J Korean Med Sci.  2001 Apr;16(2):245-249. 10.3346/jkms.2001.16.2.245.

Multiple intracranial aneurysms associated with branchio-oto-dysplasia

Affiliations
  • 1Department of Neurosurgery, Hanyang University Kuri Hospital, Hanyang University College of Medicine, Kuri, Korea. KCH5142@email.hanyang.ac.kr

Abstract

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.

Keyword

Branchio-Oto-Renal Syndrome; Intracranial Aneurysm; Subarachnoid Hemorrhage

MeSH Terms

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