title: Abdominal Arterial Anomalies in Children With Alagille Syndrome: Surgical Aspects and Outcomes of Liver Transplantation.
abstract: Angiogenic defects secondary to gene mutations of JAG1 and NOTCH2, causing arterial anomalies in Alagille syndrome (AGS), are well described in the literature. The study analyzes the frequency of abdominal arterial anomalies in children with AGS with an emphasis on outcomes following liver transplantation (LT).
  
  
  
  Between 1988 and 2013, 242 children with AGS were treated at our institution. We performed a retrospective analysis of 55 who underwent LT during the study period. Preoperative abdominal arterial findings, operative reports, arterial reconstruction technique, and early as well as late complications following LT were reviewed specifically focusing on arterial thrombosis.
  
  
  
  Twenty-five patients had preoperative imaging available for analysis. Twelve of these patients showed celiac trunk stenosis (48.0%), 2, a superior mesenteric artery stenosis (8.0%) and one a stenosis of both renal arteries. Twenty patients (36.3%) underwent standard hepatic reconstruction using the native recipient hepatic artery. Thirty-five patients (63.7%) underwent aortic conduit reconstruction (ACR) from the infrarenal aorta using donor arterial conduits. Hepatic artery thrombosis occurred in 9 patients (16.3%). This number was higher in the standard arterial anastomosis group 7/20 (35.0%) than in those with ACR 2/35 (5.7%, P = 0.0079).
  
  
  
  In this series, children with AGS pretransplant have a high prevalence of abdominal arterial anomalies. Preoperative abdominal vascular imaging makes it possible to anticipate whether or not a classical arterial revascularization can be performed or whether an ACR is required.
authors.affiliation: *Department of Pediatric Surgery †Department of Pediatric Radiology ‡Department of Pediatric Hepatology, Hôpitaux Universitaires Paris-Sud, Bicêtre Hospital, AP-HP, Université Paris XI, Le Kremlin-Bicêtre, France.
authors.forename: Jules
authors.initials: J
authors.lastname: Kohaut
authors.forename: Romain
authors.initials: R
authors.lastname: Pommier
authors.forename: Florent
authors.initials: F
authors.lastname: Guerin
authors.forename: Danièle
authors.initials: D
authors.lastname: Pariente
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Jacquemin
authors.forename: Hélène
authors.initials: H
authors.lastname: Martelli
authors.forename: Sophie
authors.initials: S
authors.lastname: Branchereau
mesh_terms: D000293:Adolescent; D016738:Alagille Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000601:surgery; D002445:Celiac Artery / Q000002:abnormalities* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D017538:Mesenteric Artery, Superior / Q000002:abnormalities* / Q000601:surgery; D015995:Prevalence; D012077:Renal Artery / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery