Abstract
INTRODUCTION: Type1 Neurofibromatosis (NF1) is an autosomal dominant disorder with a great variety of clinical features. Vascular manifestations appear in less than 7%, and venous complications are extremely rare. CASE PRESENTATION: We report a case of an enormous cervical hematoma caused by bleeding from the internal jugular vein in a NF1 patient. As the patient refused surgery, natural evolution was marked by spontaneous fistulization and drainage of the mass. DISCUSSION: Several cases of fragility of both the vessel wall and the surrounding tissue are discussed and two jugular vein aneurysms were reported. Management of such cases remains unclear and some reports describe defective hemostatic control at surgery. CONCLUSION: Surgery in NF1 vascular involvement should be considered on a case by case basis especially when the risk of iatrogenesis is high. Surgeons need to be aware of hemorrhagic risks, which could occur because of vessel friability.