Esophageal replacement in two siblings with XXXX .

@entity1 @entity834 @entity2318 @entity3986 @entity4240 @entity2640 @entity3685 @entity74 @entity1217 @entity850 @entity130
@entity4240 is an @entity74 of squamous epithelium that results in @entity3986 of the skin after minor @entity130 . Although severe @entity2318 of the skin is the most common and obvious complication of the disease, esophageal @entity2318 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 @entity834 , which sealed spontaneously during a period of parenteral nutrition and @entity3685 . Both subsequently developed cervical @entity850 . These were successfully managed by dilatations, and both @entity1 are now tolerating a solid diet. Neither @entity1 experienced problems from endotracheal intubation. Epithelial loss occurred in the skin adjacent to the surgical incision but healed quickly with topical @entity1217 therapy. This report confirms previous reports that esophageal replacement is well tolerated by @entity1 with @entity2640 and suggests that the reversed gastric tube technique, not previously reported used for this disease, may be preferable to colon esophagoplasty.
