Surgical considerations for syndactyly of the hand in harlequin ichthyosis: a 5-year experience of sequential surgical treatment in a single patient

Abstract

Harlequin ichthyosis (HI) is a rare congenital disease that primarily affects the skin. Its complications include syndactyly, which can cause severe constriction. Surgical release is thought to be helpful, but the details remain unclear due to a lack of data. We share our 5-year experience of a patient with HI with successful outcomes from five syndactyly release operations involving division, local flap interposition, and full-thickness skin grafts. A visible decrease in the ulnar deviation of the metacarpophalangeal joints and a wider range of motion were noted. There were no complications, except for one case of donor site dehiscence that was resolved after minor revision. The surgical design must conform to the anatomical structure, as it can be easily mistaken due to masking of the skin. A suitable donor site is a flat, non-folding surface, such as the lateral thigh.