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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
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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 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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
24
Chalazion 25
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
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
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
30
Keratoacanthoma 31
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
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
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
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
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
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
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).
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
FIGURE 3-8. Kaposi’s sarcoma. Lesion o the lower lid in a patient with AIDS.
C H AP T ER
Eyelid rauma
48
Marginal Eyelid Laceration 49
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
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
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
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
ENTROPION History
ecen surgery on he eye or recen onse
ACUTE SPASTIC ENT OPION o ocular irri a ion
56
Entropion 57
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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 5-16. 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
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
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
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
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
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.
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
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
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
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
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
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
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
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
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
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
Examination
CONGENITAL NASOLACRIMAL
DUCT OBSTRUCTION Diagnosis is based mainly on he his ory.
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
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
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
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
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
134
Dacryocystitis 135
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
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
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
142
Orbital Cellulitis 143
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
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
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
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
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. 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
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
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.
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. (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
mos can be mild or the eye can be so small that Dif erential Diagnosis
it cannot be seen at all. Anophthalmos
170
Microphthalmos 171
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
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
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
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
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
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
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
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
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
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
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 14-9. 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
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
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
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
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
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
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
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
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 14-18. 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
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
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
258
Orbital Fractures 259
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
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
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
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 15-5. 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
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
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.
285
286 INDEX
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