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无症状性阿伯内西畸形的偶然影像学诊断——两例报告

Incidental radiological diagnosis of asymptomatic Abernethy malformations-two case reports.

作者信息

Shah Ali, Aziz Abdul, Awwad Amir, Ramjas Greg, Higashi Yutaro

机构信息

Trauma and Orthopaedics, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK.

Interventional Radiology, Queen's Medical Centre, Nottingham University Hospitals, Nottingham, UK.

出版信息

BJR Case Rep. 2016 Jul 29;3(1):20150496. doi: 10.1259/bjrcr.20150496. eCollection 2017.

Abstract

The diagnosis of the rare congenital extrahepatic portosystemic shunts is of clinical significance because of the risk of hepatic encephalopathy; liver dysfunction; and associated cardiac, gastrointestinal, vascular, skeletal and genitourinary anomalies. This article describes two varying cases showing the same type of the extrahepatic congenital shunts (Type II). Both the patients were clinically asymptomatic. The first patient initially presented with unprovoked deep venous thrombosis and a staging CT scan was performed to identify any potential underlying malignancy. The second was a polytrauma patient in whom a congenital extrahepatic portosystemic shunt was identified on the CT scan performed to investigate the trauma-related injuries. The first case underwent hepatological investigations, including a fibroscan to rule out liver fibrosis, and was diagnosed as having a Type II congenital malformation, while the second case is under observation post recovery from his traumatic injuries and will be subsequently referred to the hepatology team in the future. Although uncommon, extrahepatic portosystemic shunts can cause significant morbidity and mortality, and all new cases diagnosed radiologically should be further investigated by referring them to a hepatologist.

摘要

罕见的先天性肝外门体分流的诊断具有临床意义,因为存在肝性脑病、肝功能障碍以及相关的心脏、胃肠道、血管、骨骼和泌尿生殖系统异常的风险。本文描述了两例不同的病例,均表现为同一类型的肝外先天性分流(II型)。两名患者临床上均无症状。首例患者最初表现为不明原因的深静脉血栓形成,并进行了分期CT扫描以确定是否存在任何潜在的恶性肿瘤。第二例是一名多发伤患者,在为调查创伤相关损伤而进行的CT扫描中发现了先天性肝外门体分流。首例患者接受了肝脏检查,包括进行肝脏硬度值测定以排除肝纤维化,并被诊断为患有II型先天性畸形,而第二例患者在创伤性损伤恢复后正在接受观察,随后将转诊至肝病科团队。虽然肝外门体分流不常见,但可导致严重的发病率和死亡率,所有经影像学诊断的新病例都应转诊给肝病专家进行进一步检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bbbf/6159285/1984d30c4c9f/bjrcr.20150496.g001.jpg

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