# Abdominal hemorrhage and vascular fragility associated with neurofibromatosis type 1

**Authors:** Haruka Sato, Fumito Okada, Yoshiki Asayama

PMC · DOI: 10.1007/s11604-025-01738-0 · Japanese Journal of Radiology · 2025-01-24

## TL;DR

A 67-year-old woman with neurofibromatosis type 1 experienced recurrent abdominal bleeding due to fragile blood vessels, highlighting the need for careful management in similar cases.

## Contribution

This case report highlights NF-1 vasculopathy as a rare but critical cause of abdominal hemorrhage requiring tailored medical strategies.

## Key findings

- Computed tomography identified a pseudoaneurysm and fragile vessels in a patient with NF-1.
- Emergency surgery confirmed vascular fragility leading to recurrent abdominal bleeding.
- NF-1 vasculopathy is a rare complication that requires careful handling during medical procedures.

## Abstract

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.

## Linked entities

- **Diseases:** neurofibromatosis type 1 (MONDO:0018975)

## Full-text entities

- **Genes:** NF1 (neurofibromin 1) [NCBI Gene 4763] {aka NFNS, VRNF, WSS}
- **Diseases:** vascular fragility (MESH:D005600), vascular abnormalities (MESH:D014652), vascular complication (MESH:D003925), Abdominal hemorrhage (MESH:D000007), bleeding (MESH:D006470), NF-1 vasculopathy (MESH:D009456), pseudoaneurysm of the (MESH:D017541), hemorrhagic ascites (MESH:D001201), malignancies (MESH:D009369), autosomal dominant disorder (MESH:D030342)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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Source: https://tomesphere.com/paper/PMC12125140