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<p>We report a case of recurrent abdominal bleeding associated with vascular fragility in a 67-year-old woman with neurofibromatosis type 1 (NF-1). Computed tomography (CT) scan revealed hemorrhagic ascites and a pseudoaneurysm of the sigmoid colon artery, which was suspected to be the source of bleeding. Emergency laparotomy confirmed extremely fragile vessels, requiring repeated surgeries for recurrent bleeding. The patient was diagnosed with NF-1 vasculopathy, a rare vascular complication of this autosomal dominant disorder. NF-1, which affects 1 in 3000–5000 individuals, is associated with reduced life expectancy due to malignancies and vascular diseases, including NF-1 vasculopathy. This condition involves structural vascular abnormalities and increased fragility affecting vessels of all sizes. Recognition of this fragility is critical during invasive procedures, such as interventional radiology or surgery, to reduce the risk of bleeding and ensure optimal management. This case highlights the importance of considering NF-1 vasculopathy in patients with abdominal bleeding and adopting tailored strategies to address its challenges.</p>

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Abdominal hemorrhage and vascular fragility associated with neurofibromatosis type 1

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年1月24日

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  • Haruka Sato,
  • Fumito Okada,
  • Yoshiki Asayama

摘要

We report a case of recurrent abdominal bleeding associated with vascular fragility in a 67-year-old woman with neurofibromatosis type 1 (NF-1). Computed tomography (CT) scan revealed hemorrhagic ascites and a pseudoaneurysm of the sigmoid colon artery, which was suspected to be the source of bleeding. Emergency laparotomy confirmed extremely fragile vessels, requiring repeated surgeries for recurrent bleeding. The patient was diagnosed with NF-1 vasculopathy, a rare vascular complication of this autosomal dominant disorder. NF-1, which affects 1 in 3000–5000 individuals, is associated with reduced life expectancy due to malignancies and vascular diseases, including NF-1 vasculopathy. This condition involves structural vascular abnormalities and increased fragility affecting vessels of all sizes. Recognition of this fragility is critical during invasive procedures, such as interventional radiology or surgery, to reduce the risk of bleeding and ensure optimal management. This case highlights the importance of considering NF-1 vasculopathy in patients with abdominal bleeding and adopting tailored strategies to address its challenges.