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Angiogram. A: Left common carotid angiogram left anterior oblique (LAO) view showing a huge aneurysm at the extracranial internal carotid artery (ICA). B: Capillary phase of Fig. 3A showing slow flow fo the left ICA without pulsation. C: Left external carotid angiogram (lateral view) showing the collateral flow from left external carotid artery to left ICA. D: Right common carotid angiogram (anteroposterior view) showing the collateral flow from right carotid artery to left ICA.
Source publication
Objective: We report a case of neurofibromatosis type 1 with ruptured extracranial giant internal carotid artery (ICA) aneurysm, treated by endovascular trapping using a triple coaxial catheter system.
Case presentation: A 41-year-old female presented with dysphagia, and acutely appeared and enlarged left neck mass. Cerebral angiography, MRI, and c...
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Citations
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.
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.
