Subarachnoid Hemorrhage Because of Distal Superior Cerebellar Artery Dissection in Neurofibromatosis Type 1

World Neurosurg. 2017 Sep:105:1036.e15-1036.e19. doi: 10.1016/j.wneu.2017.06.052. Epub 2017 Jun 16.

Abstract

Background: Neurofibromatosis type 1 (NF1) is a rare disease with an incidence of 1 in every 3000 births. Numerous studies have focused on the main function of NF1 as a tumor suppressor, whereas few have examined the cerebrovascular abnormalities observed in patients with NF1. It is worth noting that intracranial aneurysms are uncommon in this condition.

Case description: We report a case of NF1 with a dissection of the distal segment of the superior cerebellar artery. A 36-year-old woman presented with a distal superior cerebellar artery (SCA) dissection causing subarachnoid hemorrhage. Subsequently, because of the rich collateral blood flow distal to the dissection, N-butyl cyanoacrylate (NBCA) glue embolization was unsuccessful. Therefore, direct trapping of the artery was necessary. The patient was discharged after an uneventful postoperative period, and has remained without complications.

Conclusions: In the treatment of subarachnoid hemorrhage because of a distal SCA dissection in patients with NF1, NBCA glue embolization may be a safer option than microsurgery or coil embolization, in the acute phase, considering the possible vulnerability of the vessel wall, accessibility, morphology of the lesions, and the risk of developing unpredictable infarcts in the case of parent artery occlusion. However, regular reevaluation of the blood flow is necessary to monitor recurrence, given the rich collateral circulation.

Keywords: Dissection; Neurofibromatosis type 1; Subarachnoid hemorrhage; Superior cerebral artery.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aortic Dissection / complications
  • Aortic Dissection / diagnostic imaging
  • Aortic Dissection / surgery*
  • Cerebellum / blood supply*
  • Cerebellum / diagnostic imaging
  • Cerebellum / surgery*
  • Female
  • Humans
  • Intracranial Aneurysm / complications
  • Intracranial Aneurysm / diagnostic imaging
  • Intracranial Aneurysm / surgery*
  • Neurofibromatosis 1 / complications
  • Neurofibromatosis 1 / diagnostic imaging
  • Neurofibromatosis 1 / surgery*
  • Subarachnoid Hemorrhage / diagnostic imaging
  • Subarachnoid Hemorrhage / etiology
  • Subarachnoid Hemorrhage / surgery*