MULTIPLE GASTROINTESTINAL ATRESIAS, WITH INTRALUMINAL CALCIFICATIONS AND CYSTIC DILATATION OF BILE-DUCTS - NEWLY RECOGNIZED ENTITY RESEMBLING A STRING OF PEARLS

  • 1 January 1976
    • journal article
    • research article
    • Vol. 57  (2) , 268-271
Abstract
The siblings reported here represent a new and complex congenital 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 the 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.

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