Esophageal replacement in two siblings with epidermolysis bullosa

Dystrophic epidermolysis bullosa is an inherited disorder of squamous epithelium that results in dystrophic scarring of the skin after minor trauma. Although severe scarring of the skin is the most common and obvious complication of the disease, esophageal scarring with formation of long strictures may also develop. In two affected siblings, such esophageal strictures occurred and progressed in severity to degrees necessitating esophageal replacement. Each received a preoperative period of total parenteral nutrition, which was continued into the postoperative period. In one, an isoperistaltic segment of right colon was placed substernally in a single stage procedure. In the other, a substernal reversed gastric tube was constructed, also in a single stage. This latter procedure was shorter and technically simpler than the colon interposition. The early postoperative courses in both were complicated by cervical anastomotic leaks, which sealed spontaneously during a period of parenteral nutrition and gastrostomy feedings. Both subsequently developed cervical anastomotic strictures. These were successfully managed by dilatations, and both patients are now tolerating a solid diet. Neither patient experienced problems from endotracheal intubation. Epithelial loss occurred in the skin adjacent to the surgical incision but healed quickly with topical silver sulfadiazine therapy. This report confirms previous reports that esophageal replacement is well tolerated by patients with epidermolysis bullosa and suggests that the reversed gastric tube technique, not previously reported used for this disease, may be preferable to colon esophagoplasty.