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12 岁结节性硬化症男孩胸主动脉腹主动脉瘤的开放性急诊修复。

Open Emergency Repair of a Thoracoabdominal Aortic Aneurysm on a 12-Year-Old Boy With Tuberous Sclerosis.

机构信息

Vascular and Endovascular Surgery Research Group, Fundación CardioInfantil- La Cardio, Bogotá D.C, Colombia.

Vascular and Endovascular Surgery Fellowship, Fundación CardioInfantil- La Cardio, Bogotá D.C, Colombia.

出版信息

Vasc Endovascular Surg. 2024 Jan;58(1):85-89. doi: 10.1177/15385744231184509. Epub 2023 Jun 15.

DOI:10.1177/15385744231184509
PMID:37318445
Abstract

Tuberous sclerosis complex is a neurocutaneous syndrome caused by an autosomal dominant genetic disorder. This condition can lead to the expression of many vascular anomalies especially, in the pediatric population. Likewise, it has been linked with aortic aneurysm development. We report a case of a 12-year-old boy who presented a 97 × 70 mm Crawford type IV thoracoabdominal aortic aneurysm. Satisfactory open surgical repair was performed with an 18-mm multibranched dacron tube graft. Clinical and imaging findings revealed a de novo tuberous sclerosis diagnosis. The patient was discharged uneventfully during a 1-month follow-up.

摘要

结节性硬化症复合征是一种常染色体显性遗传疾病引起的神经皮肤综合征。这种情况可能导致许多血管异常的发生,尤其是在儿科人群中。同样,它也与主动脉瘤的发展有关。我们报告了一例 12 岁男孩,他患有 97×70mm 的 Crawford Ⅳ型胸腹主动脉瘤。使用 18mm 的多分支涤纶管移植物进行了满意的开放性外科修复。临床和影像学检查结果显示为新诊断的结节性硬化症。患者在 1 个月的随访中顺利出院。

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