MORNING GLORY SYNDROME

Signs and Symptoms

Morning glory syndrome (MGS) is a congenital optic disc anomaly that can be discovered at any age, though most patients are usually made aware of the condition at their first eye examination. The incidence is unknown and the condition is found equally in males and females.1,2 MGS can be either bilateral or unilateral.2-8 When the condition is bilateral, visual acuity is typically good.4,9 However, most patients with unilateral MGS have markedly reduced visual acuity, often to the level of hand motion vision.4,10 While reports are often contradictory regarding the level of visual function, it can safely be stated that MGS has a spectrum of severity, with most patients retaining useful vision.11

There will be a noticeably enlarged anomalous disc and peripapillary retinal changes. The nerve will appear larger than the fellow eye’s nerve in unilateral cases. The condition gets its name from its resemblance to a tropical flower of the same name. It is characterized by a funnel-shaped excavated and enlarged dysplasic optic disc, with white tissue surrounded by an elevated pigmented peripapillary annulus. White glial tissue is present at the bottom of the cup and represents an important diagnostic criterion.

The retinal vessels arise from the periphery of the disc anomaly and run an abnormally straight, radial course over the peripapillary retina. The origin of the vessels is obscured by the central tuft of glial tissue. This can give the morning glory disc a pseudo-glaucomatous appearance.1,6,12-14 There will appear to be an excessive number of retinal vessels; however, this is simply due to the fact that glial tissue obscures the branching of the vessels within the optic cup. Retinal detachment may develop during the clinical course.15-21 Strabismus is frequently encountered in patients with MGS as well.22

Many ocular conditions have been found in association with MGS, including microophthalmos, cataracts, myopia, ciliary body cysts, Bergmeister’s papilla and hypertelorism.12,23 Numerous systemic abnormalities have also been identified in association with MGS, including Goldenhar’s syndrome; sphenoidal encephalocele; porencephaly and hydronephrosis; renal failure; cerebral malformation; frontonasal dysplasia; endocrine irregularities; neurofibromatosis type 2; midline craniofacial defects such as basal encephalocele, cleft lip and palate; Chiari type I malformation; and agenesis of the corpus callosum.3,5,6,10,15,24,25 More recently, MGS has been reported in association with Down’s syndrome, primary open angle glaucoma and multiple sclerosis.26-28

Despite numerous reported associations, these comorbidities seem to be mostly anecdotal cases. Thus, MGS is considered to be an isolated ocular abnormality. Further, in the absence of consistent systemic associations, perhaps the term “syndrome” does not apply to this condition. However, one study reported on 22 eyes with MGS and persistent hyperplastic primary vitreous, giving some credence to a possible association between the two entities.29

Pathophysiology

Morning glory syndrome is a nonprogressive congenital optic nerve anomaly. The condition has been shown to be limited to the eye with no involvement of the retrobulbar nerve and brain.2,15,23 MGS has long been considered to be a variant of optic nerve coloboma.23 However, more recent findings suggest this may not be true. The central glial tissue, vascular anomalies, scleral defects, adipose and smooth muscle tissue within the peripapillary sclera are more consistent with a mesenchymal abnormality.24,30 An alternate theory suggests that abnormal enlargement of the distal optic stalk during development allows formation of the characteristic excavation seen in MGS.24 Spectral-domain and swept-source OCT has demonstrated a preretinal tractional membrane and inferiorly decentered excavation in MGS.31

Visual dysfunction arises from an undeveloped optic nerve with fibers never reaching the lateral geniculate nucleus. The main associated pathology that occurs in association with MGS is retinal detachment. OCT has demonstrated slit-like retinal breaks within or at the edge of the disc excavation. These slit-like breaks provide a direct communication between the subretinal space and the vitreous cavity, permitting fluid from vitreous syneresis to evolve tissue separation.16-21,32,33

Management

Management of morning glory syndrome typically does not extend beyond proper diagnosis. While the appearance can be quite dramatic, extensive neurological evaluation can be avoided, as this is a non-acquired, nonprogressive disc anomaly. While there have been many associated systemic abnormalities reported, there is not enough consistency to consider these comorbidities anything but coincidental, making extensive evaluation unwarranted. Glaucoma treatment based solely upon the disc appearance should be avoided. Protective eyewear should be recommended in order to safeguard the better-seeing eye in unilateral cases.

The patient must be monitored and educated about the signs and symptoms of retinal detachment. Management of this type of retinal detachment varies, potentially involving pars plana vitrectomy with posterior hyaloid removal, fluid/air exchange, endolaser in the area of the retinal break, and a long-acting gas-bubble injection or silicone oil tamponade.17,18,34,35

Clinical Pearls

The neuroretinal rim of the morning glory disc is recessed and not readily visible. This has been mistakenly identified as acquired thinning of the rim, as seen in glaucoma. Morning glory syndrome has frequently been misdiagnosed and mistreated as normal tension glaucoma. Always rule out MGS in cases of suspected normal tension glaucoma. Impulsive diagnoses should be avoided.

In cases where there is reduced visual acuity, MGS may be misdiagnosed as amblyopia.

While dramatic in appearance, morning glory syndrome does not progress. There is no necessary treatment unless retinal detachment develops.

 

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