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Camptodactyly

Christian Dumontier MD, PhD


Centre de la Main
Guadeloupe - FWI

Available at www.diuchirurgiemain.org
Camptodactyly
• Camptodactyly = Bent finger [from
the greek κάµπτω (to bend) and
δάκτυλος (finger)]

• 1st description by Tamplin, 1846

• 1st use of the name by Landouzy-1906

• Nontraumatic finger PIP joint


flexion deformity, often occurring
bilaterally (≈ 2/3).

• Mostly the little finger (94% of cases -


Engber)

Yannascoli SM, Goldfarb CA. Treating Congenital Proximal Interphalangeal Joint Contracture. Hand Clin 34 (2018)
237–249
Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg Am
1977;2(3):216–24.
Camptodactyly

• Camptodactyly is a clinical
sign, possibly part of a
syndrome, but is never a
disease in its own right
(Flatt).

• Its cause remains debatable to


date

Flatt AE: The care of congenital hand anomalies. St. Louis, 1977, The CV Mosby Co, pp 147-54
Camptodactyly

• Unknown incidence but


probably less than 1% (0,83%
in 2000 newborn - Gnamey)

• Males = females or Females >


males according to series

• 45/46 were caucasians


( Engber)

Gnamey D. Fréquence des malformations congénitales et héréditaires des doigts. Lille Médical 1973;18(8):950–2.
Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg Am
Clinical presentation
• Most common: sporadic without family history

• Familial variant : autosomal dominant pattern of inheritance with


variable penetrance

• Association with clinodactyly, syndactyly, polydactyly,


brachydactyly, or longitudinal ray defects is a different form

• Associated with various syndromes including arthrogryposis,


mucopolysaccharidoses, Marfan syndrome, chromosomal trisomy
disorders, oculodentaldigital dysplasia, Freeman- Sheldon
syndrome, windblown hand, and camptomelic dysplasia..
Camptodactyly
• Bimodal age distribution during
periods of rapid growth.

• < 2 years old, infantile


camptodactyly (M = F), ≈ 80% of Infantile presentation from Yannascoli 2018
cases.

• > 10 years old, adolescent


camptodactyly (F > M), ≈ 20% of
cases.

Yannascoli SM, Goldfarb CA. Treating Congenital Proximal Interphalangeal Joint Contracture. Hand Clin 34 (2018)
237–249
Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg Am
1977;2(3):216–24.
Classification

• Many used
Type I Infantile
• Age at presentation
and associated Type II Adolescent
anomalies are more
frequently preferred Type III Syndromic

Benson LS, Waters PM, Kamil NI, et al. Camptodactyly: classification and results of nonoperative treatment. J Pediatr
Orthop 1994;14(6):814–9
Causes suspected

• Tuberculosis and rheumatoid disorders - Landouzy


1906

• Sluggish peripheral circulation - Oldfield 1954

• Volar skin tightness + abnormal insertion of the 4th


lumbrical (2 out of 3 cases) - Courtemanche

Courtemanche AD. Campylodactyly: etiology and management. Plast Reconstr Surg 1969;44(5):451–4.
Oldfield MC. Campylodactyly: flexor contracture of the fingers in young girls. Br J Plast Surg 1956; 8(4):312–7.
Cause: abnormal volar structures
• Abnormal lumbrical insertion (96% of his cases),
47% FDS anomaly -Mc Farlane

• Volar skin tightness and very few lumbrical


insertion anomaly (10%) - Siegert

• Hypoplastic FDS tendon in 5 out of 6 patients-


Ogino
McFarlane RM, Curry GI, Evans HB. Anomalies of the intrinsic muscles in camptodactyly. J Hand Surg Am 1983;8(5
Pt 1):531–44.
McFarlane RM, Classen DA, Porte AM, et al. The anatomy and treatment of camptodactyly of the small finger. J Hand
Surg Am 1992;17(1):35–44.
Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br 1990;15(2): 181–9.
Ogino T, Kato H. Operative findings in camptodactyly of the little finger. J Hand Surg Br 1992;17(6): 661–4.
From
Ogino

• Baker et al reported up to 34% of defective FDS V over 526 hands.

• Austin et al. reported 58% independent, 21% connected and 21 % absent FDS V (50 dissections)

Baker DS, Gaul JS Jr, Williams VK, Graves M. The little finger superficialis – clinical investigation of its anatomic and
functional shortcomings. J Hand Surg 1981; 6A: 374-8.
Austin GJ, Leslie BM, Ryby LK. Variations of the flexor digitorum superficilais of the small finger. J Hand Surg 1989; 14A:
262-267.
Lumbrical muscles anomalies
• 21 cases out of 21 with anomalous insertion of lumbrical IV

• 15 insertions onto the flexor sheath +/- MP joint capsule

• 4 insertions onto the extensor apparatus

• 3 insertions on the FDS

• 5 anomalies of the palmar interosseous muscle out of 10

• Camptodactyly is thought to be the result of an inefficient


intrinsic function

Mc Farlane, RM, Curry GI, Evans HB. Anomalies of the intrinsic muscles in camptodactyly. J Hand Surg 1983;
8:531-544
Wilhelm A, Kleinschmidt W: Neue atiologische und therapeutische Gesichtspunkte bei der Kamptodaktylie und
Tendovaginitis stenosans. Chir Plast Reconstr 5:62, 1968.
Minami A, Sakai T. Camptodactyly caused by abnormal insertion and origin of lumbrical muscle. J Hand Surg Br 1993;
18B: 310-311.
However

• Lumbrical I inserted only into the extensor expansion of the


index finger in 95% of cadaver hands,

• Normal insertion of lumbrical II was noted in 90%, of


lumbrical III in 48%, and of lumbrical IV in 17% (Mehta).

• The most common anomaly was a split insertion, either into


bone and tendon of the same digit or into the extensor
expansions of adjacent digits.

Mehta HJ, Gardner WU: A study of lumbrical muscles in the human hand. Am J Anat 109:227, 1961
Cause- abnormal dorsal structures

• Anomalies of the extensor mechanism

• Absence of lateral bands- Smith

• Absence of extensor mechanism - Carneiro

• Extensor mechanism anomalies- Koman

Smith PJ, Grobbelaar AO. Camptodactyly: a unifying theory and approach to surgical treatment. J Hand Surg Am
1998;23(1):14–9.
Carneiro RS. Congenital attenuation of the extensor tendon central slip. J Hand Surg Am 1993;18(6): 1004–7.
Koman LA, Toby EB, Poehling GG. Congenital flexion deformities of the proximal interphalangeal joint in children: a
subgroup of camptodactyly. J Hand Surg Am 1990;15(4):582–6.
As a consequence
• If surgery is considered

• « We believe that rather than


isolated anatomic factors that
differ in each individual, it is
involvement of all the anatomic
factors previously discussed to
a variable degree that occurs in
all patients » (Smith) from Smith and Grobbelaar

Smith PJ, Grobbelaar AO. Camptodactyly: a unifying theory and approach to surgical treatment. J Hand Surg Am
1998;23(1):14–9.
Most authors now favor a stepwise surgery

• Stepwise assessment of all potentially involved


structures (skin, fascia, the FDS tendon,
lumbricals, interossei, lateral bands, volar plate,
accessory collateral ligaments, joint surfaces, and
central slip insertion) is necessary

• And treatment is tailored to the involved structures


diagnosed pre-op and/or found at surgery
Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm
based on clinical examination. Plast Reconstr Surg 2006;117(6): 1897–905.
Hamilton KL, Netscher DT. Evaluation of a stepwise surgical approach to camptodactyly. Plast Reconstr Surg
2015;135(3):568–76.
Netscher DT, Hamilton KL, Paz L. Soft-tissue surgery for camptodactyly corrects skeletal changes. Plast Reconstr Surg
2015;136(5):1028–35.
Patient clinical presentation
• Painless deformity

• Compensatory MP extension

• May present with DIP extension - rare (beware


of a boutonniere)

• PIP deformity may be reducible or not ( a fixed


deformity may not be an end stage of the
disease ?)

• Mostly the little finger (> 55% of cases), often


bilateral and asymmetric.

• Aggravation during period of rapid growth


Physical examination

• Eliminate differential diagnosis (trigger finger and


distal arthrogryposis are the main diagnosis)

• Identify all structures that can be involved in the


deformity in order to address them
Physical examination steps

May require Z-plasty, skin graft or


Skin tightness or a pterygium
rotational flap

Yannascoli SM, Goldfarb CA. Treating Congenital Proximal Interphalangeal Joint Contracture. Hand Clin 34 (2018)
237–249
Physical examination steps
PIP contracture with MCP and
wrist flexion to assess volar plate
and intrinsic joint structures
Physical examination steps
May require Z-plasty, skin graft or
Skin tightness or a pterygium
rotational flap
PIP contracture with MCP and wrist flexion to
assess volar plate and intrinsic joint structures

PIP passive extension with the wrist any change during wrist extension
in extension indicates tightness of flexor tendons
Bouvier’s maneuver (active PIP To identify attenuation of the central
extension with the MP flexed) slip of the extensor mechanism
DIP hyperextension ?

if planning to divide the FDS, FDP


FDP strength
must be intact and strong
FDS function of the small finger ? Testing of FDS V

Yannascoli SM, Goldfarb CA. Treating Congenital Proximal Interphalangeal Joint Contracture. Hand Clin 34 (2018)
237–249
FDS V testing

• Independant and functional FDS V - 51-58%

• Common FDS 4 and 5 - 15-27%

• « Absent » functional FSD V - 34% (Baker)

• An « absent » FDS V or a common tendon with the FDS IV


will diminish grip strength of about 10%.

Bowman P, Johnson L, Chiapetta A, Mitchell A, Belusko E. The clinical impact of the presence or absence of the fifth finger
flexor digitorum superficialis on grip strength. J Hand Ther 2003; 16(3): 245-8.
Radiographic evaluation

• Flattening and beaking of the


P1 head

• Indentation of P2 phalangeal
base
Radiographic evaluation

• Present in 29% of Foucher’s


series (up to 58% if the
contracture was fixed) and
68% in Ogino’s series

Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm
based on clinical examination. Plast Reconstr Surg 2006;117(6): 1897–905.
Ogino T, Kato H. Operative findings in camptodactyly of the little finger. J Hand Surg Br 1992;17(6): 661–4.
Treatment

Patients (and parents) should be


• Abstention aware
To accept mild deformity
To strictly adhere to therapy
• Non operative protocol often sufficient with
treatment acceptable results
That a normal finger are not the
• Surgery goal of surgical correction and
that surgery can be delayed
Abstention
• Easy to perform

• No cost

• No complication

• No functional impairment

Possible aggravation
with time
Non-operative treatment

• Of 62 patients, only 5 failed conservative treatment

• Capener-type splint worn day & night until regaining complete


extension, then 8 hours/night until late teenage

Miura T, Nakamura R, Tamura Y. Long-standing extended dynamic splintage and release of an abnormal restraining
structure in camptodactyly. J Hand Surg Br 1992;17B:665-672.
Non-operative treatment
• Passive stretching protocols
( 4 times / day, 5 minutes)

• Active extension
strentghening
FromYannascoli
• Static splinting at night
(sometimes dynamic splinting
at day)

A lack of motivation from the patient or family to comply with these


regimens excludes the patient as a surgical candidate
Non-operative treatment

• Passive manipulation: 5
minutes, 20 times a day to
take advantage of the creep
phenomenon,

• The stretching force should


be maintained for a prolonged
time.

Rhee SH et al. Effect of Passive Stretching on Simple Camptodactyly in Children Younger Than Three Years of Age. J
Hand Surg 2010;35A:1768–1773
Non-operative treatment
Before TTT
After

• Only passive manipulation

• 61 digits in 22 patients Mild

• < 3 years of age: long finger Moderate


in 27 cases, little finger in 15
cases, ring finger in 14 cases,
and the index finger in 5 Severe
cases.
0 17,5 35 52,5 70

Rhee SH et al. Effect of Passive Stretching on Simple Camptodactyly in Children Younger Than Three Years of Age. J
Hand Surg 2010;35A:1768–1773
Non-operative treatment may
improve bone remodeling

• 48 fingers in 20 patients

• Statistically significant
radiological parameters
improvement

• PIP contracture improved


from 34° +/- 13° to 6° +/- 7°

Hong SW et al. Radiographic Remodeling of the Proximal Phalangeal Head Using a Stretching Exercise in Patients With
Camptodactyly . J Hand Surg Am. 2019 (in Press)
Surgery : Contra-indications ?

• Non-motivated/compliant patient

• No functional impairement

• Contracture less than 30-60°

• Long-standing articular deformity ?


Operative treatment
• Skin tightness ➠ Zplasty, rotational
flaps, FTSG,…

• As all different • Lumbrical insertion ➠ excision +/- re-


anatomical structures insertion onto the extensor mechanism
have been involved, so • FDS ➠ FDS tenotomy +/- transfer
techniques have been
onto the extensor mechanism
designed to treat the
suspect anatomical • PIP contracture ➠ volar plate release
anomaly +/- temporary K-wire

• Extensor weakness ➠ FDS transfer,


Lasso,…or postop splinting
Limited expected outcomes
• In 18 patients, improvement in 35%, no difference in 30%, and worse results in
35% (Engber). Worse results associated with phalangeal joint recontouring

• 0 excellent, 7 good, 6 fair, and 25 poor results (Siegert). 50% of patients


considered worsened by surgery. Results deteriorate with longer follow-up.

• With FDS sectioning without transfer ➠ Patients improved in active extension


but noted a loss of flexion (Ogino), ➠ no improvement in 8 digits, but
improvement if transfer was done in 12 cases (Koman) .

• In 35 cases of isolated small finger camptodactyly, only 50% had less than 15°
flexion deformity with 33% unable to regain full flexion, average dpc1.8 cm (Mc
Farlane).

Engber WD, Flatt AE. Camptodactyly: an analysis of sixty-six patients and twenty-four operations. J Hand Surg Am 1977;2(3):216–24.
Siegert JJ, Cooney WP, Dobyns JH. Management of simple camptodactyly. J Hand Surg Br 1990;15(2): 181–9.
Ogino T, Kato H. Operative findings in camptodactyly of the little finger. J Hand Surg Br 1992;17(6): 661–4.
McFarlane RM, Classen DA, Porte AM, et al. The anatomy and treatment of camptodactyly of the small finger. J Hand Surg Am 1992;17(1):35–44.
Koman LA, Toby EB, Poehling GG. Congenital flexion deformities of the proximal interphalangeal joint in children: a subgroup of camptodactyly. J
Hand Surg Am 1990;15(4):582–6.
Operative treatment

• Adverse results such as loss of flexion and scar


formation have been commonly reported
• « Surgical correction does not improve overall arc of
motion but places the existing arc of motion in a more
functional, hygienic position for improved large-object
grasp ».

Evans BT, Waters PM, Bae DS. Early results of surgical management of camptodactyly. J Pediatr Orthop 2017;37(5):
317–20.
Stepwise approach
• 83% good to excellent results (Smith)

• Radiographic improvement in 15/16 patients, a


decrease deformity from 62° to 4° post, 83% had
full active extension (Netscher)

• Foucher improved his results from 55% to 86%


using a stepwise approach
Smith PJ, Grobbelaar AO. Camptodactyly: a unifying theory and approach to surgical treatment. J Hand Surg Am
1998;23(1):14–9.
Netscher DT, Hamilton KL, Paz L. Soft-tissue surgery for camptodactyly corrects skeletal changes. Plast Reconstr Surg
2015;136(5):1028–35.
Foucher G, Lorea P, Khouri RK, et al. Camptodactyly as a spectrum of congenital deficiencies: a treatment algorithm
based on clinical examination. Plast Reconstr Surg 2006;117(6): 1897–905. 

1.Skin Stepwise approach:
2.Subcutaneous and fascia Goldfarb proposal
3.Lumbrical and FDS

4.FDS tenotomy
Step 1 to 4, and step 9
5.Release of PIP joint contracture always performed
6.Correction of PIP extension lag
Step 5 to 8 are
7.PIP joint pinning
performed according to
8.Terminal tenotomy prep examination and
9.Release of the tourniquet intraoperative findings
Yannascoli SM, Goldfarb CA. Treating Congenital Proximal Interphalangeal Joint Contracture. Hand Clin 34 (2018) 237–249
Wall LB, Ezaki M, Goldfarb CA. Camptodactyly Treatment for the Lesser Digits. J Hand Surg Am. 2018;43(9):874.e1-e4.
Skin and subcutaneous release

• Z-plasty may be insufficient


in severe deformities and a
flap should be preferred
Skin and subcutaneous release

• Then release subcutaneous


tissues and fascia
Assessment of lumbricals and FDS anatomy

• Trace lumbricals distally and


remove any abnormal
insertion

Kamnerdnakta S, Brown M, Chung KC. Camptodactyly Correction. Operative Techniques in Hand and Wrist Surgery,
Chund KC (eds). 2018. pp842-849
Assessment of lumbricals and FDS anatomy

• FDS division at Chiasma camper (if FDP has enough strength


preop)

Kamnerdnakta S, Brown M, Chung KC. Camptodactyly Correction. Operative Techniques in Hand and Wrist Surgery,
Chund KC (eds). 2018. pp842-849
Release of PIP contracture

• Done if no full extension is


made possible preoperatively
with wrist and MP flexion

• Cave !: preserve the arterial


arch (Edwards) for the
vincula and the
vascularisation of the PIP +++
Correction of PIP extension lag
• If preop Bouvier maneuver shows
a mild extension lag (<60°), the
patient receives a postoperative
splint for 4 weeks.

• If the preoperative Bouvier


maneuver shows a severe extension
lag (>70°), a tendon transfer using
the released FDS to the extensor
mechanism is performed.

• A K-wire is inserted for 7-10 days


to maintain the correction
Tendon transfer

• Few authors recommend it actually

• McFarlane used the FDS IV

• Gupta suggested the use of EIP

Gupta A, Burke FD. Correction of camptodactyly. J Hand Surg 1990;15B:168-170.


Release of the tourniquet

• Release tourniquet before the


end of the procedure to assess
digit perfusion

• Sometimes the neurovascular


bundles will not allow full
extension
Postop regimen
• Permanent splint except for hourly exercices:

• Active and passive MCP joint range of motion.

• Active PIP joint extension with MCP held in flexion.

• Ranges of motion for the PIP joint and DIP joint are initiated via proximal and
middle phalanx blocking exercises.

• Passive PIP joint flexion is avoided if an extension transfer has been performed.

• Four weeks postoperative : start to remove the splint

• Six weeks postoperative : passive flexion of the PIP joint for patients with tendon
transfer. A dynamic finger extension splint may be initiated. The static extension splint
is now worn only at night.

• Twelve weeks postoperative: splint are discontinued


Netscher DT, Staines KG, Hamilton KL. Severe camptodactyly: A systematic surgeon and therapist collaboration
Journal of Hand Therapy 28 (2015) 167-175
Other techniques: Anterior
tenoarthrolysis

• Glicenstein performed a
anterior release of all
structures from the bone. The
principles are to re-
equilibrate flexors and
extensors.

• 30 fingers were improved, 7


identical and 2 aggravated

Glicenstein J, Haddad R, Guéro S. Traitement chirurgical des camptodactylies. Ann Chir Main 1995;14(6):264-271.
Io = splint, infantile
IIa: surgery, adolescent, supple
IIb: surgery, late presentation, stiff

• The stiffer the finger, the


better the results of surgery ?

Goffin D et al. Camptodactylie. Classification et résultats thérapeutiques. Ann Chir Main 1994; 13(1): 20-25
Radiological changes with
surgery ?

• Netscher et al reported recovery of volar subluxation of the


middle phalanx and angulation of the proximal phalanx.

Netscher DT, Hamilton KL, Paz L. Soft-tissue surgery for camptodactyly corrects skeletal changes. Plast Reconstr Surg.
2015;136(5):1028-1035.
Conclusion
• Camptodactyly is a clinical sign, not a symptom

• Most patient are no or little impairment

• Non surgical treatment has proven effective but parents’


adhesion to the treatment plan is the key to success.

• Surgery can improve patients but will not give a normal finger,
and can sometimes aggravate the symptoms

• If surgery is performed, a stepwise approach seems logical as not


a single anomaly can be expected to be the cause of the deformity

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