Introduction
An uncommon congenital abnormality of the optic nerve is known as the morning glory anomaly. Its similarity to the morning glory flower led to its naming. When it is accompanied by systemic signs and symptoms, it is referred to as morning glory syndrome.
What Is Morning Glory Syndrome?
Morning glory syndrome (MGS) is a congenital (present at birth) defect of the optic nerve that resembles the "morning glory" flower. The optic disk, which is where the fibers of the optic nerve exit the retina, has an enlarged, funnel-like cavity that serves as a defining feature of this condition. The disk is enlarged and has what appears to be a white pupil in its white center. The blood vessels are more numerous and curved as they emerge from the enlarged disk with the white center, giving this malformation the appearance of flower petals.
What Is the Etiology and Epidemiology of Morning Glory Syndrome?
Although the embryonic origins of the morning glory anomaly are unknown, it is thought that either a primary mesenchymal anomaly or a defect in fetal fissure closure is responsible for it.
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Sex - Both genders are impacted; however, cases of female predilection have been reported more.
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Race - Occurrence is less among black people.
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Age - Typically, it is identified by the age of two. However, around the age of five, late presentations are occasionally observed. This mainly occurs as the patients are from rural areas and have lower socioeconomic status, which results from a lack of awareness.
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The syndrome mainly occurs unilaterally. Bilateral occurrence is less common.
What Is the Pathogenesis of Morning Glory Syndrome?
It might be caused by a primary mesenchymal abnormality, a lack of closure of the posterior scleral wall, and lamina cribrosa (a structure that resembles a mesh that allows optic nerve fibers to move through the sclera) development. Primary neuroectodermal dysgenesis may be indicated by the abnormal vascular pattern and central gliosis. Eight individuals who had optic nerve abnormalities, as well as PAX6 gene mutations, were reported by Azuma and colleagues. The central nervous system and several ocular tissues both express this gene, which is involved in the development of the eyes. Nallathambi et al., however, were unable to detect PAX6 mutations in their patients. The majority of cases that are reported are female, sporadic (no other instances in the family), and unilateral (affecting only one eye).
What Are the Signs and Symptoms of Morning Glory Syndrome?
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Poor visual acuity and poor vision in the impacted eye.
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Morning glory syndrome can happen on its own or in combination with other visual abnormalities like amblyopia or crossed or lazy eyes or with non-ocular issues like brain disorders.
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People with non-ocular findings frequently have a wide head, a depressed nasal bridge, and a defect or cleft in the middle of their upper lip.
What Are the Complications of Morning Glory Syndrome?
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The failure of the optic nerve to fully form while the baby is developing appears to be the cause of morning glory syndrome. Retinal detachment, which can happen in about 26 to 38 percent of morning glory syndrome patients, is the most serious complication. Sometimes, an optic nerve coloboma is mistakenly diagnosed as morning glory syndrome.
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Amblyopia brought on by long-term strabismus and an untreated refractive error may be difficult to treat. Retinal breaks may result from traction caused by peripapillary tissue over the retina. Retinal breaks can happen in the optic disk's tissues or near the excavation's edge.
How Is Morning Glory Syndrome Evaluated?
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Optical Coherence Tomography (OCT)- The optic disk anomaly can be assessed using optical coherence tomography (OCT). Studies have revealed that the epiretinal membrane clearly pulls the retina in a centrifugal direction.
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Optical Coherence Tomography (OCT) Angiography- Features of OCT angiography are present in individuals who have contractile optic disks. A dense microvascular network in the peripapillary capillary layer surrounding the optic nerve, with no distinction between the superficial and deep vascular plexuses.
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Fundus Fluorescein Angiography- There have been reports of peripheral capillary nonperfusion in morning glory syndrome, which is detected by fundus fluorescein angiography.
What Is the Management or Treatment of Morning Glory Syndrome?
In morning glory syndrome, early detection and treatment are crucial. Surgery is the treatment option that might lead to recovery of vision. In order to prevent amblyopia (a visual impairment that usually only affects one eye, but occasionally both, it happens when the connection between the brain and the eye breaks down and the brain is unable to recognize vision from a single eye), the strabismus (a condition where neither set of eyes points in the same direction), and anisometropia (a medical condition where refractive indices are not equal for both eyes) should be treated. Complete cycloplegic refraction (a technique that measures a person's overall refractive error by briefly paralyzing the eye muscles that help focus vision) is performed, and glasses are given. Surgery may be used to treat squints. Another related condition that needs surgical treatment is retinal detachment.
Some patients need to be referred to a variety of specialties, including neurosurgery, interventional neuroradiology, otolaryngology (a field of medicine where the ears, nose, and throat are the primary focus), and dentistry, depending on the other associated abnormalities. Brain imaging tests should be performed on everyone who has been identified as having a morning glory disk anomaly.
In unilateral instances of morning glory syndrome, the fellow eye must be closely monitored. A retinal detachment or cataract could develop in the other eye. In regard to this, either phacoemulsification (modern cataract surgery techniques such as phacoemulsification involve drawing and emulsifying the internal lens of the eye using an ultrasonic handpiece. the lens out of the eye), lens placement, or vitreoretinal surgery is performed.
What Is the Prognosis of Morning Glory Syndrome?
In people with morning glory syndrome, the visual prognosis is typically not good. Amblyopia is a result of the high refractive errors and strabismus in these patients. Due to the abnormality of the optic disk, there is a high risk of retinal detachment. Children who have chronic retinal detachment are significantly more likely than others to be discovered incidentally with decreased vision.
Conclusion:
The patient needs to receive appropriate guidance regarding the MGS prognosis and complications. A comprehensive ocular and systemic assessment is necessary in these cases. An ophthalmologist should also examine any family members. An evaluation by a multidisciplinary team consisting of an ophthalmologist, a neurologist, and a doctor should be given to any patient who presents with MGS. It might be necessary to consult a neuro-ophthalmologist and a vitreoretinal specialist. Ocular issues are the most frequent reason for patients to see the primary clinician, so these specialists ought to be conscious of the condition. Morning glory syndrome can be accompanied by systemic abnormalities like moyamoya disease or encephalocele, so prompt referral to a neurologist is required. Their primary clinicians can then monitor these patients.
It is important to guarantee treatment compliance. Patients on follow-up are first introduced to the nursing staff in the department. They can assess patient adherence to medication and lifestyle recommendations and alert the primary care clinician to any problems. The best possible patient outcomes may be obtained through this collaborative and multidisciplinary approach to care.
