Multiple gastrointestinal atresias with cystic dilatation of the biliary duct.
Pediatr Surg Int
; 16(3): 211-3, 2000.
Article
en En
| MEDLINE
| ID: mdl-10786985
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.
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Banco de datos:
MEDLINE
Asunto principal:
Enfermedades de los Conductos Biliares
/
Quistes
/
Atresia Intestinal
Tipo de estudio:
Prognostic_studies
Límite:
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
Pediatr Surg Int
Asunto de la revista:
PEDIATRIA
Año:
2000
Tipo del documento:
Article
País de afiliación:
Estados Unidos