Massive intratumor hemorrhage in facial plexiform neurofibroma.

Abstract

Plexiform neurofibromatosis is a feature of von Recklinghausen's disease. Head and neck lesions may produce varying degrees of cosmetic and functional deformity. However, life-threatening hemorrhage into facial plexiform neurofibromas has not been previously reported. We report two patients with von Recklinghausen's disease who experienced massive hemorrhage into facial neurofibromas, one following a blunt injury and the other without a known initiating event. Conservative management did not stop the hemorrhage into facial neurofibromas in either instance. Surgical exploration was mandated for hemorrhage control and evacuation of blood clots. Hemostasis was attained by a combination of hypotensive anesthesia and chromic catgut suture ligatures. These case reports demonstrate a potentially lethal complication in patients with facial plexiform neurofibroma. Where a competent and experienced interventional neuroradiologist is not available, surgical exploration should be undertaken to control bleeding.