A Case of Neurofibromatosis Type 1 Whose Life Was Saved after Rapid Cervical Swelling and Airway Stenosis Caused by Multiple Arterial Ruptures

Tsutsumiuchi Toshiki, Imoto Yoshimasa, Narita Norihiko, Fujieda Shigeharu

doi:10.2468/jbes.73.21

<p>Neurofibromatosis type 1 (NF-1) is said to be rarely associated with vascular lesions such as arterial stenosis, aneurysm, arteriovenous fistula, or arteriovenous malformation. These vascular lesions, which are thought to be attributable to vascular fragility, cause fatal complications depending on their location and conditions. We experienced a case of ruptures of the thoracoacromial artery and ascending pharyngeal artery associated with NF-1 whose life was saved by emergency tracheostomy and endovascular treatment. As it is rare for arterial bleeding from two places to occur at the same time and the patient's life is saved, here we report our case together with a review of the literature. The case was a 61-year-old woman with NF-1 brought to our hospital due to rapid swelling after mild cervical irritation. She underwent an emergency tracheostomy for airway stenosis, and significant bleeding from the wound was found. Contrast-enhanced CT performed to search for the source of the bleeding revealed a huge hematoma and leakage of contrast medium in the right neck. She was suspected of having had sudden cervical swelling due to an arterial rupture. Subsequently she underwent angiography and transcatheter arterial embolization (TAE) on the bleeding source, and hemostasis was obtained and her life was saved. In NF-1 cases, bleeding from the neck may require urgent airway management, so otolaryngologists and head and neck surgeons should be familiar with these complications.</p>

A Case of Neurofibromatosis Type 1 Whose Life Was Saved after Rapid Cervical Swelling and Airway Stenosis Caused by Multiple Arterial Ruptures

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