Multiple intracranial aneurysms associated with branchio-oto-dysplasia

J Korean Med Sci. 2001 Apr;16(2):245-9. doi: 10.3346/jkms.2001.16.2.245.


Branchio-oto-dysplasia is characterized by abnormalities of embryonic branchial arch system and deafness inherited as autosomal dominant with variable gene expression. We present a rare case of multiple intracranial aneurysms associated with branchio-oto-dysplasia. A 40-yr-old man with severe headache presented as spontaneous subarachnoid hemorrhage on brain computed tomographic scan. The patient also manifested clinical features of branchio-oto-dysplasia and right hemifacial hypoplasia. Carotid angiogram confirmed an aneurysm in the anterior communicating artery. Intraoperative findings demonstrated multiple aneurysms in the anterior communicating artery and in the left posterior communicating artery, which were clipped successfully. Postoperative course was uneventful. This condition has not been reported previously. We also reviewed literatures to discuss whether the intracranial aneurysm was as a coincidental finding or a part of this malformation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Branchio-Oto-Renal Syndrome / complications*
  • Cerebral Angiography
  • Humans
  • Intracranial Aneurysm / diagnostic imaging
  • Intracranial Aneurysm / etiology*
  • Subarachnoid Hemorrhage / diagnostic imaging
  • Subarachnoid Hemorrhage / etiology
  • Temporal Bone / abnormalities
  • Tomography, X-Ray Computed
  • Zygoma / abnormalities