Moyamoya disease complicated by life-threatening epistaxis: first report of a case.

Objective: Subacute haemorrhage is a common emergency in otorhinolaryngology. Rapid evaluation of the aetiology and localization is a precondition for suitable treatment. We demonstrate a rare case of primarily intractable epistaxis associated with occlusion of the circle of Willis (moyamoya disease).

Patient: A 38-year-old man presented with a 24-h history of recurrent epistaxis. Anamnesis revealed long-term anticoagulation after heart valve transplantation and arterial hypertension.

Results: As a result of several re-bleedings after anterior nasal packing, a re-packing was followed by surgical treatment under general anaesthesia. Four days after discharge the patient presented to the intensive care unit with severe re-bleeding. After removal of a temporary Bellocq packing, interdisciplinary treatment was necessary. Emergency angiography revealed advanced moyamoya disease, with occlusion of both internal carotid arteries. The cerebral blood supply was sustained by an excessive collateral network originating from external carotid anastomoses. This complicated the endovascular treatment, which consisted of embolization of the infraorbital and maxillar arteries with liquid material and coils flanked by Bellocq packing. The patient was doing well at follow-up after 12 months.

Conclusion: Epistaxis complicating moyamoya disease is rare, and endovascular treatment is difficult due to the high risk of cerebral embolism. Malformations of the cerebral arteries should be considered in the differential diagnosis of intractable epistaxis.