From the University of Pennsylvania Scheie Eye Institute (NC); and the Department of Ophthalmology (AAC, KAL), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Netan Choudhry, MD, Massachusetts Eye & Ear Infirmary, 12th Floor, 243 Charles Street, Boston, MA 02141. E-mail: email@example.com
Morning glory anomaly is a sporadic congenital abnormality of the optic disc that is often unilateral. Heterotropic smooth muscle in these eyes can spontaneously contract, resulting in transient vision loss. We report a case of morning glory disc anomaly with contractility induced by ocular massage.
A 2-year-old girl was referred for amblyopia of the left eye. Her medical and ocular history was unremarkable. On examination, her visual acuity (LEA symbols) was 20/30 in the right eye and light perception without projection in the left eye with an afferent pupillary defect. Intraocular pressures were normal in both eyes. External examination revealed microphthalmia with pseudoptosis of the left upper eyelid. Slit-lamp examination and dilated fundus examination was normal in the right eye. Slit-lamp examination of the left eye revealed posterior opacification of the lens with a persistent hyaloid artery remnant. The left optic nerve appeared excavated with the blood vessels arising from the disc periphery, displaced by white gliotic tissue (Fig. 1). A diagnosis of morning glory disc anomaly with persistent hyaloid artery was made.
Figure 1. Fundus Photograph of the Left Eye Demonstrating a Morning Glory Disc with a Persistent Hyaloid Artery Remnant.
During examination under anesthesia, pressure of the RETCAM II probe (Clarity Medical Systems, Pleasanton, CA) on the left eye elicited repetitive contraction of the optic disc, which contracted over 7 seconds before returning to baseline. Applying minimal and constant pressure with the Retcam probe on the corneal surface, three episodes of such optic disc contraction were observed over a period of 1 minute. These episodes were cyclic and did not seem to occur as a response to differences in the pressure applied over the eye (Fig. 2 and video). The macula and peripheral retina were normal. An MRI/MRA of the brain and orbits showed no evidence of a basal encephalocele or moyamoya disease1; however, the scan did reveal left microphthalmos, a funnel-shaped enlargement of the optic nerve at its junction with the globe, and thinning of its intracanalicular portion consistent with morning glory disc anomaly (Fig. 3).
Figure 2. Fundus Photograph of the Left Eye Demonstrating a Morning Glory Disc in Contracted State.
Figure 3. A Magnetic Resonance Imaging T1-Weighted Image of the Orbits Demonstrated Microphthalmia, Funnel-Shaped Enlargement of the Optic Nerve at Its Junction with the Globe and Thinning of Its Intracanalicular Portion.
Descriptions of the morning glory disc first appeared in the literature in the early 1900s, but it was not until 1970 that Kindler coined the term morning glory disc, after the flower of the same name.2 Kindler described the morning glory disc as a “funnel-shaped, excavated nerve-head defect, surrounded by an elevated annulus of chorioretinal pigment disturbance and subretinal fibroglial tissue.”2 The etiology of this condition remains controversial and is centered around a sole defect in mesodermal differentiation or a defect in the relative speed of differentiation of the mesoderm versus ectoderm.3,4 This faulty differentiation subsequently gives rise to a defect in the posterior scleral wall and lamina cribosa. As a consequence, the optic nerve is retrodisplaced and retinal tissue herniates through this defect, straightening the retinal vessels and giving rise to the morning glory flower appearance. It has also been proposed that regression of the hyaloidal vasculature may be compromised, which is suggested by the associated finding of persistent fetal vasculature seen in this case and reported previously.5
The contraction of the optic disc in this condition has been reported in few morning glory disc cases worldwide.6–11 Theories regarding the etiology of this contraction include pressure balance or muscular contraction.7,8,12 Evidence lending support for muscular contraction emerged with the discovery of heterotropic smooth muscle cells in the sclera of an eye with an optic disc coloboma at the Armed Forces Institute of Pathology.12
Histopathological studies have also identified heterotropic fibroadipose tissue in the optic nerve meninges, which are derived from mesoderm.13 The presence of nonvascular contractile smooth muscle cells in the posterior choroid and sclera of normal eyes has been reported.14,15 These cells have been found to be distributed in a U-shaped pattern around the short posterior ciliary arteries, larger caliber vortex veins, and in the temporal quadrant of the fovea.15 They possess sympathetic nerve terminals in the choroid and nitroxidergic parasympathetic terminals in the sclera that are functionally contractile.15 Immunohistochemical staining with smoothelin, a cytoskeletal protein only found in smooth muscle cells, has reinforced these findings.15 Although smooth muscle cells have been found in both normal eyes and eyes with morning glory disc anomaly, this contractility has only been reported in patients with the morning glory disc anomaly.4,16
An analysis of contractile movements in a case of morning glory disc anomaly investigated the rates of change in the size of the optic disc, cup, surrounding pigmented ring, and cup/disc ratio.11 A 21.5% reduction of the optic cup and 39.9% change in size of the optic disc was observed, which may explain transient vision loss in patients with morning glory anomaly.6,17 Further imaging of the contraction of the optic disc in this condition, as with optical coherence tomography, could prove useful in understanding the effects of the contraction on neighboring structures, including the peripapillary retina and subretinal space. Recently, spectral-domain optical coherence tomography was performed on 9 eyes with morning glory disc anomaly, 3 of which had contractility, and no differences were found regarding the presence of subretinal fluid or cerebrospinal communications between contractile and non-contractile morning glory discs or any other anomaly that could explain the contractile behavior of some but not all morning glory discs.6
Despite several reports of optic disc contractility in morning glory disc anomaly, the exact etiology remains controversial. It is unclear whether the apparent contractility is a result of stimulation of non-vascular smooth muscle or a result of an increase in intraocular pressure from manual stimulation possibly potentiating further herniation of neural tissue through the existing scleral defect, giving the appearance of disc contraction.5,11
- Massaro M, Thorarensen O, Liu GT, Maguire AM, Zimmerman RA, Brodsky MC. Morning glory disc anomaly and moyamoya vessels. Arch Ophthalmol. 1998;116:253–254.
- Kindler P. Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol. 1970;69:376–384.
- Dempster AG, Lee WR, Forrester JV, McCreath GT. The morning glory syndrome: a mesodermal defect?Ophthalmologica. 1983;187:222–230. doi:10.1159/000309330 [CrossRef]
- Manschot WA. Morning glory syndrome: a histopathological study. Br J Ophthalmol. 1990;74:56–58. doi:10.1136/bjo.74.1.56 [CrossRef]
- Lee BJ, Traboulsi EI. Update on the morning glory disc anomaly. Ophthalmic Genetics. 2008;29:47–52. doi:10.1080/13816810801901876 [CrossRef]
- Cennamo G, de Crecchio G, Iaccarino G, Forte R, Cennamo G. Evaluation of morning glory syndrome with spectral optical coherence tomography and echography. Ophthalmology. 2010;117:1269–1273. doi:10.1016/j.ophtha.2009.10.045 [CrossRef]
- Pollock S. The morning glory disc anomaly: contractile movement, classification and embryogenesis. Doc Ophthalmol. 1978;65:439–460. doi:10.1007/BF00143047 [CrossRef]
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- Chuman H, Nao-I N, Sawada A. A case of morning glory syndrome associated with contractile movement of the optic disc and subretinal neovascularization [article in Japanese]. Nippon Ganka Gakkai Zasshi. 1996;100:705–709.
- Wise JB, MacLean AL, Gass JD. Contractile peripapillary staphyloma. Arch Ophthalmol. 1966;75:626–630.
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- Willis F, Zimmerman LE, O’Grady R, Smith RS, Crawford B. Heterotropic adipose tissue and smooth muscle in the optic disc: association with isolated colobomas. Arch Ophthalmol. 1972;88:139–146.
- Koenig SB, Naidich TP, Lissner G. The morning glory syndrome associated with sphenoidal encephalocele. Ophthalmology. 1982;89:1368–1373.
- Poukens V, Glasgow BJ, Demer JL. Nonvascular contractile cells in sclera and choroids of humans and monkeys. Invest Ophthalmol Vis Sci. 1998;39:1765–1774.
- May CA. Non-vascular smooth muscle cells in human choroids: distribution, development and further characterization. J Anat. 2005;207:381–390. doi:10.1111/j.1469-7580.2005.00460.x [CrossRef]
- Font RL, Zimmerman LE. Intrascleral smooth muscle in coloboma of the optic disk: electron microscopic verification. Am J Ophthalmol. 1971;72:452–457.
- Graether JM. Transient amaurosis in one eye with simultaneous dilation of retinal veins in association with a congenital anomaly of the optic nerve head. Arch Ophthalmol. 1963;70:342–345.