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 Table of Contents  
PHOTO ESSAY
Year : 2022  |  Volume : 34  |  Issue : 2  |  Page : 176-177

Bilateral lens coloboma in child with Marfan's syndrome treated with lens surgery


Department of Pediatric Ophthalmology and Strabismus, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission18-May-2021
Date of Decision04-Oct-2021
Date of Acceptance26-May-2021
Date of Web Publication30-Aug-2022

Correspondence Address:
Dr. Sandra C Ganesh
C-2, Staff Quarters, Aravind Eye Hospital, Avinashi Road, Coimbatore - 641 014, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_120_21

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  Abstract 


Lens coloboma can occur with ocular or systemic associations like Marfan's and Stickler's syndrome. Causes of poor vision include refractive error, anisometropia and amblyopia. Visual rehabilitation and surgery is technically challenging in these patients. Here we report a child with progressive defective vision with Marfans syndrome and lens coloboma who underwent successful surgery with intraocular lens (IOL) placement. The bag was stabilised with capsule tension ring and iris hooks, and the IOL was further stabilised with optic capture. Postoperatively, the child regained good vision in the operated eye with very good centration of the IOL at three month follow up visit.

Keywords: Capsular tension ring, lens coloboma, Marfan's syndrome


How to cite this article:
Ganesh SC, Krishnababu, Elizabeth S, Jogitha. Bilateral lens coloboma in child with Marfan's syndrome treated with lens surgery. Kerala J Ophthalmol 2022;34:176-7

How to cite this URL:
Ganesh SC, Krishnababu, Elizabeth S, Jogitha. Bilateral lens coloboma in child with Marfan's syndrome treated with lens surgery. Kerala J Ophthalmol [serial online] 2022 [cited 2022 Dec 4];34:176-7. Available from: http://www.kjophthal.com/text.asp?2022/34/2/176/355042




  Introduction Top


Lens coloboma in Marfan's syndrome is infrequently reported.[1],[2] This is due to defective or absent development of the zonules and ciliary body.[3] Surgeryis technically challenging and successful, intraocular lens (IOL) placement depends on coloboma's extent and usage of capsular supportive devices like iris hooks, capsular tension ring (CTR), Cionnis ring or segments. We present the successful visual rehabilitation following IOL placement in the left eye of a child with the above condition.


  Case Report Top


A 13-year-old boy [Figure 1] with Marfans syndrome presented with progressively decreasing vision in his left eye of unknown duration. His best corrected visual acuity (BCVA) was 20/40 (−0.75/−[email protected]) and 20/125 (−8.0/−[email protected]) in his right eye (RE) and left eyes (LE), respectively. Slit lamp evaluation under mydriasis showed clear lens with coloboma extending from 12 to 2 o'clock (RE) and 9–1 o'clock (LE) [Figure 2] with few intact zonules. As left eye BCVA was low, we decided on surgery with IOL placement, after explaining possibility of subnormal visual outcome due to amblyopia. Following lens aspiration the capsular bag was stabilised with CTR (Aurolab, Madurai, India) [Figure 3], inserting which was technically challenging due to small and irregular colobomatous bag. Three iris hooks were used to stabilise the bag prior to three piece acrylic IOL (Alcon Labs, Texas, USA) implanted in sulcus with optic capture [Figure 4] and [Figure 5].
Figure 1: (a) Photograph showing a 13-year-old male with tall and thin stature. He had disproportionately long arms (b) with hyper extensibility of fingers

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Figure 2: Picture showing lens coloboma in left eye from 9 to 1 o' clock position with intact zonules. The vision in his left eye was 20/125 (−8.0/−[email protected])

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Figure 3: The capsular bag was stabilised with the capsular tension ring (Aurolab, Madurai, India), prior to aspiration of the lens cortex

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Figure 4: Three Iris hooks were used for dilatation and stabilisation of capsular bag prior to insertion of intraocular lens (Alcon Labs, Texas, USA)

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Figure 5: Picture showing a well centred Intra ocular lens with capsular tension ring in the bag. Best corrected vision has improved from 20/125 to 20/63 with negligible astigmatism postoperatively (−[email protected])

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Postoperatively, visual acuity in his LE improved to 20/63 (−[email protected]) with well centered IOL.


  Discussion Top


Visual rehabilitation is technically challenging due to capsular fornix aspiration, zonular dialysis extension, vitreous herniation and IOL decentration.[3] Lens coloboma in Marfan's poses the difficulty of a small sized capsular bag with notching, in addition to the difficulties encountered in subluxated lens management. Also there exists a possibility of late decentation of IOL placed entirely in bag due to progressive myopia. Hence we decided on bag sulcus fixation of IOL, to aid in long term stability and centration.

The improvement in BCVA post surgery implies that the high myopia causing vision loss may have occurred beyond the amblyogenic years of his life.

IOL implantation can thus be an effective option to provide optimal visual rehabilitation in eyes with progressive vision loss in lens coloboma, in expert hands.

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 understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gupta G, Goyal P, Malhotra C, Jain AK. Bilateral lens coloboma associated with Marfan syndrome. Indian J Ophthalmol 2018;66:1192.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Thapa BB, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2014-207112.  Back to cited text no. 2
    
3.
Wang JK, Ma SH. Lens coloboma treated with lens surgery. BMJ Case Rep. 2015 Sep 29;2015:bcr2015210559. doi: 10.1136/bcr-2015-210559. PMID: 26420693; PMCID: PMC4593262.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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