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P6.10 Multi-faceted peri-transplant strategy for biliary atresia candidates for liver transplantation; effect on access to transplantation and outcome

Serena Abbate, Italy



Multi-faceted peri-transplant strategy for biliary atresia candidates for liver transplantation; effect on access to transplantation and outcome

Jean de Ville de Goyet1, Francesco di francesco1, Serena Abbate1, Francesco Cirillo1, Leone Valentina1, Silvia Riva1, Marco Sciveres1.

1Department of Pediatrics, ISMETT IRCCS, Palermo, Italy

Introduction: Biliary atresia patients (BAP) represent the largest group of single-etiology candidates for liver transplantation (LT) as a whole, and the majority of indications for LT under the age of 3 years. Because of the many challenges relating to the young age, small weight, and relative urgency for LT in many of them, BAP infants represent the largest proportion of those who die while waiting for the LT, and after LT. Most LT centres still face the latter situation.  
Method:  Retrospective review of all consecutive BAP assessed and listed for LT during a 5 year period with a holistic approach: – early referral, - optimization of nutritional support, - dual donor strategy (deceased (DD) and living donors (LD)), - prevention of graft technical complications, and – FK monotherapy (basiliximab induction). 
Results:  Starting January 2017, 50 BAP were listed for LT. 41/50 had advanced biliary cirrhosis (polysplenia malformative syndrome in 9/41 cases (22%)), and were aged < 3 years at assessment (21 cases (52%) were assessed before the age of 6 months, and another 17 cases before the age of 12 months). Other main reason for liver replacement (9 cases) was: hepatopulmonary syndrome in 1 case, portal hypertension (with or without minor cholestatic features) in 4 cases, recurrent cholangitis (n=2) or pruritus (n=2). Of these 9, mean age at listing was 35,5 months; 2/9 improved while waiting and were removed from the list. 
All others (41 BAP with cirrhosis/liver failure) were also transplanted; mean waiting time was 79 days (from 9 to 356 days). During waiting time, all these 41 but 3 needed medical management of ascites; nutritional invasive support was added in 22/41 (54%), consisting in enteral nutrition in 12, parenteral nutrition in 5 and combined enteral/parenteral nutrition in 5. The latter 41 LT consisted of 6 left split DD livers and 35 LD left lobe grafts. Mean age, weight and PELD at the day of LT were  11.1 months, 7,9 Kgs and 22, respectively. Biliary complications were 2/41 (1 fistula and 1 anastomostic stricture), both successfully managed; percutaneous angioplasty of portal or hepatic vein anastomosis was performed successfully in 4 and 1 cases, respectively. A meso-Rex bypass was performed in 2 patients (portal vein thrombosis). There was no hepatic arterial thrombosis.

Overall, all 50 patients are currently alive; 2 are not candidates for transplant any more, while 48 have been transplanted and all are well with first and well-functioning grafts (Mean follow-up: 29 months (minimum = 3 months, and Maximum = 55 months).
Conclusion: In literature, early hepatic failure in BAP is associated with high risk of pre- and post-LT morbidity and mortality. Very early referral to LT centre, combined with flexible graft offer (to allow shorter waiting time) and aggressive pre-emptive peri-transplant medical management may allow decreasing, possibly avoiding, pre and post-LT mortality.