A Case of Pseudoaneurysm in Neurofibromatosis Type I / 대한이비인후과학회지

ABSTRACT

Neurofibromatosis type I(NF-1) is a genetic disorder that usually identified clinically by cafeau-lait spots, and cutaneous neurofibromas. Vascular abnormalities are also associated with von Rechlinghausen neurofibromatosis. Whereas renal and gastrointestinal system lesions are common, involvement of the craniocerebral vessels is relatively rare. More than 85% of the reported lesions are of a purely occlusive or stenotic nature, including progressive arterial occlusive disease. Aneurysms are the second most frequently reported vascular abnormality in NF-1. Recent reported cases of extracranial vertebral artery aneurysms and carotid artery aneurysms have been treated by endovascular detachable balloon technique, and yielded good result. The authors experienced a case of pseudoaneurysm in NF-1 patient who complained of sudden-onset neck swelling. That was identified by doppler sonography, and then angiographic embolization was done.