Intracranial vascular anomalies in patients with morning glory disk anomaly.

PURPOSE: An association between morning glory disk anomaly (MGDA) and intracranial vascular anomalies including Moyamoya disease has been recognized. We evaluated a series of patients with MGDA to ascertain the frequency of cerebrovascular anomalies.

DESIGN

Retrospective observational case series.

METHODS

We reviewed the neurologic histories and neuroimaging studies of twenty patients with MGDA at two institutions between 1982 and 2004. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain was performed on all patients who had not undergone neuroimaging. MRI/MRA studies done for 40 pediatric patients without MGDA were also evaluated for cerebrovascular anomalies. The prevalence of anomalies in the two groups was compared by Fisher exact test.

RESULTS

Nine of 20 patients (45%) with MGDA had cerebrovascular anomalies. Anomalies ranged from agenesis of the A1 segment of the anterior cerebral artery to bilateral stenosis of the internal carotid arteries with moyamoya disease. Three patients underwent revascularization procedures. Ten of 40 patients (25%) in the control group had any intracranial vascular anomaly, whereas only two of 40 (5%) had an abnormality of the anterior circulation, the most common finding in the MGDA group.

CONCLUSION

We recommend that all patients with MGDA undergo MRI/MRA or computerized tomographic angiography to detect vascular and structural brain anomalies. It may be unclear whether cerebrovascular anomalies represent isolated congenital anomalies or findings of progressive occlusive cerebrovascular disease. Follow-up imaging should be considered in patients with cerebrovascular anomalies and is clearly indicated if neurologic signs or symptoms are present.