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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 283-284

Iridolenticular coloboma with butterfly shaped chorioretinal coloboma

1 Department of Ophthalmology, Adesh Institute of Medical Sciences and Research, Bathinda, Punjab, India
2 Department of Ophthalmology, Gian Sagar Medical College and Hospital, Banur, Punjab, India

Date of Submission10-Jul-2021
Date of Acceptance16-Aug-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Rajwinder Kaur
Department of Opthalmology, Adesh Institute of Medical Sciences and Research, Bathinda, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1846_21

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Keywords: Coloboma, microcornea, nystagmus, retinal detachment

How to cite this article:
Gupta E, Kaur R, Khan B, Anupriya. Iridolenticular coloboma with butterfly shaped chorioretinal coloboma. Indian J Ophthalmol Case Rep 2022;2:283-4

How to cite this URL:
Gupta E, Kaur R, Khan B, Anupriya. Iridolenticular coloboma with butterfly shaped chorioretinal coloboma. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:283-4. Available from: https://www.ijoreports.in/text.asp?2022/2/1/283/334916

We report an interesting case of iridolenticular coloboma with butterfly-like chorioretinal coloboma. Light perception was absent in the right eye (RE); visual acuity in the left eye (LE) was 20/200. Slit-lamp biomicroscopy revealed RE nystagmus, microcornea, typical iris coloboma, neovascularization of iris in pupillary zone and ciliary zone, 360° pupillary ruff atrophy, congenital cataract, polychromatic crystals in the anterior chamber (AC), angles in the pupillary area and anterior vitreous cavity, but posterior segment details could not be analyzed. LE revealed nystagmus, microcornea, iris coloboma, minimal congenital cataract superiorly, and shallow AC. Ciliary processes and lens coloboma were identified [Figure 1]. Fundoscopy revealed large chorioretinal coloboma involving macula with intercalary membrane adjoining optic nerve head giving it a butterfly-like appearance [Figure 2]. Intraocular pressure was 10 mm Hg (RE) and 26 mm Hg (LE). Central corneal thickness (CCT) was 653 m (RE) was 674 m (LE). Prophylactic green laser barrage (spot size = 100 m and power = 150 mW) sparing macula was done and the patient was advised for surgery in the left eye.
Figure 1: (a) Microcornea, typical iris coloboma, pupillary ruff atrophy, and congenital cataract, polychromatic crystals in anterior chamber and angle, neovascularization of iris in the right eye. (b) microcornea, typical iris coloboma in left eye. (c) and (d) gonioscopy of left eye: ciliary processes and lens coloboma

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Figure 2: (a) Slit-lamp fundus examination with Goldmann 3 mirror through central mirror revealed butterfly-shaped retinochoroidal coloboma with intercalary membrane, captured with 7SE iPhone camera (inverse image). (b) SD–OCT showing “hump effect.” (c) B-scan ultrasonography showing closed funnel RD in the right eye. (d) B-Scan ultrasonography showing well-defined excavated area with posterior hump and absence of retinochoroidal layer, suggestive of fundal coloboma adjacent to ONH with superonasal traction suggestive of superonasal RD in the left eye

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  Discussion Top

Choroidal coloboma is a congenital defect caused by defective closure of embryonic fissure which normally closes during the sixth week of gestation.[1] The incidence of congenital colobomata is 0.5–0.7/10,000 births.[2] Histologically, RPE, choroid, and retina are poorly developed in the area of coloboma. The continuation of the neurosensory retina into the colobomatous area is termed as intercalary membrane (ICM). Detachment or break in ICM usually leads to RD in these colobomatous areas due to a schisis-like split of the neurosensory retina at the margins of coloboma.[1] This is seen in our case where one eye was lost to chronic RD and start of superonasal RD in the other eye. Uhumwangho et al.[3] reported that retinal detachment occurred in 24.1% of eyes that did not have prophylactic laser photocoagulation compared to 2.9% of eyes that received prophylactic photocoagulation.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Gopal L, Khan B, Jain S, Vilakkumadadhil S, Prakash VS. A clinical and optical coherence tomography study of the margins of choroidal colobomas. Ophthalmology 2007;114:571-80.  Back to cited text no. 1
Hornby SJ, Adolph S, Gilbert CE, Dandona L. Visual acuity in children with coloboma: Clinical features and a new phenotypic classification system. Ophthalmology 2000;107:511-20.  Back to cited text no. 2
Uhumwangho OM, Jalali S. Chorioretinal coloboma in a paediatric population. Eye 2014;28:728-33.  Back to cited text no. 3


  [Figure 1], [Figure 2]


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