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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 188-189

Anterior pyramidal cataract: A rare presentation

Aravind Eye Hospital, Madurai, Tamil Nadu, India

Date of Submission19-Jul-2022
Date of Acceptance16-Aug-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Sivadharshini Maheshbabu
Aravind Eye Hospital, No. 1 Anna Nagar, Madurai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1665_22

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Keywords: Anterior pyramidal cataract, anterior segment dysgenesis, nystagmus

How to cite this article:
Maheshbabu S, Rajendran J. Anterior pyramidal cataract: A rare presentation. Indian J Ophthalmol Case Rep 2023;3:188-9

How to cite this URL:
Maheshbabu S, Rajendran J. Anterior pyramidal cataract: A rare presentation. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 9];3:188-9. Available from: https://www.ijoreports.in/text.asp?2023/3/1/188/368156

An eight-year-old male child presented with complaints of shaking movements of the eyes, noted since four months of age. An ophthalmic evaluation revealed the best-corrected visual acuity (BCVA) to be 4/60 and 6/36p in the right and left eye, respectively. Anterior segment examination showed partial aniridia with persistent pupillary membrane and pendular nystagmus with jerk component in both eyes and anterior pyramidal cataract in the right eye [Figure 1]a and [Figure 1]b. Ultrasound biomicroscopy (UBM) and anterior segment optical coherence tomography (AS-OCT) images were taken of the right eye [Figure 1]c and [Figure 1]d. Fundus examination was normal except for a dull foveal reflex. The ancillary ophthalmic evaluation showed a short axial length of 19.71 mm and 19.25 mm in the right and left eye, respectively. OCT of both eyes showed an epiretinal membrane. When subjected to electroretinogram (ERG), as the vision loss was disproportionate to the clinical findings, the patient was diagnosed with achromatopsia and was treated with best refractive correction and tinted glasses.
Figure 1: (a and b) Slit-lamp photography of the right eye showing anterior pyramidal cataract. (c) Ultrasound biomicroscopy (UBM) image of the right eye depicting anterior pyramidal cataract. (d) AS-OCT image of the right eye showing anterior pyramidal cataract

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  Discussion Top

An anterior polar pyramidal cataract is a rare congenital cataract. It is usually bilateral and can have dominant inheritance.[1] They can progress and become visually significant and becomes a point of concern. They may be associated with other ocular abnormalities like aniridia, persistent pupillary membrane, and remnants of persistent fetal vasculature.[2] The ocular phenotype involving partial or complete absence of the iris, anterior polar cataract, microphthalmia, optic nerve, foveal hypoplasia, nystagmus, and glaucoma are all common associations of PAX6 heterozygous mutations.[3] Histologically, they consist of hyperplastic lens epithelium in a collagenous matrix.[4] Anterior pyramidal cataract is also reported in patients with retinoblastoma and Ehlers–Danlos syndrome.[5],[6] These patients present to us with some form of refractive errors that can cause amblyopia, strabismus, and nystagmus. Rarely, the pyramid may spontaneously dehisce into the anterior chamber and may present as an anterior chamber foreign body.[7] They need regular follow-up for the prevention of long-term visual complications. We present a case of congenital nystagmus in both eyes, with developmental cataract and anterior segment dysgenesis. Regular follow-up is advocated to prevent further vision loss with glaucoma and cataract progression.

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

Amaya L, Taylor D, Russell-Eggitt I, Nischal KK, Lengyel D. The morphology and natural history of childhood cataracts. Surv Ophthalmol 2003;48:125-44.  Back to cited text no. 1
Khokhar SK, Pillay G, Dhull C, Agarwal E, Mahabir M, Aggarwal P. Pediatric cataract. Indian J Ophthalmol 2017;65:1340-9.  Back to cited text no. 2
[PUBMED]  [Full text]  
Chauhan BK, Medsinge A, Baumgartner MP, Scanga HL, Kamakari S, Gajdosova E, et al. Case series: Pyramidal cataracts, intact irides and nystagmus from three novel PAX6 mutations. Am J Ophthalmol Case Rep 2018;10:172-9.  Back to cited text no. 3
Wheeler DT, Mullaney PB, Awad A, Zwaan J. Pyramidal anterior polar cataracts. Ophthalmology 1999;106:2362-7.  Back to cited text no. 4
Kostick AM, Romanchuck KG, Beebe DC. Anterior pyramidal cataracts in Ehlers-Danlos syndrome. Can J Ophthalmol 1996;31:133-6.  Back to cited text no. 5
Brown GC, Shields JA, Oglesby RB. Anterior polar cataracts associated with bilateral retinoblastoma. Am J Ophthalmol 1979;87:276.  Back to cited text no. 6
Brown N, Ellis P. Anterior polar pyramidal cataract. Presenting as an anterior chamber foreign body. Br J Ophthalmol 1972;56:57-9.  Back to cited text no. 7


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