Epiphrenic diverticulum composed of airway components attributed to a bronchopulmonary-foregut malformation: report of a case.
Bronchopulmonary-foregut malformation (BPFM), defined originally as pulmonary sequestration with or without communication to the esophagus, has been acknowledged to include congenital foregut diverticula. We present herein the case of a 43-year-old woman with a 9-year history of dysphagia, in whom a barium meal examination demonstrated a 2.5-cm epiphrenic diverticulum and several fistulae. A laparotomy was performed and the lower esophagus without communication to the lung was pulled down and resected, followed by an esophagogastrostomy carried out with fundopexy. Since her operation, the patient has been free of symptoms. Histologically, the diverticulum was observed to be lined by stratified squamous cells, but its shape was formed by mural cartilage, smooth muscle cells, and three ciliated-cell cysts. The dysphagia was considered to have been derived from the kinked esophagus created by the rigid diverticulum, being the possible developmental arrest of a supernumerary lung bud. These findings indicate that this case may involve BPFM in the broad sense. Although several cases of bronchogenic cysts located beneath or across the diaphragm have been reported as a subgroup of BPFM, congenital epiphrenic diverticula has rarely been described.