Multiple gastrointestinal atresias with cystic dilatation of the biliary duct

Charles L. Snyder, Matthew Mancini, Alfred P. Kennedy, Raymond A. Amoury

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.

Original languageEnglish (US)
Pages (from-to)211-213
Number of pages3
JournalPediatric Surgery International
Volume16
Issue number3
DOIs
StatePublished - Jan 1 2000
Externally publishedYes

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Dilatation
Survivors
Drainage
Intestinal Atresia
Duodenal Obstruction
Abdominal Muscles
Gastrostomy
Total Parenteral Nutrition
Intestinal Obstruction
Biliary Tract
Jejunum
Duodenum
Rectum
Laparotomy
Signs and Symptoms
Immune System
Transplantation
Newborn Infant
Antral

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Multiple gastrointestinal atresias with cystic dilatation of the biliary duct. / Snyder, Charles L.; Mancini, Matthew; Kennedy, Alfred P.; Amoury, Raymond A.

In: Pediatric Surgery International, Vol. 16, No. 3, 01.01.2000, p. 211-213.

Research output: Contribution to journalArticle

Snyder, Charles L. ; Mancini, Matthew ; Kennedy, Alfred P. ; Amoury, Raymond A. / Multiple gastrointestinal atresias with cystic dilatation of the biliary duct. In: Pediatric Surgery International. 2000 ; Vol. 16, No. 3. pp. 211-213.
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