Spontaneous intracranial dissections are a rare cause of subarachnoid hemorrhage (SAH), accounting for 2% to 3% of cases. 1,2 Dissecting aneurysms most commonly affect the large proximal intracranial vessels, although small distal cerebral vessels may rarely be involved. [1][2][3][4] However, to the best of our knowledge, dissecting aneurysms of the recurrent artery of Heubner (RAH), a large named perforator arising from the anterior cerebral artery (ACA), have not been previously observed. We present an exceptional case of ruptured dissecting aneurysm of the RAH in a patient with osteogenesis imperfecta (OI), an inherited disorder of collagen synthesis.
CASE REPORTA 50-year-old woman was transferred to us for evaluation and management of spontaneous SAH and intraventricular hemorrhage. She had been found unresponsive by her family and was initially seen in an outside hospital, where she was found to have a Glasgow coma score of 5 (decorticate posturing) and was thus intubated. Head computed tomography (CT) scan revealed diffuse cisternal SAH predominating in the anterior interhemispheric fissure and tetraventricular hemorrhage with hydrocephalus ( Figure 1A,B). A right frontal external ventricular drain was placed emergently, revealing an opening pressure of 20 cm of water. Following cerebrospinal fluid diversion, her neurological examination improved slightly, measured by her withdrawing all four extremities to pain.CT angiography was obtained, demonstrating a small fusiform aneurysmal dilation of the well-developed left RAH ( Figure 1C). Cerebral angiography confirmed the presence of a 4 mm × 1.5 mm fusiform aneurysm arising from the proximal portion of the left RAH overlying the A1 segment of the left ACA ( Figure 1D). The A1 vessel was mildly deformed, suggesting that the aneurysm was actually larger in size and had a thrombosed, nonangiographically visible portion ( Figure 1D). Given the "pearl-on-string" appearance of the aneurysm and its atypical location, a dissecting nature was strongly suspected. Moreover, there was angiographic evidence of an old healed dissection of the right internal carotid artery, just proximal to its entrance into the carotid canal, and occlusion of the proximal right vertebral artery at its V1-V2 junction, just proximal to its entrance into the right C6 foramen transversarium, also suggesting an old dissection ( Figure 1E,F). Consideration was given for obtaining a brain magnetic resonance imaging to demonstrate the intramural thrombus and confirm the diagnosis of intracranial dissection. However, given the patient's critical condition and the expectation that magnetic resonance imaging findings would not drastically change our management strategy, this study was not performed in order to avoid delaying definitive treatment.Further questioning of the family revealed that the patient had type I (autosomal dominant) OI, as did roughly 50% of her siblings and children. Interestingly, the patient's mother had died of aneurysmal SAH and one of her maternal aunts had had surgical cli...