A Case of Neurofibromatosis Type I Requiring rapid Airway Management due to Ruptured Pseudo-Aneurysm in the Buccal Region

Abstract

Neurofibromatosis type I (NF1) refers to neurofibromatosis associated with the presence of café-au-lait spots, and carries a relatively good prognosis. Pseudo-aneurysm formation in the head and neck region is relatively rare in patients with NF1, but could prove fatal by causing acute airway obstruction. We report a case of NF1 who presented with airway stenosis due to formation of a pseudo-aneurysm in the buccal region.

A 33-year-old man with NF1 presented with acute onset of swelling in the right buccal region. Computed tomography (CT) revealed a contrast-enhancing mass lesion measuring 4.0×4.0×7.5 cm in size in the right masseter muscle. Angiography showed an aneurysm of the maxillary artery, with extravasation of the contrast medium. The patient was admitted to the hospital and endovascular coil embolization of the aneurysm was performed by neurosurgeons. However, the facial swelling began to worsen gradually after about seven hours and bruising was found in the neck region. CT revealed re-bleeding in the right buccal region and tracheal deviation due to acute hemorrhage. Therefore, the patient was admitted to the ICU, intubated and initiated on mechanical ventilation. A repeat angiography showed a pseudo-aneurysm in the transverse facial artery, which was different from the pseudo-aneurysm treated earlier. This aneurysm had multiple feeding arteries, and was treated by coil embolization as well as embolized with N-butyl-2-cyanoacrylate (NBCA). Three days after the embolization, the patient was extubated and the postoperative course was satisfactory. No recurrence of the pseudo-aneurysms has been observed until date, more than half a year after the treatment. In patients with NF1 presenting with rupture of a pseudo-aneurysm in the head and neck region, prompt airway management is essential.