Abstract
With appropriate surgery prior to 8 wks of age, over 70% of patients with BA become jaundice-free & 90% survive beyond 10yrs. of age1. To assess the frequency of significant congenital anomalies outside the hepatobiliary system & their effect on management, we have retrospectively analysed the case records of 237 cases seen between June '70 & June '85. Major anomalies occurred in 47 (20%) 27 having more than one, 16 had one or more cardiac anomalies (Pulmonary stenosis 7, VSD 5, ASD 3, PDAS, others 7) requiring specific investigation which sometimes delayed biliary surgery. Operative management was complicated in 3 with intestinal atresia re-requiring prior surgery & a further 16 had intestinal malrotation. 17 (7%) had polysplenia & 2 asplenia. In 10 of these the portal vein was pre-duodenal. 9 had abdominal situs inversus making dissection & identification of structures in the portahepatis difficult. In the 171 cases operated on by one surgeon, age @ surgery rather than the presence or absence of anomalies was crucial in obtaining a jaundice-free state. This was achieved in 73% of those operated on by 8weeks of age, as opposed to 27% with later surgery. 8 of 31 (26%) with anomalies & 50 of 140 (33%) without are jaundice free. With more than 70% having surgery after the optimum age, earlier recognition & referral of suspected cases is essential.
1. Ohi R et al. World J.Surg. 9, 285-293 (1985)
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Er, H., Tr, S. & Ap, M. CONGENITAL ANOMALIES IN EXTRAHEPATIC BILIARY ATRESIA (BA): AN ADDITIONAL REASON FOR EARLY RECOGNITION AND REFERRAL. Pediatr Res 20, 694 (1986). https://doi.org/10.1203/00006450-198607000-00052
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DOI: https://doi.org/10.1203/00006450-198607000-00052