Learn all about morning glory syndrome. In 1970 Kindler described an unusual congenital disc anomaly as “morning glory syndrome” because of its resemblance to the morning glory flower.
It is characterized by an enlarged, funnel-shaped excavation that incorporates the optic disc. The disc itself is enlarged, and orange or pink in color within a surrounding area of peripapillary chorioretinal pigmentary changes. The overall diameter of the disc depends on the size of the excavated posterior scleral opening. Occasionally the disc itself may appear elevated. Within the center of the disc is a white glial tuft. Similar to petals on a flower, the blood vessels are increased in number and curve as they emanate radially from the disc, rather than in the usual central branching pattern. They then straighten, and it is often difficult to distinguish the arteriolar from the venous circulation. There are also small peripapillary arteriovenous communications. Depending on the size of papillary chorioretinal involvement, the macula may be incorporated into the excavation, which is termed “macular capture.
Morning glory syndrome is typically unilateral with equal involvement of the right and left eyes. Bilaterality of this condition can occur. The visual prognosis is usually poor, in the 20/100 to 20/200 range. Serous retinal detachments can occur in 30% of individuals. Although not completely understood, it is postulated that there is a communication between the subarachnoid space within the posterior excavation and the subretinal space. Cerebrospinal fluid can then leak subretinally and can extend to a complete retinal detachment. Cases to support this hypothesis include resolution of the serous detachment following optic nerve sheath fenestration, and demonstration of a gas bubble in the subarachnoid space following vitrectomy with gas injection and optic nerve sheath fenestration.
Contractile movements within the morning glory disc anomaly, leading to an opening and closing of the central cup that corresponds to pulse pressure, can also be seen. The visual prognosis in individuals with morning glory disc anomaly is poor. In addition to the abnormal disc itself and the predilection for serous retinal detachments, there is an added compounding variable of high refractive errors, amblyopia, and strabismus. Although ocular realignment surgery and treatment of anisometropic amblyopia is recommended and may result in some recuperation of vision, it is rare to see dramatic improvements.
Treatment of the other associated abnormalities requires an interdisciplinary approach, often including neurosurgery, interventional neuroradiology, otolaryngology, and dentistry. All patients discovered on routine ophthalmic examination to have morning glory disc anomaly should have brain MRI, MRA, and timely referrals to the appropriate subspecialist.
What is Morning Glory Syndrome?
A birth defect of the optic nerve (the nerve to the eye) in which there is a coloboma (cleft) of the optic disc. The coloboma results in a funnel-shaped optic nerve head with a white dot in the center, an elevated ring of pigment around the disk, and vessels radiating out from the ring like spokes. Reflection from within the eye may give the appearance of a white pupil. Vision in the affected eye is severely impaired. There is an increased risk of retinal detachment. It is also called morning glory anomaly.
Morning Glory Syndrome Definition
Morning glory syndrome (MGS) is an optic neuropathy characterized by a congenital funnel shaped excavation of the posterior fundus that incorporates the optic disc malformation (resembling the morning glory flower) MGS is usually unilateral and may result in a decrease in best-corrected visual acuity (BCVA). MGS either occurs isolated or associated to other ocular or non-ocular anomalies.
Morning Glory Syndrome Symptoms
Typically, only one eye is affected in Morning Glory Syndrome. But, in some rare cases, both eyes may be affected, causing significant signs and symptoms. The signs and symptoms of this eye disorder are usually detected in a child, after the first 12 months of the child’s life. The signs and symptoms of Morning Glory Syndrome are outlined below.
• Poor vision
• Lazy eye (amblyopia)
• Color perception difficulties
• Squinting of the eye (strabismus) – eye that drifts severely away from the child’s fixed point of reference
• Other neurological symptoms, depending on associated congenital conditions
• Feeding difficulties, if associated with cleft palate.
Morning Glory Syndrome Causes
The cause of Morning Glory Syndrome developmental defect is unknown. A developmental defect is a birth defect that affects the baby in the mother’s womb, during pregnancy. The exact reason why these defects occur in some babies is unknown
Some studies have shown a mutation in PAX6 gene, to be the probable cause of Morning Glory Optic Disc Anomaly. Research is being performed to arrive at a definitive cause of this condition.
Morning Glory Syndrome Treatment
Morning glory syndrome has not any specific treatment. However it is important to optimize visual acuity to prevent amblyopia. Dilated fundus exams should be done to detect serous retinal detachments that tend to originate in the peripapillary area and extends to the posterior pole. Brain imaging (MRI, MRA and/or CTA) should be done to rule out CNS involvement, including basal encephalocele, Moyamoya and other structural or vascular abnormalities. Surgical correction of the transsphenoidal basal encephalocele can be extremely difficult or even contraindicated due to the vital structures that may be found in the herniation, included the optic chiasm, the hypothalamus, and the anterior cerebral artery.