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TIMING OF FIRST ESOPHAGEAL VARICEAL HEMORRHAGE IN CHILDREN WITH BILIARY ATRESIA SPLENIC MALFORMATION SYNDROME

OR Guttman, SC Ling, EA Roberts, VL Ng
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario

BACKGROUND: Biliary atresia (BA) is the leading indication for liver transplantation in children. Through progressive scarring and obliteration of the biliary ducts, patients usually progress to chronic liver failure despite palliative Kasai portoenterostomy. Previous reviews of BA patient series have reported median age at the time of first variceal hemorrhage ranging from 3.3 years to 9.8 years.
HYPOTHESIS: Although BA occurs as an isolated anomaly in the majority of infants, a distinct phenotypic subgroup has been described with other associated congenital abnormalities including polysplenia, situs inversus, malrotation, and intra-abdominal vascular anomalies. Although still controversial, it has been proposed that patients with this phenotype have poorer outcomes than those with isolated BA. The aim of this report was to describe two children with biliary atresia splenic malformation syndrome who developed esophageal variceal hemorrhage in infancy, significantly younger than reported means.
CASE: The first infant underwent Kasai portoenterostomy at 11 weeks of age without re-establishment of biliary drainage. She was found to have polysplenia, annular pancreas and non-rotated duodenum as well as interrupted IVC and a hypoplastic portal vein. The Kasai was performed on the second child at 13 weeks of age and initially drained successfully. This child also had polysplenia, situs ambiguous with midline liver and IVC interruption. Both infants were female. Esophageal variceal hemorrhage occurred in the first child at the age of 8.5 months and in the second child at 5.5 months. Both infants underwent sclerotherapy as secondary prophylaxis. The first child has since undergone successful liver transplantation at the age of 12 months and the second child has been listed for transplant.
CONCLUSIONS: These cases suggest an association not previously reported between biliary atresia polysplenia syndrome and earlier onset of esophageal variceal hemorrhage. Review of existing databases of children with BA will be undertaken to determine if this association is confirmed.

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