Author Affiliations: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania (Drs Rojanaporn, Kaliki, C. L. Shields, and J. A. Shields), and Retina Service, Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand (Dr Rojanaporn).
In 1970, Kindler1 first described the morning glory disc anomaly in 10 patients with unusual congenital anomaly of the optic disc. Morning glory disc anomaly manifests with features of a congenitally enlarged optic disc with a central funnel-shaped excavation and overlying thin glial membrane. The anomalous disc is often surrounded by retinal pigment epithelial hyperplasia and occasionally surrounded by exudation and subretinal fluid. The descriptive name “morning glory” depicts the similarity of the recognizable retinal vascular pattern radially emerging from the optic disc, similar to the arrangement on the morning glory flower. These disc vessels are supernumerous and appear straightened and radially course to the retinal periphery.1 The morning glory disc anomaly can be associated with neurologic abnormalities, so recognition of the ocular finding and appropriate neuroimaging is important.2
Peripheral retinal vascular nonperfusion in children has been recognized with several congenital conditions, such as retinopathy of prematurity, Coats disease, familial exudative vitreoretinopathy, facioscapulohumeral muscular dystrophy, and incontinentia pigmenti.2 To our knowledge, there have been no published reports on peripheral fluorescein angiographic findings in morning glory disc anomaly, perhaps because of the limited availability of fluorescein angiography in children and the difficulty in imaging the peripheral retina. Herein, we report the association of morning glory disc anomaly and peripheral retinal nonperfusion in 4 consecutive patients.
There were 4 patients with morning glory disc anomaly, diagnosed at a mean age of 19 months (median, 11.5 months; range, 4-48 months). In all 4 cases, the morning glory disc anomaly was unilateral. There was no history of prematurity, low birth weight, nonperfusion in the opposite eye, or familial exudative vitreoretinopathy. Brain magnetic resonance imaging was normal in all cases. Demographic and clinical features are listed in Table 1. Retinal vascular features are listed in Table 2.
In all 4 cases, fundus findings, documented with the Retcam camera (Massie Industries) showed peripheral retinal nonperfusion involving 360° in 3 cases and 90° (inferotemporally) in 1 case. The zone of nonperfusion measured mean 7 mm (median, 6.3 mm; range, 6.3-8.3 mm). At the junction between the retinal perfusion and retinal nonperfusion zone, there were brushfire retinal vessels in 3 cases (75%) and looping of retinal vessels in 1 case (25%). There was no fibrous or fibrovascular traction seen clinically, but somehow, retinal vessels appeared to be dragged toward the periphery, resulting in vascular straightening in all 4 cases (100%). No patient showed visible ischemic or neovascular retinal signs (Figure).
Figure. A 13-month-old white boy showing an enlarged optic disc, radially emanating vessels, central glial membrane, surrounding shallow subretinal fluid with 3 radial retinal folds (A and B) and peripheral retinal nonperfusion (C). A 10-month-old Asian girl showing morning glory optic disc anomaly with subretinal exudates and subretinal fluid around the disc (D), radial orientation of the retinal vessels (E), and peripheral retinal nonperfusion (F). A 4-month-old white boy showing morning glory optic disc anomaly with central glial tuft, surrounding retinal pigment epithelial hyperplasia (G), radial orientation of the retinal vessels (H), and peripheral retinal nonperfusion (I).
Morning glory disc anomaly was first described by Kindler.1 Later, it was shown to be associated with retinal, cerebral, and carotid vascular anomalies.2 There are only a few large case series on morning glory disc anomaly and, to our knowledge, there is no previous documentation of peripheral nonperfusion.1- 5 Kindler first reported 10 patients with unusual congenital optic disc anomaly and named it “morning glory syndrome.” He noted white fluffy tissue in the optic disc center (100%), annulus retinal pigment epithelial disturbance around the optic disc (100%), multiple branching and radial vessels from the disc (70%), and retinal detachment (20%).1 Haik et al3 reported clinical features of 30 patients with morning glory disc anomaly, noting central glial tuft (100%) and associated retinal detachment (37%). Harasymowycz et al4 reported clinical features of 20 patients with morning glory disc anomaly and found disc telangiectasia (14%), vascular straightening and sheathing (14%), and retinal detachment (14%). Beyer et al,5 in 8 cases, noted vascular microtelangiectasia (67%), vascular sheathing (70%), vascular stretching (80%), and vascular looping (80%). On fluorescein angiography, arteriolar anastomoses near the optic disc were noted,5 but there was no comment on the peripheral retina. In these 4 case series and additional literature review by Lee and Traboulsi,6 there was no comment on peripheral fundus angiography and peripheral retinal nonperfusion.1- 5 Kim et al7 recently reported a case of unilateral peripapillary staphyloma that showed immature retina at birth in both eyes, but later, retinopathy of prematurity developed only in the eye with peripapillary staphyloma. They commented that retinal vascular development involves migration of astrocytes and a vascular precursor through the optic nerve; therefore, the optic nerve anomaly could affect the retinal vascular development.
Retinal detachment (RD) is the most serious retinal complication of morning glory disc anomaly, with an incidence of 14% to 37%.1- 4 The pathogenesis is speculated to involve a juxtapapillary retinal break or abnormal communication between the subarachnoid space and subretinal space.2 Spontaneous retinal reattachment can occur in up to 36% of cases over a slow course, averaging 7.5 years.3 In our series, all 4 patients had associated RD. Three were confined to the peripapillary region with shallow RD, and 1 patient had total bullous RD. Spontaneous resolution of RD without treatment occurred in all 4 patients.
The new observation in our series was the presence of asymptomatic peripheral retinal nonperfusion in all 4 cases. Of these, 3 showed “brushfire” retinal vessels at the border of perfused and nonperfused retina and 1 patient showed vascular looping. There were no cases of telangiectasia, neovascularization of disc, retina, or choroid or progression of peripheral nonperfusion over a mean follow-up period of 20.3 months. Based on our personal observation, the pattern of peripheral retinal nonperfusion was strikingly similar to the nonperfusion found with congenital Coats disease. This new observation could be related to improved technology of peripheral retinal imaging with intravenous fluorescein angiography in infants. We postulate that the retinal vascular maldevelopment found at the optic disc also occurs at the peripheral fundus, leading to nonperfusion.
Correspondence: Dr C. L. Shields, Ocular Oncology Service, Ste 1440, Wills Eye Institute, 840 Walnut St, Philadelphia, PA 19107 (firstname.lastname@example.org).
Author Contributions: Dr C. L. Shields has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Financial Disclosure: None reported.
Funding/Support: Support was provided by the Eye Tumor Research Foundation (Drs C. L. Shields and J. A. Shields) and Lucille Wiedman fund for pediatric eye cancer (Drs C. L. Shields and J. A. Shields).
Role of the Sponsors: The funders had no role in the design and conduct of the study; the collection, analysis, and interpretation of the data; and preparation, review, or approval of the manuscript.
Rojanaporn D, Kaliki S, Shields CL, Shields JA. Morning Glory Disc Anomaly With Peripheral Retinal Nonperfusion in 4 Consecutive Cases. Arch Ophthalmol. 2012;130(10):1327-1330. doi:10.1001/archophthalmol.2012.505