A 64-year-old woman with neurofibromatosis type 1 was admitted because of a sudden hematoma in her right neck and throat. To prevent an airway obstruction, tracheostomy was performed, but bleeding into the trachea persisted and was difficult to stop. Bleeding arose from a vessel lesion in the right parapharyngeal space, passed through the paratracheal space, and finally reached the tracheostomy wound. An arteriovenous fistula (AVF) in the maxillary artery was revealed by angiography. We diagnosed rupture of the varix resulting from venous high pressure caused by the AVF. The patient was treated by vascular interventional radiology (IVR). A large number of platinum coils were inserted and N-butyl-2-cyanoacrylate (NBCA) was embolized in the AVF and varix. Although innovative vascular IVR was better than surgery to treat AVF of the head and neck lesion, some problems were encountered. The cost for the provided medical services was high, and NBCA was not approved by the pharmaceutical affairs law in Japan.

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