Successful pregnancy outcome in a patient with congenital biliary atresia

Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai pr...

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Published inObstetrics and gynecology (New York. 1953) Vol. 80; no. 3 Pt 2; p. 492
Main Authors McMichens, T T, Robichaux, 3rd, A G, Smith, J W
Format Journal Article
LanguageEnglish
Published United States 01.09.1992
Subjects
Adult
Biliary Atresia - epidemiology
Biliary Atresia - surgery
Esophageal and Gastric Varices - etiology
Female
Gastrointestinal Hemorrhage - etiology
Humans
Hypersplenism - etiology
Hypertension, Portal - etiology
Pregnancy
Pregnancy Complications - epidemiology
Pregnancy Complications - etiology
Pregnancy Outcome
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Summary:Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai procedure. Surgical treatment of congenital biliary atresia is associated with substantial perioperative morbidity and mortality, as well as long-term sequelae. Portal hypertension and hypersplenism can complicate the course of up to 50% of patients who survive for more than 2 years. Not surprisingly, few of these patients reach adulthood. We report the case of a woman with congenital biliary atresia surgically corrected with the Kasai operation who subsequently reached adulthood and became pregnant. Despite a prenatal course complicated by portal hypertension, bleeding esophageal varices, and hypersplenism, the woman delivered a healthy infant at 36.5 weeks' gestation.
ISSN:0029-7844