Rare portal hypertension caused by Abernethy malformation (Type IIC): A case report.

IF 1.4 Q3 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING World journal of radiology Pub Date : 2023-08-28 DOI:10.4329/wjr.v15.i8.250
Xin Yao, Yang Liu, Li-Dan Yu, Jian-Ping Qin
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Abstract

Background: Abernethy malformation is a rare congenital vascular malformation with a portosystemic shunt that may clinically manifest as cholestasis, dyspnea, or hepatic encephalopathy, among other conditions. Early diagnosis and classification are very important to further guide treatment. Typically, patients with congenital portosystemic shunts have no characteristics of portal hypertension. Herein, we report an 18-year-old female with prominent portal hypertension that manifested mainly as rupture and bleeding of esophageal varices. Imaging showed a thin main portal vein, no portal vein branches in the liver, and bleeding of the esophageal and gastric varices caused by the collateral circulation upwards from the proximal main portal vein. Patients with Abernethy malformation type I are usually treated with liver transplantation, and patients with type II are treated with shunt occlusion, surgery, or transcatheter coiling. Our patient was treated with endoscopic surgery combined with drug therapy and had no portal hypertension and good hepatic function for 24 mo of follow-up.

Case summary: This case report describes our experience in the diagnosis and treatment of an 18-year-old female with Abernethy malformation type IIC and portal hypertension. This condition was initially diagnosed as cirrhosis combined with portal hypertension. The patient was ultimately diagnosed using liver histology and subsequent imaging, and the treatment was highly effective. To publish this case report, written informed consent was obtained from the patient, including the attached imaging data.

Conclusion: Abernethy malformation type IIC may develop portal hypertension, and traditional nonselective beta-blockers combined with endoscopic treatment can achieve high efficacy.

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罕见的由阿伯内蒂畸形(IIC型)引起的门静脉高压1例。
背景:Abernethy畸形是一种罕见的先天性血管畸形,伴有门静脉系统分流,临床表现为胆汁淤积、呼吸困难或肝性脑病等。早期诊断和分类对进一步指导治疗非常重要。通常,先天性门静脉分流患者没有门静脉高压的特征。在此,我们报告一位18岁的女性,她有明显的门静脉高压,主要表现为食管静脉曲张破裂和出血。影像学显示门静脉主干细,肝脏内无门静脉分支,由门静脉主干近端上行侧枝循环引起食管和胃静脉曲张出血。Abernethy畸形I型患者通常采用肝移植治疗,II型患者采用分流闭塞、手术或经导管盘绕治疗。患者经内镜手术联合药物治疗,随访24个月无门静脉高压症,肝功能良好。病例总结:本病例报告描述了我们对一名18岁女性IIC型阿伯内蒂畸形合并门静脉高压症的诊断和治疗经验。这种情况最初被诊断为肝硬化合并门静脉高压。患者最终通过肝脏组织学和随后的影像学诊断,治疗非常有效。为了发表本病例报告,获得了患者的书面知情同意,包括所附的影像学数据。结论:IIC型Abernethy畸形可发展为门静脉高压症,传统非选择性β受体阻滞剂联合内镜治疗可取得较高疗效。
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来源期刊
World journal of radiology
World journal of radiology RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING-
自引率
8.00%
发文量
35
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