It is a congenital (present at birth) hand deformity where wrist is bent in the direction of the pinky finger. Spectrum of abnormalities varies from hypoplasia of medial (ulnar) digits to absence of ulna.  Hand and wrist bone ( carpus   )are always affected, missing digits, syndactyly, thumb abnormalities.

SYMPTOMS 

Depend on severity of disease

 Mild tilt of the wrist to a severe bending on medial side

 Malformed hand and possibly missing fingers

Medial forearm long bone( ulna ) can also be absent 

 OPERATIVE PROCEDURE 

Based on age, severity and degree of functional deficit, surgery usually performed around 12 months.

Physiotherapy and splint age should start few weeks after the birth 

Release of the fibrous anlage with realignment of the wrist bone and forearm 

Hand function can be improved by separating any joined fingers (syndactyly ) and by deepening the first webs pace.

FAQS

1. At what age surgery should be performed?

Usually surgery is performed at 1 year when thumb function is developing but splint age and physiotherapy should start a few weeks after birth.

2. How many surgeries will be necessary?

It depends on deformity, stage reconstruction (realignment of the wrist bone and forearm, separating any joined fingers (syndactyly ) and by deepening the first webs pace) is required; however few touch up procedures can be required later on.

3. What is the approximate duration of the procedure?

Two to three hours, depending on severity of deformity.

4. What setup is required for the procedure?

A patient is required for the procedure and usually the patient admits for one to two days.

 

Written by :  Dr. Afaq Saleem

Copyrights: Pakistan association of Plastic Surgeons