes depends on
he number o lashes and dura ion. T ere may Chronic progressive in amma ory
be jus SPK or here may be corneal scarring. diseases, such as ocular cica ricial
pemphigoid, will o en have recurren
Special Considerations lashes ha can be very di cul o com-
Impor an o di eren ia e richiasis rom ple ely eradica e.
abnormal eyelid posi ions, such as en ropi- richiasis rela ed o rauma or o her non-
ons, which secondarily resul in eyelashes progressive scarring usually responds well o
rubbing on he cornea. rea men .
Trichiasis 73

FIGURE 5-9. richiasis. A. Eyelashes are growing pos eriorly, con ac ing he cornea rom he upper eyelid.
In rue richiasis, he eyelid margin is normal. Of en, wi h conjunc ival scarring disease, here will be some
accompanied in- urning o he eyelid margin. B. Lower eyelid richiasis rom conjunc ival scarring.
74 5 EYELID MALPO SITIO NS

PTOSIS Dif erential Diagnosis


I he p osis is congeni al wi h poor leva or
CONGENITAL MYOGENIC PTOSIS unc ion, here is lit le else in he di eren-

C ongeni al myogenic p osis is he mos


common congeni al p osis and resul s
rom a dysgenesis o he leva or muscle. I may
ial. Bir h rauma can resul in a p osis, bu
here is usually good leva or unc ion and
he leva or muscle is no f bro ic. Marcus
be unila eral or bila eral and can vary in sever- Gunn jaw wink needs o be considered in
i y rom very mild p osis o very severe. all cases o congeni al p osis (see sec ion
“Marcus Gunn Jaw Wink”).
Epidemiology and Etiology T ere are o her orms o myogenic p osis
Age: Bir h ha are acquired, no congeni al, such as
Gender: Equally a ec s males and emales in muscular dys rophy, chronic progressive
ex ernal oph halmoplegia (CPEO), myas he-
E iology: T e leva or muscle developmen
nia gravis, or oculopharyngeal dys rophy.
is abnormal, resul ing in f brosis and at y
inf l ra ion o he leva or muscle. Laboratory Tests
History Skele al muscle biopsy and elec rophysi-
ologic es ing may be needed. An elec ro-
P osis no ed a bir h or soon a er.
cardiogram should be done i CPEO is
Child may have chin up head posi ion, suspec ed.
especially i bila eral.
Paren s may no e he child’s eyes are open Treatment
while asleep. Fron alis suspension surgery using su ure,
a silicone rod, or ascia la a
Examination
T e age o do surgery depends on he
P osis is no ed o be ei her unila eral or severi y o he p osis and any underlying
bila eral. amblyopia.
T ere is lit le or no leva or unc ion, resul - Amblyopia may need rea men wi h
ing in a f bro ic, s i eyelid wi h a airly f xed pa ching a er he eyelid is li ed.
posi ion as he eye moves rom up o down
T ere is con roversy in rea ing unila eral
gaze.
congeni al p osis regarding whe her o do a
T e lid crease is o en poorly ormed. ron alis suspension only on he p o ic eye or
Depending on he severi y o he p osis, i he normal eye should have excision o he
here may be amblyopia wi h unila eral p osis leva or and a ron alis suspension as well o
( Fig. 5-10). provide symme ry.

Special Considerations Prognosis


T ere will be abnormal superior rec us Surgery is very success ul in li ing he lid
unc ion in 16% o pa ien s; his makes expo- above he pupillary axis. T e eyelid will some-
sure problems a er repair and s rabismus a imes all wi h ime and repea surgery may be
concern. needed la er.
Ptosis 75

FIGURE 5-10. Congenital myogenic ptosis. Modera e congeni al p osis in a child. No e he ex reme use o he
eyebrows o lif he eyelids. T ere is a prominen eyelid crease in his child, bu i is usually poorly de ned in
congeni al p osis. Leva or unc ion was 3 mm.
76 5 EYELID MALPO SITIO NS

ACQUI ED MYOGENIC PTOSIS I is heredi ary in 50% o he cases.

A cquired myogenic p osis is an unusual


cause o p osis rela ed o developmen o
a muscular disease ha can be localized or may
I is progressive un il he eyes are f xed in
a sligh ly downward direc ion wi h a severe
p osis.
be sys emic. Hear block, re ini is pigmen osa, abnor-
mal re inal pigmen a ion, and various neu-
Epidemiology and Etiology
rologic signs have been associa ed wi h his
Age: Acquired bu can presen in children syndrome.
or adul s
E iology: Sys emic muscular diseases ha Dif erential Diagnosis
can cause acquired myogenic p osis include Congeni al p osis
muscular dys rophy, CPEO, myas henia gra- Neurogenic p osis
vis, and oculopharyngeal dys rophy.
Laboratory Tests
History
Skele al muscle biopsy and elec rophysi-
Progressive p osis ha is o en associa ed ologic es ing may be needed.
wi h o her muscular dys unc ion
An ECG should be done i CPEO is
Examination suspec ed.
P osis is no ed wi h decreased leva or
Treatment
unc ion.
Surgery is needed or correc ion.
T ere may be abnormal eye movemen s
and abnormal acial one. Evalua ion and rea men o any
sys emic abnormali ies mus be addressed
Myas henia gravis may have double vision
f rs .
as par o he presen a ion.
Depending on he severi y o he p osis
Chronic progressive ex ernal oph hal-
and he amoun o leva or unc ion, ei her
moplegia has decreased eye movemen s, bu
leva or resec ion or ron alis suspension is
here is no diplopia.
indica ed.
Care ul evalua ion o he abili y o close
Fron alis suspension wi h a silicone rod
he eyes is needed, as poor closure will
will work well in many o hese pa ien s.
increase he risk o pos opera ive corneal
exposure ( Fig. 5-11). Prognosis
Special Considerations Mos pa ien s can achieve an eyelid level
Chronic progressive ex ernal oph halmo- ha is unc ional.
plegia is a gradual, bila eral p osis ha begins in Mos will no be able o achieve an
childhood or young adul hood and is progres- eyelid level or unc ion ha is considered
sive wi h involvemen o ex raocular muscles. normal.
Ptosis 77

FIGURE 5-11. Acquired myogenic ptosis. A. Pa ien wi h muscular dys rophy and severe p osis. T ere is very
lit le leva or unc ion, and he pa ien is barely able o keep his eyelids above he pupil wi h ex reme eyebrow
eleva ion. B.T e same pa ien af er ron alis suspension surgery. He can now e ec ively lif his eyelids by
eleva ing his eyebrows.
78 5 EYELID MALPO SITIO NS

APONEU OTIC PTOSIS T e p osis may be worse in downgaze

A poneuro ic p osis is he mos common


ype o p osis and is he resul o disin-
ser ion o he leva or aponeurosis. T is ype o
( Fig. 5-12).

Special Considerations
p osis may be caused by normal aging changes, Myas henia gravis mus be considered in
swelling, or repe i ive s re ching o he upper all cases.
eyelid. T e onse o p osis is gradual.
Dif erential Diagnosis
Epidemiology and Etiology Congeni al p osis (di eren ia e by poor
Age: arely congeni al. Mos common in leva or unc ion)
older pa ien s. Myas henia gravis
Gender: Equal rauma ic p osis
E iology: Aponeuro ic p osis is due o an
Laboratory Tests
abnormali y o he leva or aponeurosis or i s
inser ion. T is resul s rom normal involu ional None
changes and/ or repe i ive rac ion such as eye-
Treatment
lid rubbing, eyelid swelling, and eye surgery.
Ex ernal leva or resec ion and müllerec-
History omy are bo h good surgical approaches or
Gradual, progressive droopiness o he success ul repair.
eyelids is he mos common his ory. Dry eyes, poor eye closure, and poor Bell’s
ecen eye surgery or eyelid swelling can phenomenon mus all be recognized preoper-
exacerba e he p osis. a ively. T ese condi ions make pos opera ive
corneal exposure more likely.
Examination
Mild o severe p osis wi h normal leva or Prognosis
unc ion and o en a high eyelid crease or less Excellen prognosis or success ul surgical
commonly a poor eyelid crease. correc ion
Ptosis 79

FIGURE 5-12. Aponeurotic ptosis. Bila eral p osis rom dehiscence o he leva or aponeurosis. No e he high,
very de ned upper eyelid crease. Leva or unc ion was 18 mm.
80 5 EYELID MALPO SITIO NS

NEUROGENIC PTOSIS Pa ien s younger han 50 years o age need


neuroimaging unless hey have signif can
THI D NE VE PALSY vascular disease.
hird cranial nerve palsy is usually mani es Vasculopa hic causes o hird nerve palsies
as sudden or progressive onse o p osis should resolve wi hin 3 mon hs.
wi h an underlying s rabismus. De ermining
he e iology is he f rs priori y, as some causes Dif erential Diagnosis
o a hird cranial nerve palsy are li e- hrea en- Myas henia gravis
ing. rea men is di cul . Chronic progressive ex ernal
oph halmoplegia
Epidemiology and Etiology
Age: Any age. are in children. Laboratory Tests
Gender: No emale versus male di erence M I wi h MR , or an angiogram i he
no ed. hird nerve palsy involves he pupil

Etiology Pathophysiology
Ischemic microvascular disease In errup ion o he hird nerve may be
caused by compression o he nerve or isch-
Compressive: aneurysm, umor emia. Ischemia will no cause pupillary dila-
rauma ion and will resolve wi hin 3 mon hs.
Oph halmoplegic migraine: children
Treatment
History T e majori y o pupil-sparing hird nerve
Acu e onse o p osis wi h double vision palsies will resolve in 3 mon hs.
when he eyelid is li ed. T ese pa ien s should be given adequa e
May or may no be associa ed wi h pain. ime or spon aneous resolu ion be ore surgi-
cal correc ion is per ormed.
Examination T e underlying s rabismus mus be rea ed
Comple e p osis wi h he eye posi ioned be ore at emp ing o li he eyelid.
down and ou ( Fig. 5-13). P osis surgery requires ron alis suspen-
T ere is an inabili y o eleva e, depress, or sion, bu here is risk o corneal exposure.
adduc he eye. Fron alis suspension wi h a silicone rod is
T e pupil may or may no be dila ed. a sa er surgical approach.
Aberran regenera ion o he hird nerve
Prognosis
should be ruled ou .
Many hird nerve palsies will resolve in 3 o
Special Considerations 6 mon hs.
I he pupil is dila ed, he pa ien needs T ose ha do no resolve are di cul
emergen neuroimaging o rule ou a pos e- o ge in o normal eyelid posi ion wi hou
rior communica ing ar ery aneurysm. causing an unaccep able amoun o corneal
Nonresolving hird nerve palsies, incom- exposure.
ple e hird nerve palsies, and any hird nerve Pa ien s will o en have residual diplopia
palsy wi h aberran regenera ion requires rom he mo ili y problems when he eyelid
neuroimaging. is raised.
Neurogenic Ptosis 81

FIGURE 5-13. T ird nerve palsy. A. Comple e p osis o he upper eyelid wi h no leva or unc ion. B.E leva ion
o he p o ic eyelid reveals ocular misalignmen consis en wi h a hird nerve palsy.
82 5 EYELID MALPO SITIO NS

MYASTHENIA GR VIS T e ice es involves placing an ice pack on

M yas henia gravis is an au oimmune dis-


order in which au oan ibodies at ack
he recep ors o he neuromuscular junc-
he eyelids or 2 minu es, i he p osis is sec-
ondary o myas henia gravis, i will improve.
ensilon es ing involves IV adminis ra-
ion. T e resul is usually a sys emic muscular ion o edrophonium chloride ( ensilon).
weakness ha can be li e- hrea ening due o Improvemen o he p osis or diplopia
respira ory compromise. P osis wi h or wi h- indica es he e iology is myas henia gravis.
ou diplopia can o en be he ini ial presen- T e use ulness o ensilon es ing is lim-
a ion. rea men o he sys emic disease is i ed because o he po en ial adverse e ec s
maximized be ore any surgery is done on he including bradycardia and even respira ory
eyelids. arres ( Fig. 5-14).
A combina ion o single-f ber EMG and
Epidemiology and Etiology an ibody es ing is mos commonly used o
Age: Any age make he diagnosis.
Gender: More common in emales
Special Considerations
E iology: Au oimmune disease; may be
Pa ien s wi h newly diagnosed myas henia
associa ed wi h hymoma or an eceden
gravis need a C or M I o he ches o rule
in ec ion.
ou hymoma.
History Pa ien s wi h any signs or symp oms o
Insidious onse o droopy eyelids, double respira ory compromise need immedia e neu-
vision, or bo h, which are o en worse a he rologic evalua ion or possible hospi al admis-
end o he day. sion and rea men .
Pa ien s may have acial weakness, proxi- Dif erential Diagnosis
mal limb weakness, or di cul y swallow-
Ea on–Lamber syndrome
ing and brea hing. Symp oms are ypically
in ermit en . Chronic progressive ex ernal
oph halmoplegia
Examination T ird nerve palsy
T e disease is mos o en generalized and
sys emic bu may presen ini ially wi h p osis Laboratory Tests
and/ or double vision. Ace ylcholine recep or an ibody assay and
P osis is worsened wi h sus ained upgaze single-f ber EMG
and diplopia is worse wi h con inual eye
Pathophysiology
movemen s ( a igue).
An au oimmune disorder in which au oan-
Weakness o he orbicularis muscles is usu-
ibodies at ack he recep ors o he neuromus-
ally ound.
cular junc ion
T ere may also be acial and proximal limb
weakness. Treatment
Diagnosis may be made wi h he ice es , rea men o he disease is sys emic and may
ace ylcholine recep or an ibody es ing, include pyridos igmine bromide (Mes inon),
single-f ber EMG, or ensilon es ing. prednisone, and possible hymec omy.
Neurogenic Ptosis 83

rea men should be coordina ed by a Fron alis suspension is usually required.


neuro-oph halmologis or neurologis . Once
maximum medical improvemen has been Prognosis
achieved, surgical correc ion o he p osis can Variable depending on he severi y o he
be at emp ed. disease

FIGURE 5-14. Myasthenia gravis. Bila eral p osis wi h inabili y o keep he lids rom covering he pupils. T ere
is also decreased one o he acial muscles.
84 5 EYELID MALPO SITIO NS

MA CUS GUNN JAW-WINKING Movemen o he mandible la erally, o he


SYND OME con rala eral side mos commonly, resul s in
his syndrome may be very mild or very eleva ion o he eyelid ( Fig. 5-15).
drama ic wi h eleva ion o he eyelid
Special Considerations
wi h each jaw movemen when chewing ood.
rea men is required i he p osis or he eyelid T e amoun o p osis and he amoun
movemen is signif can . o synkine ic movemen will de ermine he
rea men .
Epidemiology and Etiology
Dif erential Diagnosis
Age: Presen a bir h
Congeni al p osis
E iology: A congeni al synkine ic syndrome
caused by a congeni al aberran connec ion o Laboratory Tests
he oculomo or nerve f bers ha innerva e he None
leva or muscle and he rigeminal nerve f bers
o he muscles o mas ica ion. Treatment
I he amoun o synkine ic movemen
History
is small, hen a ron alis suspension is done.
A care aker o en f rs no ices his condi ion I he synkine ic movemen is large, hen
when eeding he baby. he leva or muscle mus be disinser ed and
T e a ec ed eyelid will move up and down excised be ore he ron alis suspension can
wi h he jaw movemen during eeding. be done.

Examination Prognosis
A unila eral p osis wi h poor leva or unc- I may be di cul o ge good symme ry
ion. T e unila erally p o ic eyelid eleva es in he eyelids unless bo h eyes are opera ed
wi h movemen o he jaw. on.
Neurogenic Ptosis 85

FIGURE 5-15. Marcus Gunn jaw winking syndrome. A. Pa ien wi h severe p osis wi h ull eyebrow eleva ion and
chin up posi ion o keep his eyelids above he pupil.
( continued)
86 5 EYELID MALPO SITIO NS

FIGURE 5-15. (Continued) Marcus Gunn jaw winking syndrome. B. Wi h opening o his mou h, he eyelids go
up and he is able o normalize his head posi ion. C and D. More common unila eral jaw winking where lef upper
lid open wi h opening o he mou h.
( continued)
Neurogenic Ptosis 87

FIGURE 5-15. (Continued)


88 5 EYELID MALPO SITIO NS

HO NE ’S SYND OME O her pharmacologic es ing will di eren-

H orner’s syndrome classically presen s


wi h mild p osis (2 mm) and pupillary
miosis. A er any serious e iology has been
ia e f rs - and second-order neuron in errup-
ion rom hird-order neuron.
Hydroxyamphe amine drops will no
ruled ou , hese cases respond very well o sur- dila e he pupil o a hird-order neuron
gical correc ion wi h a müllerec omy. Horner’s syndrome.
A hird-order neuron Horner’s syndrome
Epidemiology and Etiology is generally o benign e iology.
Age: May be congeni al or acquired
Dif erential Diagnosis
E iology: E iology o acquired cases
includes rauma, surgical procedures in he Aponeuro ic p osis wi h pupillary
neck area, apical lung malignancies, aneurysm, anisocoria
dissec ion o he caro id ar ery, and idiopa hic.
Laboratory Tests
History Ches C and M I/ MR wi h gadolin-
Mild p osis is no ed. T e pupillary miosis ium o he neck and brain in all pa ien s wi h
may or may no be no ed un il he examina ion. f rs - or second-order neuron involvemen
Many neurologis s will image all pa ien s
Examination wi h Horner’s syndrome.
P osis, pupillary miosis, and anhidrosis are
he hree f ndings. Pathophysiology
T e p osis is usually mild (1 o 2 mm). In errup ion o he sympa he ic innerva-
ion o Müller’s muscle resul s in he p osis,
In congeni al Horner’s syndrome, here is
whereas he dila or muscle o he iris resul s
also decreased pigmen a ion o he iris on he
in he miosis.
involved side ( Fig. 5-16).
Treatment
Special Considerations
Once de ermined o be o benign e iology,
Cocaine es ing is used o conf rm he a müllerec omy is he procedure o choice.
diagnosis.
A 4% o 10% cocaine solu ion will dila e Prognosis
a normal pupil bu will ail o dila e a pupil T e p osis responds well o surgical
a ec ed by Horner’s syndrome. correc ion.
Neurogenic Ptosis 89

FIGURE 5-16. Horner’s syndrome. A. P osis wi h miosis on he lef side. T e amoun o p osis is more han is
of en seen in Horner’s syndrome and here may be some aponeuro ic dehiscence as well. B. Congeni al Horner’s
syndrome wi h p osis, miosis, and iris hypopigmen a ion.
90 5 EYELID MALPO SITIO NS

MECH ANICAL PTOSIS Eyelid scarring in ernally requires palpa-


ion and s re ching and eversion o he eyelid

M echanical p osis is drooping o he eye-


lid rela ed o res ric ion o he eyelid
rom ei her scar issue or he weigh o a mass
o iden i y i .

Special Considerations
or swelling. Imaging, such as C , may be needed
o de ermine he ex en o he mass or o
Epidemiology and Etiology rule ou a oreign body or a rac ure a er
Age: Any age rauma.
Gender: Equal Dif erential Diagnosis
E iology: T e increased weigh o any mass rauma ic p osis
will weigh he eyelid down. T is can include Aponeuro ic p osis
chalazion, skin carcinoma, gian papillary
conjunc ivi is, hemangiomas, neurof bromas, Laboratory Tests
and so or h. es ric ion o eyelid movemen None
by scar issue will also produce his orm o
p osis. Treatment
Addressing he cause is he primary
History rea men .
apidi y o onse varies according o he Managemen can include medical rea -
process men or surgical excision o a lesion or scar
Chalazion will have rapid onse , whereas a issue.
large basal cell carcinoma may slowly worsen Some pa ien s may require a second
over years. surgery o correc he p osis i removing he
Examination mechanical cause is no cura ive.
P osis wi h an eyelid mass or evidence o Prognosis
scar issue ( Fig. 5-17) ela ed o prognosis o he mass or
T e cause can be ex ernal or under he swelling ha is causing he p osis. I i is he
eyelid and di cul o see such as in severe resul o a recurren process, he prognosis
gian papillary conjunc ivi is. is poor.
Mechanical Ptosis 91

FIGURE 5-17. Mechanical ptosis.N euro broma o he lef upper lid weighing down he eyelid and causing a
p osis.
92 5 EYELID MALPO SITIO NS

TRAUMATIC PTOSIS Any residual swelling, scarring, and lag-


oph halmos should also be documen ed
rauma ic p osis has mul iple causes; ( Fig. 5-18).
however, exac ly which causes are pres-
en in each case can be di cul o de ermine. Special Considerations
Allowing 6 mon hs or spon aneous resolu- Mus wai 6 mon hs or possible resolu ion
ion be ore correc ion is he rule in all rau- o he p osis be ore repairing.
ma ic cases. A number o cases will improve or resolve
during his ime.
Epidemiology and Etiology Any injury ha sugges s direc cut ing o
Age: All ages he leva or should have explora ion o he
Gender: Males more common leva or a he ime o rauma repair i possible.
E iology: May include mul iple causes
Treatment
including myogenic, neurogenic, and aponeu-
ro ic injuries Moni or or improvemen over he 6 mon hs
ollowing he rauma.
History I he lid is s ill p o ic a er 6 mon hs, surgi-
rauma o he eyelid wi h residual p osis cal correc ion wi h ex ernal leva or resec ion
a er he swelling resolves or ron alis suspension depending on leva or
unc ion is indica ed.
Examination
Documen a ion o he severi y o p osis Prognosis
will allow moni oring or improvemen . Good i here is good leva or unc ion. I
Assessmen o leva or unc ion also helps here is poor leva or unc ion wi h scarring,
de ermine e iology. he prognosis is no as good.
Traumatic Ptosis 93

FIGURE 5-18. raumatic ptosis. Eyelid and eyebrow lacera ions resul ing in a rauma ic p osis. T is p osis did
no improve over 6 mon hs and required surgical repair.
94 5 EYELID MALPO SITIO NS

PSEUDOPTOSIS Evalua ion o he eyelid skin rela ive o


he rue eyelid margin posi ion is impor an

P seudop osis is he alse illusion o p osis


caused by malposi ion o he eye, con-
rala eral eyelid, or lack o orbi al volume. I
( Fig. 5-19A).
Eyelid re rac ion on he con rala eral side
mus also be considered.
is very impor an o recognize a pseudop o-
sis o avoid doing unnecessary or he wrong Special Considerations
surgery. C scanning may be needed i a mass
pushing he globe up is suspec ed.
Epidemiology and Etiology
Age: Any age Dif erential Diagnosis
Gender: Equal Enoph halmos ( Fig. 5-19B)
E iology: T e eyelid posi ion is normal Hyper ropia
bu o her ac ors resul in he appear- Globe malposi ion
ance o p osis. T e mos common is an Lid re rac ion o he con rala eral eyelid
abnormal posi ion o he eye. Eleva ion
Derma ochalasis
o he eye or he eye sinking in will bo h
give a alse p osis. Derma ochalasis (see Treatment
“Derma ochalasis”) is also a common cause
o pseudop osis. Depends on he cause o he pseudop osis
Enoph halmos, microph halmos, ph hisis
History bulbi: build up he orbi
Pa ien presen s wi h he complain o a Hyper ropia: consider s rabismus surgery
droopy eyelid or a variable period. Globe eleva ion: remove mass
Examination Con rala eral eyelid re rac ion: correc
In all pa ien s wi h p osis, evalua ion eyelid re rac ion
mus include no ing he posi ion o he Derma ochalasis: blepharoplas y
eye. Enoph halmos, hyper ropia, and a
globe pushed superiorly all cause Prognosis
pseudop osis. Good
Pseudoptosis 95

FIGURE 5-19. Pseudoptosis. A. Derma ochalasis is he mos common cause o pseudop osis. I he eyelid skin
is eleva ed, he eyelid is in a normal posi ion under he skin. B. Ano her orm o pseudop osis is enoph halmos
resul ing in drooping o he eyelid. T is pa ien may also have some componen o leva or aponeurosis disinser ion.
96 5 EYELID MALPO SITIO NS

BROW PTOSIS T is may give he appearance o excess


upper eyelid skin. Pa ien s will also develop

B row p osis is drooping o he eyebrows.


I is o en a par o derma ochalasis bu
mus be recognized and rea ed separa ely or
urrows o he orehead rom chronically
eleva ing heir eyebrows ( Fig. 5-20).

he bes resul s. Many pa ien s will have some Special Considerations


degree o brow p osis and no have symp oms. Brow droop will add o redundan skin o
Symp oms can include loss o superior visual he upper eyelids.
f eld, brow ache and a igue, and rhy ids o I is impor an o recognize how much
he orehead and brow. T ose wi h signif can o he excess skin on he upper eyelids
brow p osis may require eyebrow surgery wi h will go away i he eyebrows are eleva ed.
or wi hou eyelid surgery. T is excess rom he brow droop should
no be excised during blepharoplas y or
Epidemiology and Etiology
he eyebrows will appear oo close o he
Age: More common as pa ien s age eyelids.
Gender: Equal. Females are more likely o Ideally, he brow should be li ed and hen
be symp oma ic. he blepharoplas y per ormed.
Epidemiology: Wi h aging, gravi y, invo-
lu ional changes, and loss o elas ici y resul Treatment
in drooping o he eyebrows. Depending on Brow li using one o he ollowing
mul iple ac ors, his drooping will become echniques; each has i s own indica ions and
symp oma ic in hose 50 years o age or older. advan ages.
History Endoscopic eyebrow li
Pa ien s will at emp o li heir eyebrows Coronal eyebrow li
resul ing in deep urrows o he orehead, Mid orehead eyebrow li
brow ache, and even headaches. Direc eyebrow li
Examination Prognosis
Eyebrows si below he superior orbi al rim. Good
BrowPtosis 97

FIGURE 5-20. Brow ptosis.T e eyebrows are well below he orbi al rim in his pa ien , adding o he apparen
amoun o derma ochalasis.
98 5 EYELID MALPO SITIO NS

DERMATO CH ALASIS History


Symp oms are brow ache, heaviness

D erma ochalasis is a very common con-


di ion which increases as people age.
Loss o elas ici y rom ul raviole exposure
around he eyes, and loss o superior visual
f eld. T ese have a slow, insidious onse .

and aging resul s in excess skin o he upper Examination


and lower lids. I severe enough, he upper Excess skin o he upper eyelids o varying
eyelid skin may cause unc ional blockage o degrees
he superior visual f eld. More commonly, he I becomes especially bo hersome a er he
excess skin and he associa ed an erior pro- skin con ac s he eyelashes.
lapse o orbi al a is a cosme ic de ormi y. Derma ochalasis is o en associa ed wi h
an erior prolapse o orbi al a .
Epidemiology and Etiology Underlying he excess skin, he possibili y
Age: Older pa ien s o a rue p osis mus be evalua ed ( Fig. 5-21).
Gender: Equal male- o- emale ra io Treatment
E iology: E iology is loss o eyelid skin Blepharoplas y, which may be unc ional
elas ici y rom aging and ul raviole ligh or cosme ic in na ure.
exposure. T ere may be a heredi ary compo-
nen o derma ochalasis, especially when i Prognosis
occurs a a younger age. Good
Dermatochalasis 99

FIGURE 5-21. Dermatochalasis.T ere is a large amoun o overhanging skin o bo h upper eyelids. T e
underlying eyelid posi ion is normal. T ere is also some brow droop, which was addressed surgically a he
same ime as he blepharoplas y.
100 5 EYELID MALPO SITIO NS

BLEPH ARO CH ALASIS T ere may also be rue p osis, lacrimal gland
prolapse, and prominen vessels o he lids.

B lepharochalasis is a rare, amilial vari-


an o angioneuro ic edema ha occurs
in younger individuals. I is charac erized by
Mos commonly, hese f nding are unila -
eral. Pa ien s may also be seen a he ime o
swelling wi h a swollen, uid-f lled lid wi h
recurren episodes o in amma ory edema o very lit le in amma ory signs.
he eyelids ha resul s in s re ching o he is-
sues; over ime, he eyelids ake on he appear- Dif erential Diagnosis
ance o derma ochalasis commonly seen in Derma ochalasis
much older pa ien s. T yroid-rela ed oph halmopa hy
Orbi al in amma ory disease
Epidemiology and Etiology
Age: Onse in eens o 20s Pathophysiology
Gender: More common in emales Unknown
E iology: Unknown; a varian o angioneu- Treatment
ro ic edema rea men is surgical excision o he skin
and correc ion o p osis.
History
Surgical repair can be complica ed by
ecurren episodes o eyelid swelling, usu- recurren edema, which may resul in recur-
ally unila eral rence o he problem.
Examination Prognosis
Excess skin o he eyelids ha is very hin Variable, depending on whe her he edema
and paper-like ( Fig. 5-22) con inues o recur or burns ou
Blepharochalasis 101

FIGURE 5-22. Blepharochalasis.T is is a 25-year-old pa ien wi h recurren swelling o he righ eyelids


resul ing in he hin, s re ched, redundan skin o he upper and lower eyelid.
102 5 EYELID MALPO SITIO NS

EYELID RETRACTION Dif erential Diagnosis


Malposi ion o he globe

E yelid re rac ion is displacemen o he


eyelid oward he respec ive superior or
in erior orbi al rim, resul ing in scleral show.
Hypo ropia or hyper ropia
Con rala eral p osis
T is condi ion can be mild and wi hou Laboratory Tests
symp oms or can resul in corneal exposure.
T yroid oph halmopa hy is he mos common T yroid unc ion es s unless pa ien has
cause and eyelid re rac ion is o en he ini ial known, con rolled hyroid abnormali ies or
sign o his disease. here is a known surgical cause o he eyelid
re rac ion.
Epidemiology and Etiology
Pathophysiology
Age: Adul hood. are in children. Age o
T yroid oph halmopa hy resul s in
onse dependen on e iology.
chronic in amma ion o he lid re rac ors
Gender: Females more common leading o scar issue orma ion and re rac ion
E iology: Eyelid re rac ion is caused by o he eyelids.
hyroid oph halmopa hy mos commonly,
ollowed by overaggressive eyelid surgery and Treatment
ver ical rec us muscle surgery ( Fig. 5-23). In hyroid oph halmopa hy, he disease
Upper eyelid re rac ion may be he resul o mus be rea ed so i is inac ive be ore any
con rala eral p osis. Lower lid re rac ion can surgical correc ion.
be a normal ana omic varian . Parinaud syn- rea corneal exposure wi h lubrica ion
drome is a cen ral nervous sys em cause o while wai ing or he disease o become
upper eyelid re rac ion. inac ive.
History Mos causes o eyelid re rac ion will require
In hyroid oph halmopa hy, he pa ien no es surgical rea men i hey are signif can .
slow onse o one or bo h eyes appearing oo ecession o he re rac ors is he mos
wide open or having he “hyper hyroid s are” common procedure used.
O en, redness and irri a ion o he T is works or mild o modera e
a ec ed eye accompany hese symp oms. re rac ion.
Pa ien s may have a his ory o sys emic More severe re rac ion o he lower eye-
hyroid abnormali ies. lids requires eyelid spacer ma erial o be
T ose pa ien s wi h a surgical cause will implan ed.
give he his ory o eyelid or eye muscle surgery. Excess excision o skin o he upper or
lower lids during blepharoplas y may require
Examination in ernal spacers or rarely skin gra s.
Documen he amoun o eyelid re rac ion
and whe her here is re rac ion or p osis on Prognosis
he o her side. Generally, re rac ion can be rea ed suc-
Upper eyelid lag on down gaze, prop osis, cess ully wi h surgery.
and dysmo ili y all go along wi h hyroid oph- Corneal exposure is o en an ongoing
halmopa hy. No e signs o corneal exposure problem ha is improved wi h rea men bu
and previous eye or eyelid surgery. no comple ely cured.
Eyelid Retraction 103

FIGURE 5-23. Eyelid retraction. A. Lef , lower eyelid re rac ion rom scarring o he eyelid o he orbi al rim
and a i anium pla e af er rauma and orbi al rac ure repair. B.T yroid-rela ed oph halmopa hy wi h upper
eyelid re rac ion.
104 5 EYELID MALPO SITIO NS

EYELID DYSKINESIS T e spasms are bila eral, al hough hey can


some imes be more severe on one side han
BENIGN ESSENTIAL he o her.
BLEPHA OSPASM Spasms can involve he lower ace and

B enign essen ial blepharospasm is a bila -


eral condi ion charac erized by involun-
ary spasms o he orbicularis oculi, procerus,
neck wi h ime.
Spasms do no occur during sleep, unlike
in hemi acial spasm.
and corruga or muscles. T is condi ion may
s ar as mild wi ching o he eyelids and can Special Considerations
progress so ha hese con rac ions leave he Mus rea any condi ion ha may cause
pa ien s unc ionally blind during he con rac- ocular irri a ion and hus worsen he blepha-
ion. Pa ien s canno predic when he spasms rospasm such as dry eyes.
will occur and he orced closure o he eyelids
Blepharospasm can have a al conse-
can be li e- hrea ening i hey occur during
quences i no con rolled and he pa ien
driving, crossing s ree s, and so or h.
drives.
Epidemiology and Etiology Dif erential Diagnosis
Age: Onse is when pa ien s are 40 years o Hemi acial spasm
age or older.
Severe dry eyes or o her ocular irri a ion
Gender: Women more commonly a ec ed
han men Treatment
E iology: Unknown, bu probably o cen- Bo ulinum oxin injec ion. T ese
ral nervous sys em origin, mos likely in he injec ions are e ec ive or mos pa ien s
basal ganglia. T e process causes involun ary and las 3 o 4 mon hs be ore reinjec ion
spasms o he orbicularis oculi, procerus, and is required.
corruga or muscles. Wi h ime, in some pa ien s hese injec-
History ions may become less e ec ive and par ial
surgical excision o orbicularis muscle and
T e spasms s ar as mild wi ches and pro- o her pro rac ors will be required.
gressively worsen wi h ime.
Bo ulinum oxin injec ion may s ill be
Pa ien s o en do no presen un il he needed bu will be more e ec ive a er
spasms are severe enough o in er ere wi h surgery.
ac ivi ies o daily living.
Muscle relaxan s and seda ives are occa-
Examination sionally used in his disease bu are o lit le
Pa ien s have in ermit en episodes o benef .
orced eyelid closure ha usually las or min-
Prognosis
u es ( Fig. 5-24). Be ween spasms, he exami-
na ion may be normal. T us, he diagnosis is Good wi h bo ulinum oxin injec ion
o en based on his ory. are cases may no respond o rea men .
Eyelid Dyskinesis 105

FIGURE 5-24. Benign essential blepharospasm.T is pa ien would develop blepharospasm wi h any at emp o
ouch he eyes. T e spasm can go on o involve o her acial muscles.
106 5 EYELID MALPO SITIO NS

HEMIFACIAL SPASM Dif erential Diagnosis


Epidemiology and Etiology Benign essen ial blepharospasm
Age: Adul hood Myokymia
Gender: Equal male o emale ra io
Treatment
E iology: Vascular compression o he
acial nerve a he level o he brains em T e pa ien needs M I o he cerebellar–
pon ine angle o rule ou a mass lesion.
History Bo ulinum oxin is hen generally he rea -
Unila eral spasm o one side o he ace men o choice.
Neurosurgical decompression o he acial
Examination nerve is some imes considered.
T e pa ien may have mild acial palsy on
he a ec ed side. T e spasm may be seen on Prognosis
examina ion or no ed by he pa ien ’s his ory Bo ulinum A oxin con rols he spasm
( Fig. 5-25). bu requires repea injec ion every 3 o
6 mon hs.
Special Considerations
Spasms are presen during sleep, unlike
benign essen ial blepharospasm where hey
are absen .
Eyelid Dyskinesis 107

FIGURE 5-25. Hemifacial spasm. Hemi acial spasm o he lef side o he ace. T e spasm is usually in ermit en .
C H AP T ER

Congeni al Eyelid Anomalies

BLEPH AROPHIMOSIS mo ili y disorders, and various degrees o


men al def ciency ( Fig. 6-1).

B lepharophimosis is a congeni al eyelid


syndrome ha has a charac eris ic eyelid
appearance and includes elecan hus, epican-
Dif erential Diagnosis
No o her syndrome gives hese charac-
hus inversus, and severe myogenic p osis. eris ic changes. Mus di eren ia e rom
simple epican hus (see Fig. 6-2) and
Epidemiology and Etiology elecan hus.
Age: Congeni al Treatment
Gender: Equal Mul iple s ages o recons ruc ion are
Inheri ance: Au osomal dominan required.
E iology: Unknown Ini ial surgery is aimed a he elecan hus
and epican hus inversus.
History T is may require a simple Z-plas y, Y–V
O en have amily members wi h he same plas y, or ransnasal wiring.
syndrome T e second s age is correc ion o he
p osis, which usually requires ron alis
Examination suspension.
Charac eris ic eyelid f ndings include ele- Finally, o her eyelid abnormali ies are
can hus, epican hus inversus, and severe addressed.
p osis.
O her f ndings, which may or may no be Prognosis
presen , include lower eyelid ec ropion, a Signif can improvemen can be made wi h
poorly developed nasal bridge, hypoplasia o surgery. Depending on he severi y, here will
he superior orbi al rims, hyper elorism, always be some eyelid changes ha remain.

108
Blepharophimosis 109

FIGURE 6-1. Blepharophimosis. T is child has classic changes o blepharophimosis with ptosis, telecanthus,
and epicanthus inversus. T is must be dif erentiated rom simple epicanthus ( see Fig. 6-2) .
110 6 CO NGENITAL EYELID ANO MALIES

EPICANTHUS Epican hus palpebralis: Fold is equally


on he upper and lower eyelids ( Fig. 6-2).

E pican hus is a medial can hal old ha is


usually caused by imma ure mid acial
bones. T e condi ion is usually bila eral and
Epican hus superciliaris: Fold runs rom
he eyebrow region o he lacrimal sac.

will resolve as he child’s ace ma ures. Special Consideration


Epican hus is associa ed wi h cer ain eyelid Epican hus arsalis can be a normal varian
syndromes. o he Asian eyelid. Epican hus inversus is
par o blepharophimosis syndrome.
Epidemiology and Etiology
Age: Congeni al Dif erential Diagnosis
Gender: Equal Blepharophimosis
E iology: Imma ure mid acial bones are
Treatment
considered o be he cause.
Mos cases o epican hus resolve wi h nor-
History mal acial ma ura ion, so any po en ial rea -
No ed a bir h men should be delayed un il he child is
ma ure.
Examination T e excep ion is epican hus inversus,
T ere is an ex ra old o skin and subcu a- which rarely disappears wi h acial
neous issue medially involving he eyelids. ma ura ion.
T is may make he child appear eso ropic. I surgical rea men is required, hese
Four ypes o epican hus have been cases respond well o a Y–V-plas y or Z-plas y.
described:
Epican hus arsalis: Fold is more promi- Prognosis
nen on he upper eyelid. Excellen . Mos cases resolve as he child
Epican hus inversus: Fold is more grows.
prominen on he lower eyelid. I surgery is required, he resul s are good.
Epicanthus 111

FIGURE 6-2. Epicanthal folds. T ese olds can o en be seen as an isolated nding in young children. Unless
severe, these epicanthal olds will lessen and even disappear as the child’s ace matures.
112 6 CO NGENITAL EYELID ANO MALIES

EPIBLEPH ARON I here are corneal changes, considera ion


mus be given o surgical correc ion ( Fig. 6-3).

E piblepharon is override o he pre arsal


muscle and skin, which causes he cilia o
assume a ver ical posi ion al hough he eyelid
Special Considerations
I is o en very di cul o di eren ia e epi-
margin is in a normal posi ion. T is disorder is blepharon rom congeni al en ropion, espe-
usually asymp oma ic wi h no corneal s aining cially in an awake child who is squeezing his
and requires no rea men . or her eyes shu .
I here are signif can corneal changes,
Epidemiology and Etiology hen i is likely he eyelid is en ropic and sur-
Age: Congeni al gery is required.
Gender: Equal Dif erential Diagnosis
E iology: Imma ure acial bones are el o Congeni al en ropion
allow or his excess skin and muscle.
Treatment
History No rea men is needed in mos cases.
T ere are usually no symp oms. I here are corneal changes, hen excision
Examination o he excess skin and muscle is he rea men
o choice.
T ere is an excess o skin overriding he eye-
lid margin, which may even come in con ac Prognosis
wi h he eye. I his skin can be pulled back, he Excellen . Mos cases resolve as he acial
eyelid margin under i is in a normal posi ion. bones ma ure. T e rare case ha requires
T ere is rarely any corneal s aining. surgery will respond well.
Epiblepharon 113

FIGURE 6-3. Epiblepharon (A&B). Excess skin o the lower eyelid rolls in and touches the cornea. T e eyelid is
in a normal position and the cornea is normal. T is condition must be dif erentiated rom congenital entropion
(see Fig. 6-4).
114 6 CO NGENITAL EYELID ANO MALIES

CONGENITAL ENTROPION is awake and an at emp is made o examine


he eyelid, he child squeezes he eye shu and

C ongeni al en ropion is rare bu can also


be di cul o diagnose in an in an . An
eye ha is always irri a ed and ha he child
a normal eyelid may urn in.
Evidence o corneal scarring or an epi he-
lial de ec on he in erior cornea is enough o
does no wan o open is a clue o look or an suspec an en ropion ( Fig. 6-4).
epi helial de ec or corneal scar.
Epidemiology and Etiology Dif erential Diagnosis
Age: A bir h Epiblepharon
Gender: Equal
E iology: Usually rela ed o an abnormal- Treatment
i y o he eyelid re rac ors or arsus. Very Surgical correc ion is required.
rarely, here can be conjunc ival scarring Excision o pre arsal skin and orbicularis
causing he en ropion. wi h igh ening o he re rac ors is he rea -
men o choice.
History
T e child’s eye is always irri a ed, and he
Prognosis
child does no wan o open he eye.
Good. Some chance o corneal scarring i
Examination no diagnosed early.
I is di cul o examine he eyelid o an
in an unless hey are asleep. When he child
Congenital Entropion 115

FIGURE 6-4. Congenital entropion. A. It is di cult to examine a child’s eyelid to determine whether it is
entropic, especially when the eye is already irritated. In this child, there is corneal scarring rom a congenital
entropion. B. Child immediately a er placing rotating sutures in the lower eyelid. C. Postoperative picture
4 weeks a er entropion surgery. T e eyelid is in a normal position and the corneal opacity is resolving.
116 6 CO NGENITAL EYELID ANO MALIES

CONGENITAL COLOBOMA Colobomas in his loca ion are no usually


associa ed wi h any o her abnormali y. A col-

C ongeni al colobomas are ull hickness


de ec s in he eyelid. Even larger de ec s
are usually well olera ed over he shor erm
oboma o he lower eyelid is more likely o
be par o a acial cle syndrome and may
have o her acial de ec s and lacrimal
un il he de ec can be repaired. Upper eyelid abnormali ies.
colobomas are usually no associa ed wi h At en ion mus be given o he cornea or
o her sys emic abnormali ies, whereas lower signs o exposure, al hough exposure is rare
eyelid colobomas are more commonly associ- ( Fig. 6-5).
a ed wi h acial cle syndromes.
Dif erential Diagnosis
Epidemiology and Etiology Bir h rauma o he eyelid
Age: Apparen a bir h
Gender: Equal Treatment
E iology: Abnormal embryonic develop- Surgical repair o he coloboma is usually
men resul s in hese eyelid de ec s. s raigh orward and can be done wi hou any
aps ha would occlude he eye and cause
History amblyopia.
Eyelid de ec is usually no ed a bir h or
Prognosis
soon a er. T ere are ew symp oms.
Colobomas do very well wi h surgical
Examination repair.
T e ull- hickness de ec is mos com- O her acial de ec s may no be as easy o
monly medially on he upper eyelid. repair.
Congenital Coloboma 117

FIGURE 6-5. Congenital coloboma. A. Child born with a coloboma o the upper eyelid. T is may be a totally
isolated nding, but coloboma and a preauricular skin tag (B) are consistent with Goldenhar’s syndrome.
118 6 CO NGENITAL EYELID ANO MALIES

CONGENITAL DISTICHIASIS Dif erential Diagnosis


Congeni al en ropion

D is ichiasis is a rare condi ion where an


ex ra row o eyelashes replaces he mei-
bomian gland openings on he eyelids.
Conjunc ival scarring causing richiasis

Treatment
Epidemiology and Etiology rea men is based on he symp oms and
Age: Presen a bir h individualized. I rea men is required,
op ions are variable.
Gender: Equal
Conserva ive rea men wi h lubrica ion
E iology: Embryonic pilosebaceous uni s
and con ac lenses is o en no success ul.
improperly di eren ia e in o hair ollicles.
Eyelash abla ion wi h cryo herapy or
History elec rolysis o en resul s in recurrence o
T e ex ra row o lashes may be no ed or eyelashes bu will be adequa e in some
eye irri a ion may promp oph halmic evalua- pa ien s.
ion a which ime he problem is discovered. Surgical excision o he eyelashes and
recons ruc ion—some imes using buccal
Examination mucosal gra s—works in he mos severe
A second row o eyelashes is no ed grow- cases.
ing pos erior o he normal eyelash posi ion
( Fig. 6-6). T ese lashes may be in con ac Prognosis
wi h he cornea causing symp oms o eye irri- Usually good, bu mul iple procedures
a ion, corneal punc a e s aining, and scarring. may be required and here may be undesirable
A good corneal evalua ion is impor an . cosme ic de ec s a er surgery.
Congenital Distichiasis 119

FIGURE 6-6. Congenital distichiasis. All our eyelids have these extra rows o eyelashes growing out o the
position where the meibomian glands should be.
120 6 CO NGENITAL EYELID ANO MALIES

ANKYLOBLEPH ARON T e eye and orbi may be normal or have


associa ed abnormali ies ( Fig. 6-7A).

C ongeni al ankyloblepharon is a ailure o


he eyelids o separa e during embryonic
developmen . Ankyloblepharon may also be
Acquired ankyloblepharon shows usion o
he eyelids rom scar issue. T e eye i sel is
no able o be seen ( Fig. 6-7B).
acquired due o scarring, which resul s in
adherence o he eyelids o each o her and o Dif erential Diagnosis
he globe. Cryp oph halmos
Epidemiology and Etiology Microph halmos
Age: Congeni al. Acquired cases can occur
Treatment
a any age depending on he cause.
Congeni al ankyloblepharon: lysis o he
Gender: Equal
bands holding he eyelids oge her
E iology: Congeni al ailure o he eyelids
O her recons ruc ive procedures may be
o separa e during embryonic developmen .
needed depending on severi y.
Acquired ankyloblepharon is mos com-
monly he resul o progressive conjunc ival Acquired ankyloblepharon: de ermining
scarring resul ing in usion o he eyelids. he e iology o he scarring is done f rs .
Some causes include ocular cica ricial pem- I his process needs rea men o quie
phigoid, S evens–Johnson syndrome, chemi- any in amma ion, hen ha mus be done
cal burns, and herpes zos er. f rs .
At emp s o recons ruc he eyelids and
History resur ace he cornea can hen be at emp ed.
Congeni al cases no ed a bir h T is requires coordina ion be ween oculo-
Acquired cases will usually have a his ory plas ic and corneal surgeons.
o progressive scarring rela ed o he primary
Prognosis
disease.
Congeni al ankyloblepharon: good
Examination Acquired ankyloblepharon: poor in mos
Congeni al ankyloblepharon may have cases. T e process causing he scarring will
comple e usion o he eyelids or jus a ew o en hamper he healing a er eyelid and cor-
bands holding he eyelids oge her. neal recons ruc ive surgery.
Ankyloblepharon 121

FIGURE 6-7. Congenital ankyloblepharon. A. Unilateral combined ankyloblepharon and cryptophthalmos.


(Courtesy Richard W. Hertle, MD.) Ankyloblepharon. B. Acquired ankyloblepharon as the result o scarring rom
ocular cicatricial pemphigoid. T e eyelids are used, and there is likely scarring o the eyelids to the globe as well.
C H AP T ER

Miscellaneous Eyelid Condi ions

OCULAR CICATRICIAL Some pa ien s will have ulcera ion o o her


PEMPHIGOID mucosal sur aces such as oral, esophageal, or
geni al lesions.

O cular cica ricial pemphigoid (OCP) is a


conjunc ival scarring disease ha occurs
in older adul s. I can be mild or can be pro-
Skin lesions may also be par o he
presen a ion.
A signif can number o hese pa ien s have
gressive and lead o corneal scarring and blind- used or are curren ly using an iglaucoma drops.
ness. OCP con inues o be a con using, poorly
unders ood condi ion ha can be very di - Examination
cul o rea in some pa ien s. Findings range rom mild, sub le conjunc i-
val scarring in he early s ages o severe scar-
Epidemiology and Etiology ring where he eyelid is s uck o he cornea.
Age: Older adul s Cica ricial en ropion, richiasis, and severe
dryness all add o he poor ocular sur ace. T e
Gender: More common in emales
condi ion o he cornea is impor an in guid-
E iology: An au oimmune process in ing rea men .
which an ibodies bind o he conjunc ival
Evalua ion o he mou h and skin or o her
basemen membranes, resul ing in in amma-
lesions is impor an ( Fig. 7-1).
ion and scarring.
Special Considerations
History
Some pa ien s on an iglaucoma medica-
T ere may be a long his ory o ocular ions will ge conjunc ival scarring ha is no
irri a ion and epila ion o eyelashes progressive i he medica ion is s opped. T is
over many years. T e o her ex reme is f nding was more common in pa ien s using
rapidly progressive conjunc ival and even mio ics, such as pilocarpine, bu also seems
corneal scarring wi h very red in amed o be associa ed wi h some o he modern
eyes. an iglaucoma drops.

122
Ocular Cicatricial Pemphigoid 123

I is no clear whe her hese pa ien s have membrane ha resul s in in amma ion
OCP or i he scarring is en irely rela ed o and even ual scarring. T is des roys he
he drops. ear glands o he conjunc iva and causes
in- urned eyelids, lashes and corneal
Dif erential Diagnosis
scarring.
S evens–Johnson syndrome
Acid and alkali burns Treatment
Previous eyelid surgery T e in amma ion mus be quie ed f rs .
T is may require only doxycycline in very
rachoma
mild cases or s rong medica ions such as
A opic disease cyclophosphamide, mycophenola e mo e il
Laboratory Tests or aza hioprine in re rac ory cases.
Immuno uorescence es ing o he con- A er he in amma ion is quie , eyelid prob-
junc iva will reveal immunoglobulins a he lems, such as richiasis or en ropions, can be
basemen membrane in OCP. addressed surgically. All pa ien s will require
aggressive lubrica ion and/ or punc al occlusion.
A posi ive biopsy is diagnos ic, bu a nega-
ive biopsy does no rule ou OCP because
Prognosis
here are a signif can number o biopsy-
nega ive OCP cases. Variable. Some pa ien s’ disease will burn
ou or respond o rea men wi hou signif -
Pathophysiology can ocular injury. In o her pa ien s, he dis-
An au oimmune process in which immune ease can progress no mat er wha rea men
complexes bind a he conjunc ival basemen is used.
124 7 MISCELLANEO US EYELID CO NDITIO NS

FIGURE 7-1. Ocular cicatricial pemphigoid. A. Scarring o the eyelid to the cornea in this advanced case. B. Earlier
in the disease, the conjunctival scarring is less obvious and may even be overlooked i the conjunctiva in the ornices
is not care ully examined.
( continued)
Ocular Cicatricial Pemphigoid 125

FIGURE 7-1. ( Continued) Ocular cicatricial pemphigoid. C.T e lower eyelid is entropic secondary to
conjunctival scarring rom pemphigoid. D. Oral ulcerations are of en ound in active ocular cicatricial pemphigoid
and help solidi y the diagnosis.
C H AP T ER

Lacrimal Obs ruc ions

CONGENITAL Mos obs ruc ions will spon aneously


OBSTRUCTIONS resolve by he age o 6 o 12 mon hs.

Examination
CONGENITAL NASOLACRIMAL
DUCT OBSTRUCTION Diagnosis is based mainly on he his ory.

C ongeni al nasolacrimal duc obs ruc ion


is seen in 2% o 6% o newborns bu
resolves in he f rs 3 o 4 weeks in mos in an s.
Examina ion may reveal increased ear f lm
and some crus ing o he eyelashes.
Mucus re ux wi h pressure over he
T e chronic purulen discharge is he main lacrimal sac conf rms he diagnosis, bu is no
problem or caregivers, bu , wi h addi ional always presen . Examina ion mus rule ou a
ime, a large percen age o hese obs ruc ions dacryocys ocele or any sign o in ec ion
will resolve on heir own. ( Fig. 8-1).
Epidemiology and Etiology
Dif erential Diagnosis
Age: Congeni al
Chronic conjunc ivi is
Gender: Equal in males and emales
Punc al dysgenesis
E iology: Incomple e developmen o he
En ropion
dis al lacrimal passage wi h a membranous
block a he valve o Hasner richiasis

History Treatment
Paren s will no e a chronic mucous dis- iming o he rea men is con roversial.
charge wi h mat ing o he eyelashes a 3 o Nine y percen o all congeni al nasolacri-
4 weeks o age in 2% o 6% o ull- erm in an s mal duc obs ruc ions will resolve by age
in one or bo h eyes. 12 mon hs.

126
Congenital Obstructions 127

Many physicians will use conserva ive T e rare pa ien will require a
rea men un il his ime. T is managemen dacryocys orhinos omy.
consis s o massage wi h opical an ibio ics as
needed o con rol he mucus discharge. Prognosis
Probing and irriga ion under general anes- rea men is very success ul. Wai ing or
hesia will success ully rea 90% o pa ien s. spon aneous resolu ion while he child has
T ose pa ien s no responsive o probing and chronic discharge is o en di cul or he
irriga ion may require in uba ion wi h silicone caregivers.
ubes wi h or wi hou a balloon dacryoplas y.

FIGURE 8-1. Congenital nasolacrimal duct obstruction. T ere is redness, crusting, and irritation o the right
eyelids rom the chronic discharge. T e tear f lm is also increased. In many patients with congenital nasolacrimal
duct obstruction, there will be no external signs and the diagnosis is based on the history the caregivers report.
128 8 LACRIMAL O BSTRUCTIO NS

DACRYOCYSTOCELE I a mass is no ed above he medial

A dacryocys ocele is a rare lesion no ed a


bir h in he medial can hal area. I repre-
sen s uid and mucus rapped in he lacrimal
can hal endon, ano her e iology mus be
considered.
I bila eral consider nasal exam and chance
sac. Dacryocys oceles will resolve bu mus be o respira ory obs ruc ion.
observed care ully because hey can become
in ec ed.
Dif erential Diagnosis
Epidemiology and Etiology Hemangioma
Age: Congeni al Meningoencephalocele
Gender: Equal in males and emales Dacryocys i is
E iology: Blockage o he lacrimal sys em
dis ally, a he valve o Hasner, and proxi- Treatment
mally, a he valve o Rosenmüller, resul ing in Observa ion or he f rs 1 o 2 weeks wi h
rapped amnio ic uid and/ or mucus pro- massage
duced by he lacrimal sac goble cells.
Many will resolve on heir own.
History Probing is required i here is any sign
Cys ic swelling o he medial can hus o in ec ion or i here is no resolu ion a er
below he endon no ed a bir h 2 weeks.
Examination
Prominen cys ic mass below he medial Prognosis
can hal endon ( Fig. 8-2) Excellen

FIGURE 8-2. Dacryocystocele. T e large, distended right lacrimal sac is easily seen and is f rm to palpation.
T is child underwent probing and irrigation, which resolved the obstruction.
Congenital Obstructions 129

LACRIMAL FISTULA Examination

A lacrimal f s ula is an ex ra opening o he


lacrimal sys em on o he skin usually
loca ed in erior-nasal o he lacrimal punc um.
Small cu aneous opening in erior and
nasal o he medial can hal angle
May or may no have ears exi ing rom i
One hird o f s ulas will have an associa ed ( Fig. 8-3)
lacrimal obs ruc ion wi h chronic mucous
discharge. T e o her pa ien s are o en Dif erential Diagnosis
asymp oma ic. Mus de ermine i here is associa ed
dacryos enosis
Epidemiology and Etiology
Age: ypically congeni al, bu acquired f s- Treatment
ulas can occur a an age. I symp oma ic, he epi helial lined
Gender: Equal in males and emales is ula can be excised. I here is also an
E iology: Abnormal embryonic develop- associa ed dacryos enosis, a dacryocys o-
men o he lacrimal sys em. Cases o rhinos omy and excision o he is ula is
acquired f s ulas are rela ed o dacryos enosis indica ed.
wi h dacryocys i is. Acquired f s ulas will disappear when he
dacryocys i is resolves.
History
O en asymp oma ic unless here is an Prognosis
associa ed dacryos enosis Excellen

FIGURE 8-3. Congenital lacrimal f stula. Note the very small opening in erior-nasal to the puncta, which is
connected to the lacrimal system.
130 8 LACRIMAL O BSTRUCTIO NS

ACQUIRED OBSTRUCTIONS Def ni ive diagnosis made wi h irriga ion


o he lacrimal sys em, which will demon-
ACQUIRED NASOLACRIMAL DUCT s ra e obs ruc ion o ow ( Fig. 8-4).
OBSTRUCTION
Dif erential Diagnosis
A cquired nasolacrimal duc obs ruc ion
becomes more common as pa ien s age.
T e obs ruc ion mos commonly occurs in he
O her causes o earing such as:
Kera i is sicca
nasolacrimal duc . Pa ien s may presen wi h Blephari is
earing or an in ec ion. Many pa ien s may
Ec ropion
have an obs ruc ion and be wi hou symp oms.
Punc al abnormali ies
Epidemiology and Etiology
Age: Older pa ien s Special Tests
Gender: Females mos commonly Dacryocys ography may help def ne lacri-
mal s enosis in di cul cases and in par ial
E iology: Involu ional changes in he lacri- obs ruc ions.
mal duc / sac is he mos common cause.
Naso-orbi al rauma or surgery, sinusi is, and Treatment
dacryocys i is are also causes.
Symp oma ic comple e obs ruc ion
History requires a dacryocys orhinos omy.
Con inual earing, which may have been Par ial obs ruc ions can be rea ed wi h
preceded by in ermit en episodes o earing. balloon dacryoplas y.
T e process is mos commonly unila eral bu
Prognosis
may be bila eral.
Dacryocys orhinos omy is success ul 90%
Examination o he ime or more o en.
Increased ear f lm on sli lamp examina- Balloon dacryoplas y is 70% o 80% suc-
ion wi h abnormal dye disappearance es cess ul bu is less invasive.
Acquired Obstructions 131

FIGURE 8-4. Acquired nasolacrimal duct obstruction. T ere are o en no external signs o acquired nasolacrimal
duct obstruction. T ere are excess tears running down the cheek and slight injection o the right eye. I there is
some dacryocystitis associated with the blockage, the eye may be red.
132 8 LACRIMAL O BSTRUCTIO NS

CANALICULAR OBSTRUCTION Special Considerations


Epidemiology and Etiology T ere may be an addi ional, more dis al,
Age: Any obs ruc ion in he lacrimal sac and duc in
some o hese cases.
Gender: More common in emales
E iology: rauma, ex ernal conjunc ival Dif erential Diagnosis
in ec ions (Epidemic Kera oconjunc ivi is, Kera i is sicca
herpes), canaliculi is, sys emic chemo herapy Blephari is
Ec ropion
History
O her lacrimal sys em abnormali ies
Onse o earing may be gradual or
acu e. Treatment
Silicone in uba ion wi h or wi hou a
Examination dacryocys orhinos omy
Increased ear f lm, normal eyelid Prognosis
posi ion, and evidence o canalicular Canalicular obs ruc ions have a poorer prog-
obs ruc ion on probing o he canaliculi nosis han more dis al obs ruc ions. Success is
( Fig. 8-5) in he range o 50%, depending on he e iology.
Acquired Obstructions 133

FIGURE 8-5. Canalicular obstruction. External signs o herpes simplex are the only sign o the canalicular
obstruction. Probing demonstrates canalicular scarring as a result o herpes simplex.
C H AP T ER

Lacrimal In ec ions

DACRYOCYSTITIS Orbi al celluli is mus be considered i he


in ec ion is severe ( Fig. 9-1B).
Epidemiology and Etiology Pa ien s wi h a low-grade chronic in ec ion
Age: Mos common in older adul s bu can may have mucus/ pus expressible hrough he
be seen a any age. canaliculi wi h pressure over he lacrimal sac.
Gender: More common in emales T e conjunc iva may be injec ed.
E iology: Nasolacrimal obs ruc ion rom Probing and irriga ion should no be done
various causes wi h s asis o f uid in he lacri- in he set ing o an in ec ion.
mal sac and even ual in ec ion. Special Considerations
History I a pa ien complains o blood expressed
rom he lacrimal sys em, a lacrimal sac
May have acu e onse o pain and swelling
umor mus be considered and imaging
over he lacrimal sac. O hers may give he his-
done. In ec ions can give bloody discharge
ory o chronic earing wi h chronic mucous dis-
as well.
charge and a ender lump over he lacrimal sac.
T ere may be a prolonged his ory o a Dif erential Diagnosis
chronic conjunc ivi is. Lacrimal sac umor
Examination Laboratory Tests
enderness over he lacrimal sac is he Cul ure and sensi ivi y o any ma erial
mos common nding. expressed or drained rom he lacrimal
T e lacrimal sac may be enlarged wi h sac
signi can swelling, or i may be rela ively
small ( Fig. 9-1A). Similarly, he amoun o Imaging
periorbi al swelling varies wi h he severi y o C or MRI scanning may be needed i a
he in ec ion. lacrimal sac umor is suspec ed.

134
Dacryocystitis 135

Treatment Rare pa ien s will have an open lacrimal


rea men o he acu e in ec ion is he rs sys em a er he in ec ion is gone and will no
priori y. Sys emic an ibio ics and warm com- require a dacryocys orhinos omy.
presses are he rea men o choice. Pa ien s who have had a dacryocys i is and
I here is a ormed abscess o he sac, inci- have an obs ruc ed lacrimal sys em have an
sion and drainage is indica ed. increased risk o recurren dacryocys i is.

Ul ima ely, when he in ec ion has Prognosis


resolved, mos pa ien s will require a Excellen unless he pa ien is
dacryocys orhinos omy. immunocompromised
136 9 LACRIMAL INFECTIO NS

FIGURE 9-1. Dacryocystitis. A.T is 68-year-old man has a ormed lacrimal sac mass that is tender with a
surrounding mild cellulitis. B. A more severe dacryocystitis with surrounding cellulitis.
Canaliculitis 137

CANALICULITIS T ere may be a ollicular conjunc ivi is


and a chronic mucous discharge.

C analiculi is is a rare in ec ion involving


he proximal lacrimal sys em. T e in ec-
ion can be bac erial or ungal and is usually
Pressure over he canaliculus may express
pus or concre ions ( Fig. 9-2A and B).
T ere is more likely o be enderness over
indolen . Diagnosising canaliculi is can be he canaliculus wi h an in ec ious e iology
di cul because i o en presen s as a chronic and less likely when rela ed o re ained punc-
conjunc ivi is and no un il la e does he lacri- al plugs ( Fig. 9-2C).
mal in ec ion become apparen .
Dif erential Diagnosis
Epidemiology and Etiology
Chronic conjunc ivi is
Age: Usually older adul s
Migra ed punc al plug
E iology: Some abnormali y o he lacrimal
sys em leads o concre ion orma ion and a Laboratory Tests
chronic in ec ion. In ra-canalicular plugs have
become a more common cause o canaliculi is. Cul ure and sensi ivi y o ma erial in cana-
liculus is help ul in de ermining rea men .
History
Chronic mucous discharge, earing, Treatment
and conjunc ivi is unresponsive o opical Warm compresses and opical and sys-
an ibio ics emic an ibio ics are he ini ial rea men .
Ask abou any his ory o punc al plug Mos pa ien s will have concre ions or a
placemen and he ype o plug placed. plug in he canaliculus and he process will
recur un il hese are removed wi h incision
Examination and drainage o he canaliculus.
T e diagnosis can be di cul o con rm
unless i is suspec ed. Prognosis
An ery hema ous, pou ing, dila ed punc- Good once recognized. A second obs ruc-
um, which is o en ender o palpa ion and ion lower in he lacrimal sys em may resul in
very ender o probing, is o en presen . a recurrence.
138 9 LACRIMAL INFECTIO NS

FIGURE 9-2. Canaliculitis. A. A red, tender upper canaliculus with expression o pus with pressure over the
canaliculus. B.T e lacrimal stones ound on opening the canaliculus.
( continued)
Canaliculitis 139

FIGURE 9-2. (Continued) Canaliculitis. C. Intra-canalicular plug removed af er causing canaliculitis.


C H AP T ER

Lacrimal Sac umors

L acrimal sac umors are rare and he e iol-


ogy is widely varied rom benign o malig-
nan . Any dacryos enosis or dacryocys i is has
Classically, he mass may be above he
medial can hal endon bu early in he course
may presen like a dacryocys i is.
he po en ial o be a lacrimal sac umor. When
Examination
Findings vary rom being iden ical o dac-
ryocys i is o a palpable mass in he lacrimal
sac area.
T e umor may be ound during dacryo-
cys orhinos omy when here was no evidence
o a umor preopera ively.
I a umor is suspec ed, nasal examina ion
by an o olaryngologis may help def ne i s
ex en , along wi h C and/ or MRI scanning
( Fig. 10-1).
Dif erential Diagnosis
Dacryocys i is
Laboratory Tests
Biopsy o he lacrimal sac or any abnor-
mal appearing lacrimal sac. Dacryocys ogram
may be help ul.
Imaging
C or MRI scanning is needed i a lacri-
mal sac umor is suspec ed. I may no be able
o di eren ia e a umor rom an enlarged sac

140
Lacrimal Sac Tumors 141

secondary o in ec ion bu will show a large Care ul long- erm ollow-up is impor an
erosive mass. or any lacrimal sac umor.

Treatment Prognosis
Comple e excision o any benign or malig- Recurrence is no uncommon.
nan umor is impor an . Fi y percen o ransi ional and squamous
Frozen sec ion con rol is required o ry o cell carcinomas will recur, and 50% o hese
assure comple e excision. recurrences will be a al.
Benign papillomas may recur wi h malig-
nan rans orma ion. Lymphomas are sensi-
ive o irradia ion.

FIGURE10. 1 Lacrimal sac tumor. A.T e patient has ullness o the lef lacrimal sac area and bloody discharge.
B. An axial C scan showing a mass in the lacrimal sac ossa, which was a lymphoma on biopsy.
C H AP T ER

Orbi al In ec ions

ORBITAL CELLULITIS Examination


Ery hema, swelling, chemosis, res ric ed

O rbi al celluli is is a real oph halmic emer-


gency ha needs promp recogni ion and
rea men . T e in ec ion can progress rapidly
mo ili y, pain on eye movemen , and prop o-
sis charac erize orbi al celluli is.
T ese symp oms are progressive over 24
over a ew hours in severe cases wi h po en ial o 48 hours.
li e- hrea ening complica ions.
As he in ec ion advances, vision can be
af ec ed.
Epidemiology and Etiology Pa ien s may or may no have a ever and
Age: All ages leukocy osis.
Gender: Equal incidence in males and I is very impor an o make he dis inc-
emales ion be ween he signs o orbi al celluli is and
E iology: Sinusi is is he mos common presep al celluli is where here is jus swelling
cause bu o her causes include skin in ec- and redness o he eyelids ( Fig. 11-1).
ions or skin wounds, den al in ec ions, and Imaging
dacryocys i is.
C scanning is no required o make he
diagnosis o orbi al celluli is bu is needed o
History look or he source o in ec ion (e.g., sinusi is,
One o 3 days o progressive swelling orbi al abscess) and o rule ou o her pro-
around he eye cesses such as an orbi al umor.
T e process may be preceded by an upper A C scan will show sinusi is, which can
respira ory in ec ion. require drainage.
T e pa ien may have a his ory o sinus Orbi al oreign bodies or an orbi al abscess
in ec ions. can require addi ional surgery.

142
Orbital Cellulitis 143

Special Considerations Laboratory Tests


Aggressive and promp rea men o Comple e blood coun : Whi e coun may
orbi al celluli is is required o preven pos- be normal.
erior ex ension o he in ec ion, which can Blood cul ures are o ques ionable value.
resul in cavernous sinus hrombosis, which is
li e- hrea ening. Treatment
Immedia e broad-spec rum IV an ibio ics,
Dif erential Diagnosis
orbi al imaging, and care ul moni oring or
Presep al celluli is improvemen in he rs 24 o 48 hours.
Orbi al pseudo umor
Prognosis
Orbi al abscess
Good. Rare complica ions rom devel-
Phycomycosis
opmen o an abscess or cavernous sinus
Me as a ic orbi al umor hrombosis.

FIGURE 11-1. Preseptal cellulitis. A. Child with a scratch on the lateral lef upper eyelid that resulted in
preseptal cellulitis 2 days later. Ocular motility is normal. T e patient responded to antibiotics within 48 hours.
( continued)
144 11 O RBITAL INFECTIO NS

FIGURE 11-1. (Continued) Preseptal cellulitis. B. Early cellulitis related to a subconjunctival abscess that
required drainage and oral and topical antibiotics. C–F. Patient with 2 days o swelling o the lef eye with orbital
cellulitis. T e eye is swollen shut but, with lif ing, the eyelid ocular motility is limited and there is chemosis. T e
patient responded with improvement in 48 hours on IV antibiotics.
( continued)
Orbital Cellulitis 145

FIGURE 11-1. (Continued) Preseptal cellulitis. G. C scan shows proptosis and sinusitis and is consistent with
the clinical diagnosis o orbital cellulitis.
146 11 O RBITAL INFECTIO NS

ORBITAL ABSCESS Imaging


C scanning will demons ra e a sub-

A n orbi al abscess is a rare complica ion


o sinusi is and orbi al celluli is. Orbi al
celluli is ha does no improve on broad-
perios eal opaci y usually adjacen o an
in ec ed sinus. Rarely, he abscess may be
in raconal.
spec rum IV an ibio ics needs care ul imaging
o look or an orbi al abscess. Dif erential Diagnosis
Orbi al celluli is
Epidemiology and Etiology Phycomycosis
Age: Any Cavernous sinus hrombosis
Gender: Equal Orbi al pseudo umor
E iology: Sinus disease is he mos com-
mon source o a subperios eal abscess. Rarely, Laboratory Tests
an orbi al oreign body can be he cause and Comple e blood coun ; cul uring o he
mus be suspec ed i he abscess is in raorbi al abscess con en s.
(in raconal).
Treatment
History Mos pa ien s will require immedia e sur-
gical drainage o he abscess and rea men
Orbi al celluli is wi h no sign o improve-
wi h broad-spec rum IV an ibio ics.
men on appropria e an ibio ics
Some abscesses have been rea ed wi h
IV an ibio ics alone and close observa ion in
Examination
children younger han age 9 years.
Signs are hose o orbi al celluli is ha
do no improve on appropria e IV Prognosis
an ibio ics. Promp and aggressive rea men usually
T e globe may be displaced away rom he allows success ul rea men .
abscess. An orbi al abscess does have he po en ial
T e abscess is diagnosed on C scanning o resul in visual loss, mo ili y problems, or
( Fig. 11-2). even severe CNS morbidi y.
Orbital Abscess 147

FIGURE 11-2. Orbital abscess. A. A patient with a 2- to 3-day history o swelling o the lef eye. B.T ere is 5
mm o proptosis and limited motility.
( continued)
148 11 O RBITAL INFECTIO NS

FIGURE 11-2. (Continued) Orbital abscess. C. C scan shows pan sinusitis with a medial orbital abscess that
required surgical drainage.
( continued)
Orbital Abscess 149

FIGURE 11-2. (Continued) Orbital abscess. D. A patient with weeks o a red irritated eye. E. C scan shows an
abscess around an old orbital oor implant.
150 11 O RBITAL INFECTIO NS

PHYCOMYCOSIS Imaging
( MUCORMYCOSIS) C scanning will show evidence o sinus
disease, which a imes can be very mild

P hycomycosis is a rare, o en a al ungal


in ec ion ha occurs in very sick, immu-
nocompromised pa ien s, mos commonly
( Fig. 11-3B).
MRI wi h gadolinium should be done
o look or evidence o ex ension in o he
in poorly con rolled diabe ics. T is in ec ion cavernous sinus.
s ar s in he nasopharynx or sinuses and sec-
ondarily invades he orbi . Aggressive rea - Pathology
men has improved he survival in his o en Diagnosis is made on biopsy.
a al condi ion.
Nonsep a e, large branching hyphae ha
s ain on hema oxylin and eosin s aining,
Epidemiology and Etiology
unlike mos ungi, are ound.
Age: Adul s
Gender: Equal male and emale Dif erential Diagnosis
occurrence Orbi al celluli is
E iology: Fungi invade he orbi rom he Orbi al pseudo umor
sinuses or nose. T e ungi invade blood vessel Cavernous sinus hrombosis
walls and produce hrombosis, ischemia, and
allow spread o he ungi. Laboratory Tests
History Evalua ion or diabe ic con rol, leukocy e
coun
Pa ien s o en have a his ory o severe
sinus pain and progressive orbi al swelling. Pathophysiology
T e pa ien s who develop his in ec ion Oppor unis ic ungal in ec ion ha grows
are immunocompromised in some way. in an immunocompromised hos .
T e mos common underlying condi ion
is severe diabe es wi h poor con rol bu o h- Treatment
ers include malignancy, chemo herapy, and Con rol sys emic disease, IV ampho ericin
chronic s eroid use. B, and surgical debridemen o necro ic is-
sue, which can involve orbi al exen era ion.
Examination
Prop osis is he mos common nding Prognosis
wi h an orbi al apex syndrome. Poor
Black eschar in he nasal cavi y is a Depending on s a e o he pa ien ’s
la e nding and is no a reliable diagnos ic sys emic disease, his condi ion can o en
sign. be a al.
Pa ien s are very sick sys emically Even i he disease is con rolled, vision is
( Fig. 11-3A). o en los in he af ec ed eye.
Phycomycosis (Mucormycosis) 151

FIGURE 11-3. Phycomycosis. A. A patient with poorly controlled diabetes with a 1-week history o sinusitis.
T e patient has a rozen globe and a central retinal artery occlusion. T ere is a dusky erythema o the cheek.
B. C scan shows di use sinus disease with orbital involvement. Biopsy o the sinus revealed ungus consistent
with phycomycosis.
152 11 O RBITAL INFECTIO NS

ASPERGILLOSIS MRI can be help ul in de ning ex en o


he disease in he orbi and looking or pos-

A spergillosis occurs in wo orms. One


orm, very similar o phycomycosis,
occurs in immunocompromised pa ien s and
sible CNS ex ension.
T e allergic orm shows he sinus lled
wi h mot led area o increased at enua ion on
has a poor prognosis. T e second orm occurs nonenhanced C scan.
in heal hy pa ien s wi h chronic sinus disease T ere may be areas o bone remodeling
and allergies. T is orm has a good prognosis. and even erosion.
Epidemiology and Etiology MRI imaging shows a signal void on 2
Age: Adul s images.
Gender: Equal male and emale incidence Pathology
E iology: An oppor unis ic in ec ion ha Diagnosis is made by biopsy.
grows in he sinuses and secondarily invades he Sep a e branching hyphae o uni orm
orbi . I can occur in wo orms. One orm ac s wid h are seen on Gomori’s me henamine
like phycomycosis and hus occurs in immuno- silver s aining.
compromised hos s. T e second, “allergic” orm
occurs in immune compe en hos s wi h chronic Dif erential Diagnosis
sinus disease and allergies. T e sinus is lled wi h Sinusi is wi h mucocele
mucin and ungus and may have bone erosion. Phycomycosis
History Me as a ic orbi al umor
Aspergillosis occurring in immunocompro- Laboratory Tests
mised hos s presen s similar o phycomycosis.
Immunocompromised pa ien s will have
T e allergic orm will presen as chronic associa ed blood nding such as ke oacidosis,
sinus problems bu will invade he orbi wi h leukopenia, and so or h, depending on he
ime in 17% o pa ien s, resul ing in orbi al e iology o he immunode ciency.
signs depending on he sinus involved.
Pa ien s wi h allergic orm may have a
Examination peripheral blood eosinophilia, eleva ed o al
Findings on examina ion are dependen immunoglobulin E, and posi ive allergy skin
on he orm o in ec ion. es ing or ungus.
T e presen a ion o aspergillosis in he immu- Treatment
nocompromised hos is he same as ha o phy-
Immunocompromised pa ien s wi h asper-
comycosis and is only dif eren ia ed on biopsy.
gillosis are rea ed he same as phycomycosis
T e allergic orm will only presen wi h (see previous discussion).
orbi al ndings in he minori y o cases.
Cleaning ou he mucin and ungus rom
T e signs vary rom displacemen o he he af ec ed sinus and orbi de ni ively rea s
globe o an orbi al apex syndrome, depend- he allergic orm.
ing on he loca ion o he in ec ion and exac
direc ion o invasion ( Fig. 11-4). Prognosis
Poor in he immunocompromised orm
Imaging
Good in he allergic orm wi h appropria e
C scan will show sinus disease wi h sec-
rea men
ondary orbi al invasion.
Aspergillosis 153

FIGURE 11-4. Aspergillosis. A. A 45-year-old patient with loss o vision in the lef eye and very mild proptosis
on the lef . T ere are no other orbital signs. B. C scan shows a large mass o the sphenoid sinus with erosion
into the cavernous sinus. C. On MRI, the central area o signal void is classic or aspergillosis. T is mass was
simply cleaned out via transnasal sinus surgery and the vision returned to normal.
C H AP ER

Orbi al Inf amma ion

THYROID-RELATED hyroid imbalance, bu up o 30% may be


OPHTH ALMOPATHY eu hyroid a he onse o symp oms.

Examination
hyroid-rela ed oph halmopa hy ( RO)
is he mos common cause o prop osis in T e earlies signs o RO are very nonspe-
adul s. T e disease can range rom mild eyelid ci c and i can be di cul o make he diag-
re rac ion o severe prop osis wi h op ic nerve nosis early in he disease.
compression and corneal exposure. Early in he Eyelid re rac ion and eyelid lag are
disease course, RO can be di cul o diag- also early signs ha will help con rm he
nose bu la er he ocular signs become classic. diagnosis.
As he disease progresses, chemosis, pro-
Epidemiology and Etiology p osis, and mo ili y res ric ion wi h diplopia
Age: Rare in children, mainly adul s will become apparen .
Gender: Women a ec ed ve o eigh La e signs are decreased vision rom
imes more o en han men op ic nerve compression and severe corneal
E iology: Poorly unders ood au oimmune exposure ( Fig. 12-1A–F).
inf amma ory process ha a ec s he eyelid
Imaging
and orbi al issues
C scan will show enlargemen o he
History rec us muscles wi h endon sparing.
Ini ial onse o nonspeci c ocular irri a ion T e in erior rec us is he mos commonly
ollowed by eyelid re rac ion, lid lag, eyelid involved muscle ollowed by medial rec us
swelling, and bulging o he eyes. and superior rec us.
Pa ien s will no e symp oms o be worse in T e la eral rec us is rarely involved. C
he morning and improve over he day. Many scan is no needed o make he diagnosis o
pa ien s will have he his ory o a sys emic RO, as his is a clinical diagnosis.

154
T yroid-Related Ophthalmopathy 155

C scanning is help ul o con rm long- erm use, hey are usually limi ed o use
unusual cases, evalua e op ic nerve compres- as a emporary, shor - erm rea men .
sion, and be ore surgery or irradia ion ( Fig. Orbi al irradia ion in some pa ien s is
12-1G, H). e ec ive a s opping he progression o he
disease bu no e ec ive a reversing any o
Special Considerations he changes ha have occurred.
T e course and severi y o disease is Any pa ien wi h signi can , ac ive disease
widely variable. is a po en ial candida e or irradia ion (excep
Pa ien s may have a ew mon hs o mild pa ien s wi h diabe ic re inopa hy).
inf amma ion wi hou any sequelae, whereas T e use o orbi al irradia ion is
o hers can have severe inf amma ion ha can con roversial.
lead o severe prop osis, double vision, and
visual loss over a ew mon hs or years. S eroid injec ions in o he orbi and a
shor course o high-dose IV s eroids are
Pa ien s who smoke have a longer and o her rea men s.
more severe course.
Immune modula ors such as ri uximab are
Dif erential Diagnosis also being explored o s op his au oimmune
process.
Orbi al pseudo umor
A er he inf amma ory phase is over,
Orbi al celluli is
surgical correc ion o residual prop osis,
Orbi al lymphoma diplopia, and eyelid de ormi ies can be
considered.
Laboratory Tests
T is is done via a combina ion o orbi al
T yroid-s imula ing hormone decompression and eye muscle and eyelid
surgery.
Pathophysiology
Pa ien s presen ing wi h severe inf am-
Chronic inf amma ory process leads o ma ion and an op ic neuropa hy or corneal
deposi ion o glycosaminoglycans in he decompensa ion can require an urgen orbi al
muscles and orbi al a wi h even ual scarring decompression.
and dys unc ion o hese issues.
Prognosis
Treatment
Good, bu some pa ien s may require
Limi ing he inf amma ion will limi he mul iple surgical procedures over years as
scarring and severi y o he disease. par o he rea men . Pa ien s wi h signi -
Sys emic s eroids will decrease inf am- can disease o en have a prolonged course o
ma ion, bu because o he side e ec s rom rea men .
156 12 O RBI AL INFLAMMA IO N

FIGURE 12-1. T yroid-related ophthalmopathy. A. A patient with very early thyroid-related ophthalmopathy
with slight lid retraction on the lef . B. In down gaze, there is eyelid lag. C. A 20-year-old patient with severe
proptosis, eyelid retraction, and corneal exposure.
( continued)
T yroid-Related Ophthalmopathy 157

FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. D–F. A 45-year-old patient with progressive


swelling o the eyes with double vision and recent decreased vision. T ere is proptosis, chemosis, and limitation
o motility. Vision was 20/ 80 rom optic nerve compression.
( continued)
158 12 O RBI AL INFLAMMA IO N

FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. G and H. C scans show enlargement o all rectus
muscles with crowding at the orbital apex. T e patient required an orbital decompression and her vision returned
to normal.
( continued)
T yroid-Related Ophthalmopathy 159

FIGURE12- 1. (Continued) T yroid-related ophthalmopathy. I. A patient with severe thyroid-related


ophthalmopathy. J.A f er 3 years and multiple surgeries, there is signi cant improvement.
160 12 O RBI AL INFLAMMA IO N

IDIOPATHIC ORBITAL Special Considerations


INFLAMMATION (ORBITAL Rarely, here may be very ew inf amma-
PSEUDOTUMOR) ory signs and a more chronic bro ic process
ha is ermed sclerosing inf amma ory orbi al
Epidemiology and Etiology pseudo umor.
Age: Children and adul s T is condi ion is no very responsive o
rea men as rea men is geared oward elimi-
Gender: Equal incidence in males and
na ing inf amma ion and here is very lit le
emales
inf amma ion in his process.
E iology: T is inf amma ory process
Sys emic condi ions, such as sarcoidosis,
is by de ni ion unrela ed o any sys-
may cause a very similar pic ure.
emic abnormali y and he cause remains
unknown. Dif erential Diagnosis
Orbi al celluli is
History
T yroid-rela ed oph halmopa hy
Acu e onse o orbi al pain o en associ-
a ed wi h prop osis, ery hema, swelling, and Lymphoma
res ric ed eye movemen s. Sarcoidosis
T e symp oms depend on he exac loca- Wegener’s Granuloma osis
ion o he process bu pain is common o all Rup ured dermoid cys
presen a ions.
Me as a ic disease
Adul s more commonly have unila eral
disease bu in children his can be a bila eral Laboratory Tests
process. Pa ien s may have a leukocy osis, periph-
eral blood eosinophilia, eleva ed ESR, and a
Examination posi ive ANA. None o hese are diagnos ic.
T e acu e inf amma ory process can occur
Pathophysiology
an eriorly and presen wi h acu e ery hema
and swelling o he lids and globe. A pleomorphic cellular inf amma ory
response occurs and i no rea ed or no
I may presen as a myosi is wi h
responsive o rea men here will be a resul-
res ric ed mo ili y and pain wi h eye move-
an bro ic response ha will progress wi h
men , as a scleri is, a dacryoadeni is, or in
ime and resul in chronic scarring.
he orbi al apex wi h ew ex ernal signs bu
signi can pain, dysmo ili y, and decreased Treatment
vision. Sys emic s eroids are he mains ay o
T e presen a ion is variable depending rea men .
on he issues a ec ed. Pa ien s wi h orbi al T ere should be an improvemen in
pseudo umor can have a ever and a leukocy- symp oms in 24 o 48 hours.
osis ( Fig. 12-2).
T e longer he process has been presen ,
Imaging he longer i can ake or a clinical response.
C scanning will show hickening o he A er here is a good clinical response, he
a ec ed issues such as enlarged muscles, s eroids are apered over 4 o 6 weeks.
hickened sclera, enlarged lacrimal gland, or Pa ien s wi hou response o s eroids or
an in l ra e in he orbi al a . wi h mul iple recurrences o he inf amma ion
Idiopathic Orbital Inf ammation (Orbital Pseudotumor) 161

require an orbi al biopsy o con rm he Prognosis


diagnosis. Excellen prognosis in mos acu e cases.
I con rmed, hey are hen candida es or T ere may be recurrences.
orbi al irradia ion.
Cases ha are chronic wi h less inf amma-
Immune suppressive agen s may also be ory response are less responsive o rea men
used in some pa ien s wi h recurrences. and can be progressive.

FIGURE 12-2. Orbital pseudotumor. A. A 33-year-old man with a 5-day history o swelling, erythema, and
pain that is worse with eye movement. T e eye is red with orbital swelling and tenderness to palpation. Eye
movements are limited by pain. B.D i use in ltration o the orbit and slight enlargement o the medial rectus.
T e clinical presentation along with the C scan are consistent with orbital pseudotumor. T e patient responded
within 24 hours to oral prednisone.
( continued)
162 12 O RBI AL INFLAMMA IO N

FIGURE 12-2. (Continued) Orbital pseudotumor. C.T is is a scleritis: Scleritis with some anterior orbital
swelling. D.D i use scleral thickening on lef side.
( continued)
Idiopathic Orbital Inf ammation (Orbital Pseudotumor) 163

FIGURE 12-2. (Continued) Orbital pseudotumor. E. C shows a di use enlargement o the lateral rectus muscle
consistent with a myositis. T e patient had limited adduction and abduction as well as pain with eye movement.
F. C scan showing in ammation at the orbital apex. On examination, the eye may be white and quiet with
minimal proptosis. T ere is of en decreased vision and motility dys unction consistent with an orbital apex
syndrome.
164 12 O RBI AL INFLAMMA IO N

SARCOIDOSIS Ches x-ray or a ches C is needed o


evalua e he lungs or possible pulmonary

S arcoidosis can occur in he orbi in mul iple


orms and wi h varying amoun s o inf am-
ma ion.Mos commonly,i presen s wi h lacrimal
sarcoidosis.

Special Considerations
gland enlargemen wi h very mild inf amma ory Mos pa ien s wi h sarcoidosis will have
signs. Sarcoidosis may have a much more acu e sys emic disease wi h pulmonary ndings.
swelling and can a ec he sclera, ex raocular Some pa ien s will have he disease
muscles, or o her orbi al issues. isola ed o he orbi wi hou sys emic
indings.
Epidemiology and Etiology
Age: Any age bu mos common in Dif erential Diagnosis
adul hood Idiopa hic orbi al pseudo umor
Gender: Equal Dacryoadeni i is
E iology: Mul isys em inf amma ory dis- Laboratory Tests
ease ha occurs primarily in individuals o
A rican and Scandinavian descen . Angio ensin-conver ing enzyme may be help-
ul in es ablishing he diagnosis.
History
Mos commonly presen s wi h lacrimal Treatment
gland enlargemen wi h varying amoun o A biopsy o he a ec ed issue is usu-
inf amma ory signs ally required o con rm he diagnosis o
sarcoidosis.
Examination When presen , a conjunc ival nodule is
Bila eral lacrimal gland enlargemen is he simple o biopsy. O herwise, he a ec ed is-
mos common presen a ion. sue is biopsied.
Ex raocular muscles, he op ic nerve, and A er he diagnosis is es ablished, care-
eyelid skin are less commonly a ec ed. ul sys emic evalua ion is needed o look or
Inf amma ion in adjacen sinuses may sec- sarcoid.
ondarily a ec he orbi . rea men is mos commonly sys emic
T e en ire eye mus be evalua ed or signs prednisone al hough o her immunosuppres-
o sarcoidosis causing uvei is (an erior or sive agen s have been used.
pos erior), iris nodules, or re inal vascular rea men is usually aimed a disease con-
changes. rol whe her i is or con rol o orbi al inf am-
Conjunc ival granulomas as well as sarcoid ma ion or o con rol pulmonary disease.
skin lesions can help con rm he diagnosis
( Fig. 12-3). Prognosis
Mos pa ien s do well, bu rare pa ien s can
Imaging have signi can sys emic mani es a ions.
C scanning will show enlargemen o T e orbi al disease can be chronic and
he lacrimal gland, muscle, or o her a ec ed recurren .
s ruc ure.
Sarcoidosis 165

FIGURE 12-3. Orbital sarcoidosis. A and B. A patient with proptosis and double vision with some mild aching
o the right eye. Motility shows poor adduction o the right eye.
( continued)
166 12 O RBI AL INFLAMMA IO N

FIGURE 12-3. (Continued) Orbital sarcoidosis. C. C scan shows an enlarged medial rectus muscle. T is
myositis responded to oral prednisone but recurred. Biopsy showed sarcoidosis. D. Bilateral lacrimal gland
enlargement on the external photograph.
( continued)
Sarcoidosis 167

FIGURE 12-3. (Continued) Orbital sarcoidosis. E and F. Axial and coronal C scans show enlarged lacrimal
glands. Biopsy showed sarcoidosis. G.I n ltration o the eyelid and anterior lacrimal tissue by sarcoidosis. Under
the eyelid, the in ltration is yellow-brown with prominent blood vessels.
168 12 O RBI AL INFLAMMA IO N

WEGENER’S Examination
GRANULOMATOSIS Findings include scleri is, which may be
an erior or pos erior and is o en necro izing.

W egener’s granuloma osis can involve


he eye and orbi as a secondary ex en-
sion rom he sinuses or i can presen involv-
Prop osis wi h or wi hou orbi al inf am-
ma ion may be presen ( Fig 12-4A).

ing he eye i sel wi h scleri is, kera i is, uvei is, Imaging
and so or h. T e sys emic disease can be li e- C scans show bone erosion rom sinus
hrea ening and he ocular involvemen can ex ension o he disease ( Fig 12-4B and C).
cause blindness and loss o he eye.
Dif erential Diagnosis
Epidemiology and Etiology Malignan umor o he sinus
Age: Mainly adul s Laboratory Tests
Gender: Equal occurrence in males and An ineu rophil cy oplasmic an ibodies
emales (speci cally c-ANCA) are o en presen wi h
E iology: A sys emic necro izing granulo- Wegener’s disease.
ma ous vasculi is ha classically a ec s he
upper and lower respira ory rac and can Pathology
a ec he small vessels o any major organ Vasculi is, granuloma ous inf amma ion,
sys em. and issue necrosis are ound on pa hologic
evalua ion.
History
Diagnosis may or may no already be made Treatment
when he pa ien presen s wi h eye ndings. Immunosuppressive medica ion, speci -
Mos commonly, here is bony erosion via cally cor icos eroids and cyclophosphamide,
ex ension o he disease in o he orbi rom is he rea men o choice.
he sinus cavi y.
Prognosis
Pa ien s can also have a necro izing scleri-
Variable. T e disease can be progressive
is, which can be severe.
and a al.

FIGURE 12-4. Wegener’s granulomatosis. A. A patient with orbital pseudotumor-like picture.


( continued)
Wegener’s Granulomatosis 169

FIGURE 12-4. (Continued) Wegener’s granulomatosis. B and C. C scans show in ltration along the in erior–
medial orbit and into the sinus. Poor response to prednisone and a positive ANCA led to a biopsy, which was
consistent with Wegener’s granulomatosis.
C H AP T ER

Congenital Orbital Anomalies

MICROPHTH ALMOS T ere may be an accompanying cyst,


which may be quite large.

M icrophthalmos is a de ect in the eye


development. T e eye is small and there
are usually structural de ects. T e microphthal-
T is condition is usually unilateral, rarely
bilateral.

mos can be mild or the eye can be so small that Dif erential Diagnosis
it cannot be seen at all. Anophthalmos

Epidemiology and Etiology Treatment


Age: Congenital reatment is aimed at stimulating the orbit
to grow and mature normally.
Gender: Equal occurrence in males and
emales I the eye is only slightly small or i there
is a large cyst, orbital growth may continue as
Etiology: Developmental de ect with
normal.
ailure o the choroidal ssure to close as an
embryo. T is results in a small eye with struc- However, i the eye is very small, expand-
tural abnormalities ( Fig. 13-1) ing con ormers should be used to try to
stimulate orbital growth.
Examination Dermis at graf s are used sometimes.
T e eye may be small to virtually
nonexistent. Prognosis
T ere are structural abnormalities within T ese patients of en have some orbital
the eye and the eye usually has poor or no asymmetry even with aggressive treatment.
vision. T e cosmetic result is generally acceptable.

170
Microphthalmos 171

FIGURE 13-1. Microphthalmos. A. A child wi h microph halmos wi h cys .


( continued)
172 13 CO NGENITAL O RBITAL ANO MALIES

FIGURE 13-1. (Continued) Microphthalmos. B. CT scan shows a small eye and at ached cys .
( continued)
Microphthalmos 173

FIGURE 13-1. (Continued) Microphthalmos. C. Pa hologic specimen shows he cys ic ou pouching coming
from he abnormally developed eye.
C H AP T ER

Orbital Neoplasms

CONGENITAL ORBITAL adulthood when they have become large, start


TUMORS to leak, or rupture rom trauma.

Examination
DERMOID CYSTS

D
T e classic location or a superf cial der-
ermoid cysts are relatively common,
moid cyst is at the lateral brow over the ron-
benign, orbital tumors in children.
tozygomatic suture.
Classically, they are present at birth, are
located superior temporally at the orbital rim, Less commonly, they can be superior
and enlarge with time. medial or even in the lower lid.
T ey are smooth, painless masses that
Epidemiology and Etiology slowly enlarge.
Age: Congenital and enlarge with age T ey can be reely mobile or f xed to the
bony suture.
Gender: Equal occurrence in males and
emales Deeper dermoids can be in the superior
and/ or lateral orbit.
Etiology: Epidermal elements are le dur-
ing embryonic development in deeper tissues. “Dumbbell” dermoids occur in the tem-
poral ossa and have a component in the orbit
T ese epidermal elements then orm a cyst
and a part in the temporal ossa.
that enlarges with time.
Deeper dermoids present with proptosis
or with symptoms o orbital in ammation
History
as the dermoid cyst either leaks or ruptures
More superf cial dermoids are o en noted ( Fig. 14-1C).
in the f rst 1 to 2 years o li e as they grow and
become more noticeable (Fig. 14-1A, B). Imaging
T e dermoids that are deeper, such as in C scan: nonenhancing cystic mass
the orbit, may not become symptomatic until (Fig. 14-1D).

174
Congenital Orbital Tumors 175

MRI: hypoin ense on 1; hyperin ense on Treatment


2(Fig. 14-1E). Comple e surgical excision wi h an in ac
capsule is he surgery o choice.
Dif erential Diagnosis
T is procedure should be done when
When loca ed super icially and empo-
he po en ial or cys rup ure becomes a
rally, here are very ew lesions his can be
risk.
con used wi h. Imaging usually helps make
he diagnosis i loca ed deep in he orbi . T is mos o en occurs when he child
begins o walk and be more ac ive.
Pathology
T e cys is lined by kera inizing epidermis Prognosis
wi h dermal appendages such as hair ollicles Excellen or superf cial dermoids.
and sebaceous glands. Good or deep dermoids as long as he
T e cys is f lled wi h kera in and oil. en ire cys is removed

FIGURE 14-1. Dermoid cyst. A. Sof , mobile mass along he superior emporal rim in a 1-year-old pa ien . T is
has been presen since bir h.
( con inued)
176 14 O RBITAL NEO PLASMS

FIGURE 14-1. (Con inued) Dermoid cyst. B. Excision o he dermoid hrough a lid crease incision. Ca nd
D. Prop osis and globe displacemen caused by a deep orbi al dermoid, which was no ed a age 5 years. T e
ossa orma ion caused by hese lesions is seen on he C scan. T e deep orbi al loca ion means hey are of en
no no iced un il he child is older. T is was comple ely excised and he pa ien did well wi hou any ur her
problems.
( con inued)
Congenital Orbital Tumors 177

FIGURE 14-1. ( Con inued) Dermoid cyst. D. C scan show cys ic lesion wi h bony ossa orma ion. E. MRI o a
dermoid cys . On a 2-weigh ed image, he cys is hyperin ense o a and muscle.
178 14 O RBITAL NEO PLASMS

LIPODERMOIDS Dif erential Diagnosis

L ipodermoids are congeni al solid umors


loca ed emporally below he conjunc iva.
T ese are some imes no no ed un il la er in li e.
Fa prolapse
Lymphoma
Prolapsed lacrimal gland
T ey should be le alone in almos all cases.

Epidemiology and Etiology Pathology


Age: Congeni al Kera inizing squamous epi helium wi h
adnexal s ruc ures
Gender: Equal occurrence in males and
emales T e underlying dermis usually con ains a
and connec ive issue.
E iology: Developmen al anomaly

History Treatment
Presen a bir h and generally does no No rea men
change wi h ime At emp ed excision can damage he
adjacen lacrimal duc s and rec us or leva or
Examination
muscles.
Yellowish, pink lesion over he la eral sur ace
In rare cases when he lipodermoid is
o he globe deep o he conjunc iva ( Fig. 14-2)
very large, he an erior por ion can be
T ey vary in size and o en have hairs on debulked leaving he conjunc iva
he sur ace. unresec ed.
Imaging
Prognosis
I large, C scan will show a mass wi h a
densi y. Excellen i le alone
Congenital Orbital Tumors 179

FIGURE 14-2. Lipodermoid. A. Classic loca ion or a lipodermoid, which has been presen since bir h. B. Close
inspec ion of en shows hairs on he lesion. Despi e he cosme ic appearance, hese are bes lef alone.
180 14 O RBITAL NEO PLASMS

VASCULAR ORBITAL Di eren ia ion be ween rhabdomyosar-


TUMORS coma and deep capillary hemangioma can
only be made wi h biopsy.
CAPILLARYHEMANGIOMAS
Imaging

C apillary hemangiomas are benign umors


o he orbi ha appear in he f rs ew
weeks o li e and enlarge over he f rs 6 o 12
C scan reveals a mass ha can be well or
poorly margina ed wi h enhancemen wi h
con ras ( Fig. 14-3D, E).
mon hs. T ey hen end o shrink over ime
bu he ini ial presen a ion can be drama ic. MRI is hypoin ense on 1 and hyperin-
ense on 2.
Epidemiology and Etiology T e lesion enhances wi h gadolinium.
Age: No ed in he f rs year o li e Dif erential Diagnosis
Gender: Equal occurrence in males and Rhabdomyosarcoma
emales
E iology: Abnormal grow h o blood ves- Pathology
sels wi h varying degrees o endo helial cell Proli era ion o endo helial cells organized
proli era ion in o a ne work o basemen membrane–lined
vascular channels.
History
Lesions are o en no ed in f rs ew weeks Treatment
o li e and hey grow, some imes rapidly, over T ese lesions will regress so hemangiomas are
weeks o mon hs. observed or regression unless hey cause visual
T ey can presen deeper in he orbi wi h obs ruc ion or as igma ism leading o amblyopia.
prop osis or more superf cially as an expand- In his case, rea men is required.
ing mass. Orbi al lesions causing severe prop osis
T e hemangioma will hen involu e over may also require rea men .
mon hs o years. Seven y-f ve percen o Orbi al biopsy is required i he
lesions will resolve over 4 years. lesion canno be di eren ia ed rom a
rhabdomyosarcoma.
Examination
rea men op ions include in ralesional
T e lesion appearance is dependen on he s eroid injec ion, sys emic s eroids, or, in
loca ion (Fig. 14-3A-C, F, G). selec cases, surgical excision.
T e more common superf cial lesions Recen s udies sugges sys emic proprano-
produce an eleva ed, dimpled, s rawberry- lol may become he rea men o choice.
colored lesion.
Care ul moni oring o hese babies on pro-
Deeper lesions may give a bluish pranolol mus be done by pedia ric cardiology.
discolora ion.
Deep orbi al lesions may only give symp- Prognosis
oms o an expanding orbi al mass. Good
Vascular Orbital Tumors 181

FIGURE 14-3. Capillary hemangioma. A. Subcu aneous capillary hemangioma o he righ eyebrow ha
increased in size over 6 mon hs. T e lesion becomes more prominen and red wi h crying. T is lesion resolved
over 3 years. B. A small hemangioma on he child’s arm.
( con inued)
182 14 O RBITAL NEO PLASMS

FIGURE 14-3. (Con inued) Capillary hemangioma. C.S uper cial orbi al hemangioma ha had increased in size
and was causing amblyopia rom 7 diop ers o induced as igma ism. D and E. C scans show his an erior orbi al
mass, which is well circumscribed and enhances wi h con ras . T is was excised because o he as igma ism and
amblyopia.
( con inued)
Vascular Orbital Tumors 183

F G

FIGURE 14-3. (Con inued) Capillary hemangioma. F. Large cu aneous capillary hemangioma wi h visual
obs ruc ion. G.T is lesion responded well o a series o in ralesional s eroid injec ions.
184 14 O RBITAL NEO PLASMS

CAVERNOUS HEMANGIOMAS Imaging

C avernous hemangiomas can presen as


asymp oma ic, very insidious onse pro-
p osis. More commonly, hese lesions pres-
C scan shows an encapsula ed, homoge-
neous, round mass wi h variable enhancemen .
MRI: isoin ense on 1 and hyperin ense
en wi hou any symp oms and are ound on on 2(Fig. 14-4D, E).
imaging done or unrela ed reasons. T ey are
Marked enhancemen wi h gadolinium
slowly growing masses ha are generally easy
o remove depending on heir loca ion. Special Considerations
Rarely, lesions may grow rapidly during
Epidemiology and Etiology
pregnancy.
Age: Adul s
Gender: Mos commonly middle-aged Dif erential Diagnosis
women Hemangiopericy oma
E iology: Unknown Schwannoma
Fibrous his iocy oma
History
Very slow grow h usually means he Pathology
pa ien is unsure o he onse or dura ion o Encapsula ed umor consis ing o large
he lesion. endo helial lined channels wi h abundan ,
Mos commonly, he presen a ion is pro- loosely dis ribu ed smoo h muscle in he vas-
p osis bu rarely here can be symp oms o cular walls and smoo h muscle.
visual loss.
Treatment
Examination Surgical excision is he rea men o choice.
Axial prop osis is he common T ese lesions are easily removed once
presen a ion. exposed.
I he lesion is a he apex or is very large, T ey do no regress and slowly enlarge so
i can cause op ic nerve compromise ( Fig. observa ion only delays surgery.
14-4A-C) or s rabismus.
Lesions can rarely cause orbi al pain or he Prognosis
appearance o a choroidal mass. Excellen
Vascular Orbital Tumors 185

FIGURE 14-4. Cavernous hemangioma. A. A pa ien wi h prop osis o he righ eye o unknown dura ion and
no o her visual or orbi al complain s. B. C scan shows a well-circumscribed in raconal orbi al mass.
( con inued)
186 14 O RBITAL NEO PLASMS

FIGURE 14-4. (Con inued) Cavernous hemangioma. C.T e mass was excised and was a cavernous hemangioma.
D. MRI o a cavernous hemangioma. T e 1-weigh ed image shows he lesion isoin ense o muscle and
hypoin ense o a .
( con inued)
Vascular Orbital Tumors 187

FIGURE 14-4. ( Continued) Cavernous hemangioma. E. On the T 2-weighted image, the lesion is hyperintense to
fat and muscle.
188 14 O RBITAL NEO PLASMS

LYMPHANGIOMAS Care ul evalua ion or evidence o a

L ymphangiomas are rare vascular ham-


ar omas ha can behave in many di er-
en ways depending on loca ion and grow h
lymphangioma superf cially should be done
in hese cases. Imaging will aid in he diagno-
sis i he lesion is en irely orbi al.
pat erns. T is condi ion can vary rom mild
Imaging
ra her asymp oma ic lesions, o progressively
growing, inf l ra ive lesions, o acu e prop o- C scan: Poorly circumscribed, he eroge-
sis and visual loss rom bleeding in o hese neous mass
lesions. MRI: Hyperin ense on 1; very hyperin-
ense on 2, wi h possible area o uid and
Epidemiology and Etiology blood ( Fig. 14-5D, E)
Age: Usually no ed in he f rs decade o li e
Special Considerations
Gender: More common in emales
Surgery per ormed on a lymphangioma
E iology: Congeni al lesion increases he chances o spon aneous bleeds
wi hin he lesion.
History
Surgery should only be done i absolu ely
T ese lesions are o en no ed associ- necessary.
a ed wi h a spon aneous bleed o he lesion
al hough hey likely were presen or years Dif erential Diagnosis
prior. Diagnosis can usually be made wi h
T ey can grow slowly and hen suddenly MRI.
hemorrhage.
Lymphangiomas can mani es as pain, Pathology
subconjunc ival hemorrhage, or as prop osis. Nonencapsula ed mass wi h large
Less commonly, he cys s o hese lesions are serum- illed spaces lined by la endo helial
no ed subconjunc ivally. cells
T ese lesions enlarge wi h upper respira- T e in ers i ium has scat ered lymphoid
ory in ec ions. ollicles.

Examination Treatment
T e f ndings on examina ion are depen- Observa ion unless he spon aneous
den on he loca ion o he lesion. bleeding causes visual loss, corneal exposure,
or severe cosme ic disf guremen .
T e mos common presen a ion is
associa ed wi h sudden bleeding in o he Generally, wi h ime he blood will
lymphangioma. resorb.
I he bleed is superf cial, hen a subcon- When an orbi al hemorrhage causes visual
junc ival bleed is seen and he cys s o he loss, drainage o he hemorrhage should be
lymphangioma are o en ound ( Fig 14-5A, per ormed.
B). I he hemorrhage is in he orbi , he f nd- Debulking o he lesion or orbi al decom-
ings may only be prop osis ( Fig 14-5C). pression are o her rea men op ions.
Vascular Orbital Tumors 189

Lymphangiomas are inf l ra ive so excision Prognosis


is very di cul and here is usually signif can Variable depending on he grow h o he
bleeding associa ed wi h excision, which is lymphangioma
only per ormed as a las resor .
Progressive lesions have a high incidence
o visual disabili y and poor cosme ic resul .
190 14 O RBITAL NEO PLASMS

FIGURE 14-5. Lymphangioma. A. Pa ien wi h sudden onse o orbi al discom or and prop osis. Medially, a
small area o hemorrhage is no ed wi h subconjunc ival cys s consis en wi h a lymphangioma. B. More obvious
hemorrhage was no ed along wi h he onse o deep orbi al pain. Mul iple cys s can be seen in he hemorrhage.
Imaging was consis en wi h a lymphangioma.
( con inued)
Vascular Orbital Tumors 191

FIGURE 14-5. (Con inued) Lymphangioma. C. Prop osis o he lef eye wi h recurren episodes o orbi al pain.
T e pain was usually associa ed wi h an increase in he prop osis. D and E. MRIs show a superior orbi al mass
wi h area o resh and old blood consis en wi h a lymphangioma.
192 14 O RBITAL NEO PLASMS

HEMANGIOPERICYTOMA MRI: isoin ense on 1; hyperin ense on 2

H emangiopericy oma is a rare lesion ha


can mimic a cavernous hemangioma bu
has more rapid grow h and is more likely o
(Fig. 14-6C, D).
Enhances wi h gadolinium
Special Considerations
cause symp oms. T ese can recur and have a
chance o me as asis. T ese lesions have he po en ial or recur-
rence locally whe her he pa hology is benign
or malignan .
Epidemiology and Etiology
Malignan lesions can recur and can also
Age: Middle age
me as asize.
Gender: Equal occurrence in males and
emales Dif erential Diagnosis
E iology: umor origina es rom he peri- Cavernous hemangioma
cy e. T is is a rare orbi al umor. Fibrous his iocy oma
Schwannoma
History
Insidious onse o prop osis and mass Pathology
e ec bu usually more rapid onse han a cav- Uni orm spindle-cell umor wi h a sinu-
ernous hemangioma soidal vascular pat ern; can be divided in o
benign, in ermedia e, and malignan orms.
Examination
Treatment
Prop osis is o en he only f nding.
Comple e excision in he capsule is he
T ese lesions appear more o en in he bes rea men .
superior orbi bu in raconal loca ion is also
Recurrence, i malignan , may require
common ( Fig. 14-6A, B).
exen era ion.
Imaging Prognosis
C scan: well-circumscribed, encapsu- Variable. Pa ien s mus be ollowed or a
la ed mass leas 10 years or local recurrence or me as asis.
Vascular Orbital Tumors 193

FIGURE 14-6. Hemangiopericytoma. A and B. A 55-year-old man presen ed wi h increasing prop osis over 6
mon hs and diplopia. T ere is axial prop osis on he lef and a well-circumscribed mass on C scan. Pa hologic
examina ion revealed a hemangiopericy oma.
( con inued)
194 14 O RBITAL NEO PLASMS

FIGURE 14-6. ( Con inued) Hemangiopericytoma. C. 1-weigh ed coronal MRI wi h con ras and a suppression
shows a hemangiopericy oma nex o he righ lacrimal gland.
( con inued)
Vascular Orbital Tumors 195

FIGURE 14-6. (Con inued) Hemangiopericytoma. D. -weigh ed coronal MRI o he same hemangiopericy oma
2
nex o he righ lacrimal gland.
196 14 O RBITAL NEO PLASMS

ORBITAL VARICES Nondis ensible varices are more di cul

O rbi al varices will presen in he 20s and


30s wi h a his ory o years o in ermi -
en prop osis. T ese lesions can be superf cial
o diagnose.
T e pa ien will presen wi h symp oms o
an acu e hemorrhage in o he lesion as no ed
and no iceable or deep wi h only prop osis as previously.
a sign o he lesion. Mos lesions should be le T ere is generally no ex ernal hemorrhage
alone unless here is ex reme orbi al pressure presen or any sign o a varix in his ype.
wi h unc ional def ci or a severe cosme ic
disf guremen . Imaging
C scan may appear rela ively normal
Epidemiology and Etiology
or wi h jus a small di use mass on axial
Age: Usually no ed in he f rs hrough cu s.
hird decades o li e
In he dependen posi ion (coronal cu s),
Gender: Equal occurrence in males and he mass will enlarge as he varix f lls wi h
emales blood ( Fig. 14-7C, D).
E iology: Dila a ion o pre-exis ing venous Nondis ensible varices will show a
channels di use mass ha enhances wi h con ras .
History
Dif erential Diagnosis
Pa ien s wi h nondis ensible varices pres-
en wi h recurren episodes o hrombosis and Lymphangioma is he main di eren ial
hemorrhage in he lesion ( Fig. 14-7E, F). and di eren ia ion rom he nondis ensible
varix is no always possible.
T is leads o prop osis, pain, mo il-
i y res ric ion, and even decreased vision.
Pathology
T ese symp oms resolve as he hemorrhage
resolves. Well-def ned venous channels
T e dis ensible varices presen wi h pain,
Treatment
prop osis, and pressure symp oms associa ed
wi h s raining, bending orward, or Valsalva Conserva ive observa ion in mos cases
( Fig. 14-7A, B). I a nondis ensible varix bleeds and visual
T e changes in he orbi and lids associa ed or exposure symp oms require in erven ion,
wi h his venous dis ension are also no ed. drainage o he blood clo is usually he rea -
men o choice.
Examination
Dis ensible varices are diagnosed easily Prognosis
by having he pa ien s bring heir head in o a Variable. Progressive lesions can be
dependen posi ion and no e he f lling o he disf guring and success ul rea men is
varix. di cul .
Vascular Orbital Tumors 197

FIGURE 14-7. Distensible orbital varix. A. A 55-year-old woman wi h a dis ensible varix in her superior medial
orbi . B. Valsalva resul s in massive enlargemen and closure o he eye.
( con inued)
198 14 O RBITAL NEO PLASMS

FIGURE 14-7. (Con inued) Distensible orbital varix. C. C scan showing he medial orbi al varix. D.W hen
he head is placed in a dependen posi ion or he coronal C , he varix lls wi h blood, accoun ing or he
enlargemen o he lesion on he coronal cu s.
( con inued)
Vascular Orbital Tumors 199

FIGURE 14-7. ( Con inued) Distensible orbital varix. E. Nondis ensible varix may be deep in he orbi wi h only
a small an erior componen . F.D i use orbi al involvemen wi h mul iple varices.
200 14 O RBITAL NEO PLASMS

ARTERIOVENOUS pat ern) ( Fig. 14-8A, B), and eleva ed in ra-


MALFORMATIONS ocular pressure are seen in AVMs.

A r eriovenous mal orma ions (AVM)


presen wi h variable severi y. All cases
involve he connec ion o an ar erial ow in o
In high- ow s a es, he re inal vessels are
a ec ed wi h venous conges ion.

a venous drainage area such as he cavernous Imaging


sinus. T ere may be sub le swelling and red- C scan and MRI show an enlarged
ness o he eye and orbi or he presen a ion superior oph halmic vein and here may be
may be severe prop osis, exposure, and in ra- enlargemen o he ex raocular muscles
ocular vascular conges ion. ( Fig. 14-8D and E).
Orbi al Doppler shows reversal o ow in
Epidemiology and Etiology
he superior oph halmic vein and is diagnos-
Age: Older adul s, excep a er rauma, ic o an AVM ( Fig. 14-8C).
which can occur a any age
Gender: Equal occurrence in males and Dif erential Diagnosis
emales Orbi al pseudo umor
E iology: rauma (basal skull rac ure) Orbi al celluli is
resul s in high- ow f s ulas T yroid-rela ed oph halmopa hy
Degenera ive vascular process in pa ien s Chronic conjunc ivi is
wi h hyper ension and a herosclerosis resul s
in a low- ow f s ula. Treatment
Low- ow AVMs will o en resolve
History
spon aneously.
Abrup onse o prop osis, chemosis, ar eri-
T e signs may worsen as he f s ula
aliza ion o he conjunc ival vessels in one eye.
closes o . High- ow lesions o en require
T is occurs in a high- ow AVM. at emp ed selec ive emboliza ion o close
High- ow AVMs will have more severe symp- he f s ula.
oms bu o en have he his ory o head rauma. T is may also be needed in low- ow
Low- ow AVMs are in older pa ien s, are lesions ha resul in uncon rolled glaucoma,
slower in onse , and he symp oms are less diplopia, or vascular occlusion.
drama ic.
Prognosis
Examination Variable. Many low- ow AVMs will close
Prop osis, chemosis, dysmo ili y, ar eriali- on heir own. rea men or AVMs is success-
za ion o he conjunc ival vessels (corkscrew ul bu does have a risk o visual loss.
Vascular Orbital Tumors 201

FIGURE 14-8. Arteriovenous mal ormation. A and B. A pa ien wi h a 3- o 4-week his ory o swelling and
redness o he lef eye. Mo ili y is limi ed as no ed in at emp ed upgaze.
(con inued)
202 14 O RBITAL NEO PLASMS

FIGURE 14-8. ( Con inued) Arteriovenous mal ormation. C. Color Doppler imaging shows ar erializa ion o he
superior oph halmic vein, which is diagnos ic o an ar eriovenous mal orma ion. D and E. C scan shows enlarged
superior oph halmic vein and engorged rec us muscles, which is usually seen wi h an AVM.
( con inued)
Vascular Orbital Tumors 203

FIGURE 14-8. ( Con inued)


204 14 O RBITAL NEO PLASMS

NEURAL TUMORS T e malignan orm in adul s may show


in amma ory signs along wi h signs o an
OPTIC NERVE GLIOMAS op ic neuropa hy and prop osis.

O p ic nerve glioma is a glial umor ha


mos commonly presen s in children
and includes painless prop osis and visual
Imaging
C scan demons ra es usi orm enlarge-
loss. T ese can ini ially involve he op ic chi- men o he op ic nerve ( Fig. 14-9B).
asm or grow o involve i . rea men remains MRI is he imaging o choice o evalua e he
con roversial. ex en and grow h o an op ic nerve glioma.
1 imaging is iso- o hypoin ense, whereas
Epidemiology and Etiology 2 imaging shows prolonged relaxa ion imes

Age: Predominan ly in children during he ( Fig. 14-9C, D).


f rs decade o li e. Malignan gliomas occur in Special Considerations
middle-aged males.
Neurof broma osis ype 1 is associa ed
Gender: Equal occurrence in males and wi h 25% o 50% o op ic nerve gliomas.
emales
E iology: Unknown Dif erential Diagnosis
Op ic nerve meningioma; he di eren ial
History diagnosis is more rela ed o how ex ensive he
In children, gliomas presen wi h umor is and no wha i is.
gradual, painless, unila eral, axial, prop osis
Pathology
wi h loss o vision and an a eren pupillary
de ec . T ese are in radural lesions ha are juve-
nile pilocy ic as rocy omas.
T e malignan orm in adul s presen s wi h
symp oms o op ic neuri is bu rapidly prog- Treatment
ress o blindness and dea h.
Con roversial and mus be individualized.
Mos gliomas can be observed because hey
Examination
are usually very slow-growing lesions.
Axial prop osis wi h visual loss, a eren
I here is signif can grow h, surgical
pupillary de ec , op ic a rophy, or nerve swell-
excision is he bes rea men . I he umor is
ing are all f ndings ( Fig. 14-9).
unresec able, radia ion is considered.
T ere are no in amma ory signs or pain.
Diagnosis is usually made on he basis o Prognosis
orbi al imaging. Variable. Some gliomas grow aggressively;
o hers can remain s able or years.
Neural Tumors 205

FIGURE 14-9. Optic nerve glioma. A. A 6-year-old girl wi h painless prop osis and visual loss. B. C scan
shows usi orm enlargemen o he op ic nerve consis en wi h an op ic nerve glioma.
( con inued)
206 14 O RBITAL NEO PLASMS

FIGURE 14-9. (Con inued) Optic nerve glioma. C. -weigh ed axial MRI shows again shows usi orm
1
enlargemen .
( con inued)
Neural Tumors 207

FIGURE 14-9. (Con inued) Optic nerve glioma. D. -weigh ed axial MRI o he same pa ien . ( C and D
2
cour esy o Ka e Lane, MD.)
208 14 O RBITAL NEO PLASMS

NEUROFIBROMAS Imaging

N eurof bromas are composed o proli er-


a ing Schwann cells wi hin heir nerve
shea h. T ere are mul iple orms o neurof -
C and MRI show a di use inf l ra ing
lesion in plexi orm neurof bromas.
An isola ed neurof broma will be well-
bromas. Plexi orm neurof bromas are o en circumscribed wi h charac eris ics similar o a
associa ed wi h neurof broma osis ype 1. schwannoma.

Epidemiology and Etiology Special Considerations


Age: Plexi orm neurof bromas are usually Any pa ien wi h a plexi orm neurof -
seen in he f rs decade. Isola ed lesions occur broma mus be care ully evalua ed or neuro-
in he hird hrough f h decades. f broma osis i hey are no known o have he
Gender: Equal occurrence in males and disease.
emales Soli ary neurof bromas are rare orbi al
E iology: Plexi orm neurof bromas are he umors ha can be excised and are unlikely o
mos common neurof broma o involve he be associa ed wi h neurof broma osis. T ese
orbi and are associa ed wi h neurof broma- end o occur in middle age.
osis ype 1.
Dif erential Diagnosis
History Lymphangioma
Pa ien s will o en already have he diagno- Orbi al pseudo umor
sis o neurof broma osis and will develop hick- Isola ed lesions mus consider
ening and hyper rophy o he a ec ed nerve. schwannoma, cavernous hemangioma,
T ey may presen wi h hickening o eyelid f brous his iocy oma
or periorbi al skin, or wi h prop osis. Isola ed
neurof bromas are no usually associa ed wi h Pathology
neurof broma osis. Proli era ing, in er wining bundles o
Schwann cells, axons, and endoneural f bro-
Examination
blas s wi hin he nerve shea hs.
Findings will vary depending on he nerve
or nerves ha are involved. Treatment
T e involved nerves grow as a or uous, Observa ion wi h surgical debulking only
ropy angle o nerves. as a las resor .
T is grow h is usually slow bu progressive T ese umors canno be comple ely
and resul s in hickening o involved perior- excised and recur and regrow wi h ime. Rare,
bi al and orbi al issues, prop osis, and orbi al isola ed lesions can be comple ely excised.
bony abnormali ies ( Fig. 14-10A). T ese umors are o en very vascular and
T ese bony changes include orbi al bleed during excision.
enlargemen , abnormali ies o he sphenoid
wing, and hypoplasia o he e hmoid and Prognosis
maxillary sinuses ( Fig. 14-10B). Generally poor cosme ic and unc ional
Isola ed neurof bromas presen wi h mass resul s because o he progressive na ure o
e ec . Prop osis, diplopia, and decreased hese inf l ra ive umors.
vision may occur. Isola ed lesions have a good prognosis.
Neural Tumors 209

FIGURE 14-10. Neurof broma. A. Severe prop osis, comple e p osis, and orbi al in l ra ion by a plexi orm
neuro broma. B. C scan shows orbi al in l ra ion as well as absence o par o he sphenoid bone o he orbi .
All is consis en wi h neuro broma osis.
210 14 O RBITAL NEO PLASMS

MENINGIOMAS Op ic nerve meningiomas presen wi h

M eningiomas are invasive umors ha


arise in racranially and secondarily
invade he orbi . T ey are usually slowly
decreased vision, an a eren pupillary de ec ,
prop osis, and possible oph halmoplegia
( Fig. 14-11E–J).
progressive umors ha are very di cul o T e op ic nerve may be normal, swollen, a ro-
comple ely excise because o heir inf l ra ive phic, or have shun vessels.
na ure.
Imaging
Epidemiology and Etiology C scan: Hyperos osis, calcif ca ion, wi h
Age: Bimodal peak in he second and f h adjacen so issue ullness
decades MRI: Use ul o de ec grow h along he
Gender: More common in women dura
E iology: T ese umors arise rom arach- Gadolinium enhancemen and a suppres-
noid villi. Mos commonly, hese umors s ar sion echniques help def ne hese lesions in
as in racranial umors and ex end in o he he orbi .
orbi secondarily. A primary orbi al orm arises
rom he op ic nerve shea h arachnoid issue. Dif erential Diagnosis
Op ic nerve glioma
History Lymphangioma
Meningiomas will have a gradual onse o
symp oms as hey slowly ex end rom heir Pathology
in racranial origin in o he orbi . hese umors are composed o cells
Oph halmic mani es a ions are dependen ha can be round, polygonal, or spindle
on he loca ion o he umor. shaped.
Mos common orbi al presen a ion is In addi ion, here are varying admix ures
umors arising near he p erion ha presen as o blood vessels, f broblas s, and psammoma
a emporal ossa mass and prop osis ha can bodies.
o en be suddenly no iced bu have been pres- T e di eren pat erns o meningiomas
en or years ( Fig. 14-11A–D). show varying mix ures o hese componen s.
Op ic nerve meningiomas presen wi h
Treatment
slow, painless, progressive visual loss and
prop osis. In racranial meningiomas ha ex end in o
he orbi are usually rea ed surgically.
Examination I well encapsula ed, hese can be com-
Findings on examina ion depend on he ple ely excised wi h a neurosurgical and
loca ion o he meningioma. orbi al approach.
I loca ed in he emporal ossa, f ndings Meningiomas can be inf l ra ive and
include emporal ossa ullness, prop osis, involvemen o vi al s ruc ures may preven
eyelid edema, and chemosis. comple e excision and allow or debulking
I he meningioma arises near he sella and only.
op ic nerve, early f ndings will be visual loss rea men o op ic nerve shea h menin-
wi h op ic nerve edema or a rophy. giomas is required i here is aggressive
Neural Tumors 211

grow h, hrea o in racranial spread, or Prognosis


visual loss. umors are generally progressive bu very
Radia ion is he rea men o choice in slowly.
progressive umors. Radia ion will slow or some imes s op
Radia ion has been shown o slow grow h grow h.
o hese umors. More rapid grow h is unusual and he
Surgical excision or biopsy is rarely needed. diagnosis should be ques ioned.

FIGURE 14-11. Sphenoid wing meningioma. A. A pa ien wi h a lef -sided prop osis o gradual progressive
onse . No e he emporal ossa ullness. B. C scan shows hyperos osis and an associa ed sof issue mass, all
consis en wi h a sphenoid wing meningioma.
( con inued)
212 14 O RBITAL NEO PLASMS

FIGURE 14-11. (Con inued) Sphenoid wing meningioma. C. 1 -weigh ed coronal pos con ras MRI wi h a
suppression shows lef sphenoid wing meningioma.
( con inued)
Neural Tumors 213

FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. D. 2-weigh ed coronal MRI shows lef sphenoid
wing meningioma. E. A pa ien wi h axial prop osis and visual loss.
(con inued)
214 14 O RBITAL NEO PLASMS

FIGURE 14-11. ( Continued) Sphenoid wing meningioma. F. MRI scan shows a usi orm enlargement o the
optic nerve and is consistent with a meningioma. G. More commonly, there is dif use thickening o the optic
nerve. T e thickened right optic nerve has a central lucency, termed the “railroad track” sign.
( continued)
Neural Tumors 215

FIGURE 14-11. ( Continued) Sphenoid wing meningioma. H.T e -weighted image can be hypointense to
2
hyperintense to at and muscle as seen on the lef side.
( continued)
216 14 O RBITAL NEO PLASMS

FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. I. MRI 1-weigh ed coronal image wi h con ras and
a suppression shows a more globular op ic nerve meningioma on he lef .
( con inued)
Neural Tumors 217

FIGURE 14-11. ( Con inued) Sphenoid wing meningioma. J. MRI 2 -weigh ed coronal image shows a more
globular op ic nerve meningioma on he lef .
218 14 O RBITAL NEO PLASMS

SCHWANNOMAS Special Considerations

S chwannomas presen as well-encapsu-


la ed, slowly growing masses ha ac very
much like a cavernous hemangioma. T ese
Eigh een percen o pa ien s wi h
schwannomas have neurof broma osis.

masses are usually easily excised and cause no Dif erential Diagnosis
subsequen problems. Capillary hemangioma
Hemangiopericy oma
Epidemiology and Etiology
Fibrous his iocy oma
Age: 20 o 50 years o age
Gender: Equal occurrence in males and Pathology
emales Proli era ion o Schwann cells in a peri-
E iology: Eccen ric grow hs rom periph- neural capsule is seen.
eral nerves T ese may be in a igh ly ordered arrange-
History men (An oni A) or loose arrangemen
(An oni B).
Slow, insidious onse o prop osis over years
Treatment
Examination
Surgical excision is he rea men o
Prop osis wi h he direc ion dependen on
choice.
he umor posi ion (mos commonly in ra-
conal) ( Fig. 14-12A). Because hey are ou pouchings o a nerve,
hey can o en be s ripped o he nerve.
Less commonly, here may be eyelid swell-
ing, diplopia, visual dis or ion. Recurrence o he umor, even i here is
only par ial resec ion, is very rare.
Imaging
C and MRI scan show a well-circum- Prognosis
scribed, round lesion ( Fig. 14-12B–D). Excellen
Neural Tumors 219

FIGURE 14-12. Schwannoma. A and B. A 45-year-old woman wi h gradual onse o righ eye prop osis. C
scan shows a well-circumscribed mass in he superior orbi . T ere is some bony ossa orma ion. On excision,
his was a schwannoma. Schwannomas are mos commonly in raconal.
( con inued)
220 14 O RBITAL NEO PLASMS

FIGURE 14-12. ( Con inued) Schwannoma. C. MRI shows a well-circumscribed lesion. T is -weigh edi mage
1
shows he lesion isoin ense o muscle and hypoin ense o a .
( con inued)
Neural Tumors 221

FIGURE 14-12. ( Con inued) Schwannoma. D. On he -weigh ed image, he lesion is hyperin ense o a and
2
muscle.
222 14 O RBITAL NEO PLASMS

MESENCHYMAL TUMORS Special Considerations


Acu e prop osis in a child is an emergency.
RHABDOMYOSARCOMA A er a rhabdomyosarcoma is suspec ed,

R habdomyosarcoma is he mos common


primary orbi al malignancy o child-
hood. Classically, he child presen s wi h
he lesion needs o be biopsied wi hin
24 hours ollowed by promp ini ia ion o
rea men .
sudden onse o prop osis over days o weeks
wi h an orbi al mass ound on imaging. Once Dif erential Diagnosis
suspec ed, he lesion should be immedia ely Capillary hemangioma
biopsied so rea men can be s ar ed as quickly
Orbi al pseudo umor
as possible.
Orbi al celluli is
Epidemiology and Etiology Rup ured dermoid cys
Age: Average age 7 o 8 years Me as a ic umor
Gender: Equal occurrence in males and
Pathology
emales
T ere are our dis inc orms o
E iology: Rhabdomyosarcomas develop
rhabdomyosarcoma.
rom undi eren ia ed pluripo en ial mesen-
chymal cells. Embryonic: poorly di eren ia ed
spindle cells
History Alveolar: shows rounded
Classically, rapid onse o unila eral prop osis rhabdomyoblas s
is seen over days o a week, bu some pa ien s Pleomorphic: rounded or s rap-like
may presen less rapidly over many weeks. cells wi h cross-s ria ions
Examination Bo ryoid: rare orm wi h grape-like
clus ers
Prop osis is seen wi h variable amoun s o
adnexal response, such as edema, ery hema, Treatment
and globe displacemen , and some imes a
Once suspec ed, a C scan is done o
palpable mass ( Fig. 14-13A, C, D).
iden i y he lesion.
T e superior nasal orbi is he mos com-
Urgen orbi al biopsy wi h pa hologic eval-
mon loca ion.
ua ion is hen done o conf rm he diagnosis
Imaging and classi y he ype o rhabdomyosarcoma.
C scan: Homogenous mass ha may Sys emic evalua ion by a pedia ric oncolo-
have bony erosion ( Fig. 14-13B, E). gis is hen done. Radia ion and sys emic che-
mo herapy are he mains ays o rea men .
MRI: Hypoin ense on 1 and hyperin-
ense on 2 ( Fig. 14-13F). Prognosis
Variable enhancemen wi h gadolinium Survival ra es are 90% wi h his rea men .
Mesenchymal Tumors 223

FIGURE 14-13. Rhabdomyosarcoma. A. An 8-year-old girl wi h 3-week his ory o progressive swelling o he
righ eye. B. C scan shows large mass o he orbi , which on biopsy was a rhabdomyosarcoma.
( con inued)
224 14 O RBITAL NEO PLASMS

FIGURE 14-13. ( Con inued) Rhabdomyosarcoma. C. and D. A 3-mon h-old girl wi h 1- o 2-week his ory o
swelling o he righ eye.
( con inued)
Mesenchymal Tumors 225

FIGURE 14-13. (Con inued) Rhabdomyosarcoma. E. C scan shows well-circumscribed mass wi h inden a ion
o he globe. Biopsy o he mass revealed a rhabdomyosarcoma. T e ac ha his pa ien is younger han he
ypical rhabdomyosarcoma pa ien shows ha rhabdomyosarcoma can presen a various ages. F. MRI o a
rhabdomyosarcoma. T e 2-weigh ed image shows he lesion is hyperin ense o muscle and a .
226 14 O RBITAL NEO PLASMS

FIBROUS HISTIOCYTOMA Special Considerations

F ibrous his iocy oma is a umor ha can be


benign, locally aggressive, or malignan .
I no comple ely excised, he benign orms
Incomple e excision can lead o
recurrence and he recurrence can be
malignan .
can become malignan . T e umor is usually T e malignan , mos aggressive orm can
inf l ra ing and no encapsula ed. I usually me as asize.
presen s wi h prop osis and is o en accompa-
nied by various orms o orbi al dys unc ion Dif erential Diagnosis
depending on he umor loca ion.
Hemangiopericy oma
Epidemiology and Etiology Capillary hemangioma
Age: Middle age Schwannoma
Gender: Equal occurrence in males and
Pathology
emales
Fibrous-appearing his iocy ic cells ha
E iology: Arises de novo rom mesenchy-
orm a charac eris ic car wheel or s ori orm
mal issue. T e umor can be benign, in erme-
pat ern
dia e, or malignan .
T e availabili y o immunohis ochemical
History s udies has allowed or more accura e classif -
Usually slow onse o prop osis wi h no ca ion o mesenchymal umors.
def ni ive onse in he leas aggressive orm. Many o hese umors in he pas may have
T e malignan orm can presen more rap- been incorrec ly classif ed.
idly accompanied by diplopia, pain, swelling, T is brings in o ques ion some o he
and res ric ed eye movemen s. prognos ic predic ions or hese umors.
For example, some hemangiopericy omas
Examination
ha are aggressive and even po en ially malig-
Prop osis wi h ew o her orbi al signs in nan umors may have classif ed as f brous
he benign orm his iocy omas.
T e more aggressive orms show signs o
in amma ion, res ric ed eye movemen s, che- Treatment
mosis, and swelling ( Fig. 14-14A). Comple e surgical excision. T e his ology
will reveal he prognosis.
Imaging
C scan: Well-circumscribed orbi al mass Prognosis
bu he in ermedia e and malignan orms can
Depends on his ologic ype. I he
be more inf l ra ive ( Fig. 14-14B).
benign or locally aggressive orms are com-
MRI: isoin ense on 1 and hyperin ense on 2 ple ely excised, he prognosis is usually
( Fig. 14-14C) good.
Enhances wi h gadolinium
Mesenchymal Tumors 227

FIGURE 14-14. Fibrous histiocytoma. A. A 42-year-old man wi h progressive prop osis o he lef eye over 2 o
3 mon hs, increasing diplopia, and blurred vision. B. C scan shows an in raconal mass wi h possible in l ra ion
o he op ic nerve. C. On MRI, he mass abu s bu does no in l ra e he op ic nerve. T e mass was removed and
was a benign brous his iocy oma.
228 14 O RBITAL NEO PLASMS

LYMPHOPROLIFERATIVE Imaging
TUMORS C scan: A mass ha molds around orbi al
s ruc ures ra her han displacing or inf l ra -
LYMPHOID HYPERPLASIA AND ing ( Fig. 14-15D).
LYMPHOMAS MRI: Shows ex en o umor bu does no

L ymphoid lesions include a spec rum


o lesions rom benign o malignan .
Presence o even he benign lymphoid hyper-
di eren ia e orbi al in amma ion and canno
separa e lymphoid hyperplasia rom lymphoma
( Fig. 14-15F). PE scanning is used o look
plasia implies a risk in he u ure or he devel- or lymphoid lesions elsewhere in he body.
opmen o a lymphoma somewhere in he
Special Considerations
body. T ese lesions can occur in he orbi or
subconjunc ival area and end o mold around T ere are mul iple ways o evalua e a
s ruc ures ra her han displacing s ruc ures. lymphoid lesion o de ermine whe her i is a
benign or a malignan lesion.
Epidemiology and Etiology No all lesions can be clearly de ermined
Age: Older adul s o be benign or malignan .
Gender: Equal occurrence in males and Even he pa ien wi h benign lymphoid
emales hyperplasia mus be observed sys emically or
E iology: Clonal expansion o abnormal he developmen o a lymphoma elsewhere in
precursor cells. T ere is a con inuum o dis- he body over u ure years.
ease rom he benign, localized lymphoid Dif erential Diagnosis
hyperplasia hrough malignan lymphoma. Orbi al pseudo umor
History Me as a ic orbi al umor
A his ory o a painless, progressive mass Lymphangioma
T e exac his ory depends on he loca ion Pathology
o he mass. An eriorly, i can presen as a vis- A collec ion o lymphocy es is seen, which
ible, palpable mass. iden if es he lesion as lymphoid.
More pos eriorly, he symp oms will be Microscopic appearance will give some
more o prop osis or globe displacemen evidence o he lesion as benign or malignan .
depending on he loca ion.
Fresh issue is evalua ed o iden i y cell
Examination sur ace markers.
An eriorly, a visible, subconjunc ival salmon- Polyclonal lymphocy ic popula ions are
pa ch mass can be observed ( Fig. 14-15A, B). less likely o develop sys emic disease; mono-
A so , di use mass can be palpa ed i he clonal lesions are more likely o accompany
mass is in he an erior orbi bu no visible lymphoma elsewhere in he body.
( Fig. 14-15C). T e at emp o separa e lymphoid lesions
More pos erior umors will cause prop o- in o benign and malignan is no exac and is
sis wi h symp oms depending on he umor bo h con roversial and con using; however, a
loca ion ( Fig. 14-15E). his ime, i is he bes sys em available.
T ese umors end o mold around orbi al Treatment
s ruc ures and rarely displace or inf l ra e s ruc- T e lesions require biopsy o iden i y
ures, so mo ili y or visual dis urbances are rare. whe her hey are benign or malignan .
Lymphoproliferative Tumors 229

A sys emic workup is done o look or evi- Prognosis


dence o lymphoid lesions elsewhere in he body. T e prognosis is dependen on he ype o
rea men or localized benign orbi al dis- lymphoma.
ease is low-dose radia ion. Many lymphomas are very responsive o
More malignan lesions or sys emic dis- rea men bu a high-grade lymphoma can be
ease usually requires radia ion and sys emic rapidly a al, even wi h rea men .
chemo herapy.

FIGURE 14-15. Lymphoid hyperplasia and lymphoma. A and B. Subconjunc ival lymphoid in l ra es. T ese
may be isola ed or here may be orbi al ex ension. T ey may be a reac ive process or a lymphoma. Where hese
lesions all on he spec rum o benign versus malignan can only be di eren ia ed on biopsy. A was benign
reac ive lymphoid hyperplasia, and B was a low-grade lymphoma.
( con inued)
230 14 O RBITAL NEO PLASMS

FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. C. A 65-year-old woman wi h swelling and
redness o he lef eye. D. C scan shows a di use orbi al process, which on biopsy was a lymphoma. Pa ien was
rea ed wi h radia ion and chemo herapy.
( con inued)
Lymphoproliferative Tumors 231

FIGURE 14-15. ( Con inued) Lymphoid hyperplasia and lymphoma. E. Massive lid and orbi al in l ra ion by a
lymphoma on he righ . F. MRI o a lymphoma. T e 2-weigh ed image shows he lesion o be hyperin ense o
muscle and a .
232 14 O RBITAL NEO PLASMS

PLASMACYTOMA Special Considerations

P lasmacy oma is an isola ed mass o plasma


cells ha occurs in he bone. T is lesion
can ex end rom he bone in o he orbi al so
As wi h lymphoma, plasma cell umors
can be benign or malignan .
T ey are di eren ia ed rom mul iple
issue. Progression o a sys emic plasma cell myeloma on he basis o sys emic involve-
umor is ermed mul iple myeloma. men in mul iple myeloma.

Epidemiology and Etiology Dif erential Diagnosis


Age: Six h and seven h decades Mul iple myeloma
Gender: Males more commonly a ec ed Me as a ic disease
E iology: Rare proli era ion o plasma cells His iocy ic disorders
in so issues or bone o he orbi Malignan umor o he sinus

History Pathology
Pa ien s presen wi h slow onse o a mass Classic plasma cells make up he umor.
e ec wi h some in amma ory signs bu very Vary rom ma ure o larger, imma ure cells
rarely pain. depending on he umor.
Symp oms depend on he loca ion o he Di eren ia ion rom mul iple myeloma
umor. is on he basis o sys emic workup; mul iple
myeloma having o her sys emic mani es a ions.
Examination
I loca ed an eriorly, here is a palpable Treatment
mass over or adjacen o an orbi al bone. Biopsy o he lesion, and hen a comple e
T ere may be prop osis or globe displace- sys emic workup.
men depending on he loca ion I he lesion is isola ed, higher dose irradia ion
( Fig. 14-16A). is indica ed. Chemo herapy may be indica ed.

Imaging Prognosis
C scan shows a lesion in or adjacen o Variable depending on he aggressiveness
bone wi h bony des ruc ion ( Fig. 14-16B, C). o he umor.
Lymphoproliferative Tumors 233

FIGURE 14-16. Plasma cell tumor. A. A 70-year-old woman was no ed o have swelling around he lef eye.
Examina ion shows prop osis wi h downward displacemen o he lef eye.
( con inued)
234 14 O RBITAL NEO PLASMS

FIGURE 14-16. (Con inued) Plasma cell tumor. B. C scan shows a superior emporal lesion ha has eroded
bone and may even be cen ered in bone. Biopsy revealed a plasmacy oma.
( con inued)
Lymphoproliferative Tumors 235

FIGURE 14-16. ( Con inued) Plasma cell tumor. C. Axial C scan o plasmacy oma.
236 14 O RBITAL NEO PLASMS

HISTIOCYTIC DISORDERS Mos commonly in he superior, emporal

H is iocy ic disorders are a rare group


o abnormali ies o he mononuclear
phagocy ic sys em. In he orbi , hey mos
orbi .

Special Considerations
commonly presen as a uni ocal lesion o he T e older erm or hese disorders was his-
superior bone o he orbi wi h secondary pro- iocy osis X wi h specif c mani es a ions ermed
gressive prop osis. Let erer–Siwe disease, Hand–Schüller–Chris ian
disease, and eosinophilic granuloma o bone.
Epidemiology and Etiology T ese erms are replaced by dif use so - is-
Age: Children. Children younger han sue his iocy osis, mul iple eosinophilic granuloma
age 2 years are more likely o have sys emic o bone, and uni ocal granuloma o bone.
disease, which is up o 50% a al. Over he
Dif erential Diagnosis
age 2 years, he disease involves he bone
wi hou sys emic involvemen bu is o en Choles ea oma
mul i ocal. T e older he child, he more Repara ive granuloma
likely he disease will be uni ocal and less
severe. Pathology
Gender: Males more commonly a ec ed Proli era ion o dendri ic his iocy es along
wi h granulocy es and lymphocy es
E iology: Abnormal immune regula ion
resul ing in an accumula ion o proli era ing Treatment
dendri ic his iocy es
Bony lesions require a conf rma ory
History biopsy and hen debulking.
Orbi al swelling mos commonly superi- T is rea men is o en cura ive bu in
orly over days o weeks. younger children, evidence o sys emic dis-
ease mus be sough .
Examination Rarely, a s eroid or low-dose radia ion is
Superior orbi al swelling wi h a variable needed.
amoun o mass e ec is he mos common rea men or sys emic disease in younger
presen a ion ( Fig. 14-17A). children may include s eroids, irradia ion, or
Younger children are more likely o cy o oxic agen s. In some cases, he disease
have more swelling, mul i ocal bony may no respond o any hing.
involvemen , and sys emic involvemen .
Prognosis
Imaging Excellen in uni ocal disease in older children.
C scan will show a lesion adjacen o Very young children wi h sys emic disease
bone wi h bone erosion ( Fig. 14-17B, C). have a 50% mor ali y ra e.
Lymphoproliferative Tumors 237

FIGURE 14-17. Histiocytic disorder. A. An 8-year-old boy wi h a 1- o 2-week his ory o swelling o his righ
eye. Mild ery hema and swelling superiorly and downward displacemen o he globe on he righ is shown.
( con inued)
238 14 O RBITAL NEO PLASMS

FIGURE 14-17. ( Con inued) Histiocytic disorder. B and C. C scans shows a superior in l ra e wi h bony
erosion. Biopsy revealed a uni ocal granuloma o bone, which was rea ed wi h curet age.
( con inued)
Lymphoproliferative Tumors 239

FIGURE 14-17. ( Con inued)


240 14 O RBITAL NEO PLASMS

LACRIMAL GLAND TUMORS T e presence o in amma ion and he


amoun o globe displacemen is variable
EPITHELIAL TUMORS OF THE depending on he e iology o he lacrimal
LACRIMAL GLAND gland mass.

T he lacrimal gland can harbor mul iple dis-


ease processes. T e mos common is in am-
ma ory disease. Benign and malignan processes
Imaging
C scan: Pleomorphic adenomas show
a globular, circumscribed mass. T e mass
inheren o he lacrimal gland also occur. Biopsy is at ens and de orms he globe. T ere can be
o en required o iden i y hese processes. pressure expansion o he lacrimal ossa bu
Epidemiology and Etiology no erosion. Malignan lesions are no globular,
are less well def ned, and may have bony ero-
T is group includes a number o en i ies sions and calcif ca ions ( Fig. 14-18B, C, E, I).
ha involve he lacrimal gland. T is does no
include idiopa hic in amma ion or lymphoid MRI: Valuable o def ne he ex en o in ra-
inf l ra ion o he lacrimal gland. cranial ex ension in aggressive, malignan umors
( Fig. 14-18D, G).
Age: Pleomorphic adenoma occurs in
he our h and i h decades. Malignan Special Considerations
mixed umors occur a an older age. Mus comple ely excise a pleomorphic
Adenoid cys ic carcinoma has a peak adenoma or i can recur as a malignan umor.
incidence in he second and our h
Dif erential Diagnosis
decades.
Idiopa hic in amma ion o he lacrimal gland
Gender: Equal occurrence in males and
emales Lymphoid inf l ra ion o he lacrimal gland
( Fig. 14-18H).
E iology: Proli era ion o epi helial cells
Sarcoidosis
History
Pathophysiology
Pleomorphic adenomas presen wi h
progressive, downward, and inward displace- Pleomorphic adenoma: Proli era ion o epi-
men o he globe, some imes wi h axial helial cells wi h duc al and secre ory elemen s.
prop osis( Fig. 14-18A). Adenoid cys ic carcinoma: Small, benign-
T e process is painless, unlike he malig- appearing cells arranged in nes s, ubules, or
nan umors o he lacrimal gland. in a cribri orm, Swiss-cheese pat ern
Malignan mixed umors usually arise Treatment
rom exis ing pleomorphic adenomas. Pleomorphic adenoma: Comple e exci-
Adenoid cys ic carcinoma will presen sion wi hin he capsule
wi h more rapid grow h associa ed wi h sig- Malignan umors: Individualize rea men .
nif can pain( Fig. 14-18E). Generally ex ensive excision, especially wi h ade-
T is pain is wha easily separa es many noid cys ic carcinoma and high-dose radia ion.
malignan lacrimal gland umors rom a Some umors will require orbi al exen era ion.
benign pleomorphic adenoma. Prognosis
Examination Pleomorphic adenoma: Excellen i com-
Palpable mass in he superior, emporal ple ely excised
quadran wi h displacemen o he globe Malignan umors: High ra e o recurrence
down and in. over ime
Lacrimal Gland Tumors 241

FIGURE 14-18. Lacrimal gland tumor. A. A 33-year-old man wi h slowly progressive, painless prop osis over
a ew years. B and C. C scans show a round, well-circumscribed mass replacing he lacrimal gland. Comple e
excision showed a pleomorphic adenoma.
( con inued)
242 14 O RBITAL NEO PLASMS

FIGURE 14-18. (Con inued) Lacrimal gland tumor. D. MRI o a pleomorphic adenoma. T e 2 -weigh edi mage
shows he lesion is hyperin ense o a and muscle.
( con inued)
Lacrimal Gland Tumors 243

FIGURE 14-18. ( Con inued) Lacrimal gland tumor. E. A 58-year-old man wi h 6-mon h his ory o swelling and
pain around he lef eye. Massive prop osis and swelling wi h downward displacemen o he eye are seen. F.C
scan shows a large mass in he lacrimal gland area wi h bony des ruc ion. T is was adenoid cys ic carcinoma o
he lacrimal gland.
( con inued)
244 14 O RBITAL NEO PLASMS

FIGURE 14-18. (Con inued) Lacrimal gland tumor. G. MRI o an adenoid cys ic carcinoma. T e 2-weigh ed
image shows he lesion is hyperin ense o muscle and a . H.T is 75-year-old man had swelling and downward
displacemen o he lef eye or 2 o 3 mon hs.
( con inued)
Lacrimal Gland Tumors 245

FIGURE 14-18. ( Con inued) Lacrimal gland tumor. I. C scan shows an enlarged lacrimal gland, which was a
lymphoma on biopsy. Con ras he shape o his mass wi h he very round con our in B and C.
246 14 O RBITAL NEO PLASMS

MISCELLANEOUS ORBITAL Sinus umors ha ex end in o he orbi


TUMORS may only show prop osis, o en wi h some
direc ional displacemen o he globe.
SECONDARYORBITAL TUMORS
Imaging
S econdary orbi al umors are umors ha
invade he orbi rom adjacen s ruc ures
including sinus umors, eyelid umors, and
C scan: Dependen on he primary
source. A umor rom he sinuses will show
umors ha ex end in o he orbi rom wi hin sinus changes.
he globe. MRI: May help o def ne ex raocular
ex ension o a primary ocular umor.
Epidemiology and Etiology
E iology: Includes umors ha invade he Dif erential Diagnosis
orbi rom he sinus, eyelid, or globe. Sinus I he primary umor is known, he sec-
processes include mucoceles and squamous ondary process is easily suspec ed.
cell carcinoma. Eyelid umors include basal
cell carcinoma ( Fig. 14-19A–C), sebaceous Pathology
adenocarcinoma, and squamous cell carci- T e pa hology is specif c or each o he
noma ( Fig. 14-19E–G). Re inoblas oma individual processes.
and choroidal melanoma ( Fig. 14-19D) can
ex end rom he globe in o he orbi . Treatment
Age and gender: Variable based on pri- rea men is aimed a comple e excision i
mary umor possible.
History I he umor is resec able and here is no
evidence o dis an me as asis, hen ha is he
O en, here is a his ory o ei her a
rea men o choice.
neglec ed primary malignancy or a his ory o
previous rea men o he primary malignancy. Depending on he umor, irradia ion a er
excision can be used.
T e ime course o symp oms and grow h
is dependen on he primary malignancy. For hose umors unresec able, he use
o irradia ion and chemo herapy can be
Examination considered.
T ere may be obvious ex ernal signs o he rea men mus be individualized.
primary umor such as in a neglec ed basal
cell carcinoma. Prognosis
Likewise, in raocular umors ha ex end Generally poor. Even i he umor is
ou side he globe usually have ex ernal signs hough o be removed, here is o en
o in amma ion. recurrence.
Miscellaneous Orbital Tumors 247

FIGURE 14-19. Basal cell carcinoma with orbital invasion. A. A pa ien wi h a neglec ed medial can hal basal
cell carcinoma presen s wi h prop osis. B and C. Axial and coronal C scans show a large medial orbi al mass
ha was basal cell carcinoma on biopsy. T e pa ien required an orbi al exen era ion.
( con inued)
248 14 O RBITAL NEO PLASMS

FIGURE 14-19. (Con inued) Basal cell carcinoma with orbital invasion. D. A pa ien wi h a large limbal mass.
On undus examina ion, here was a large choroidal melanoma ha had ex ended ex rasclerally.
( con inued)
Miscellaneous Orbital Tumors 249

FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. E. A 77-year-old man wi h an
orbi al apex syndrome.
( con inued)
250 14 O RBITAL NEO PLASMS

FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. F. C scan shows an in l ra ing
mass a he apex wi h hickening along he superior orbi . Biopsy showed squamous cell carcinoma ha had
spread in o he apex along he ron al nerve. T e pa ien had he his ory o a squamous cell carcinoma o he
orehead excised 2 years prior.
( con inued)
Miscellaneous Orbital Tumors 251

FIGURE 14-19. ( Con inued) Squamous cell carcinoma with perineural spread. G. Coronal C showing lesion
a apex.
252 14 O RBITAL NEO PLASMS

METASTATIC ORBITAL TUMORS Imaging

M e as a ic orbi al umors usually pres-


en wi h orbi al in amma ion, pain,
prop osis, and bony des ruc ion. Mos cases
C scan: Highly variable appearance
o hese lesions. May be discre e or inva-
sive, cause bony erosion or hyperos osis
will have a known primary malignancy bu (pros a e), and may only show muscle
in up o 25%, he primary si e is unknown enlargemen .
( Fig. 14-20A–G). MRI: No diagnos ic and does no
de ine bone well. May show ex en in o so
Epidemiology and Etiology issues.
Age: Mos commonly in he f h, six h,
and seven h decades Dif erential Diagnosis
E iology and gender: Breas me as asis is Lymphoma
he mos common in women. Lung me as a- Wegener’s granuloma osis
sis is he mos common in men and second Orbi al pseudo umor
in women. O her e iologies include pros a e
in men, gas roin es inal, and many have an Pathology
unknown primary si e. Special s ains and marker s udies can help
iden i y he issue o origin in hose cases ha
History do no have a primary malignancy already
T e majori y o pa ien s will have a known iden if ed.
primary malignancy a he ime o orbi al
occurrence. Treatment
T e onse o symp oms ends o be more Biopsy o iden i y he umor as me as a ic
rapid han in mos orbi al umors and can be Sys emic rea men o he carcinoma is
accompanied by pain. hen ha o he primary malignancy.
Orbi al irradia ion will o en shrink he
Examination
orbi al umor and diminish symp oms, ei her
Prop osis is he mos common f nding. alone or in combina ion wi h chemo herapy.
T is can be axial or displace he globe.
Prognosis
A palpable mass may be presen ha is
usually f rm. P osis, mo ili y dis urbances, T e prognosis is ha o he primary umor
and decreased vision may be presen because wi h me as asis.
o he inf l ra ive na ure o he me as asis. In mos cases, he prognosis is poor.
Miscellaneous Orbital Tumors 253

FIGURE 14-20. Metastatic breast carcinoma. A and B. A 65-year-old woman wi h known his ory o breas
cancer presen s wi h swollen, pain ul righ eye. T e pa ien has prop osis wi h a rozen globe and a corneal ulcer.
C scan shows di use in l ra ion o he orbi wi h me as a ic breas carcinoma. Fur her workup revealed o her
areas o me as a ic disease.
( con inued)
254 14 O RBITAL NEO PLASMS

FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. C. MRI 1 -weigh ed image shows he lesion
hypoin ense o a and muscle.
( con inued)
Miscellaneous Orbital Tumors 255

FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. D.T e 2 -weigh ed image shows he lesion
hyperin ense o a and muscle.
( con inued)
256 14 O RBITAL NEO PLASMS

FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. E and F. A 68-year-old man wi h known me as a ic
lung adenocarcinoma presen s wi h prop osis and pain. No e he la eral mass on he righ . C scan shows a
la eral orbi al mass wi h bone des ruc ion.
( con inued)
Miscellaneous Orbital Tumors 257

FIGURE 14-20. ( Con inued) Metastatic breast carcinoma. G. An 8-year-old child wi h known neuroblas oma
wi h orbi al me as asis. T e pho ograph shows he classic orbi al ecchymosis along wi h prop osis.
C H AP T ER

Orbi al rauma

ORBITAL FRACTURES T e pa ien will of en have double vision


af er he injury.
O BITAL FLOO FR CTU E Less commonly, he pa ien may no e

O rbi al oor rac ures are he mos com-


mon ype o orbi al rac ure. T is is he
resul o a blow o he eye i sel or o he bony
orbi al swelling af er he rauma rom orbi al
emphysema af er blowing he nose.

rim. Many rac ures only resul in swelling and Examination


ecchymosis o he orbi al issues. T ose wi h Orbi al swelling and ecchymosis are vari-
en rapped issue and persis en diplopia, or able. Some rac ures have very lit le.
wi h a large rac ure and enoph halmos, will In raorbi al hypes hesia and res ric ed mo il-
require repair. i y wi h diplopia are he mos speci c signs.
As he orbi al swelling decreases, large
Epidemiology and Etiology rac ures will develop enoph halmos.
Age: Mos common in second o our h Variable degrees o crepi ance may be
decades presen as an indica ion o he rac ure.
Gender: More common in males
Imaging
E iology: Direc orce o he in erior
orbi al rim wi h buckling and rac ure o he C scanning shows a rac ure o he
oor is one mechanism. T e second mecha- orbi al loor o en wi h blood in he
nism consis s o orces ha raise he in raor- sinuses.
bi al pressure and hen “blow-ou ” he hin A rac ure ha is very small is more likely
orbi al oor. o have en rapmen o orbi al issue han a
very large rac ure.
History T e in erior rec us is almos never in he
rauma such as s , ngers, elbow, hi wi h rac ure i sel bu issues around he muscle
a ball, and so or h are en rapped.

258
Orbital Fractures 259

MRI does no image bone well and Treatment


should no be used ini ially af er rauma. Open repair is required or pa ien s wi h
T e excep ion is he Whi e-Eyed Blowou unc ional diplopia ha does no improve as
Frac ure(WEBOF) where he muscle may be he swelling resolves.
in he rac ure(below).
Frac ures involving more han 50% o he
Special Considerations oor will resul in signi can enoph halmos
and also should be considered or repair.
Children and eenagers may sus ain an
orbi al oor rac ure wi h no ecchymosis, bu Frac ures should be repaired wi hin
wi h severe en rapmen o he in erior 2 weeks o rauma wi h he excep ion o a
rec us muscle and associa ed pain, nausea, WEBOF, which is an emergency.
and vomi ing. Mos rac ures ha are repaired will
T is is called a whi e-eyed blowou rac ure require an implan o some ype.
(WEBOF) ( Fig. 15-1). T ese pa ien s are
very uncom or able and di cul o examine. Prognosis
T e en rapmen needs o be released Good i repaired wi hin 2 weeks
wi hin 24 o 48 hours, as he muscle is severely Some pa ien s will have direc muscle or
en rapped and will become ischemic i no nerve injury and ei her will no improve or
released. T is requires emergen surgery. may ake mon hs o improve.

FIGURE 15-1. White-eyed blowout racture. A and B. A 9-year-old boy wi h a his ory o being hi wi h
an elbow 1 day prior. He has pain, worse wi h eye movemen ; double vision; and has had nausea and some
vomi ing. T e pho ograph shows his lack o ecchymosis and swelling as well as severe res ric ion o upgaze.
( continued)
260 15 O RBITAL TRAUMA

FIGURE 15-1. (Continued) White-eyed blowout racture. C. C scan shows a small rap-door f oor rac ure wi h
issue en rapmen . T is rac ure needs o be repaired promp ly as he en rapped muscle may become ischemic.
( continued)
Orbital Fractures 261

FIGURE 15-1. (Continued) Orbital f oor racture. D. T is 72-year-old woman ell and hi her eye on her bedpos .
She has ull mo ili y bu signi can swelling, ecchymosis, and in raorbi al hypes hesia. E. C scan shows a large
orbi al f oor rac ure. T e pa ien is a risk or developmen o enoph halmos rom he large rac ure.
262 15 O RBITAL TRAUMA

MEDIAL WALL FR CTU E uid rhinorrhea, and damage o he lacrimal

M edial wall rac ures can be isola ed rac-


ures o he medial wall only or hey
can be a par o larger rac ures involving he
drainage sys em.

Imaging
nose and sinuses. Isola ed rac ures are rea ed C scanning will show he ex en o he
much like orbi al oor rac ures ( Fig. 15-2). rac ure and assis wi h po en ial planning o
Larger rac ures usually involve a mul idisci- he repair.
pline approach o he repair o he rac ures. MRI does no image bone well and should
no be used ini ially af er rauma.
Epidemiology and Etiology
Age: Mos common in second hrough Special Considerations
our h decades Medial wall rac ures wi h en rapmen
Gender: More common in males o he medial rec us need o be repaired
E iology: Direc rac ures occur rom s rik- sooner han oor rac ures (wi hin 1 week) i
ing a solid objec . Indirec (blow-ou ) rac- possible.
ures occur in associa ion wi h and by similar
Treatment
mechanisms as orbi al oor rac ures.
I isola ed, medial wall rac ures of en do
History no need repair.
rauma his ory is variable. Medial rec us en rapmen wi h diplopia is
Symp oms include diplopia and cosme ic one indica ion or repair.
de ormi ies depending on he ex en o he I he rac ure is large, enoph halmos
nasal rac ures. can develop and require surgery o build
up he orbi . Implan s are some imes
Examination placed.
Medial rec us en rapmen wi h diplopia Larger rac ures involving he nasal bridge
and even ual enoph halmos are he wo ocu- and medial orbi require repair and pla ing,
lar mani es a ions ha may occur. usually in conjunc ion wi h an o olaryngol-
Direc rac ures of en have signi can dam- ogy specialis .
age o he nasal bridge and medial orbi . T e
nasal bridge may be depressed wi h elecan hus. Prognosis
O her ndings ha can occur include Good. Larger rac ures may require mul-
epis axis, orbi al hema oma, cerebral spinal iple surgeries and revisions.
Orbital Fractures 263

FIGURE 15-2. Medial wall racture. A o C. A 55-year-old man s ruck in he ace wi h an unknown objec
presen ed wi h horizon al diplopia. Mo ili y is res ric ed in he righ eye in bo h adduc ion and abduc ion.
( continued)
264 15 O RBITAL TRAUMA

FIGURE 15-2. (Continued) Medial wall racture. D and E. C scans show a medial wall orbi al rac ure wi h he
medial rec us muscle pulled in o he rac ure.
( continued)
Orbital Fractures 265

FIGURE 15-2. (Continued)


266 15 O RBITAL TRAUMA

O BITAL OOF FR CTU E En rapmen o he superior rec us or supe-

O rbi al roo rac ures ( Fig. 15-3) are rare


rac ures ha need o be recognized
because o he po en ial or li e- hrea ening
rior oblique muscle is ex remely rare.

Imaging
neurologic sequelae. T ere may jus be a small C scanning will show he rac ure usually
rac ure wi h no neurologic problems or here jus inside he orbi al rim.
may be signi can in racranial air and bleeding. MRI does no image bone well and should
rea men is in conjunc ion wi h neurosurgery. no be used ini ially af er rauma.
MRI can be o value o evalua e in racra-
Epidemiology and Etiology
nial injury.
Age: Mos common in second hrough
our h decades Special Considerations
Gender: More common in males Impor an o consul neurosurgery or he
E iology: Blun rauma or direc injury by po en ial o CNS complica ions wi h a roo
a hin objec ha goes above he globe under rac ure
he superior orbi al rim. An isola ed roo rac-
ure is rare. Treatment
History Repair o a roo rac ure is usually done or
neurologic reasons ra her han ocular.
rauma his ory will of en sugges high-
energy orces ha caused he injury. Any pla ing and repair is done via
cranio omy.
T ese include hydraulic air hoses, a blun
objec wi h high veloci y, and so or h. Nondisplaced rac ures do no require
repair.
Examination
Poor upgaze, supraorbi al hypes hesia, and Prognosis
more swelling superiorly han in eriorly sug- Variable depending on he ex en o asso-
ges an orbi al roo rac ure. cia ed CNS injuries
Orbital Fractures 267

FIGURE 15-3. Orbital roo racture. A.T e pa ien was s ruck in he eye wi h a hydraulic air hose. Examina ion
shows signi can swelling, wi h decreased upgaze and supraorbi al hypes hesia. B. C scan shows an orbi al roo
rac ure wi h in racranial hemorrhage.
268 15 O RBITAL TRAUMA

ZYGOMATIC FR CTU E Imaging

Z ygoma ic rac ures are he resul o sig-


ni can rauma ic orce o he zygoma ic
area ( Fig. 15-4A–C). T e resul injury and
C scanning shows rac ure o he
zygoma ic arch a he ron al-zygoma ic
su ure and a he maxillary–zygoma ic
symp om depend on he direc ion and amoun su ure.
o displacemen o he bone. Repair should be T e zygoma ic arch is displaced in vari-
done wi hin he rs week when needed. ous direc ions depending on he direc ion o
rauma.
Epidemiology and Etiology
T ere is usually an associa ed orbi al oor
Age: Young adul s rac ure ( Fig. 15-4D–E).
Gender: Males mos common MRI does no image bone well and should
E iology: rauma wi h orce direc ed a no be used ini ially af er rauma.
he zygoma
Treatment
History
Repair is required or mos rac ures wi h
rauma wi h signi can orce
any signi can displacemen .
Pa ien s of en complain o pain and di -
T is should be done as soon as he swelling
cul y opening heir mou h and chewing.
has lessened.
Examination T is is done wi h open reduc ion and
Ini ial ndings may be minimal i here pla ing as needed. Nondisplaced rac ures do
is signi can swelling and ecchymosis o he no require repair.
orbi and cheek.
Depressed cheek, orbi al rim s ep o , and Prognosis
inabili y o open he mou h wide are com- Excellen i repaired promp ly
mon ndings.
Orbital Fractures 269

FIGURE 15-4. Zygomatic racture. A. A 43-year-old man s ruck on he righ cheek and eye wi h a ba . T ere is
f at ening o he cheek wi h rismus. B and C. C scans show a zygoma ic rac ure.
( continued)
270 15 O RBITAL TRAUMA

FIGURE 15-4. (Continued) Zygomatic racture. D and E. C scans show a smaller minimally displaced
zygoma ic rac ure wi h associa ed orbi al f oor rac ure.
( continued)
Orbital Fractures 271

FIGURE 15-4. (Continued)


272 15 O RBITAL TRAUMA

MISCELLANEOUS TRAUMA he hemorrhage is di use wi hin he


issues
O BITAL HEMO HAGE MRI: Acu e hemorrhage is hypoin ense

O rbi al hemorrhage as he resul o orbi al


rauma is common and rarely requires
any speci c rea men ( Fig. 15-5A&B).
on 1 and hyperin ense in 2. When blood is
more han 7 days old, i will become hyperin-
ense on 1 and variable on 2.
Spon aneous orbi al hemorrhage is rare and
requires evalua ion o he orbi or a source Special Considerations
o bleeding, al hough none may be ound. In spon aneous hemorrhage, an orbi al
Orbi al or eyelid surgery is also a cause. T e vascular mal orma ion needs o be looked or.
need o drain an orbi al bleed is very rare. I no hing is seen on imaging af er he
acu e hemorrhage, a ollow-up MRI wi h
Epidemiology and Etiology
gadolinium may iden i y a lesion.
Age: Any
Gender: Males more common because o Dif erential Diagnosis
he higher incidence o rauma Spon aneous hemorrhage (no rauma)
E iology: rauma , surgery ( Fig. 15-5E) or includes he ollowing.
orbi al vascular lesion such as lymphangioma or Lymphangioma
vascular mal orma ion Venous mal orma ion and varix
History Ar eriovenous mal orma ion
His ory is ha o rauma.
Treatment
Wi h vascular mal orma ions, here is sud-
den onse o orbi al pain, pressure, prop osis, Observa ion, unless here is visual loss
and some imes ecchymosis. Mild visual loss needs moni oring wi h
in ravenous s eroids, ace azolamide, and pos-
Examination
sible la eral can ho omy.
Examina ion reveals prop osis wi h vari-
I visual loss is more severe, immedia e la -
able symp oms depending on he severi y o
eral can holysis is indica ed along wi h high-
he hemorrhage.
dose in ravenous s eroids.
T ere can be o her ocular and orbi al inju-
Orbi al imaging is done o look or locu-
ries i he cause is rauma.
la ed blood.
Mild hemorrhage may only reveal
T e blood is usually wi hin he orbi al is-
prop osis.
sues and orbi al drainage or even decompres-
Severe hemorrhage can resul in no ligh sion is rarely o value.
percep ion vision, wi h severe prop osis,
T e excep ion would be a locula ed hem-
corneal exposure, rozen globe, eleva ed in ra-
orrhage such as in a lymphangioma.
ocular pressure, and inabili y o close he eye
because o he severe prop osis Prognosis
( Fig. 15-5C–D).
Chance o permanen visual loss is presen
Imaging wi h severe hemorrhage.
C scan: May show a discre e mass or Less severe hemorrhages resolve wi hou
more in l ra ive lesion. Mos commonly sequelae.
Miscellaneous Trauma 273

FIGURE 15-5. Orbital hemorrhage. A. A 17-year-old girl wi h orbi al hemorrhage a er being poked in he
eye wi h a eld hockey s ick. T ere was no o her injury no ed on C scan. Vision was normal bu he orbi was
modera ely igh . She was observed or progressive hemorrhage bu he ullness o he orbi resolved overnigh .
B.T ere was a small corneal delle ha resolved wi h lubrica ion and resolu ion o he hemorrhage.
( continued)
274 15 O RBITAL TRAUMA

FIGURE 15-5. (Continued) Orbital hemorrhage. C. Severe orbi al hemorrhage a er re robulbar injec ion in
pa ien on Coumadin. D. C scan shows di use hemorrhage wi hin he issue and no locula ed blood. No e he
s re ching and s raigh ening o he op ic nerve.
( continued)
Miscellaneous Trauma 275

FIGURE 15-5. (Continued) Orbital hemorrhage. E. Bila eral orbi al hemorrhage a er blepharoplas y.
276 15 O RBITAL TRAUMA

O BITAL FO EIGN BODIES Imaging

O rbi al oreign bodies mus always be


suspec ed in any kind o orbi al rauma
( Fig. 15-6A–J). Mos oreign bodies are
Imaging is key o iden i ying and localizing
an orbi al oreign body and C scanning is
he imaging modali y o choice.
removed surgically wi h he excep ion o MRI should never be done af er rauma
cer ain iner ma erials ha are deep in he unless a me allic oreign body has been ruled ou .
orbi .
Glass, plas ic, and organic oreign bodies
may no show up well wi h C scanning.
Epidemiology and Etiology T ese can be bet er visualized wi h MRI
Age: Any age scanning, bu even MRI may no show hese
oreign bodies.
Gender: More common in males
E iology: Foreign bodies can en er Treatment
he orbi be ween he globe and he Orbi al oreign bodies should be removed
orbi al wall or by double per ora ion o i hey are organic, cause symp oms, or i hey
he globe. have sharp edges so ha migra ion could
cause damage.
History T e posi ion o he oreign body can a ec
T e his ory may be o a speci c oreign he decision o remove a oreign body. T e
body en ering he orbi . more pos erior i is, he more di cul i will
be o remove i .
he more di icul si ua ion is where
here is rauma wi h a poor his ory bu Any oreign body lef in place requires
wi h wounds ha could sugges a oreign counseling o he pa ien abou he po en ial
body. or u ure ex rusion or in ec ion.
I a oreign body is suspec ed, wound and
orbi al explora ion is required even i imaging
Examination
is nega ive.
I he oreign body is an erior, i may be
palpable or even visible. Prognosis
I i is deeper, here may very ew signs Good. Organic oreign bodies can some-
excep an en rance wound, or here may be imes be re ained and lead o chronic in am-
signi can hemorrhage and swelling . ma ion or in ec ion.
Miscellaneous Trauma 277

FIGURE 15-6. Orbital oreign body. A. A 12-year-old sho wi h a BB gun 2 weeks prior. T e child has a long-
s anding eso ropia. No e he mild ery hema o he la eral righ globe. B. C scan shows he BB in he an erior
la eral orbi . BBs can be le in he orbi wi hou a problem. In his case, because o he an erior loca ion and
rela ive ease o removal, he BB was removed.
( continued)
278 15 O RBITAL TRAUMA

FIGURE 15-6. (Continued) Orbital oreign body. C and D. Mul iple eyelid lacera ions rom being s ruck wi h a
wineglass ha broke. A re ained oreign body mus always be suspec ed wi h broken glass. On C scan, a oreign
body is no ed.
( continued)
Miscellaneous Trauma 279

FIGURE 15-6. ( Continued) Orbital oreign body. E.T e glass oreign body ha was removed. T e wineglass was
leaded crys al, which is why i showed up so well on C scan. F.T e pa ien was s ruck in he eye wi h a pencil 4
mon hs prior. He presen ed wi h mild irri a ion o he le orbi . No e he lump in he le medial can hus.
( continued)
280 15 O RBITAL TRAUMA

FIGURE 15-6. (Continued) Orbital oreign body. G. C scan shows a medial orbi al opaci y. H.T e medical
orbi al opaci y urned ou o be par o a pencil.
( continued)
Miscellaneous Trauma 281

FIGURE 15-6. ( Continued) Orbital oreign body. I and J.T e pa ien ran in o a bush and an eyelid lacera ion was
revealed on examina ion. I. C scan showed no oreign body. J. Explora ion showed mul iple wood ragmen s.
282 15 O RBITAL TRAUMA

MUCOCELE Examination

D es ruc ion o he sinus os ium rom


rauma or sinus disease can resul in a
mucous- lled sinus ha can hen expand in o
Findings will depend on he loca ion o
he mucocele.
T e mass expands slowly and will displace
he orbi ( Fig. 15-7). Symp oms depend on he globe, cause prop osis, and, i pos erior,
he loca ion o he mucocele. rea men is may cause op ic nerve compression and/ or
usually aimed a excision o he cys and obli - an orbi al apex syndrome.
era ion o he sinus.
Special Considerations
Epidemiology and Etiology Fron al and e hmoidal sinus mucoceles
Age: Any age bu mos common age is 40 are he mos common, wi h sphenoid sinus
o 70 years mucoceles being less common.
Gender: More common in males
Dif erential Diagnosis
E iology: Blockage o he sinus os ium
Orbi al abscess
resul s in a mucous- lled cys ha can expand
wi h ime. Primary sinus umor wi h orbi al
ex ension
History
Treatment
T ere is usually a his ory o rauma o he
Surgical excision wi h obli era ion o he
sinuses or a long his ory o sinus disease.
a ec ed sinus
T e orbi al process shows very slow insidi-
ous onse o prop osis or globe displacemen . Prognosis
More pos erior mucoceles can presen wi h T ere can be recurrences. Mos mucoceles
slow visual loss. are easily rea ed surgically unless ex remely
Rarely, i he mucocele becomes in ec ed, large.
he symp oms can have a rapid progression.
Miscellaneous Trauma 283

FIGURE 15-7. Orbital mucocele. A. A pa ien wi h a his ory o prior acial rac ures presen s wi h he
complain ha his le eye is “ou o place.” T e dura ion o his condi ion is unknown. B. C scan shows a large
ron al sinus mucocele displacing he globe downward.
Index
NO E: Locators ollowed by ‘ ’ re er to f gures.

A Brow ptosis, 96–97 Corneal abrasion, 57


Acid and alkali burns, 123 Burns, 54–55 CPEO, see chronic progressive
Acquired myogenic ptosis, 76–77 external ophthalmoplegia
Acquired nasolacrimal duct (CPEO)
obstruction, 130–131 C Cryptophthalmos, 120
Actinic keratosis, 32–33 , 34, 40 Canalicular eyelid laceration, 50–51 Cutaneous horn, 6–7
pigmented, 4 Canalicular obstruction, 132–133
Acute spastic entropion, 56–57 Canaliculitis, 137–138, 139
Ankyloblepharon, 120–121 Capillary hemangiomas, 180–183 , D
Anophthalmos, 170 218, 222, 226 Dacryoadenititis, 164
Antibiotic ointment, 54 Cavernous hemangiomas, 184–187 , Dacryocystitis, 134–135, 136 , 140
Apocrine hydrocystoma, 16–17 192, 208 Dacryocystocele, 128
Aponeurotic ptosis, 78–79 , 90 Cavernous sinus thrombosis, 146, Dacryocystorhinostomy, 130
Arteriovenous mal ormations 150 Dermal nevus, 2
(AVM), 200–203 , 272 Central eyelid laceration, 49 Dermatochalasis, 98–99 , 100
Aspergillosis, 152–153 Chalazion, 8, 24–25 , 46 Dermatof broma, 20
Atopic disease, 123 Cherry angioma. see Hemangioma Dermoid cysts, 174–177
AVM. see Arteriovenous Cholesteatoma, 236 Discoid lupus, 32
mal ormations (AVM) Chronic blepharitis, 42 Distichiasis, 118–119
Chronic chalazion, 42 Dog bites, 48, 52
Chronic conjunctivitis, 126, 137 with eyelid lacerations, 53
B Chronic progressive external
Balloon dacryoplasty, 130 ophthalmoplegia (CPEO),
Basal cell carcinoma, 18, 20, 24, 30, 74, 82 E
36–39 , 40, 42 Cicatricial ectropion, 62, 66–67 , 68 Eaton–Lambert syndrome, 82
cystic, 16 Coloboma, 116–117 Eccrine hydrocystoma, 16
pigmented, 4, 44 Congenital distichiasis, 72 Ectropion
Bell’s palsy, 64 Congenital entropion, 112, 114–115 cicatricial, 66–67
Benign adenoma, 14 Congenital eyelid anomalies involutional, 62–63
Benign essential blepharospasm, ankyloblepharon, 120–121 mechanical, 68–69
104–105 , 106 blepharophimosis, 108–109 paralytic, 64–65
Benign eyelid lesions coloboma, 116–117 symblepharon, 70–71
apocrine hydrocystoma, 16–17 distichiasis, 118–119 trichiasis, 72–73
cutaneous horn, 6–7 entropion, 114–115 Electrical burn with necrosis, 55
epidermal inclusion cyst, 8–9 epiblepharon, 112–113 Entropion, 58, 60, 114–115
hemangioma, 22–23 epicanthus, 110–111 acute spastic, 56–57 , 58
molluscum contagiosum, Congenital myogenic ptosis, 74–75 cicatricial, 56, 58, 60–61 , 72, 122
10–11 Congenital nasolacrimal duct congenital, 112, 114–115
nevocellular nevi, 20–21 obstruction, 126–127 involutional, 56
papilloma, 2–3 Congenital orbital anomalies spastic, 72
seborrheic keratosis, 4–5 microphthalmos, 170–173 Epiblepharon, 112–113 , 114
syringoma, 14–15 Congenital orbital tumors Epicanthus, 110–111
trichoepithelioma, 18–19 dermoid cysts, 174–177 Epidermal inclusion cyst, 8–9 ,
xanthelasma, 12–13 lipodermoids, 178–179 10, 18
Birth trauma, 116 Congenital ptosis, 76, 78 Epithelial tumors, 240–245
Blepharitis, 130, 132 Conjunctival granulomas, 164 Eyelid
Blepharophimosis, 108–109 , Conjunctival papillomas, 2 abscess, 26
110 Contralateral ptosis, 102 burns, 54–55
Botulinum toxin injection, 104 Copious corneal lubrication, 54 in ammation

285
286 INDEX

Eyelid (cont.) congenital myogenic, 74–75 Lacrimal obstructions


chalazion, 24–25 traumatic, 92–93 acquired nasolacrimal duct,
oppy eyelid syndrome, retraction, 102–103 130–131
28–29 canalicular, 132–133
hordeolum, 26–27 congenital nasolacrimal duct,
neoplasms F 126–127
actinic keratosis, 32–33 Fat prolapse, 178 dacryocystocele, 128
basal cell carcinoma, 36–39 Fibrous histiocytoma, 184, 192, 208, lacrimal f stula, 129
Kaposi’s sarcoma, 46–47 218, 226–227 Lacrimal sac tumor, 134, 140–141
keratoacanthoma, 30–31 Floor racture, 258–261 Lentigo maligna, 34–35
lentigo maligna, 34–35 Floppy eyelid syndrome, 28–29 Lipodermoids, 178–179
malignant melanoma, 44–45 Fractures, orbital trauma Lymphangiomas, 188–189,
sebaceous adenocarcinoma, oor, 258–261 190 –191 , 196, 208, 210,
42–43 medial wall, 262–265 228, 272
squamous cell carcinoma, orbital roo , 266–267 Lymphoid hyperplasia and
40–41 zygomatic, 268–271 lymphomas, 228–231
retraction, 102–103 Lymphoid inf ltration o lacrimal
trauma gland, 240
canalicular eyelid laceration, H Lymphoma, 160, 178, 252
50–51 Hemangioma, 22–23 , 46 Lymphoproli erative tumors
dog bites, 52–53 Hemangiopericytoma, 184, 192– histiocytic disorders, 236–239
eyelid burns, 54–55 195 , 218, 226 lymphoid hyperplasia and
marginal eyelid laceration, Hemi acial spasm, 104, 106–107 lymphomas, 228–231
48–49 Histiocytic disorders, 232, plasmacytoma, 232–235
Eyelid malpositions 236–239
Brow ptosis, 96–97 Hordeolum, 26–27
dermatochalasis, 98–99 Horner’s syndrome, 88–89 M
dyskinesis Hyperkeratotic actinic keratosis, 30 Malignant melanoma, 20, 34, 44–45
benign essential Hypotropia, 102 survival in relation to tumor
blepharospasm, 104–105 depth, 44t
hemi acial spasm, 106–107 Marcus gunn jaw winking syndrome,
ectropion I 84–87
cicatricial, 66–67 Idiopathic orbital in ammation. see Marginal eyelid laceration, 48–49
involutional, 62–63 Orbital pseudotumor Mechanical ectropion, 68–69
mechanical, 68–69 Idiopathic orbital pseudotumor, 164 Mechanical ptosis, 90–91
paralytic, 64–65 Intracranial meningiomas, 210 Medial wall racture, 262–265
symblepharon, 70–71 Involutional ectropion, 62–63 , 68 Melanocytic nevus, 46
trichiasis, 72–73 Involutional entropion, 56, 58–59 , Melanoma, 22
entropion 60, 72 Meningiomas, 210–217
acute spastic entropion, Mesenchymal tumors
56–57 f brous histiocytoma, 226–227
cicatricial entropion, 60–61 K rhabdomyosarcoma, 222–225
involutional entropion, 58–59 Kaposi’s sarcoma, 46–47 Metastatic breast carcinoma, 253
mechanical ptosis, 90–91 Keratitis sicca, 130, 132 Metastatic disease, 160, 232
neurogenic ptosis Keratoacanthoma, 10, 30–31 , 40 Metastatic orbital tumors, 143, 152,
Horner’s syndrome, 88–89 228, 252–257
marcus gunn jaw winking Metastatic tumor, 222
syndrome, 84–87 L Microphthalmos, 120, 170–173
myasthenia gravis, 82–83 Lacrimal f stula, 129 Migrated punctal plug, 137
third nerve palsy, 80–81 Lacrimal gland tumors Molluscum contagiosum, 2, 8, 10
pseudoptosis, 94–95 epithelial tumors, 240–245 eyelid margin, lesions o , 11
ptosis Lacrimal in ections Mucocele, 282–283
acquired myogenic, 76–77 canaliculitis, 137–138, 139 rontal and ethmoidal sinus, 282
aponeurotic, 78–79 dacryocystitis, 134–135, 136 sinusitis with, 152
INDEX 287

Mucormycosis. see Phycomycosis lacrimal gland tumors Phycomycosis, 143, 146, 150–151 ,
Multiple myeloma, 232 epithelial tumors, 240–245 152
Myasthenia gravis, 74, 78, 82–83 lymphoproli erative tumors Pigmented actinic keratosis, 4
Myokymia, 106 histiocytic disorders, 236– Plasmacytoma, 232–235
239 Pleomorphic adenoma, 240
lymphoid hyperplasia and Polyclonal lymphocytic populations,
N lymphomas, 228–231 228
Neural tumors plasmacytoma, 232–235 Preseptal cellulitis, 26, 143
meningiomas, 210–217 mesenchymal tumors Prolapsed lacrimal gland, 178
neurof bromas, 208–209 f brous histiocytoma, Pseudoptosis, 94–95
optic nerve gliomas, 204–207 226–227 Pseudotumor, 160–163
schwannomas, 218–221 rhabdomyosarcoma, Punctal abnormalities, 130
Neurof bromas, 208–209 222–225 Punctal dysgenesis, 126
Neurof bromatosis, 204 metastatic orbital tumors, Pyogenic granuloma, 22, 46
Neurogenic ptosis, 76 252–257
Nevi. see Nevocellular nevi neural tumors
Nevocellular nevi, 20–21 meningiomas, 210–217 R
Nevus, 44 neurof bromas, 208–209 Rhabdomyosarcoma, 180,
optic nerve gliomas, 222–225
204–207 Ruptured dermoid cyst, 160, 222
O schwannomas, 218–221
OCP. see Ocular cicatricial secondary orbital tumors,
pemphigoid (OCP) 246–251 S
Ocular cicatricial pemphigoid vascular orbital tumors Sarcoidosis, 164–167 , 240
(OCP), 122–123, AVM, 200–203 Schwannomas, 184, 192, 218–221 ,
124 –125 capillary hemangiomas, 226
Oculopharyngeal dystrophy, 74 180–183 Sebaceous adenocarcinoma, 24,
Optic nerve gliomas, 204–207 , cavernous hemangiomas, 42–43
210 184–187 Seborrheic keratosis, 4–5 , 20, 34
Optic nerve meningioma, 204 hemangiopericytoma, pedunculated, 2
Orbital abscess, 143, 146–149 , 192–195 Secondary orbital tumors,
282 lymphangiomas, 188–189, 246–251
Orbital cellulitis, 142–143, 190 –191 Sinus tumor, 282
143 –145 , 146, 150, 155, orbital varices, 196–199 Solitary neurof broma, 2
160, 222 Orbital pseudotumor, 143, 146, 150, Spastic entropion, 72
Orbital oreign bodies, 276–281 155, 160–163 , 208, 222, SPK. see Superf cial punctate keratitis
Orbital hemorrhage, 272–275 228, 252 (SPK)
Orbital in ections Orbital roo racture, 266–267 Squamous cell carcinoma, 24, 32, 36,
abscess, 146–149 Orbital trauma 40–41 , 42
aspergillosis, 152–153 oreign bodies, 276–281 Stevens-Johnson syndrome, 123
cellulitis, 142–143, 143 –145 ractures Superf cial punctate keratitis (SPK),
phycomycosis, 150–151 oor, 258–261 58
Orbital in ammation medial wall, 262–265 Symblepharon, 70–71
pseudotumor, 160–163 orbital roo , 266–267 Syringoma, 8, 10, 14–15 , 18
sarcoidosis, 164–167 zygomatic, 268–271
RO, 154–155, 156 –159 hemorrhage, 272–275
Wegener’s granulomatosis, mucocele, 282–283 T
168–169 Orbital varices, 196–199 etanus immunization, 48, 52
Orbital in ammatory disease, 100 T ird nerve palsy, 80–81 , 82
Orbital lymphoma, 155 T yroid-related ophthalmopathy
Orbital neoplasms P ( RO), 100, 154–155,
congenital orbital tumors Papilloma, 2–3 156 –159 , 160
dermoid cysts, 174–177 Paralytic ectropion, 62, 64–65 , 68 rachoma, 123
lipodermoids, 178–179 Parinaud syndrome, 102 raumatic ptosis, 90, 92–93
288 INDEX

richiasis, 72–73 , 122, 126 lymphangiomas, 188–189, White-eyed blowout racture


richoepithelioma, 18–19 , 36 190 –191 (WEBOF), 259
orbital varices, 196–199
Venous mal ormation and varix, 272
V Verruca vulgaris, 4 X
Vascular orbital tumors Xanthelasma, 12–13
AVM, 200–203
capillary hemangiomas, W
180–183 WEBOF. see White-eyed blowout Z
cavernous hemangiomas, racture (WEBOF) Zygomatic racture, 268–271
184–187 Wegener’s granulomatosis, 168–169 ,
hemangiopericytoma, 192–195 252

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