Inês Pessanha1, Henrique Alexandrino2,3, Cláudia Piedade1,4, Catarina Cunha1,4, Isabel Gonçalves4, Maria Francelina Lopes1,3,4, Emanuel Furtado4
1Department of Paediatric Surgery, Paediatric Hospital, Centro Hospitalar E Universitário de Coimbra, Coimbra, Portugal
2Department of General Surgery, Centro Hospitalar e Universitário de Coimbra University Hospitals of Coimbra Coimbra Portugal
3Faculty of Medicine, University of Coimbra, Coimbra, Portugal
4Paediatric and Adult Liver Transplantation Unit Centro Hospitalar e Universitário de Coimbra Coimbra Portugal
Tóm tắt
AbstractIntroductionGallbladder agenesis (GA) is a rare congenital condition, occurring in approximately 40/100.000. It is likely due to an embryologic mishap in the development of the gallbladder bud and can be associated with other congenital variations in biliary anatomy. However, the liver likely suffers no functional impairment and can be safely used for transplantation. To the best of our knowledge, this is the first case report describing a pediatric liver transplantation (PLT) using a graft with GA.Case reportA 10‐year‐old boy with methylmalonic aciduria underwent isolated liver transplant with a deceased graft from a donor with no relevant medical or surgical history and normal laboratory tests. During the back‐table liver preparation procedure, no evidence of gallbladder was found, raising the possibility of a GA, confirmed by intraoperative cholangiography. The liver transplantation procedure was uneventful despite the particularly rare combination of biliary tree anatomic distribution found in the cholangiography. At 1 year of follow‐up, there were no clinical, laboratory, or imagological signs of bile leaks or anastomotic site stricture.DiscussionThe present report highlights the importance of the accurate knowledge of the vasculobiliary anatomic variation, particularly in extremely rare cases, such as GA, and in complex hepatobiliary procedures, such as PLT. In our opinion, grafts with GA should not be discarded for transplantation.
