A Case of Spontaneous Neck Bleeding in Neurofibromatosis Type I / 대한이비인후과학회지

ABSTRACT

It is rare to find spontaneous cervical bleedings for which a wide variety of etiologies have been reported. We experienced a case of spontaneous cervical bleeding that caused emergency operation. We present this case with a review of the literature. This case demonstrates a potentially lethal complication in patients with neurofibroma. Neurofibroma is an inherited disease, classified as 'classical (type I, von Recklinghausen, multiple or peripheral neurofibromatosis)', 'central (type II, bilateral acoustic neurofibromatosis)' and other types. The pathognomic features of classical neurofibromatosis are cafe-au-lait spots and neurofibromas. Other clinical features are variably expressed and occurnece of severe hemorrhage is an unusual complication. Conservative management do not stop the hemorrhage. Surgical exploration must be undertaken for cure.