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| PHOTO ESSAY |
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| Year : 2023 | Volume
: 3
| Issue : 1 | Page : 188-189 |
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Anterior pyramidal cataract: A rare presentation
Sivadharshini Maheshbabu, Janani Rajendran
Aravind Eye Hospital, Madurai, Tamil Nadu, India
| Date of Submission | 19-Jul-2022 |
| Date of Acceptance | 16-Aug-2022 |
| Date of Web Publication | 20-Jan-2023 |
Correspondence Address:
Sivadharshini Maheshbabu
Aravind Eye Hospital, No. 1 Anna Nagar, Madurai, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ijo.IJO_1665_22
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 |
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 | |  |
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.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Khokhar SK, Pillay G, Dhull C, Agarwal E, Mahabir M, Aggarwal P. Pediatric cataract. Indian J Ophthalmol 2017;65:1340-9. [ PUBMED] [Full text] |
| 3. | 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. |
| 4. | Wheeler DT, Mullaney PB, Awad A, Zwaan J. Pyramidal anterior polar cataracts. Ophthalmology 1999;106:2362-7. |
| 5. | Kostick AM, Romanchuck KG, Beebe DC. Anterior pyramidal cataracts in Ehlers-Danlos syndrome. Can J Ophthalmol 1996;31:133-6. |
| 6. | Brown GC, Shields JA, Oglesby RB. Anterior polar cataracts associated with bilateral retinoblastoma. Am J Ophthalmol 1979;87:276. |
| 7. | Brown N, Ellis P. Anterior polar pyramidal cataract. Presenting as an anterior chamber foreign body. Br J Ophthalmol 1972;56:57-9. |
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