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Matthieu P Robert, Nathalie Boddaert, Manoelle Kossorotoff, Diem-Trang Nguyen, Dominique Bremond-Gignac; Vascular abnormalities associated with morning glory disk anomaly are not moyamoya. Invest. Ophthalmol. Vis. Sci. 2019;60(9):3620.
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© ARVO (1962-2015); The Authors (2016-present)
The morning glory disk anomaly (MGDA) is a unilateral, sporadic, complex malformation of the optic disk. Vascular abnormalities have been reported in the ipsilateral carotid territory in children with morning glory disk anomaly and have been diagnosed as moyamoya, a progressive occlusive cerebral arteriopathy involving a progressive stenosis of the skull base vessels.Children with MGDA now all benefit from serial MRI scans with specific vascular sequences, in order to rule out moyamoya, which consequences may be life threatening in the absence of a specific treatment. Our policy so far was to renew MRI every 3 years when initially normal, and at least every other year when abnormal.We wanted to systematically evaluate the cerebral vessels in patients with confirmed MGDA, on magnetic resonance imaging.
Using our rare disease database, all cases of patients with MGDA followed up in our tertiary reference center for rare ocular diseases between 2008 and 2018 were identified. Cerebral MRI (3D T1, axial T2 and FLAIR, coronal T2, diffusion, ASL perfusion and angio-MRI 3D TOF sequences) performed in all patients were systematically reviewed.
Sixteen patients fitted the study inclusion criteria. On initial MRI, cerebral vasculature was considered normal in ten cases, abnormal in six. Serial MRI were available in 12 cases (7 of the normal ones, 5 of the abnormal ones). Abnormalities comprised: bilateral internal carotid tortuosity alone in two cases; bilateral internal carotid tortuosity and ipsilateral internal carotid hypoplasia in one case; ipsilateral venous hypoplasia in one case; bilateral complex severe arterial vasculopathy associating multiple stenosis and dilations in two cases, compatible with a moyamoya appearance. In no case where serial MRI were available was any evolution noticed, in particular in the two former cases, which were followed up over 6 and 13 years, respectively.
Patients with MGDA exhibit various vascular abnormalities in the ipsilateral carotid territory. These abnormalities may be misdiagnosed as moyamoya, however their characteristics are those of stable congenital malformations – cerebral arterial dysplasia – and do not fit the criteria for being qualified as moyamoya.In severe cases, these abnormalities require a specific neurovascular follow-up. In cases where no vascular abnormality is detected, the necessity for further imaging may be questioned.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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