Radiographic and endoscopic features of a congenital duodenal diaphragm in an adult. A case report and review of the literature.

A congenital diaphragm is an extremely rare cause of duodenal obstruction in adult life and is usually not diagnosed before operation. Halka et al. ' were the first to report the endoscopic features of this deformity, describing a "cervix sign" characterized by a narrow nipple-shaped lumen with circumferential cuffing of the duodenal muco~a at the apex of the bulb giving the appearance of a cervical os. The correct pre-operative diagnosis was also made in the patient reported by Hudson 2 who noted that, in the 11 previously recorded cases, only 1 had been recognized before operation and that a perforated web or diaphragm is probably the only congenital form of intrinsic duodenal obstruction that can remain undiagnosed until adult life. An extensive literature review by Kreig revealed that congenital diaphragms occurred in only 0.8% to 2.5% of all patients with duodenal obstruction or malformation and may be encountered throughout life. The oldest patient described was in the seventh decade, and 29% of patients did not develop symptoms until after 24 years of age. The following adult patient is reported to confirm the characteristic endoscopic and radiographic findings of this condition. The technique used for removal of the diaphragm suggests that subsequent patients might be managed entirely endoscopically with biopsy forceps and electrocautery.