Case of peripheral fibrovascular proliferative retinopathy associated with morning glory disc anomaly





Abstract


It is known that the retinal detachments (RDs) associated with a morning glory disc anomaly (MGDA) usually appear from around the disc anomaly and complications by peripheral fibrovascular proliferation and tractional RD are very rare. We report our findings in an eye with MGDA that had a tractional RD, massive exudation from a peripheral temporal fibrovascular proliferation and vasculatures stretched by this proliferation and by a contraction of the hyaloidal membrane. The RD was successfully treated by vitrectomy and encircling buckling surgery.



Introduction


Morning glory disc anomaly (MGDA) was first described by Kindler in 1970. MGDA is a congenital malformation of the optic nerve head, and the optic nerve head is covered by a fibroglial white tissue membrane with an annular retinal pigmented epithelial hypoplasia. It is known that the retinal detachments (RDs) associated with a MGDA usually appear from around a disc anomaly. , , The causes of the RDs have been suggested to be exudation from abnormal vessels or the optic nerve sheath, traction by the vitreous, or small retinal tears around the optic disc. , , Two studies have reported peripheral exudative or tractional RD in eyes with MGDA, , and She et al. reported that tractional RD was present in one of 16 cases. However, the mechanism causing the peripheral RD has not been reported, and the best treatment for the RD has not been determined.


We report our findings in a rare case of MGDA with extensive exudative and tractional RDs caused by a peripheral fibrovascular proliferation that was successfully treated by vitrectomy with scleral buckling.



Case report


A 7-year-old Japanese girl with MGDA was first examined when she was 2-years-old at another hospital because of strabismus. She was diagnosed with MGDA in her left eye at that time. She was treated with corrective spectacles for her hyperopia, and her right eye was patched intermittently. The decimal best-corrected visual acuity (BCVA) was 0.1 (+2.75 D = −3.5 DC ax180°) in the left eye, and she was followed by yearly examinations ( Fig. 1 A). When she was 6-year-old, a preretinal membrane was detected in the posterior pole of the left eye, and the arcade vessels appeared taut ( Fig. 1 B). When she was 7-years-old, her decimal BCVA decreased to 0.05 in her left eye and a RD with exudation developed. The exudation extended from the posterior pole to the peripheral temporal area in her left eye ( Fig. 1 C). The doctors in the hospital tested for anti- Toxoplasma gondii antibody, and the results were negative. She was referred to our hospital for further examinations and possible treatment because the RD had progressed.




Fig. 1


Fundus photograph of the left eye taken at the initial hospital.

A: Fundus photograph taken when the patient was 3-years-old. Morning glory disc anomaly (MGDA) can be seen.

B: Fundus photograph taken when the patient was 6-years-old. A preretinal membrane can be seen in the posterior pole, and it appears to be contracted.

C: Fundus photograph taken when the patient was 7-years-old before she came to our clinic. Exudation, preretinal membrane, and retinal detachment (RD) are present.


Our initial examination showed that her medical and family histories were unremarkable, and she was a full-term baby. The results showed that her pupillary reflexes were prompt in the right eye and sluggish in the left eye. A relative afferent pupillary defect (RAPD) was present in the left eye. The BCVA was 1.2 (+2.75 D = −0.5 DC ax147°) in the right eye and 0.04 (+2.0 D = −3.0 DC ax180°) in the left eye. The intraocular pressure was 16 mmHg in the right eye and 10 mmHg in the left eye. The anterior segment was normal bilaterally, and the lens was clear bilaterally. The fundus was normal in her right eye, but the fundus of her left eye showed MGDA with a RD with massive yellowish exudation without a retinal break. A peripheral fibrovascular proliferation which extended to the vitreous was detected in the peripheral temporal retina of her left eye. The retinal vessels from the disc to the temporal periphery appeared stretched by this proliferation and by a contraction of the hyaloidal membrane. A preretinal fibrous membrane covered the optic disc which extended to the temporal retina ( Fig. 2 A). Early phase fluorescein angiography (FA) of her left eye showed taut vessels and abnormal branching of the peripheral vessels with leakage. There was also temporal peripheral telangiectasia of the fibrovascular proliferation with massive leakage. Non-perfused retina was also observed around the abnormal vessels in the peripheral retina ( Fig. 2 B–D). The FA findings were unremarkable in the right eye. Optical coherence tomography (OCT) showed a thick preretinal membrane and retinoschisis with an abnormal retinal layer. Each layer was absent, and the separation of the layers was not present in the macular area. A RD of the inferior area of the macula was present ( Fig. 2 E and F). There was a strong traction by the fibrous membrane from the temporal proliferation and preretinal membrane and exudation from the peripheral vascular proliferation. We diagnosed the patient with MGDA associated with tractional and exudative RD with peripheral fibrovascular proliferation.


Jul 10, 2021 | Posted by in OPHTHALMOLOGY | Comments Off on Case of peripheral fibrovascular proliferative retinopathy associated with morning glory disc anomaly
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