P2.40 Better safe than sorry: Minimizing arterial complications in Alagille syndrome liver transplant recipients

Isabel González-Barba Neira, Spain

Trainee
Department of Pediatric Surgery
Vall d'Hebron University Hospital

Abstract

Better safe than sorry: Minimizing arterial complications in Alagille syndrome liver transplant recipients

Isabel González-Barba Neira1, Jose Andrés Molino 1,2, Jesus Quintero3, Cristina Dopazo2, María Marget Mercadal-Hally3, Cristina Padros 3, Itxarone Izaskun Bilbao2.

1Department of Pediatric Surgery, Vall d'Hebron University Hospital, Barcelona, Spain; 2Department of Hepatobiliary and Pancreatic Surgery and Transplants, Vall d'Hebron University Hospital, Barcelona, Spain; 3Pediatric Hepatology and Liver Transplant Unit, Vall d'Hebron University Hospital, Barcelona, Spain

Introduction: The association between Alagille syndrome (ALG) and arterial anomalies is well established in the literature. Our aim was to analyze the incidence of arterial complications after LT in our ALG series.
Methods: A retrospective analysis of receptor and graft characteristics, postoperative complications, graft and patient survival rates of ALG pediatric liver transplant (pLT) recipients from January 2015-January 2022.
Results: Eleven ALG patients were transplanted (7 female/4 male, mean age and weight of 17.6±18.9 months and 11.5±14.7). 
Primary indication for pLT was end-stage liver disease secondary to progressive cholestasis (8) and intractable pruritus (3). 1 patient had a history of partial internal biliary diversion. 
All patients were screened preoperatively for abdominal arterial anomalies with a CT angiography. One patient presented a celiac trunk stenosis  and another a hepatic artery (HA) stenosis. 
All patients were first-time graft recipients, of which 8 (72.7%) were cadaveric grafts (3 whole-organ, 1 reduced, 4 split) and 3 were living-donor grafts.  
Standard hepatic arterial reconstruction was performed in 8 cases (72.7%). The 4 remaining patients required an aortohepatic conduit. 
With a 68.6±32.4 month follow-up 2 patients presented arterial complications: 1 early hepatic artery thrombosis and 1 stenosis, which proved to be a celiac trunk stenosis that had gone unnoticed at screening. All patients have functioning grafts and are alive. 
Conclusion: Preoperative screening for abdominal arterial anomalies in ALG patients helps establish a tailored surgical plan and assess alternative sources of graft arterialization to minimize arterial complications.

References:

[1] Alagille syndrome
[2] Celiac trunk stenosis
[3] Abdominal arterial anomalies

Presentations by Isabel González-Barba Neira

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