Multiple gastrointestinal atresias, with intraluminal calcifications and cystic dilatation of bile ducts: a newly recognized entity resemblin "a string of pearls".

The siblings reported here represent a new and complex congential anomaly. A genetic basis is strongly suggested. The natural preparation of closed loop duodenal obstruction and cystic dilatation of the bile ducts is a unique finding. The hallmark of this new entity--intraluminal calcifications strung out through small and large intestine like pearls--is a striking radiographic feature and, in the proper clinical setting, pathognomonic. This entity has been uniformly lethal. Once the diagnosis is confirmed, no known therapy is useful.