Vascular type Ehlers-Danlos syndrome with intra-abdominal hemorrhage due to ruptured hepatic aneurysm: A case report

IF 1.5 Q2 MEDICINE, GENERAL & INTERNAL

Acute Medicine & Surgery Pub Date : 2024-08-29 DOI:10.1002/ams2.984

Masaou Tanaka, Kentaro Ueda, Takafumi Yonemitsu, Shinobu Tamura, Akira Ikoma, Tetsuo Sonomura, Shigeaki Inoue

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Abstract

Background

Vascular Ehlers-Danlos syndrome has a high mortality rate due to hemorrhagic complications.

Case Presentation

We report a case of vascular-type Ehlers-Danlos syndrome diagnosed due to rupture of multiple celiac aneurysms. The patient was a 25-year-old Japanese man with a history of a sigmoid perforation. He was admitted to a nearby hospital because of abdominal pain. On day 9 of hospitalization, the patient experienced shock. Enhanced abdominal computed tomography revealed a hepatic aneurysm and intra-abdominal bleeding, and the patient was transferred to our hospital. Emergency abdominal angiography revealed multiple aneurysms in the celiac, common, and right hepatic arteries. The right hepatic artery was considered responsible and was embolized. The patient had characteristic physical findings of the syndrome, aiding in confirming the genetic analysis of COL3A1 gene abnormality.

Conclusion

Juvenile-onset colonic perforation and rupture of the celiac arteries are key findings in the suspicion of vascular-type Ehlers-Danlos syndrome.

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血管型埃勒斯-丹洛斯综合征，肝动脉瘤破裂导致腹腔内出血：病例报告

背景血管型埃勒斯-丹洛斯综合征因出血并发症死亡率很高。病例介绍我们报告了一例因多发性腹腔动脉瘤破裂而被诊断为血管型埃勒斯-丹洛斯综合征的病例。患者是一名 25 岁的日本男子，曾有乙状结肠穿孔病史。他因腹痛住进了附近的一家医院。住院第 9 天，患者出现休克。增强腹部计算机断层扫描发现肝动脉瘤和腹腔内出血，患者被转入我院。急诊腹部血管造影显示腹腔动脉、肝总动脉和右肝动脉有多个动脉瘤。右肝动脉被认为是罪魁祸首，已被栓塞。患者具有该综合征的特征性体征，有助于确认 COL3A1 基因异常的遗传分析。结论幼年期发病的结肠穿孔和腹腔动脉破裂是怀疑血管型埃勒斯-丹洛斯综合征的关键发现。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acute Medicine & Surgery MEDICINE, GENERAL & INTERNAL-

自引率

12.50%

发文量

审稿时长

53 weeks