Key result
In two cases of massive bleeding caused by spontaneous arterial rupture in patients with von Recklinghausen's disease, one patient was saved by prompt surgical hemostasis while the other died of hemorrhagic shock despite endovascular treatment.
Why the study?
Neurofibromatosis type 1 (von Recklinghausen disease) is associated with vascular lesions, and reports of spontaneous arterial rupture causing massive bleeding are presented to describe management of this complication.
Case Report (n=2)
No
Spontaneous arterial rupture in patients with von Recklinghausen's disease can cause fatal massive bleeding, requiring prompt surgical or endovascular intervention.
Surgical hemostasis may be lifesaving in NF1 arterial rupture; leaves open optimal endovascular role in this rare entity.
神経線維腫症1型（von Recklinghausen disease）は常染色体優性の遺伝病で，血管病変を合併することが知られている．今回，動脈の破綻が原因で大量出血をきたした2例を経験したので報告する．症例1は57歳の女性で，右血胸の診断で緊急入院となり開胸手術を施行した．第1肋間に存在する隆起性病変が出血源で，無事止血し救命した．症例2は55歳の女性で，左腰動脈からの出血性ショックで入院後心肺停止状態となり，一旦蘇生し血管内治療を行ったが死亡した．2例ともvon Recklinghausen diseaseの診断歴があった．本疾患における動脈破裂は，止血が難しく大量出血につながるケースもあり，早急かつ適切な外科的対応が必要である．
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Miyazaki et al. (2020) conducted a case report in von Recklinghausen's disease with spontaneous arterial rupture (n=2). Surgical intervention and endovascular treatment was evaluated on Survival. In two cases of massive bleeding caused by spontaneous arterial rupture in patients with von Recklinghausen's disease, one patient was saved by prompt surgical hemostasis while the other died of hemorrhagic shock despite endovascular treatment.
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