Morning glory disc anomaly (MGDA) is a congenital anomaly of the optic disc first reported by Kindler. It is characterized by an enlarged, grayish-white optic disc area with a funnel-shaped excavation, prepapillary white tissue at the base of the excavation, peripapillary chorioretinal pigmentary disturbance, and abnormal retinal vascular course. The name derives from its morphological resemblance to the morning glory flower. When accompanied by systemic signs, it is called morning glory syndrome.
It is usually unilateral, but bilateral cases have been reported. Heredity is not clear. It is more common in females and rare in African Americans. It is considered one of the congenital optic disc anomalies that require differentiation from glaucoma3).
QIs morning glory anomaly glaucoma?
A
Morning glory anomaly is not glaucoma but a congenital anomaly of the optic disc. However, enlargement of the optic cup can be confused with glaucomatous changes, and it is listed as an important disease in the differential diagnosis of glaucoma3).
Visual acuity ranges from nearly normal to hand motion, depending on the size of the excavation, the position of the macula (relationship to the excavation), and the degree of retinal detachment. Poor vision is often present from birth. In children, it is often noticed due to poor vision in one eye or strabismus. Some cases present with leukocoria.
Enlargement of the optic disc and funnel-shaped excavation: The optic disc appears large and funnel-shaped. There is a staphyloma-like excavation around the disc, detectable by ultrasound, CT, and MRI.
White tissue at the base of the excavation: Thought to be glial proliferation or remnants of persistent hyperplastic primary vitreous. The disc morphology is obscured by this white tissue.
Abnormal retinal vascular course: Vessels originate from beneath the white tissue, are more numerous and narrower than normal, and run radially and linearly.
Peripapillary pigmentary abnormalities: Retinochoroidal pigmentation is observed. Many cases have a tongue-shaped area of retinochoroidal atrophy inferior to the disc.
Macular capture: The macula may be pulled toward the excavation and not observable.
Afferent pupillary defect (APD): May be present due to the unilateral nature.
Retinal detachment: Retinal detachment starting near the disc is often associated. About 30% have serous macular involvement.
The embryologic origin of MGDA is not clear. Hypotheses include failure of closure of the fetal fissure or primary mesenchymal abnormality. Many cases show a tongue-shaped area of retinochoroidal atrophy inferior to the disc, suggesting an association with incomplete closure of the optic cup fissure. Some cases involve posterior scleral dysplasia.
MGDA may be associated with systemic abnormalities including those of the central nervous system.
Intracranial Structural Abnormalities
Transsphenoidal basal encephalocele: A congenital anomaly where meninges protrude through a defect in the sphenoid bone, possibly involving the optic chiasm and hypothalamus1). Features include a broad head, flat nose, hypertelorism, and midline defects such as a cleft upper lip.
Agenesis of the corpus callosum: Often associated with transsphenoidal encephalocele.
Pituitary stalk duplication (PSD): Rare but reported in patients with MGDA and moyamoya disease
Cerebrovascular abnormalities
Moyamoya disease: A cerebrovascular disease characterized by progressive stenosis and occlusion of cerebral arteries
Hypoplasia of cerebral arteries: Reported in association with MGDA
PHACE syndrome: Association of posterior fossa malformations, large facial hemangiomas, arterial anomalies, cardiovascular anomalies, and eye anomalies
QIs brain examination necessary for morning glory anomaly?
A
Yes, it is necessary. There is a possibility of associated intracranial structural and vascular abnormalities such as transsphenoidal basal encephalocele and moyamoya disease1). Especially in children, evaluation with brain MRI and MRA is recommended.
Clinical diagnosis is possible by confirming characteristic optic disc findings (funnel-shaped excavation, white glial tissue, radial vascular pattern, peripapillary pigmentary changes) on fundus examination.
OCT: Useful for evaluating glial tissue on the optic disc and neurosensory retinal detachment. It can also be used to detect peripapillary choroidal neovascular membrane (PPCNVM)2)
Ultrasound: Noninvasively evaluates the depth of optic disc excavation and the presence of retinal detachment
Brain MRI/MRA: Essential for detecting intracranial abnormalities such as transsphenoidal basal encephalocele and moyamoya disease
There is no direct treatment for MGDA itself. Optimizing vision to prevent amblyopia is important; if the macula is confirmed in children under 6 years of age, amblyopia treatment should be considered.
Retinal detachment usually begins near the optic disc and is often confined to the peripapillary area. In localized cases, the subretinal fluid is likely derived from cerebrospinal fluid, and observation may be chosen. Spontaneous resolution has been reported.
In advanced retinal detachment, small tears often coexist within the optic disc excavation, and vitrectomy is performed. The surgical procedure is as follows:
Removal of the vitreous gel
Fluid-air exchange and drainage of subretinal fluid
Peripapillary photocoagulation
Tamponade with long-acting gas
In recurrent or refractory cases, application of butyl cyanoacrylate (retinal glue) to the tear site has been attempted. In pediatric cases, silicone oil tamponade may be used, but there is concern about migration into the subarachnoid space.
QHow is retinal detachment treated when it occurs?
A
Retinal detachment localized to the peripapillary area may be observed. In progressive cases, vitrectomy (fluid-air exchange, peripapillary photocoagulation, gas tamponade) is performed. Postoperative retinal reattachment progresses very slowly and may take several months to over a year.
The origin of the white tissue at the bottom of the excavation is debated between glial proliferation and persistent hyperplastic primary vitreous. The tongue-shaped chorioretinal atrophy lesion below the disc suggests an association with incomplete closure of the optic fissure. It has also been suggested that posterior scleral hypoplasia may contribute to the formation of the excavation.
Multiple mechanisms are considered for the origin of subretinal fluid.
Cerebrospinal fluid origin: In some cases, contrast medium administered into the spinal canal was observed to extend from the subarachnoid space to the subretinal space, suggesting communication between cerebrospinal fluid and subretinal fluid.
Leakage from abnormal vessels: Serous leakage from abnormal vessels in the excavation area.
Tractional: Traction by prepapillary white tissue or posterior vitreous cortex secondarily induces small tears around the optic disc.
Rhegmatogenous: In advanced cases, tears often develop near the prepapillary tissue.
In the early stage, exudative or tractional detachment may resolve spontaneously, but progression to total detachment leading to blindness is not uncommon.
Bassi et al. (2024) reported OCT findings in MGDA eyes, differentiating peripapillary choroidal neovascular membrane (PPCNVM) from peripapillary hyperreflective ovoid mass-like structures (PHOMS)2). PPCNVM is a hyperreflective structure continuous with the retinal pigment epithelium-choroid complex, and when accompanied by intraretinal cystic spaces, indicates activity. PHOMS is a marker of axoplasmic stasis and is differentiated from PPCNVM by being less hyperreflective and not associated with shadowing2). For PPCNVM, intravitreal injection of anti-VEGF agents is a treatment option.
Association with Transsphenoidal Basal Encephalocele
Bhatti et al. (2024) reported a case of transsphenoidal basal encephalocele associated with MGDA in a 20-year-old woman1). MRI confirmed an encephalocele through a sphenoid bone defect. Because the encephalocele may contain the optic chiasm, hypothalamus, anterior cerebral artery, etc., surgical correction is extremely difficult or may be contraindicated.
Bassi ST, Verma A. Optical Coherence Tomography in a Morning Glory Disc Anomaly with a Peripapillary Choroidal Neovascular Membrane. Neuro-Ophthalmology. 2024;48(1):27-29.
American Academy of Ophthalmology. Primary Open-Angle Glaucoma Preferred Practice Pattern. AAO; 2025.
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