Abstract
BACKGROUND Alagille syndrome (AGS) is an autosomal dominant hereditary disorder characterized by identifiable abnormalities in the liver, heart, face, skeleton, and eyes. Recently, liver transplantation (LT) has been proposed as a therapeutic strategy for patients with AGS complicated by end-stage liver disease, but clinical experience in performing anesthesia in LT for AGS is still scarce. We aimed to summarize our preliminary experience in the anesthetic management of LT for AGS in this study. MATERIAL AND METHODS We reviewed the cases of 11 patients with AGS who underwent LT from September 2017 to April 2019. Preoperative multi-system comorbidities, intraoperative details, and postoperative outcomes were retrospectively collected and summarized. RESULTS Cardiopulmonary abnormalities were common (81.8%) in AGS patients before LT, and the most frequent comorbidity was pulmonary artery stenosis. After careful anesthetic evaluation and perioperative management, all patients survived during the perioperative period without significant cardiovascular complications. However, there was an unexpectedly high prevalence of surgical complications and re-operations in AGS patients compared to biliary atresia recipients (54.5% vs. 22.4%, P=0.031; and 45.5% vs. 15.3%, P=0.028, respectively). CONCLUSIONS Perioperative management of LT for AGS patients can be particularly challenging, requiring a full understanding of the pathophysiology, as well as a careful preoperative evaluation of the multi-system comorbidities. The high prevalence of postoperative surgical complications should be a matter of concern.