1. Orthotopic liver transplantation for alagille syndrome
- Author
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Giuliano Torre, Paola Stroppa, Domenico Pinelli, Elena Torri, Marco Spada, Michele Colledan, Alessandro Lucianetti, Daniele Alberti, M. Zambelli, M Guizzetti, A Bertani, M. Giovanelli, V. Corno, G. Maldini, B. Gridelli, Maldini, G, Torri, E, Lucianetti, A, Guizzetti, M, Pinelli, D, Bertani, A, Corno, V, Giovanelli, M, Zambelli, M, Stroppa, P, Alberti, D, Torre, G, Spada, M, Gridelli, B, and Colledan, M
- Subjects
treatment planning ,Cardiac Catheterization ,Time Factors ,hepatic artery ,bile duct obstruction ,graft survival ,Gastroenterology ,Orthotopic Liver Transplantation ,Alagille Syndrome ,Postoperative Complications ,Actuarial Analysis ,Alagille syndrome ,anastomosis dehiscence ,postoperative complication ,surgical drainage ,Child ,conference paper ,intestine necrosi ,hemoperitoneum ,Pediatric ,clinical article ,Incidence (epidemiology) ,abdominal bleeding ,Whole liver ,cholestasi ,lymph node metastasi ,artery thrombosi ,pulmonary artery stenosi ,aorta reconstruction ,ascite ,female ,priority journal ,survival rate ,medicine.medical_specialty ,organ donor ,Orthotopic liver transplantation ,pleura effusion ,surgical infection ,reoperation ,blood vessel occlusion ,percutaneous transluminal angioplasty ,Actuarial survival ,aorta coarctation ,acute graft rejection ,male ,liver graft ,Internal medicine ,death ,heart ventricle septum defect ,liver vein thrombosi ,medicine ,Humans ,cadaver donor ,human ,portal vein thrombosi ,postoperative thrombosi ,treatment failure ,Retrospective Studies ,Transplantation ,business.industry ,recipient ,disease association ,medicine.disease ,infant ,Survival Analysis ,thorax surgery ,Surgery ,Liver Transplantation ,Hepatic artery thrombosis ,adolescent ,Split liver transplantation ,bile duct fistula ,incidence ,surgeon ,stent ,business ,Cadaveric spasm ,radiologist ,liver graft rejection ,Follow-Up Studies - Abstract
Alagille syndrome (AS) is a dominantly inherited, multisystem disorder involving the liver, heart, eyes, face, and skeleton. From October 1997 through July 2004, 260 pediatric orthotopic liver transplantations (OLTx) were performed in 231 patients. This report describes 21 patients of median age 1.95 years (range, 0.7-16.7) who had alagille syndrome. We present the technical features of the OLTx, incidence and type of complications, medical conditions related to the syndrome, need for retransplantation, as well as patient and graft survival rates. A split liver technique was used in 16 patients (76%) who received a left lateral segment (LLS) graft whereas 7 patients (33%) received a whole liver. Only cadaveric donors were used. The major surgical complications requiring reintervention in 11 patients (52%) included biliary problems (19%) and vascular complications (17%). One case of hepatic artery thrombosis required retransplantation. Three recipients (14%) died. All other patients are alive with an actuarial survival rate of 90% at 1 year and 80% at 5 years. The actuarial graft survival rate is 85% at 1 year and 75% at 5 years. Patients with AS, despite the associated cardiovascular anomalies, can be treated successfully by a combined approach between cardiologist, radiologist, cardiothoracic, and liver transplant surgeons. With careful planning and operative management, the results are comparable with those obtained with other more common cholestatic diseases.
- Published
- 2005