Abstract
BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder, characterized by neurocutaneous lesions. NF1 has a high degree of clinical variability, which can include multiple neoplasia as well as cutaneous, vascular, osseous, and cognitive features. When vascular involvement occurs, NF1 can lead to aneurysms or arteriovenous malformations, which may rupture and cause life-threatening complications. CASE SUMMARY: We present a case of primary subarachnoid hemorrhage, complicated by spontaneous and rapidly progressing hemorrhage from the left subclavian artery resulting in upper airway obstruction and hypoxia in a patient with NF1. Treatment of this patient included surgical airway management, emergency hematoma evacuation, and vascular reconstructive surgery. Close collaboration between radiology, vascular surgery, and anesthesiology was essential to prevent patient's death. CONCLUSIONS: Awareness of rare diseases such as NF1 is essential in critical care settings. Patients presenting with café-au-lait spots or cutaneous neurofibromas are at risk of vascular complications due to vascular fragility. This case of dual bleeding sources and airway obstruction from a neck hematoma underscores the need for interdisciplinary management. The role of proactive vascular screening in critically ill NF1 patients remains uncertain. Future approaches may incorporate advanced imaging and biomarker development to better stratify vascular risk and guide individualized care.