Anomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies. This case report describes the diagnosis and interventional management of a singular abnormality in the portal venous system of an 8-year-old female that led to severe portal hypertension and acute variceal bleeding. Peculiar imaging findings were not pathognomonic for any of the known congenital and acquired portal vein anomalies: absence of a normal extrahepatic portal vein; splenic and mesenteric veins merging into a dilated left gastric vein; presence of an aberrant mesenteric venous collateral with a stenotic connection with the intrahepatic right portal branch; and absence of porto-systemic shunt. The case was successfully managed with percutaneous transhepatic portography and angioplasty. Prompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding.

中文翻译：

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经皮肝穿刺治疗儿童患者门静脉高压症的独特门静脉畸形

门静脉系统异常可以是先天性的或获得性的，后者与自发性血栓形成或医源性改变有关，例如围产期脐静脉导管插入术的并发症。这些情况可以在临床上沉默多年，然后突然出现，导致严重的临床紧急情况。本病例报告描述了一名 8 岁女性门静脉系统单一异常的诊断和介入治疗，该异常导致严重的门静脉高压和急性静脉曲张出血。任何已知的先天性和获得性门静脉异常的特殊影像学表现都不是特征性的：缺乏正常的肝外门静脉；脾静脉和肠系膜静脉合并成扩张的胃左静脉；存在异常的肠系膜静脉侧支，与肝内右门静脉分支狭窄连接；并且没有门体分流术。该病例通过经皮经肝门静脉造影和血管成形术成功治疗。及时的非侵入性成像表征允许了解单一血管异常和微创介入放射学管理解决门静脉高压和静脉曲张出血。