A strange congenital disease, familial megaduodenus: review of the literature

ABSTRACT

Familial megaduodenum is an extremely rare congenital disease, with few cases described in the literatura, that is caused by chronic duodenal obstruction of functional type. It presents in the form of nonspecific clinical pseudo-obstruction from infancy, which causes a delay in its diagnosis and treatment. Conservative treatments are rarely sufficient in and of themselves to control the disease, making surgery an effective option in selected patients for relieving or avoiding obstruction, as well as improving duodenal emptying and restoring gastrointestinal tract continuity, paying special attention to the duodenal papilla. We present a case treated at the General Surgery and Digestive Apparatus Service of the Hospital of Mérida and a review of the existing literature. (AU)