Managing retinal detachment in morning glory syndrome

(Image credit: AdobeStock/vendenis)

Morning Glory Syndrome (MGS) is a rare and complex disease that necessitates a multidisciplinary approach for effective management and enhanced patient outcomes, according to Suping Wu, MD, and colleagues, who undertook a study¹ to determine the optimal treatments to obtain improved patient care.

Wu, from the Department of Ophthalmology, Binzhou Medical College, Binzhou, Shandong, PROC, was joined in this study by researchers from the Department of Ophthalmology, Weifang Eye Hospital, Weifang, Shandong, PROC; Department of Ophthalmology, Guangdong Provincial People’s Hospital, Guangzhou, Guangdong, PROC; and Department of Ophthalmology, Shandong Second Medical University, Weifang, Shangdong, PROC.

MGS, so named because of its resemblance to a morning glory in bloom, is a rare congenital malformation of the ocular fundus that results in abnormal development of the optic disc.² The incidence of MGS is approximately 3.6 in 100,000 individuals, with women affected twice as often as men. Patients with MGS typically experience low vision, often ranging from counting fingers to 20/100.2 MGS is thought to arise from mutations in the PAX6 or PAX2 genes,³ with inheritance patterns that can be autosomal dominant or recessive.

The fundus appears as a posteriorly displaced enlarged, pink, funnel-shaped optic disc; white/gray retinal pigmentary disturbances often are present around the peripapillary region.⁴ Another finding is a concentric ring of retinal-choroidal atrophy in the optic disc, which is frequently pigmented, while the peripheral area shows retinal choroidal atrophy, Wu and associates described.

Retinal detachments, which are significant complications,¹⁰ can be exudative, tractional, or rhegmatogenous, and can develop in up to 30% of cases.⁵

MGS is also associated with other ophthalmic conditions: refractive errors, strabismus, nystagmus, microcornea, pupillary membrane closure, cataracts, persistent fetal vasculature, retinal folds, retinal pigment degeneration, choroidal coloboma, scleral staphyloma, optic nerve atrophy, choroidal neovascularization, eyelid hemangiomas, and preretinal glioma.^4-6,7-11 systemic complications can include Aicardi syndrome,¹² renal coloboma syndrome,¹³ basal encephalocele,¹⁴ and moyamoya disease.⁵

Treating retinal detachments

Surgical interventions generally are performed to manage complications, especially retinal detachments, with procedures such as vitrectomy, paraoptic disc laser photocoagulation, and the use of silicone oil or gas tamponade, although the success rates can vary widely.

Regarding the treatment of MGS combined with rhegmatogenous retinal detachment, vitrectomy is performed most combined with paraoptic disc laser photocoagulation. Reattachment of the retina can be achieved in most cases, and the fundus anatomy remains stable during the follow-up period. However, some adverse outcomes can develop, such as retinal redetachment, silicone oil emulsification, and elevated intraocular pressure, among others, according to Wu and colleagues.

Regarding the treatment of MGS combined with non-rhegmatogenous retinal detachment, vitrectomy is commonly performed combined with paraoptic disc laser photocoagulation or vitrectomy alone. Both surgical methods can achieve reattachment of the retina, and the fundus anatomy is stable during the follow-up period. However, adverse outcomes also can develop in this scenario such as retinal redetachment, silicone oil emulsification, and others.

To prevent MGS combined with retinal detachment, simple paraoptic disc laser photocoagulation can be applied.

The authors noted that, “The complexity of MGS necessitates a multifaceted approach to treatment, involving advanced surgical techniques and thorough postoperative management to address complications effectively. While the visual prognosis remains variable, ongoing research into genetic factors and innovative treatment modalities holds promise for improving outcomes for individuals affected by MGS. Enhanced awareness and understanding among healthcare professionals will be pivotal in ensuring timely diagnosis and intervention, ultimately aiming to optimize the quality of life for patients with this condition.

References

1. Wu S, Wang W, Liu L, et al. Progress in the management of retinal detachment associated with Morning GlorySyndrome. Clin Ophthalmol. 2025;19:459-468. doi: https://doi.org/10.2147/OPTH.S505086

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11. Lytvynchuk LM, Glittenberg CG, Ansari-Shahrezaei S, Binder S. Intraoperative optical coherence tomography assisted analysis of pars Plana vitrectomy for retinal detachment in morning glory syndrome: a case report.BMC Ophthalmol. 2017;17:134. doi:10.1186/s12886-017-0533-0

12. Al-Moujahed A, Callaway NF, Ludwig CA, et al. Morning glory optic nerve in Aicardi syndrome: report of a case with fluorescein angiography.Eur J Ophthalmol. 2021;31:NP61. doi:10.1177/1120672120942702

13. Schimmenti LA. Renal coloboma syndrome.Eur J Hum Genet. 2011;19:1207. doi:10.1038/ejhg.2011.102

14. Ru T, Song M, Haibo Y, Weitian Z. Morning glory syndrome associated with congenital nasal anomalies.Int J Pediatr Otorhi. 2020;132:109972. doi:10.1016/j.ijporl.2020.109972