Department of Transplantation and Hepato-Pancreato-Biliary Surgery, Lahey Hospital and Medical Center, Burlington, Boston, MA, 01805, USA.
Department of Liver Transplantation, Hepatobiliary Pancreatic Surgery, King's Healthcare Partners, King's College Hospital NHS FT, Institute of Liver Studies, Denmark Hill, London, SE5 9RS, UK.
Eur J Pediatr. 2018 Mar;177(3):285-294. doi: 10.1007/s00431-017-3058-x. Epub 2017 Dec 14.
Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension.
This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.
先天性门体静脉分流是一种罕见的发育异常，导致门静脉血流分流到体循环，可分为肝外和肝内分流。它们发生在肝脏和系统静脉血管胚胎发生过程中，并与其他先天性异常相关。它们伴有更高的良性和恶性肝肿瘤风险，如果不治疗，可能导致严重的医疗并发症，包括全身脑病和肺动脉高压。
本文综述了各种类型的先天性门体静脉分流及其解剖、发病机制、症状和治疗时机及选择。
• 呈现了这种罕见先天性异常的自然病史和基本管理。
• 本文是一篇全面的综述；突出了发病机制、临床症状和治疗选择方面的重要主题；并提出了先天性门体静脉分流疾病的管理算法，以便为儿科医生提供清晰的思路。通过讨论缺乏治疗或延迟诊断的后果，努力强调儿童人群的治疗指征，并与成人组联系起来。
