Pediatric Liver Transplantation for Alagille Syndrome: Anesthetic Evaluation and Perioperative Management

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/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.

Clinical and Laboratory Characteristics in Children with Alagille Syndrome: Experience of a Single Center

“…The use of facial features in diagnostic criteria is controversial because of subjectivity and differences between observers. 20 In our study, 13 cases had characteristic facial features, including two 2-month-old infants who were treated for repeated skin yellowing. No relevant characteristic manifestations were observed in the disease onset, and the changes in facial appearance gradually became evident over time.…”

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

“…The recruited articles included retrospective studies ( n = 20) [ 5 , 10 , 13 , 15 – 17 , 20 – 23 , 25 , 26 , 28 , 30 , 32 , 36 , 37 , 39 , 41 , 42 ], case reports ( n = 13) [ 3 , 4 , 8 , 9 , 12 , 14 , 18 , 24 , 27 , 29 , 31 , 33 , 35 ], case series ( n = 2) [ 11 , 34 ], medical imaging ( n = 1) [ 19 ], pilot projects ( n = 1) [ 40 ], and a letter to the editor ( n = 1) [ 38 ]. A forest plot is shown in Figure 2 .…”

“…The age at diagnosis of Alagille syndrome and (or) pulmonary artery pathology was 1.4 ±3.1 (range: 0–12.5; median: 0.17) years ( n = 17) [ 4 , 5 , 9 – 12 , 27 , 29 , 33 , 35 , 38 ] for pediatrics and adults, and at 28.57 weeks of gestation for one fetus [ 8 ] and after birth for 3 fetuses [ 36 ]. JAG1 gene mutation was detected in 129 (32.2%) patients [ 3 , 5 , 8 , 25 , 34 , 36 , 39 ] as well as the mother of one of the fetuses [ 8 ].…”

“…Isolated pulmonary stenosis was found in 302 (75.3%) cases [ 3 , 4 , 5 , 8 , 9 , 11 – 37 , 39 – 42 ], while pulmonary stenosis with complex cardiac or vascular abnormalities was found in 86 (21.4%) cases [ 5 , 10 , 11 , 16 , 22 , 25 , 33 , 36 , 38 , 42 ]. Of them, tetralogy of Fallot was present in 71 (82.6%, 71/86) patients.…”

“…Pulmonary artery stenosis was not intervened for 264 patients and 80 (30.3%) of them received liver transplantation [ 12 – 14 , 20 , 21 , 28 , 30 , 39 , 41 ]. The outcomes of these 80 patients were not indicated for 10 of them.…”