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PHOTO ESSAY
Year : 2020  |  Volume : 68  |  Issue : 7  |  Page : 1445-1446

A rare case of temporal atypical retinochoroidal coloboma associated with posterior embryotoxon


Vitreoretina and Uveitis Services, L V Prasad Eye Institute, GMRV Campus, Visakhapatnam, Andhra Pradesh, India

Date of Submission30-Nov-2019
Date of Acceptance19-Feb-2020
Date of Web Publication25-Jun-2020

Correspondence Address:
Dr. Bhavik Panchal
Vitreoretina and Uveitis Services, L V Prasad Eye Institute, GMRV Campus, Visakhapatnam - 530 040, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2213_19

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  Abstract 


Keywords: Atypical coloboma, lens coloboma, posterior embryotoxon, retinochoroidal coloboma


How to cite this article:
Gulati M, Panchal B, Pathengay A. A rare case of temporal atypical retinochoroidal coloboma associated with posterior embryotoxon. Indian J Ophthalmol 2020;68:1445-6

How to cite this URL:
Gulati M, Panchal B, Pathengay A. A rare case of temporal atypical retinochoroidal coloboma associated with posterior embryotoxon. Indian J Ophthalmol [serial online] 2020 [cited 2020 Jul 10];68:1445-6. Available from: http://www.ijo.in/text.asp?2020/68/7/1445/287551



A 17-year-old female presented to us for routine ophthalmological evaluation. Her best-corrected visual acuity was 20/20, N06 in both the eyes. No previous history of ocular trauma or surgery was noted. Right eye anterior segment examination showed two clock hours (CH) of a prominent white line on the endothelial side just inside the temporal limbus, suggestive of posterior embryotoxon [Figure 1]a. Gonioscopy showed two CH of angle recession temporally. Temporal 3 CH of lens pseudocoloboma was noted as well [Figure 1]b. No iris coloboma was seen. The fundus examination showed a healthy disc and macula. Peripheral fundus showed temporal well defined glistening white lesion with minimal excavation and pigmented margins, suggestive of atypical retinochoroidal coloboma [Figure 1]c and intact retina in inferior quadrant [Figure 1]d. The left eye ocular examination was unremarkable.
Figure 1: Slit lamp photograph of right eye showing (a) temporal posterior embryotoxon (black arrow head); (b) temporal lens pseudocoloboma; (c) Fundus showing temporal peripheral retinochoroidal atypical coloboma; and (d) healthy optic nerve and intact inferior retina

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  Discussion Top


Typical retinochoroidal colobomas are located in the inferonasal quadrant due to the failure of embryonic fissure in the ventral side of the optic cup to close during the fifth week of gestation.[1] Embryological basis of atypical colobomas can be explained by vertebrate eye model that depicts a transient groove in the dorsal side of the optic cup called the superior ocular sulcus (SOS).[2] Hocking et al.[2] have shown that failure to close the SOS leads to superior colobomas in adult zebrafish. Several other theories explaining the formation of atypical colobomas are rotation of the fetal fissure, intrauterine inflammatory process, and developmental anomalies of the neural ectoderm, namely, faulty differentiation, and imbalance of growth in various portions of the outer and inner layer of the optic cup.[3],[4]

Posterior embryotoxon has been reported in association with retinochoroidal coloboma in Axenfeld–Reigers syndrome [5] and cholestatic disorders.[6]

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.
Onwochei BC, Simon JW, Bateman JB, Couture KC, Mir E. Ocular colobomata. Surv Ophthalmol 2000;45:175-94.  Back to cited text no. 1
    
2.
Hocking JC, Famulski JK, Yoon KH, Widen SA, Bernstein CS, Koch S, et al. FORGE Canada Consortium. Morphogenetic defects underlie Superior Coloboma, a newly identified closure disorder of the dorsal eye. PLoS Genet 2018;14:e1007246.  Back to cited text no. 2
    
3.
Barber AN. Embryology of the Human Eye. St. Louis: CV Mosby; 1955. p. 50-63.  Back to cited text no. 3
    
4.
Mann I, Ross JA. A case of atypical coloboma associated with abnormal retinal vessels. Br J Ophthalmol 1929;13:608-12.  Back to cited text no. 4
    
5.
Rennie CA, Chowdhury S, Khan J, Rajan F, Jordan K, Lamb RJ, et al. The prevalence and associated features of posterior embryotoxon in the general ophthalmic clinic. Eye (Lond) 2005;19:396-9.  Back to cited text no. 5
    
6.
Fahnehjelm KT, Fischler B, Martin L, Nemeth A. Occurrence and pattern of ocular disease in children with cholestatic disorders. Acta Ophthalmol 2011;89:143-50.  Back to cited text no. 6
    


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