Surgical management of hand deformities in hereditary dystrophic epidermolysis bullosa

摘要

In the period 1996-2001 in the Clinic for Plastic Surgery and Burns of the Military Medical Academy, 18 patients. 12 male and 6 female, with hereditary dystrophic epidermolysis bullosa (HDEB) and hand deformities were surgically treated, to achieve the complete separation of fingers, correction of the thumb adduction contracture and flexion or extension contracture of finger joints. The period of wound healing on flat surfaces after surgery, and the period between two operations was estimated. The most common deformity was the flexion contractures of metacarpophalangeal (MP) joints (45%) and one or both interphalangeal (IP) joints (types A1, A2). In 20% of the hands MP joint was streched with the flexion contracture in distal interphalangeal (DIP) or both IP joints (types B1, B2). In 35% of hands MP joint was in hyperextension with folded proximal interphalangeal (PIP) or both IP joints (C1 i C2). The adduction deformity of the thumb type 1, without the possibility of abduction, was present in 15%, type 2, when the thumb was placed above the palm in 60% and type 3, when the thumb was fused in the palm in 25%. Pseudosyndactyly of the first degree (till PIP joint) was found in 30% of hands, the second degree (till DIP joint) in 25%, and the third degree (the whole finger length) in 45% of hands. Fingers were completely separated and stretched surgically. The period of spontaneous healing was 15 days on the average. EBDC represents great medical and social problem that requires multidisciplinary approach of physicians of various specialties (surgeons, dermatologists, pediatrists, geneticists, nutritionists physiatrists, ophtalmologists, dentists, ENT, as well as specially trained persons and families). The efficient specific systemic therapy aiming to increase the skin resistence to mechanical trauma does not exist yet, and should be developed in the field of gene therapy. The surgical correction of hand deformities, acrylate glove use in the longer post operative period combined with physiotherapy, the active use of hands, the protection of injuries and skin care are the measures which prolong the period between the recurrence of contractures.