Camptodactyly

Amalia Irsha Adhari (1506668510)

Eldesta Nisa Nabila (1406642233)
Identity of The patient
Name : RAA

Gender : Male

Birth date : 28/10/2015

Age : 3 y.o

Religion : Islam

MRN : 4388198
Chief Complaint
Difficulty in moving the right 4th digit since 1 month before admission
History of present Illness
A patient, 3 y.o came to the policlinic with chief complaint of difficulty in performing finger movement for
daily activities (i.e drawing and writing) since 1 month before admission. The mother said that there is a
“Web like” structure connecting the 4th finger and the right palm which results in a flexion posture of the
right 4th finger during resting condition. The mother noticed the web like structure since the patient was 1
year old, but did not seek for any medical help because she claimed that it did not interfere with the
patient’s daily activity.

There is no redness, swelling, lump, and pain. Sensation of the finger is intact. History of hand trauma or
laceration was denied. No abnormality was found in the left palm.

Patient was referred from Hermina hospital

History of past Illness
● History of surgical procedure was denied
● History of previous hospital admission was denied

Family History
● Similar condition was denied
● Other congenital disease was denied
History of pregnancy
● Preterm (30 week), SC : Very preterm (WHO)
● BW : 1380 g(before incubator), 2100 g (1.5 mo incubator)
● No history of alcohol consumption and smoking
● No history of drug consumption during pregnancy
● History of TORCH

History of immunization
Completed
Physical Examination (General status)
● Vital sign (04/09/2019)
○ General condition : mildly ill
○ Alertness : Compos mentis
○ Blood pressure : 114/82
○ Pulse : 86 x / minute, regular rhythm
○ Temperature : 36.7 C
○ Respiratory rate : 24 x / minute, regular, rhythm, abdominothoracal
○ SaO2 : 98%
○ BW : 19 kg
○ Height : 105 cm
Head Normocephal, symmetric

Neck No palpable lymph node

Eyes No pale conjunctiva, no icteric sclera, Isochor pupil

Nose No deviation, no obstruction in nasal cavity , no

hyperemic concha, no blood or any secretion

Mouth No hyperemic pharynx, symmetric uvula, wet mucosa

of the mouth, no caries, tonsil T2-T2

Cardiac I : Ictus cordis on the 5th intercostal, left

midclavicular
P : Ictus cordis on 5th intercostal, left midclavicular
line,no tapping or waving
P : Heart border within normal range
A : S1, S2 regular, no murmur/gallop
Pulmo I : Symmetric chest, no venectation, no
chest abnormality
P : Symmetric fremitus, symmetric chest movement
P : Sonor in all field
A : Vesicular in all field

Abdomen I : Flat, no venectation

P : Supple, no superficial/deep pain, H/L not palpable
P : No shifting dullness
A : Bowel sound (+)

Extremities CRT < 2, no edema, (for upper limb,refer to next

slide)
Local Status
Look Right palm : Flexion contracture of PIP joint of the 4th digit, minimal ulnar
deviation of PIP joint of the 5th digit
Left palm : minimal ulnar deviation of PIP joint of the 5th digit

Feel Right palm : Normal sensibility, no signs of inflammation

Left palm : Normal sensibility, no sign of inflammation

Move Right palm : ROM of digiti 1-3 within normal range, limited extension of the
4th digit
Left palm : ROM within normal range
Working Diagnosis
Camptodactyly of the left digiti 4

Bilateral Clinodactyly of the 5th digit

Treatment
● Tenotomy of the hand
● application of splint
● Application of external fixation device
● Relaxation of scar or web contracture of the skin
Literature review
Embryology of the upper limb
● Limb bud (4th week)
○ Zone of polarizing (AP axis)
○ nonridge ectoderm (DV axis)
○ Apical ectodermal ridge
(PD/elongation)
● Hand plate formation (8th week)
● Digital separation completed
○ 51 days → apoptosis (distal to
proximal)
● SHH stimulation → anteroposterior bone morphogenic protein (BMP) gradient in the handplate →
formation of interdigital (ID) signalling centers, → phalanx-forming region (PFR) and repress the
interdigital apical ectodermal ridge (AER).
Congenital Hand anomaly: Epidemiology
● Congenital upper limb anomaly: 21 cases per 10.000 live births (11-year total
population study of the Stockholm region of Sweden, 2010)
● Most common congenital anomaly of upper limb: polydactyly, syndactyly,
camptodactyly
● Anomaly of upper limb differentiation (syndactyly, camptodactyly, clinodactyly) : 1 in
750 birth
Classification of congenital hand anomaly
International Federation of Societies for Surgery of the Hand (IFSSH)

● Failure of formation : Thumb hypoplasia

● Failure of differentiation : Syndactyly,
Camptodactyly, Clinodactyly
● Duplication : Polydactyly
● Overgrowth : Macrodactyly
● Undergrowth : Brachydactyly
(short fingers)
● Constriction Band Syndrome : Amniotic Band Syndrome
● Generalized skeletal abnormality : Achondroplasia
Failure of differentiation
1. Syndactyly
2. Camptodactyly
3. Clinodactyly
Camptodactyly
● deviation of the digits in the palmar-dorsal plane → flexion deformity
● Nontraumatic, frequently affects ring and little finger, PIP joint
● Mode of inheritance:
○ could be Autosomal dominant
○ Part of other syndromes i.e Down syndrome, aarskog syndrome
● Anomaly:
○ Lumbrical muscle
○ Flexor digitorium muscle
● Clinical manifestation
○ flexion of the PIP joint, hyperextension of the MCP joint
○ Painless
Clinodactyly
● Congenital deviation to the radial-
ulnar plane of the hand
● Can be inherited in an autosomal
dominant trait → bilateral
● Pathologic: Trapezoidal bone (delta
phalanx)
Diagnosis

Physical Examination
● Camptodactyly is diagnosed when
there is a finger deformity in the
flexion extension plane at the PIP
joint.
● If there is a fixed bone or joint
deformity, splinting or casting will not
correct the contracture.
● One must confirm this finding with
radiographs before initiating
treatment.
● The PIP joint is checked for
extension lag and flexion contracture
with the wrist in neutral position.
● Extension lag is the maximum
extension measurement when
performing active motion testing.
● Flexion contracture is the maximum
extension measurement when
performing passive motion testing.
● A perfectly straight PIP joint is
considered to have 0 degrees of lag
or contracture.
Physical Examination
● Extension lag and flexion contracture
measurements are not mutually
exclusive. A joint may have an
extension lag of 60 degrees, but
passive testing may reveal a joint
correctable to a 30-degree flexion
contracture.
XRay
● Three-view standard hand x-rays
(posteroanterior, noorgaard’s view, and
lateral)
● The involved joint is flexed and often
cannot be straightened. The
phalangeal head may be small and if
the flexion contracture is severe
enough, the phalangeal head may
articulating with and erode the dorsal
cortex of the neck of the proximal
phalanx.
XRay
● A true lateral radiograph of the digit gives an indication about the duration of
congenital camptodactyly. Digits that have been flexed at the PIP joints for
longer periods show the following:
● Flattening of the dorsal condylar surface of the proximal phalanx
● Widened base of the middle phalanx
● Flattening of the palmar surface of the condyle
● Indentation within the surface of the middle phalanx
● Narrowed joint space

Joseph UptonIII MD, Benjamin J. Childers MD, in Plastic Surgery Secrets Plus (Second
Edition), 2010
Treatment
Non Operative

● Stretching the PIP joint should be performed two to three times per day, taking care to deliver
dorsally directed force to the middle phalanx and not the distal phalanx. Otherwise, DIP
hyperextension deformity may result.
● Stretching exercises are supplemented with splinting. Splinting should consist of a forearm-based
splint worn a minimum of 8 hours per night, and extending up to 23 hours per day.
● If the deformity persists after 6 to 12 months of stretching and splinting, consideration is given to
operative management in moderate to severely afflicted individuals (>30° PiP flexion deformity).

Singh V, Haq A, Priyadarshini P, Kumar P. Camptodactyly: An unsolved area of plastic surgery. Archives of Plastic Surgery. 2018;45(4):363-
366.
Operative
● A patient who has functional impairment and a severe flexion deformity with extension lag more than
60 degrees or a flexion contracture more than 30 degrees
● A deformity that persists after 6 to 12 months of stretching and splinting
● Camptodactyly with a flexion contracture less than 30 degrees causes minimal function limitation for
most patients. A patient may be more concerned about the appearance of the flexed finger. Surgery
should be cautioned in these individuals because surgery may lead to a decrease in function due to
recurrent contracture.

Singh V, Haq A, Priyadarshini P, Kumar P. Camptodactyly: An unsolved area of plastic surgery. Archives of Plastic Surgery. 2018;45(4):363-
366.
Operative
● Volar plate tightening procedure
● Tenolysis
● Z-Lenghtening

Singh V, Haq A, Priyadarshini P, Kumar P. Camptodactyly: An unsolved area of plastic surgery. Archives of Plastic Surgery. 2018;45(4):363-
366.
Tenotomy
1. Skin and Facial Contracture Releasing and Dorsolateral
Flap Elevation
2. Released FDS Tendon

The Camper chiasm is divided longitudinally. Both slips of

FDS are divided transversely and cut at a level distal to the
chiasm
Tenotomy
3. Volar plate and collateral ligament
releasing
Tenotomy
3. Volar plate and collateral ligament
releasing
Tenotomy
4. FDS Transfer to Ulnar Lateral Band
When There Is Extension Lag
Tenotomy
5. Dorsolateral Flap Inset and
Incision Closure
Discussion and
Conclussion
● Patient develop a web-like structure in the left 4th digit which progress and
results in difficulty in performing finger movement  diagnosed with
camptodactyly of the left digiti 4 bilateral
● From physical examination : limited extension in the 4th digit of the left hand,
and ulnar deviation of the 5th digit of the right hand
● The patient treated with tenotomy of the hand, application of splint, Application
of external fixation device, and relaxation of scar or web contracture of the skin.