Spontaneous catastrophic cervical hemorrhage in the setting of neurofibromatosis

Neurofibromatosis type 1 (NF1) can manifest in the head and neck in a variety of ways, most commonly presenting as solitary neurofibromas. The management of these lesions require multifaceted consideration of neurovascular involvement and airway compromise. Herein, we describe a 58-year-old male with a history of NF1 and extensive cervical neurofibromas who developed a spontaneous, expanding neck hematoma secondary to his tumors. He was initially stabilized with surgical drainage and endovascular embolization, but then subsequently represented with a second rapidly expanding neck hematoma resulting in acute respiratory distress. Failed intubation due to airway edema necessitated emergent cricothyrotomy and cardiopulmonary resuscitation. Additional embolization was performed but the patient eventually succumbed to global anoxic brain injury. This case highlights the need for proactive multi-disciplinary management with specific attention to respiratory and hemodynamic stabilization in NF1 patients with large tumor burden. As with all neck hematomas, rapid respiratory decompensation may occur, requiring prompt recognition and early intervention. This case underscores the significance of a multidisciplinary approach involving emergency medicine, critical care, anesthesia, interventional radiology, and otolaryngology to recognize and address the potential for airway compromise in the face of a neck hematoma in patients with NF1. • Spontaneous neck hematoma in NF1 can cause sudden airway compromise. • Airway stabilization is critical in NF1 patients with cervical hematoma. • Prophylactic tracheostomy may prevent fatal outcomes in NF1 neck bleeding.