SUCCESSFUL SURGICAL MANAGEMENT AND LONG-TERM FOLLOW-UP OF EPIDERMOLYSIS BULLOSA

Background. Esophageal stenosis and hand deformity are serious complications of recessive dystrophic epidermolysis bullosa that influence the prognosis of patients. To control such complications we have used surgical treatments with favorable results. Additional objectives were to summarize the results of long-term follow-up. Methods. Six patients with esophageal stenosis and nine patients (13 hands) with hand deformity (contracted fingers, mitten-like deformity) were treated surgically after anemia and malnutrition were corrected by intravenous iron infusion, high-energy diet, and blood transfusion. As intubation was contraindicated, topical anesthesia was used (eg., lidocaine spray to the nasopharyngeal mucosa for esophageal dilatation and brachial block, ketamine drip, and nitrous oxide inhalation for reconstruction of contracted fingers). We established a new method for esophageal dilatation using a microvasive rigiflex balloon catheter. This catheter was advanced to the stenotic area under radiography and then expanded by injecting contrast medium into the balloon. For reconstruction of hand deformities, the epidermal glove was initially peeled off, and then the combined digits separated carefully by hand, if the release of the contracture was not sufficient, a skin incision was made avoiding injury to nerves and blood vessels. Any skin defects that appeared after the release of the contracture were covered with skin grafts taken from the abdominal wall. K-wire fixation was used to maintain the extended position of the fingers. Three weeks after the operation, the K-wires were removed and rehabilitation was commenced. Results. The esophageal stenosis was successfully dilated with the balloon catheter; in all six cases the dysphagia was relieved immediately. There was no recurrence in any of the patients on long-term follow-up. After reconstruction of the hand, daily activity improved in 12 of the 13 hands. The remaining hand was difficult to reconstruct due to severe mutilation and bone deformity. During follow-up, 6 of the 12 hands maintained successful reconstruction, whereas the remaining 6 hands showed slight to moderate recontraction of the fingers. Conclusion. Esophageal dilatation with a balloon catheter is safer and has fewer side effects compared to other surgical procedures. This method can provide favorable results and can be carried out repeatedly in a short time. Daily and social activities of patients can be improved upon reconstruction of hand deformities.