Chapter 6: Camptodactyly

Type II:  Failure of Differentiation

Camptodactyly is a genetic autosomal dominant deformity. Camptodactyly presents as a fixed flexion deformity of the proximal interphalangeal joints (PIP). The fifth finger is almost always affected. It can affect multiple joints, but in most cases this is limited to the fifth digit.

Camptodactyly can be caused by: abnormal lumbrical insertion, abnormal Flexor Digitorum Superficialis (FDS) insertion, or an abnormal volar plate. It is painless and patient usually has normal strength and sensation. This condition may or may not cause functional deficits. Camptodactyly can be mistaken for rheumatoid diseases or dupuytren contracture. Early identification is important so that it can be treated early with non-surgical management. If left untreated for several years, the flexed position will lead to a permanent contracture of the joint. There will most likely be tightness of the volar PIP joint capsule, tightness of the FDS and the skin.

Time to Play the Brain Game:

Q) What are two non- operative treatments we can employ for camptodactyly?

A) Passive stretching and orthotic management.

Classifications of Camptodactyly:

Type I:  affects the small finger,  presents in infancy ( 1st year of life)

Type II:  affects the small finger, presents in adolescence ( after 10 years of age)

Type III:  Multiple digits are involved, contractures are severe, presents at birth

Non-Operative management of Camptodactyly:

  • Most effective if PIP joint contracture/deformity is less than 30 degrees
  • Early identification is critical
  • Passive stretching of PIP joint in extension
  • Serial casting the PIP joint in extension is  an option

  • Custom extension splinting to the PIP joint,  Long term extension splinting is often required until skeletal maturity

Time to Play the Brain Game:

Q) Is Clinodactyly the same as Camptodactyly?

A) Clinodactyly refers to a curvature of a finger in the mediolateral plan and it may be radial or ulnar and involve any finger.  It usually refers to radial deviation of the 5th finger at the DIPJ.  Camptodactyly usually                involves the 5th digit and reflects a contracture at the PIPJ. NOTE: Kirners is a deformity of the distal phalanx and is NOT considered a congenital anomaly as it is not observed until approximately the age of 12.  It            is a painless curve of both DIP’s in the 5th digit and is more common in females than males.

 

  • Five examples of custom fabricated splints for PIP joint extension are shown here:

 

Hand based static PIP extension splinting


A simple gutter splint can be very effective for PIP joint extension

Digital based static PIP extension splinting, this design is comfortable for sleeping

Dynamic PIP extension design with elastic tension.

Static Progressive design for PIP extension using fishing line with hook and loop.

Operative options for Camptodactyly:     

First the surgeon must address all abnormal anatomy if it is present,

For example: bony deformities of the PIP joint, shortening of the FDS, tight volar PIP joint structures, or abnormal insertion of lumbrical muscle into the capsule of the MP joint.

Examples of possible surgeries for Camptodactyly:

a. FDS tenotomy is the surgical lengthening of the FDS to correct the flexion deformity

b. Dorsal angulation osteotomy through the neck of the proximal phalanx, this procedure

increases extension at the expense of flexion

c. Arthrodesis is the fusion of the PIP joint in a more functional position

d. Surgical releases of the tight volar soft tissue structures

e. FDS transfer

  • If full PIP extension can be achieved actively with MCP held in flexion, surgeon can transfer the abnormal FDS to the radial lateral band or to the extensor mechanism over the PIP joint.

Case Example:

Mudenda is a 10 year old boy who’s family needs for him to be able to use his hands to help on the farm.

He has Bilateral Camptodactyly with additional deformities.

Right hand shows Camptodactyly limited to small finger with flexion contracture greater than 90 degrees.

Left hand shows multiple digits with Camptodactyly flexion deformities, with small finger being contracted greater than 90 degrees.

Also noted with this case:  Mudenda’s radial collateral ligaments never formed to support his small finger MCP joints to keep them in neutral,  now the small finger extensor tendon is acting as an abductor for both of his hands.  Mudenda has difficulties with gripping.

Left hand also appears to have a rare condition called: Windblown hand (congenital ulnar drift).

Hand Surgeon’s decision for this case was to wait until this boy is about 12 – 15 years old before performing surgical fusions to the small finger PIP joints, placing the PIP joints in a more functional position at about 45-60  degrees. To correct the positioning of his small finger MCP joints, the surgeon will reconstruct the radial collateral ligaments at the MCP joints.  Reconstruction of the MCP joints will be a difficult surgery due to the rotary issues.   Mudenda may require realignment with pinning and reconstruction of his collateral ligaments or a fusion at the MCP joints depending on the issues that arise during surgery.

Time to Play the Brain Game:

Q) What is another term for Freeman-Sheldon Syndrome?

A) It is also known as whistling face syndrome or windblown hand (congenital ulnar drift of the fingers). In this syndrome the fingers flex and ulnarly deviate as if blown by the wind.