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Intracranial vascular anomalies in patients with morning glory disk anomaly
Phoebe D Lenhart1, Scott R Lambert, Nancy J Newman
1Department of Pediatric Ophthalmology, Emory Eye Center, Atlanta, GA 30322, USA.
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.
Insights
Patients with Morning Glory Disk Anomaly (MGDA) have a high frequency of cerebrovascular anomalies, including Moyamoya disease. Early detection via neuroimaging is recommended to identify these serious vascular and structural brain abnormalities.
Area of Science:
- Ophthalmology
- Neurology
- Radiology
Background:
- Morning Glory Disk Anomaly (MGDA) is a congenital optic disc abnormality.
- A known association exists between MGDA and intracranial vascular anomalies, such as Moyamoya disease.
Purpose of the Study:
- To determine the frequency of cerebrovascular anomalies in patients diagnosed with MGDA.
- To compare the prevalence of intracranial vascular anomalies in MGDA patients versus a control group.
Main Methods:
- Retrospective observational case series involving 20 patients with MGDA.
- Neurologic histories and neuroimaging (MRI/MRA) were reviewed.
- Comparison with 40 pediatric control patients without MGDA.
Main Results:
- 45% of MGDA patients (9 of 20) exhibited cerebrovascular anomalies.
- Anomalies included anterior cerebral artery agenesis and internal carotid artery stenosis with Moyamoya disease.
- The control group showed a lower prevalence of anterior circulation abnormalities (5% vs. 45% in MGDA).
Conclusions:
- Recommend MRI/MRA or CT angiography for all MGDA patients to screen for vascular and structural brain anomalies.
- The etiology of these anomalies (congenital vs. progressive) requires further investigation.
- Follow-up imaging is advised for MGDA patients with cerebrovascular anomalies, especially if neurological symptoms arise.
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