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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 465

Developmental delay, vision impairment, and the “double-ring sign”

1 Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
2 Pediatric Neurology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India
3 Department of Ophthalmology, Advanced Eye Centre, PGIMER, Chandigarh, India
4 Department of Radiodiagnosis and Imaging (Section of Neuroimaging and Interventional Radiology), PGIMER, Chandigarh, India

Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Lokesh Saini
Department of Pediatrics, Advanced Pediatric Centre, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_475_21

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How to cite this article:
Kariyappa VM, Singanamalla B, Chaurasia S, Madaan P, Saini L, Vyas S. Developmental delay, vision impairment, and the “double-ring sign”. Indian J Ophthalmol Case Rep 2021;1:465

How to cite this URL:
Kariyappa VM, Singanamalla B, Chaurasia S, Madaan P, Saini L, Vyas S. Developmental delay, vision impairment, and the “double-ring sign”. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:465. Available from: https://www.ijoreports.in/text.asp?2021/1/3/465/320141

An 11-month-old boy presented with global developmental delay, poor eye-contact, and abnormal eye movements. Examination revealed normal head size, roving eye movements, absent pupillary reflex, and central hypotonia. Fundus examination revealed double-ring sign (DRS; [Figure 1]). Rest of the systemic examination was unremarkable. Neuroimaging revealed bilateral optic nerve hypoplasia (ONH), absent septum pellucidum, and abnormal configuration of sella with small pituitary [Figure 2]. Visual evoked potentials were absent. Multisensory stimulation and rehabilitative measures were initiated. DRS, along with small optic disc and tortuous vessels, is the hallmark of ONH.[1] When ONH occurs in association with midline brain defects, septo-optic dysplasia (SOD) should be thought of. Misdiagnosis of SOD may be life-threatening, especially in the presence of pituitary hormone abnormalities that can occur at any age and are important to recognize to avoid acute decompensation.[2]
Figure 1: Fundus findings in the index patient. Fundus pictures showing a b a small pale tilted and crowded optic disc along with optic nerve hypoplasia in both right (a) and left (b) eye. Note decreased ratio of disc diameter to disc-macula distance (OD 0.25; OS 0.26). Both optic discs appear to be surrounded by double ring (double-ring sign) prominent on superior side of disc. The outer ring represents the normal junction between the sclera and the lamina cribrosa; the inner ring represents the abnormal extension of retina and retinal pigment epithelium over the outer portion of the lamina cribrosa

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Figure 2: Brain and orbit MRI of the index patient. Coronal (`) and axial (d) T2-weighted images showing hypoplasia of intra-orbital part of bilateral optic nerves (white arrows), absence of septum-pellucidum (black arrows), and squared frontal horns of lateral ventricles. Sagittal (c) T2-weighted image shows small, abnormally configured sella with small pituitary gland (white arrow)

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Authors thank the parents of the patient for images and adding to the literature.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Garcia-Filion P, Borchert M. Optic nerve hypoplasia syndrome: A review of the epidemiology and clinical associations. Curr Treat Options Neurol 2013;15:78-89.  Back to cited text no. 1
Brodsky MC, Conte FA, Taylor D, Hoyt CS, Mrak RE. Sudden death in septo-optic dysplasia. Report of 5 cases. Arch Ophthalmol 1997;115:66-70.  Back to cited text no. 2


  [Figure 1], [Figure 2]


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