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   Table of Contents      
OPHTHALMIC IMAGE
Year : 2019  |  Volume : 67  |  Issue : 7  |  Page : 1187

Double ring sign in optic nerve head hypoplasia


Department of Vitreo-Retinal Services, Aravind Eye Hospital, Madurai, Tamil Nadu, India

Date of Web Publication25-Jun-2019

Correspondence Address:
Dr. Chitaranjan Mishra
Department of Vitreo-Retinal Services, Aravind Eye Hospital, Anna Nagar, Madurai, Tamil Nadu - 625 020
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1622_18

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How to cite this article:
Deb AK, Kannan NB, Ramasamy K, Mishra C. Double ring sign in optic nerve head hypoplasia. Indian J Ophthalmol 2019;67:1187

How to cite this URL:
Deb AK, Kannan NB, Ramasamy K, Mishra C. Double ring sign in optic nerve head hypoplasia. Indian J Ophthalmol [serial online] 2019 [cited 2019 Jul 16];67:1187. Available from: http://www.ijo.in/text.asp?2019/67/7/1187/261003



Double ring sign of optic nerve head is a characteristic fundus finding in cases with optic nerve head hypoplasia (ONH).[1] ONH has been associated with congenital intracranial anomalies like thinning of corpus callosum, absence of septum pellucidum and pituitary ectopia.[2],[3] A 6-year-old male child without any significant perinatal or endocrinological history, presented with vision OD 6/6 and OS 6/18, normal anterior segments and unilateral ONH in OS [Figure 1]a. Ratio of disc-macula distance to disc-diameter i.e., DM/DD was 3.2 (more than 3 is suggestive of ONH[4]). The MRI revealed hypoplasia of left optic nerve [Figure 1]b and thinning of corpus callosum [Figure 1]c. 'De Morsier's syndrome' was ruled out due to normal endocrinological function and normal septum pellucidum. ONH has an incidence of 1/2287 live births, and 2.4/100,000 in children less than 19-year-old.[5] The differential diagnosis of ONH include optic nerve atrophy, optic nerve coloboma, morning glory anomaly, tilted disc syndrome, and glaucoma.
Figure 1: Fundus picture of left eye (a) depicting characteristic double ring sign (black arrows) of optic nerve head, coronal section of MRI (b) showing mild hypoplasia of left optic nerve (white arrow), sagittal section of MRI (c) showing thinning of distal body (white triangle) and splenium (white arrow) of corpus callosum

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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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Kaur S, Jain S, Sodhi HBS, Rastogi A, Kamlesh. Optic nerve hypoplasia. Oman J Ophthalmol 2013;6:77-82.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
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. 2
    
3.
Garcia ML, Ty EB, Taban M, David RA, Rogers D, Traboulsi EI. Systemic and ocular findings in 100 patients with optic nerve hypoplasia. J Child Neurol 2006;21:949-56.  Back to cited text no. 3
    
4.
Alvarez E, Wakakura M, Khan Z, Dutton GN. The disc-macula distance to disc diameter ratio: A new test for confirming optic nerve hypoplasia in young children. J Pediatr Ophthalmol Strabismus 1988;25:151-4.  Back to cited text no. 4
    
5.
Mohney BG, Young RC, Diehl N. Incidence and associated endocrine and neurologic abnormalities of optic nerve hypoplasia. JAMA Ophthalmol 2013;131:898-902.  Back to cited text no. 5
    


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