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 Table of Contents  
OPHTHA INSTA
Year : 2020  |  Volume : 32  |  Issue : 1  |  Page : 8-9

Cartwheel-like appearance of a variant of congenital zonular (lamellar) cataract


Department of Cataract and Iol and Pediatric and Squint Services, Aravind Eye Hospital, Pondicherry, India

Date of Submission18-Dec-2019
Date of Acceptance10-Jan-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Dr. Bharat Gurnani
Cornea Fellow, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Pondicherry - 605 007
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_93_19

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How to cite this article:
Gurnani B, Kaur K, Mohankumar A, Kannumsamy V, Gireesh P. Cartwheel-like appearance of a variant of congenital zonular (lamellar) cataract. Kerala J Ophthalmol 2020;32:8-9

How to cite this URL:
Gurnani B, Kaur K, Mohankumar A, Kannumsamy V, Gireesh P. Cartwheel-like appearance of a variant of congenital zonular (lamellar) cataract. Kerala J Ophthalmol [serial online] 2020 [cited 2020 May 28];32:8-9. Available from: http://www.kjophthal.com/text.asp?2020/32/1/8/282674




  Case Report Top


A 41-year-old male presented with complaints of defective vision in the right eye for the past 6 months. Anterior segment examination revealed developmental lamellar (spokes of a wheel) cataract [1] OD [Figure 1] and [Figure 2] involving the nucleus:
Figure 1: Image of the right eye showing developmental lamellar cataract in the form of spokes of a cartwheel

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Figure 2: Retroillumination image of the same eye showing developmental lamellar cataract in the form of spokes of a cartwheel

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  • Clinical sign Cartwheel-like appearance of a variant of congenital zonular (lamellar) cataract
  • Seen in – Rickets (first described in rickets) and tetany
  • Described by – Davidson and Horner, 1865
  • Why it occurs – Lamellar cataract is the most common type of developmental cataract producing visual impairment. Its effect on vision depends primarily on the density of its components, especially the nucleus.[2] The pathognomonic clinical appearance occurs due to concentric sharply demarcated lamellae of opacities around a central clear nuclear core with linear opacities such as spokes of a wheel (called riders) running toward the equator. Usually, inherited in autosomal dominant pattern, it may be caused by mutations in the heat-shock transcription factor-4 gene located at 16q21-q22.[3] Syndromic associations are rare
  • What else to examine – Look for nystagmus which is commonly associated with zonular cataract.


Answer to the spot diagnosis of previous issue:

Clinical condition is congenital lamellar ichthyosis (collodion baby). Main types of ichthyosis are ichthyosis vulgaris, lamellar ichthyosis, and epidermolytic hyperkeratosis. Ectropion in lamellar ichthyosis may respond to conservative management

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name 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.
Tijani M, Albaroudi N, El Bouaychi A, Laghmari M, Boutimzine N, Cherkaoui O. Congenital lamellar cataract. J Fr Ophtalmol 2017;40:527-8.  Back to cited text no. 1
    
2.
Gerth-Kahlert C, Michels R, Funk J, Gautschy U. Congenital lamellar cataract. JAMA Ophthalmol 2014;132:1122.  Back to cited text no. 2
    
3.
Bu L, Jin Y, Shi Y, Chu R, Ban A, Eiberg H, et al. Mutant DNA-binding domain of HSF4 is associated with autosomal dominant lamellar and Marner cataract. Nat Genet 2002;31:276-8.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]



 

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