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The condition is usually unilateral. Vision is generally poor, and retinal detachment occurs in over one third of cases. Some patients have associated conditions including midline facial anomalies (depressed nasal bridge and hypertelorism) basal skull defects, with herniation of pituitary and hypothalamic tissue panhypopituitarism and absence of the corpus callosum.
The cause of morning glory syndrome is unknown. It may be a form of optic disc coloboma. The retinal detachment is thought to result from a retinal tear within the excavation, providing an abnormal communication between the subretinal space and either the vitreous cavity or the subarachnoid space surrounding the optic nerve.
Symptoms
Poor vision in the affected eye.
Signs
Most cases are unilateral. Visual acuity in the affected eye usually ranges between 20/100 (6/30) and hand movements. There may be an associated strabismus and/or myopia.
The optic disc is enlarged, with a funnel-shaped central core of pale glial tissue surrounded by an elevated pigment ring. Numerous, straight blood vessels emerge from the perimeter of the excavation, rather than originating at a central blood vessel as they would in a normal eye. The increased disc diameter, the abnormal vascular pattern and excavation of the disc have some elements in common with an optic disc coloboma.
A careful retinal examination is indicated for signs of serous (non-rhegmatogenous) retinal detachment.
Prevalence
Very rare. Females are affected twice as often as males.
Significance
Vision in the affected eye is severely impaired and there is a risk of retinal detachment.
Differential diagnosis
Coloboma of the optic disc.
See also
Optic nerve head pit, Glaucoma classification, Retinal detachment classification.
Management
Advice
Being a congenital malformation, there is no specific treatment. The patient often has normal vision in the other eye. Associated conditions, including endocrine abnormalities, may require specialist assessment and treatment. The patient should be educated with regard to the symptoms of retinal detachment, and advised of the risks of contact sports and the need for eye protection.
Laser or incisional surgery
Retinal detachment may be treated with pars plana vitrectomy, laser photocoagulation, and/or intravitreal tamponade with gas or silicone oil.
Review
Routine annual review may be indicated in view of the increased risk of retinal complications.