Pharyngogastric colonic interposition for total oesophageal occlusion in epidermolysis bullosa

Abstract

Epidermolysis bullosa comprises a group of rare heritable disorders, characterized by blistering of skin and other epithelial lined structures following minor trauma. In the oesophagus, trauma from food boluses leads to bullae, ulceration and scarring, with formation of strictures. Oesophageal strictures are usually managed with balloon dilatation. We describe a case of a 19-year-old woman whose oesophageal stricture did not respond to balloon dilatation. She underwent a substernal colon interposition between the pharynx and stomach. The surgery included cervical, thoracic and abdominal approaches, with involvement of three specialist surgeons. Three months after surgery, the patient reported to be swallowing with little difficulty. Because of the high risk of morbidity and mortality associated with this surgery, we recommended that the patient should be managed in a specialist centre, with availability of intensive care facilities and the possibility of providing a multidisciplinary approach during surgery.