The efficacy of surgical shunts to treat severe portal hypertension after a Kasai procedure for biliary atresia.
BACKGROUND
To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure.


METHODS
We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS).


RESULTS
SS was performed at a median age of 5.5 years [2-13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤20 μmol/l in 24 patients at time of SS. Median follow-up was 15 years [1-32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5-22] were alive.


CONCLUSION
Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation.


LEVEL OF EVIDENCE
Type of study: Therapeutic. Level of evidence III.
['Guérin|Florent|F|', 'Charre|Lionel|L|', 'Jasienski|Stéphanie|S|', 'Duché|Mathieu|M|', 'Franchiabella|Stéphanie|S|', 'Bernard|Olivier|O|', 'Jacquemin|Emmanuel|E|', 'Agostini|Hélène|H|', 'Gauthier|Frédéric|F|', 'Branchereau|Sophie|S|']
D000293:Adolescent D001656:Biliary Atresia D002648:Child D002675:Child, Preschool D005260:Female D005500:Follow-Up Studies D006801:Humans D006975:Hypertension, Portal D008111:Liver Function Tests D016031:Liver Transplantation D008297:Male D011170:Portasystemic Shunt, Surgical D011171:Portoenterostomy, Hepatic D011183:Postoperative Complications D012008:Recurrence D015996:Survival Rate D016896:Treatment Outcome
Portal hypertension Kasai Biliary atresia Portosystemic shunt