Sharma Swapnil, Bobhate Prashant R, Sable Shailesh, Kumar Suneed, Yadav Kapildev, Maheshwari Sharad, Amin Saista, Chauhan Ashutosh, Varma Vibha, Kapoor Sorabh, Kumaran Vinay
Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India.
Department of Pediatric Cardiology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India.
Indian J Gastroenterol. 2018 Jul;37(4):359-364. doi: 10.1007/s12664-018-0884-3. Epub 2018 Sep 5.
Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. This is a retrospective analysis of data from nine patients with Abernethy malformation at a single center. This is a referral center for Pediatric Cardiology and for Hepatobiliary and Pancreatic Surgery. The patients presented to the Pulmonary Hypertension Clinic/the Hepatobiliary Surgery Clinic. Out of nine patients, four were male. Type II Abernethy malformation was present in five patients whereas three patients had type I malformation. One of the patients had communication between inferior mesenteric vein and internal iliac vein. Five out of nine patients were erroneously diagnosed as idiopathic primary pulmonary hypertension and were treated with vasodilators. One patient required living donor liver transplant. One patient was managed with surgical shunt closure whereas two patients required transcatheter shunt closure. The rest of the patients were managed conservatively. Abernethy malformation is more common than previously thought and the diagnosis is often missed. There are various management options for Abernethy malformation, which includes surgical or transcatheter shunt closure and liver transplant. Management of Abernethy malformation depends upon type, presentation, and size of shunt.
阿伯内西畸形是一种罕见的先天性异常，其中门静脉和体静脉循环之间存在直接连通。临床表现范围从影像学检查偶然发现的无症状，到疾病的继发性并发症或与相关异常有关的情况。这是对单一中心9例阿伯内西畸形患者的数据进行的回顾性分析。该中心是儿科心脏病学以及肝胆胰外科的转诊中心。这些患者就诊于肺动脉高压门诊/肝胆外科门诊。9例患者中，4例为男性。5例患者为Ⅱ型阿伯内西畸形，3例为Ⅰ型畸形。其中1例患者肠系膜下静脉与髂内静脉之间存在连通。9例患者中有5例被误诊为特发性原发性肺动脉高压，并接受了血管扩张剂治疗。1例患者需要活体供肝移植。1例患者通过手术闭合分流进行治疗，2例患者需要经导管闭合分流。其余患者采用保守治疗。阿伯内西畸形比之前认为的更为常见，且诊断常常被漏诊。阿伯内西畸形有多种治疗选择，包括手术或经导管闭合分流以及肝移植。阿伯内西畸形的治疗取决于类型、表现和分流大小。
