Finding the missing link: Vasospasm in pediatric liver transplant recipients with non-cirrhotic inborn errors of metabolism
Isabel González-Barba Neira1, Jose Andrés Molino 1,2, Jesus Quintero3, María Marget Mercadal-Hally3, Cristina Padros 3, Mauricio Larrarte3, Simone Mameli3, Cristina Dopazo2, Concepción Gómez-Gavara2, Itxarone Izaskun Bilbao2, Ernest Hidalgo2.
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: Inborn errors of metabolism (IEM) are the second most common indication for pediatric liver transplantation (pLT). This cohort of patients is characterized by its high incidence of hepatic artery thrombosis (HAT).
Methods: A retrospective analysis of pLTs (2013-2024) that exhibited intraoperative loss of arterial graft flow secondary to vasospasm.
Results: Five out of 186 pLTs (mean age and weight of 43.4±25.6 months and 15.2±4.4 kg) presented intraoperative loss of arterial flow, all of which were non-cirrhotic IEMs (3 propionic acidemias, 1 MSUD and 1 arginase deficiency). Three grafts were sourced from living donors.
Despite arterial reconstruction flow was not reestablished. In the absence of an intraluminal thrombus and anastomotic strictures the indication for intraoperative angiography was established suspecting a vasospasm.
All patients underwent a percutaneous transluminal angioplasty (PTA). Patient 1 and 2 required no further procedures. Patient 3 and 4 developed HAT: the former required an hybrid thrombectomy and underwent 2 subsequent PTAs with stenting, whilst patient 4 was subjected to 2 additional PTAs with stenting and was retransplanted 29 months later due to ischemic cholangiopathy. Patient 5 required an emergency laparotomy for damage control after the arterial anastomosis was ruptured during the initial PTA, which, despite surgical efforts, resulted in the graft’s loss.
Conclusion: Vasospasm may be the underlying cause of the high incidence of HAT in non-cirrhotic IEMs patients. Further studies are required to typify the spasmodic nature of arterial vessels in this subset of patients to adjust the surgical management and improve postoperative outcomes.
[1] Vasospasm
[2] Arterial complications
[3] Non-cirrhotic inborn errors of metabolism