Introduction: Dissecting aneurysms of the recurrent artery of Heubner (RAH) have not been previously observed. Likewise, intracranial dissections in the setting of osteogenesis imperfecta (OI) are extremely rare.
Methods: Case Report, Surgical Video, Literature Review.
Results: A 50-year old woman with OI, presented with a devastating subarachnoid and intraventricular hemorrhage, secondary to rupture of a dissecting aneurysm of the RAH. Surgical clip reconstruction of the RAH was attempted, but failed to preserve its patency, as demonstrated by intraoperative angiography. However, intraoperative motor evoked potentials remained normal, thus clips were not repositioned. Post-operatively, the patient had only limited infarction of the head of the caudate nucleus, without clinical consequences. Unfortunately, she ultimately died several days later, secondary to severe refractory cerebral vasospasm.
Conclusions: Although rare, intracranial dissecting aneurysms can occur in the setting of OI and may have a predilection to smaller and more distal arteries. Management of such aneurysms can be quite challenging, as preservation of the parent vessel is often impossible. Intraoperative angiography and neurophysiological monitoring may provide very useful feedback in this setting.
Patient Care: Improved understanding of the genetic aspects, angiographic appearance, and surgical nuances of intracranial dissecting aneurysms
Learning Objectives: By the conclusion of this session, participants should be able to:
1. Recognize the rare association of OI with intracranial dissecting aneurysms and SAH.
2. Recognize that such dissecting aneurysms in OI may occur in atypical locations, such as the recurrent artery of Heubner.
3. Understand the technical challenges when attempting to secure such highly unstable lesions, while preserving flow in the parent vessel.
4. Understand the importance of intraoperative angiography and neurophysiological monitoring in these cases.