It is a congenital (present at birth) hand deformity where the radius bone in the forearm is missing or underdeveloped, causing the hand to be bent laterally (radially deviated). Soft tissue, bones and underlying muscle (Radius, radial carpus, thumb, tendons, ligaments, muscles, nerves and blood vessels) can all be involved

SYMPTOMS 

Depend on severity of disease

 Small or absent thumb

 Lateral (radial) deviation of the hand 

Short forearm with reduced passive wrist motion

Lateral skin deficiency with relative excess on the medial side

 Flexion contracture and stiffness of the radial digits

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 birth

Surgical procedures are centralization/ radicalization of hand

Reconstruction of the thumb by shortening and rotating the index finger (pollicization) and reconstruction of deficient tendons and ligament (UCL, FPL, EPL etc.)

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 (pollicization and radial structure reconstruction) 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, it depends on thumb 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.

5. Can it be associated with other medical conditions? 

It can be isolated or can be syndromic  

Holt–Orem syndrome … Cardiac septal defects and various upper limb malformations

VACTERL association…Vertebral anomalies, Anal atresia, Cardiac defects, Tracheal anomalies, Esophageal atresia, Renal and radial abnormalities, other Limb abnormalities,

TAR syndrome … Thrombocytopenia, Absent Radius 

Fanconi’s anemia  …bone marrow failure. – Radial deficiency, aplastic anemia.

 

Written by :  Dr. Afaq Saleem

Copyrights: Pakistan association of Plastic Surgeons