A retrospective review of the outcome of children presenting to Tygerberg Children’s Hospital with biliary atresia

Karangwa, O. R. (2016-03)

Thesis (MMed)--Stellenbosch University, 2016.

Thesis

ENGLISH ABSTRACT : Abstract Background Biliary atresia (BA) is the end result of an inflammatory process leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrhosis. It is a leading cause of end-stage liver disease in children. It accounts for more than 50% of pediatric liver transplantations (LTx). Surgical treatment with Kasai portoenterostomy (PE) has improved the prognosis for patients with BA, even if most eventually need LTx. A key determinant for the post-Kasai PE patient survival is patient age at surgery as well as periand post-operative management. Objectives This study reviews the short and long-term outcome and identifies prognostic factors of childrenwith BA Study design A retrospective descriptive study Methods Folder review of all children born between May 1997 and May 2011 with biliary atresia as extracted from Tygerberg Children Hospital records database. Data collected included demographic details, clinical characteristics, liver biochemistry and liver biopsy resultsandoutcome post Kasai PE. Results Thirty seven patients with confirmed extra hepatic BA (EHBA) , 23 females and 14 males, mean age 89 days (12.7 weeks), range 7 -227 days, were identified. Twenty three patients underwent Kasai PE. Two of these underwent both Kasai PE and cystenterostomy or cholecystojejunostomy. In 15 of 23 patients, a good short term surgical outcome was achieved as supported by the establishment of bile flow,pigmented stools, resolution of jaundice and improvements in liver biochemistry. Recurrent episodes of cholangitis were experienced in 9 cases (39%) during the first 3-12months postoperatively. Seven of these patients had a progressive deterioration in clinical symptoms and liver function. Four patients died during postoperative follow up and 4 others were lost to follow up. Of the 14 patients who were not operated on, 6 died, 3 were lost tofollow up and their outcome is unknown, and 5 were still alive at last date of follow up (one underwent LTx, one is on the inactive waiting list for LTx and 3 have not yet been referred forLTx ). Conclusion Our study demonstrates that of the 23 patients in whom surgery was performed, 15(65.2%) were documented still alive (range 14 months to 14 years) of whom4 are eligible for LTx; 4(17.4%) died during the post-operative period, and 4 (17.4%) were lost to follow up. Of the 23 children operatedon, 13(56.5%) underwent surgery between 60 and 90 days of age (9 were still alive at the last follow up date, 2 were lostto follow up and 2 died),4(17.4%) operated before or at 60 days 2 survived, 1 is lost to follow upand1 died). Of the 6(26.1%) BA patients who underwent Kasai PE after 90 days of life 4 were alive, 1 lost to follow upand1 died). There was no significant difference in outcome for those who underwent early (< 60 days) and late surgery (60 to 90 days). However,timely referral and surgical correction before 90 daysand post-operative antibiotic as well as nutritional therapy were associated with a higher postKasai surgery survival rate.

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