Postoperative Hand Treatment in Children with Recessive Dystrophic Epidermolysis Bullosa

Abstract 

The purpose of this study is to give an overview of the postoperative hand treatment options in children with recessive dystrophic epidermolysis bullosa (EB) and to introduce a treatment protocol and discuss the indications and timing. Recessive dystrophic EB is a rare hereditary blistering skin condition, which leads to severe hand deformities. The aim of surgical intervention is to temporarily increase hand function and delay the recurrence of deformation. The aim of postoperative treatment is to maintain optimal range of motion of the wrist, fingers, and thumb and to delay recurrence of deformity to enlarge the possibilities of hand function. Two postoperative treatment programs are described in the literature: a program with static splinting and a program with dynamic splinting. Both splinting programs include exercises. This postoperative treatment program for EB starts with dynamic splinting, followed by static splinting in combination with exercises.