A Case of Type 1 Neurofibromatosis Associated with Rupture of the Median Sacral Artery

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  • 神経線維腫症1型に合併した正中仙骨動脈破裂の1例

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Abstract

A 38-year-old woman was referred and admitted to our hospital because of anal pain and vertigo. She had been diagnosed as having type 1 neurofibromatosis at the age of 16 years. On admission she presented with hemorrhagic shock and severe pain in the anal and right gluteal region. Her hemoglobin level was 7.9g/dL and computed tomography demonstrated hematomas in the subcutaneous space in lower pelvis and right gluteal region. Blood transfusion did not improve the anemia and pain worsened. Angiography demonstrated rupture of the median sacral artery at the lower sacral level and embolization with coils and gel foam was performed. There was no evidence of any tumor in the pre-sacral space on either CT and MRI, and we thus concluded that the rupture was due to the fragility of the arterial wall associated with type 1 neurofibromatosis.

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