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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 3  |  Page : 673-675

Cataract surgery outcomes in patients with lenticonus: A case series


Department of Cataract and IOL Services, Aravind Eye Hospital, Madurai, Tamil Nadu, India

Date of Submission26-Feb-2022
Date of Acceptance26-Apr-2022
Date of Web Publication16-Jul-2022

Correspondence Address:
Dr. Madhu Shekhar
Chief, Cataract and IOL Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai - 625 020, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_542_22

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  Abstract 


Cataract surgery in lenticonus is challenging because of increased fragility of the lens capsule. In this case series, we report three different modes of cataract surgery in 15 eyes of ten patients with lenticonus. Six patients had Alport syndrome, out of which two were diagnosed after ophthalmic examination. Two patients had posterior lenticonus, among which one had persistent fetal vasculature. There was a significant improvement in visual acuity following cataract surgery. Therefore, cataract surgery can be safely performed in patients with lenticonus, and ophthalmologists can retard the progression of renal failure by early diagnosis.

Keywords: Alport syndrome, cataract surgery, lenticonus


How to cite this article:
Rashme VL, Prasad R S, Nagu K, Shekhar M. Cataract surgery outcomes in patients with lenticonus: A case series. Indian J Ophthalmol Case Rep 2022;2:673-5

How to cite this URL:
Rashme VL, Prasad R S, Nagu K, Shekhar M. Cataract surgery outcomes in patients with lenticonus: A case series. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 17];2:673-5. Available from: https://www.ijoreports.in/text.asp?2022/2/3/673/351201



Lenticonus is a rare abnormality of the crystalline lens, and is essentially, a clinical diagnosis made by bio-microscopic examination [Figure 1]a and [Figure 1]b; [Figure 2]a. Morphologically, it is classified into anterior and posterior lenticonus. Anterior lenticonus is most commonly associated with Alport syndrome. Characteristic ocular features are anterior lenticonus, dot-and-fleck retinopathy, and posterior polymorphous corneal dystrophy (PPMD).[1] Lenticonus usually appears after the second decade of age; progressive visual loss is because of progressive lenticular myopia, and surgery is often indicated in patients with lenticonus. Surgery can be challenging because of increased fragility of the lens capsule.[2]
Figure 1: (a) Slit lamp photograph of anterior lenticonus. (b) Slit lamp photograph of anterior and posterior lenticonus

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Figure 2: (a) “Oil droplet reflex” on retro-illumination. (b) Post-operative slit lamp photograph with foldable IOL

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This paper presents cataract surgical outcomes in 15 eyes of ten patients with lenticonus. All surgeries were performed by three surgeons with an experience of more than 5000 cataract surgeries each. These cases show that lenticonus is not limited to Alport syndrome and it can occur in healthy individuals.


  Case Reports Top


Case 1

A 30-year-old male with Alport syndrome presented with complaints of decreased visual acuity in both eyes (BE). The best corrected visual acuity (BCVA) in BE was 3/60. Ocular examination revealed anterior and posterior lenticonus in BE with posterior sub-capsular cataract. Fundus examination was normal. He had 3.25D against the rule (ATR) astigmatism in the right eye (RE) and 1.5D ATR astigmatism in the left eye (LE). Femto laser-assisted cataract surgery (FLACS) was performed in BE, and Toric intra-ocular lens (IOL) was implanted in RE and monofocal IOL with arcuate keratotomy in LE. The BCVA at 1 month post surgery was 6/9 in BE.

Case 2

A 23-year-old Alport syndrome patient presented with decreased visual acuity in BE. The BCVA was 6/18 in RE and 6/12 in LE. Ocular examination in BE revealed a vesicular appearance of the cornea, suggestive of PPMD and anterior lenticonus. The fundus examination revealed a lamellar macular hole (LMH) in RE, which was confirmed with optical coherence tomography (OCT). He underwent phacoemulsification with foldable IOL in RE. The post-surgery BCVA in RE was 6/6.

Case 3

An 8-year-old boy presented with decreased visual acuity in RE. His BCVA was 6/60 in RE and 6/24 in LE. Ocular examination revealed BE posterior lenticonus, and RE had associated persistent fetal vasculature (PFV). He underwent manual small incision cataract surgery (MSICS) in RE with implantation of three-piece IOL in ciliary sulcus because of intra-operative posterior capsule rupture (PCR). Post-operatively, at 1 month, his vision in RE improved to 6/24. Vitro-retinal consultation was obtained, and he was advised observation for PFV.

Cases 4 and 5

Two female patients, 18 and 33 years old, complained of defective vision in BE. The BCVAs were 6/36 in RE and 6/24 in LE in one patient and 6/18 in RE and 6/12 in LE in the other patient. Ocular examination revealed anterior and posterior lenticonus in BE. They both had a history of deafness (sensori-neural). Urine analysis revealed hematuria and proteinuria. They were referred to a nephrologist and diagnosed to have Alport syndrome. They underwent phacoemulsification in BE with implantation of foldable IOL. The BCVA post-operatively was 6/9 in RE and 6/6 in LE in the first patient and 6/6 in BE in the second patient.

Case 6

A 14-year-old boy presented with unilateral defective vision in LE. His BCVA was 6/6 in RE and 3/60 in LE. Ocular examination revealed posterior lenticonus in LE. He underwent MSICS with three-piece PMMA IOL in ciliary sulcus because of intra-operative PCR. His vision improved to 6/9 in LE.

Cases 7 and 8

Two patients with Alport syndrome (25 years, male; 24 years, female) presented with decreased visual acuity with BCVAs of 6/12 in RE and 6/36 in LE and 6/60 in RE and 6/12 in LE, respectively. Ocular examination revealed anterior and posterior lenticonus in BE. They underwent phacoemulsification with foldable IOL. At a 1-month post-operative period, the BCVA was 6/6 in BE.

Case 9

A 51-year-old male patient presented with decreased visual acuity in LE for 7 years. The BCVA was 6/6 in RE and 6/60 in LE. Ocular examination revealed anterior lenticonus in LE. His renal parameters were normal. He underwent phacoemulsification in LE with foldable IOL. The post-operative BCVA was 6/6.

Case 10

A 66-year-old male presented with decreased visual acuity in BE. The BCVA was 6/36 in RE and 6/18P in LE. Ocular examination revealed climate droplet keratopathy and senile immature cataract with pseudo-exfoliation on the anterior lens capsule associated with anterior lenticonus in BE. No history of deafness and renal parameters were within normal limits. He underwent MSICS with implantation of PMMA IOL in RE. There was intra-operative PCR with vitreous disturbance, which was managed with automated anterior vitrectomy. At a 1-month post-operative period, his vision in RE was 6/24.


  Discussion Top


We present ten patients with lenticonus, of which six had Alport syndrome [Table 1]. Two patients were diagnosed with Alport syndrome after ophthalmic examination. One patient with Alport syndrome had associated PPMD and LMH (case 2). Two patients had only posterior lenticonus, among which one patient had PFV, and they both had intra-operative PCR. Kekunnaya et al.[3] showed 44% of intra-operative PCR among patients with posterior lenticonus. Anterior lenticonus can present in healthy individuals; in our case series, we reported two such patients similar to Setter et al.[2]
Table 1: Demographic and clinical data of patients with lenticonus

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We describe ten eyes of six patients with lenticonus who underwent continuous curvilinear capsulorhexis (CCC) and phacoemulsification with implantation of foldable IOL in the bag [Figure 2]b and achieved acceptable BCVAs [Table 2]. Our results are comparable to Zare et al.[4] CCC was started from the mid-periphery rather than conventional CCC, which is initiated at the center of the capsule. This change is important to avoid inadvertent spontaneous rupture of the lens capsule, which is thin and fragile at the poles compared to the periphery in lenticonus. None of the cases in our series had peripheral extension of CCC. We performed FLACS in two eyes. Considering increased fragility or elasticity of the lens capsule in lenticonus, real-time OCT with Femto laser can help in achieving well-centered capsulotomy avoiding the thinnest conical part of the anterior lens capsule.[5]
Table 2: Analysis of pre- and post-operative visual acuity

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  Conclusion Top


In conclusion, we ophthalmologists can retard the progression of disease to renal failure by early diagnosis and appropriate referral. Phacoemulsification is a safe and effective procedure in patients with anterior lenticonus where CCC without peripheral extension is possible starting slightly away from the center of the anterior capsule to avoid radial tears. FLACS is a good and safe option for performing cataract surgery in patients with lenticonus if there are no financial constraints.

Prior publication

Nil.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Colville DJ, Savige J. Alport syndrome. A review of the ocular manifestations. Ophthalmic Genet 1997;18:161-73.  Back to cited text no. 1
    
2.
Van Setten G. Anterior lenticonus: Histological evaluation and approach for cataract surgery. J Cataract Refract Surg 2001;27:1071-5.  Back to cited text no. 2
    
3.
Kekunnaya R, Deshmukh AV, Kulkarni S. Newer insights into the clinical profile of posterior lenticonus in children and its surgical, visual, refractive outcomes. Eye (Lond) 2022;36:985-93.  Back to cited text no. 3
    
4.
Zare MA, Rajabi MT, Nili-Ahmadabadi M, Oskouee SJ, Moghimi S. Phacoemulsification and intraocular lens implantation in Alport syndrome with anterior lenticonus. J Cataract Refract Surg 2007;33:1127-30.  Back to cited text no. 4
    
5.
Hipólito-Fernandes D, Elisa-Luís M, Alves N, Maduro V. Femtosecond laser-assisted cataract surgery for bilateral anterior lenticonus. J Cataract Refract Surg 2020;46:789-91.  Back to cited text no. 5
    


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