Intracranial aneurysms and sickle cell anemia: multiplicity and propensity for the vertebrobasilar territory

We describe a case of sickle cell anemia and multiple intracranial aneurysms and review the English-language-reported cases of sickle cell disease associated with intracranial aneurysms proven angiographically or by autopsy, to assess whether there are associations with aneurysm multiplicity and sites of aneurysm occurrence.

A 28-year-old woman with sickle cell disease and a subarachnoid hemorrhage underwent successful clipping of three intracranial aneurysms.

Among 44 reviewed cases, 57% of patients demonstrated multiple aneurysms, and aneurysms from patients with multiple aneurysms comprised nearly 80% of the total number of aneurysms. There were, on average, three aneurysms per patient for patients with multiple aneurysms. There was a predominance of female patients (female/male ratio, 1.6:1), although there existed no significant differences in age or gender for patients with single or multiple aneurysms. None of the patients with multiple aneurysms was older than 40 years of age at the time of presentation. Patients with multiple aneurysms and sickle cell disease showed a significant difference in the distribution of the aneurysm sites, with a significantly large number occurring in the vertebrobasilar axis. Multiple aneurysms associated with sickle cell disease showed a higher rate of simultaneous occurrence in the posterior and anterior circulation, compared with multiple aneurysms in the general population.

There are strong statistical associations for aneurysm multiplicity and sites of aneurysm occurrence among reported patients with sickle cell disease. Patients with sickle cell anemia and neurological symptoms should undergo magnetic resonance angiography or four-vessel angiography to detect potentially harmful, but neurosurgically treatable, pathological conditions.