Bilateral morning glory disc anomaly with unilateral internal carotid artery agenesis

Koyel Chakraborty; Sucheta Parija; Bhagabat Nayak; CS Lalitha

doi:10.4103/ijo.IJO_2247_21

PHOTO ESSAY

Year : 2022 | Volume : 2 | Issue : 2 | Page : 592-593

Bilateral morning glory disc anomaly with unilateral internal carotid artery agenesis

Koyel Chakraborty, Sucheta Parija, Bhagabat Nayak, CS Lalitha
Department of Ophthalmology, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India

Date of Submission	29-Aug-2021
Date of Acceptance	18-Nov-2021
Date of Web Publication	13-Apr-2022

Correspondence Address:
Sucheta Parija
Department of Ophthalmology, AIIMS Bhubaneswar, Sijua, Bhubaneswar - 751 019, Odisha
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2247_21

Abstract

Keywords: Internal carotid artery agenesis, morning glory syndrome, posterior staphyloma

How to cite this article:
Chakraborty K, Parija S, Nayak B, Lalitha C S. Bilateral morning glory disc anomaly with unilateral internal carotid artery agenesis. Indian J Ophthalmol Case Rep 2022;2:592-3

How to cite this URL:
Chakraborty K, Parija S, Nayak B, Lalitha C S. Bilateral morning glory disc anomaly with unilateral internal carotid artery agenesis. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 18];2:592-3. Available from: https://www.ijoreports.in/text.asp?2022/2/2/592/342938

A 28-year-old female presented with decreased visual acuity in right eye since childhood. The best-corrected visual acuity was perception of light with projection of rays accurate in all quadrants in right eye and 20/20 in left eye. Retinoscopy reported myopia of 22.0D. Pupillary reaction was ill sustained in the right eye and normal in the left eye. Fundus showed bilateral excavated optic disc with central tuft of pinkish-white glial tissue, circumpapillary atrophy, and elevated neuroretinal rim with multiple fine vessels passing radially. There was optic atrophy with posterior staphyloma and macular striae in right eye [Figure 1]a and [Figure 1]b. B-scan revealed axial length of 30 mm in right eye [Figure 1]c and [Figure 1]d. Optical coherence tomography showed a decrease in retinal nerve fiber layer in left eye [Figure 1]e. Contrast-enhanced computed tomography with angiography of brain showed absent right internal carotid artery (ICA) with posterior cerebral artery aneurysm [Figure 1]f. Patient was advised clipping of aneurysm by neurosurgeon but denied surgery.

Figure 1: (a and b): Colored fundus photograph of right eye and left eye showing morning glory disc anomaly with large disc, central glial tuft, radially oriented retinal vessels, and peripapillary pigmentary changes along with disc pallor and peripapillary staphyloma (extent marked by blue arrows) in right eye. (c, d, e): Ultrasonography B Scan of right eye and left eye shows excavation of disc along with peripapillary excavation suggestive of posterior staphyloma in right eye. Optical coherence tomography showed a decrease in retinal nerve fiber layer in left eye. (f): CT angiography brain showing the presence of only left internal carotid artery (green arrow; absent in right side), dilated tortuous P2 segment of right PCA (yellow arrow), dilated tortuous right posterior communicating artery (blue arrow), hypoplastic A1 segment of the right anterior cerebral artery (red arrow)

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Discussion

Morning glory disc anomaly (MGDA) was first described by Kinder in 1970 and derives its name from the tropical flower.^[1],[2] Prevalence is 2.6 cases per 1 million population as reported in a Swedish study.^[1] Mostly, it is unilateral but few are bilateral with female preponderance.^[2],[3],[4] Though it is sporadic, association with PAX6 mutation has been reported.^[2] The pathogenesis is unclear, but attributed to primary mesenchymal abnormality due to a neuroectodermal induction defect resulting in incomplete closure of posterior sclera and aplasia of lamina cribrosa.^[3] Ocular associations include nonrhegmatogenous retinal detachment, lid hemangioma, hyaloid remnants, strabismus, nystagmus, and Horner's syndrome.^[2],[4] Neurological and facial anomalies are also associated. Vision is poor and only 30% of cases achieve 20/40 vision. MGDA needs to be diagnosed correctly and clinically differentiated from optic nerve coloboma because of different life-threatening systemic associations, which requires prompt intervention.^[2] Hanson et al. were the first to report an association between this anomaly and carotid circulation.^[5] The vascular anomalies ranges from aplasia of the circle of Willis or segmental narrowing to complete agenesis of the ICA and can lead to ischemia, hemorrhage, or seizures.^[4] To the best of our knowledge, unilateral ICA anomaly in bilateral MGDA has been reported rarely. To conclude, early detection and low visual rehabilitation are essential in treating amblyopia along with neuroimaging for multidisciplinary management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Lee BJ, Traboulsi EI. Update on the morning glory disc anomaly. Ophthalmic Genet 2008;29:47–52.
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5.	Hanson MR, Price RL, Rothner AD, Tomsak RL. Developmental anomalies of the optic disc and carotid circulation. A new association. J Clin Neuroophthalmol 1985;5:3-8.