Paediatric and adult vascular intracranial complications of sickle-cell disease.

Background: To enumerate possible intracranial vascular sequelae of sickle-cell disease, to identify risk factors and outline management strategies.

Method: Retrospective review of a single unit experience managing vascular intracranial complications of sickle-cell disease from 1995 until 2005. Information such as homozygosity/heterozygosity, duration of disease, disease control as indicated by haematology follow-up, concurrent sickle-cell disease (SCD)-related health problems and neurosurgical management was recorded. The pattern of vascular disease was analysed to reveal possible contributory/risk factors towards development of vascular intracranial complications.

Subjects: All patients presenting with vascular intracranial complications of sickle-cell disease from 1995 to 2005 were evaluated.

Outcome Measures: Classification of vascular intracranial complications into one or more of the following categories: aneurysmal subarachnoid haemorrhage, non-aneurysmal subarachnoid/intraventricular haemorrhage and vasculitis.

Findings: There were ten patients in the study. All symptomatic vascular intracranial complications of SCD requiring neurosurgical intervention were homozygous for SCD. Aneurysms were likely to be multiple. Ruptured aneursyms in SCD were small (average 4 mm). There was a propensity for aneurysms to occur in the posterior circulation, in particular the posterior cerebral artery was frequently involved. Patients with aneurysms and Moyamoya-type vasculitis were likely to have occlusive disease of the internal carotid arteries.

Conclusions: The vascular intracranial complications of sickle-cell disease have an aggressive natural history. Tight control of SCD may reduce the possibility of complications. Complications that arise should be managed in the context of the disease entity rather than in isolation. Consideration should be given to bypass procedures, parent vessel ligations and revascularization techniques. Transcranial Doppler may be used to identify SCD patients with cerebrovascular occlusive disease, who may have increased risk of aneurysmal rupture.