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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 2  |  Issue : 1  |  Page : 8

Bilateral phacoemulsification and toric extended depth of focus intraocular lens implantation in Alport's syndromes


Department of Ophthalmology, Setubal Hospital Center, São Paulo, Brazil

Date of Submission31-Dec-2019
Date of Acceptance25-Feb-2020
Date of Web Publication24-Mar-2020

Correspondence Address:
Ines Fitas Figueiredo Matias
Rua 11, Numero 18, Bairro Da Calcada Dos Mestres, 1070-193 Lisboa
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/PAJO.PAJO_29_19

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  Abstract 


Alport syndrome is a hereditary disorder characterized by sensorineural deafness, renal disease, and ocular abnormalities, including anterior lenticonus with high refractive errors. We report a case of Alport's syndrome with bilateral anterior lenticonus that was submitted to phacoemulsification and toric extended depth of focus (EDOF) intraocular lens (IOL) implantation. A 49-year-old male patient presented with progressive bilateral visual loss for 10 years. His past medical history included a diagnosis of Alport's syndrome, with renal transplantation 20 years ago. The best-corrected visual acuity was 20/80 in the right eye and 20/63 in the left eye. Examination showed anterior lenticonus and anterior subcapsular opacities. Phacoemulsification and toric EDOF-IOL implantation were performed in both eyes. Capsulorhexis was challenging because of a highly elastic anterior capsule, which was aided by the use of a viscoadaptive ophthalmic viscosurgical device. The postoperative period was uneventful, with good refractive and visual outcomes.

Keywords: Alport syndrome, anterior lenticonus, capsulorhexis, multifocal intraocular lenses, phacoemulsification


How to cite this article:
Figueiredo Matias IF, Prata Gomes PN, da Cunha Diniz SN, Pereira Rita Neves PR, Melo de Ornelas MA. Bilateral phacoemulsification and toric extended depth of focus intraocular lens implantation in Alport's syndromes. Pan Am J Ophthalmol 2020;2:8

How to cite this URL:
Figueiredo Matias IF, Prata Gomes PN, da Cunha Diniz SN, Pereira Rita Neves PR, Melo de Ornelas MA. Bilateral phacoemulsification and toric extended depth of focus intraocular lens implantation in Alport's syndromes. Pan Am J Ophthalmol [serial online] 2020 [cited 2020 Nov 25];2:8. Available from: https://www.thepajo.org/text.asp?2020/2/1/8/281371




  Introduction Top


Alport syndrome is a hereditary condition characterized by progressive sensorineural deafness, renal disease with abnormal glomerular basement membrane,[1] and ocular abnormalities, affecting the cornea, lens, and retina.[2]

Most common ocular manifestations are anterior lenticonus and perimacular retinal flecks.[3] Other ocular features include corneal opacities, cataract, and peripheral coalescing fleck retinopathies. Corneal scarring, temporal retinal thinning, giant macular hole, and maculopathy are recently described features that broaden the ophthalmic phenotype.[2] Anterior lenticonus is a localized, cone-shaped deformation of the anterior lens surface, often bilateral and associated with Alport syndrome.[4] It produces high refractive errors and optic image degradation, leading to visual disability uncorrectable by glasses or contact lenses.[5] Treatment for symptomatic lenticonus is lens removal and intraocular lens (IOL) implantation.[2]

We report a case of Alport's syndrome with bilateral anterior lenticonus that was submitted to bilateral phacoemulsification and toric extended depth of focus (EDOF) IOL implantation, with good refractive and visual results. We describe the particularities in phacoemulsification surgery in this special case.


  Case Report Top


A 49-year-old male patient presented with progressive visual loss bilaterally for 10 years. His past medical history included a diagnosis of Alport's syndrome, with renal transplantation 7 years before and bilateral hearing loss, for which he was using a hearing aid. He was medicated with long-term immunosuppressive therapy.

On examination, the best-corrected visual acuity was 20/80 in the right eye (−10.0 D sphere/−3.0 D cyl at 160°) and 20/63 in the left eye (−12.0 D sphere/−3.5 D at 20°). Slit-lamp examination showed anterior lenticonus and anterior subcapsular opacities with oil droplet reflex [Figure 1] and [Figure 2]. Dilated fundus examination revealed a dull macular reflex bilaterally. Anterior segment imaging was performed with topographic and tomographic analysis, revealing regular corneal astigmatism and anterior lenticonus [Figure 3]. Posterior segment OCT revealed a temporal retinal thinning [Figure 4]. Optical biometry results were as follows: right eye axial length of 23.99 mm, K1 41.60 D at 15°, and K2 44.50 D at 105° and left eye axial length 23.89 mm, K1 41.81 D at 170°, and K2 44.76 D at 80°.
Figure 1: Right eye slit-lamp photograph showing anterior lenticonus and anterior subcapsular opacities

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Figure 2: Left eye slit-lamp photograph showing oil droplet reflex

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Figure 3: Anterior segment optical coherence tomography image showing bilateral anterior lenticonus (a: right eye; b: left eye)

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Figure 4: Posterior segment optical coherence tomography image showing bilateral temporal retinal thinning (a: right eye; b: left eye)

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Our diagnosis was bilateral anterior lenticonus and anterior subcapsular cataract, with severe progressive visual loss. We performed phacoemulsification and IOL implantation in both eyes, under general anesthesia.

Capsulorhexis was challenging because of highly elastic anterior capsule and bulging of the anterior surface of the crystalline. This maneuver was performed with special care and under viscoadaptive ophthalmic viscosurgical device (OVD), starting with a smaller capsulorhexis followed by a controlled slight enlargement at the end. Because of the patient's young age and corneal astigmatism (>1.5 D), we decided to implant toric EDOF IOLs in the capsular bag.

The patient was medicated with dexamethasone eye drops 4 times a day for 4 weeks, ofloxacin eye drops 4 times a day for 1 week, gentamicin ointment at night for 1 week, and flurbiprofen eye drops 4 times a day for 4 weeks.

The postoperative period was uneventful. The following day, uncorrected visual acuity was 9/10 in both eyes and intraocular pressure was 14/16 mmHg. At 4 weeks, uncorrected distance vision was 10/10 in both eyes, with uncorrected near visual acuity of J2 [Figure 5].
Figure 5: Left eye postoperative slit-lamp photograph

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  Discussion Top


Alport syndrome is a genetically heterogeneous disease, arising from mutations that impair the production, deposition, or function of the collagen IV α3α4α5 network, the major collagenous constituent of mature basement membranes in the glomerulus, cochlea, cornea, lens, and retina.[6]

Alport syndrome is estimated to affect 1 in 5000–10,000 individuals.[7] It has either an X-linked inheritance pattern in approximately 80% (COL4A5 mutations), or an autosomal inheritance pattern (COL4A3 or COL4A4 mutations), recessive in 15% and dominant in 5%.[6],[8]

Ocular anomalies have been reported in 9%–82% of Alport syndrome patients.[3] In this case, we found anterior lenticonus, cataract, and temporal retinal thinning.

Temporal retinal thinning is common in all forms of Alport; although less sensitive diagnostically than peripheral coalescing fleck retinopathy, its demonstration is more objective. When there is thinning only, vision is not affected.[2]

Lenticonus results from the protrusion of the lens through the thinnest and weakest anterior part of the capsule. The absence of the collagen IV α3α4α5 network from the capsule leads to partial splits that may rupture, and cataracts may develop from healing of small spontaneous ruptures.[2]

Lenticonus progressively worsens visual acuity, and most patients eventually require surgery.[2] Phacoemulsification with IOL implantation has been described as a safe and effective procedure in lenticonus.[9],[10]

Abnormalities in the anterior lens capsule have been described, with thinning and vertical dehiscence.[11] Capsular fragility with spontaneous rupture of the capsule, as well as highly elastic capsules, makes the capsulorhexis technically more difficult.

In our case, phacoemulsification was performed without complications. We found a highly elastic anterior capsule, so capsulorhexis was performed with special attention and using a viscoadaptive OVD.

We chose to implant a multifocal IOL because the case was a young patient looking for spectacle independence. Implantation of a toric EDOF IOL allowed us to correct the coexisting corneal astigmatism. EDOFs produce few optical phenomena, less reduction of contrast sensitivity, and better tolerance to residual astigmatism, being preferred over other multifocals in eyes with maculopathy or irregular corneas.[12]

The patient reported anxiety related to an awake procedure with topical anesthesia. HerHe chose a simultaneous bilateral approach under general anesthesia, knowing and accepting the potential risks. The simultaneous bilateral approach proved to be cost-effective, reducing the number of procedures and overall logistic expenses. It also eliminated the risk of repeated general anesthesia.

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 Top

1.
Bowling B. A systematic approach. Lens in Kanski's Clinical Ophthalmology. Ch. 9. Elsevier: London; 2016.  Back to cited text no. 1
    
2.
Savige J, Sheth S, Leys A, Nicholson A, Mack HG, Colville D. Ocular features in Alport syndrome: Pathogenesis and clinical significance. Clin J Am Soc Nephrol 2015;10:703-9.  Back to cited text no. 2
    
3.
Xu JM, Zhang SS, Zhang Q, Zhou YM, Zhu CH, Ge J, et al. Ocular manifestations of Alport syndrome. Int J Ophthalmol 2010;3:149-51.  Back to cited text no. 3
    
4.
American Academy of Ophthalmology. Lens and Cataract. In: Basic and Clinical Science Course. Sec. 11. San Francisco, CA: American Academy of Ophthalmology; 2018.  Back to cited text no. 4
    
5.
Can CU, Ilhan B, Polat S, Teberik K, Zileliolu O. Surgical management of anterior lenticonus in a patient with Alport's syndrome. Turkish J Med Sci 2008;38:269-72.  Back to cited text no. 5
    
6.
Kashtan C. Alport syndrome: Facts and opinions. F1000Res 2017;6:50.  Back to cited text no. 6
    
7.
Miner JH, Baigent C, Flinter F, Gross O, Judge P, Kashtan CE, et al. The 2014 international workshop on Alport syndrome. Kidney Int 2014;86:679-84.  Back to cited text no. 7
    
8.
Watson S, Bush J. Alport Syndrome. San Francisco, CA: StatPearls Publishing; 2019.  Back to cited text no. 8
    
9.
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. 9
    
10.
Aslanzadeh GA, Gharabaghi D, Naderi N. Clear lens phacoemulsification in the anterior lenticonus due to Alport Syndrome: Two case reports. J Med Case Rep 2008;2:178.  Back to cited text no. 10
    
11.
Choi Jh, Na Ks, Bae Sh, Roh Gh. Anterior lens capsule abnormalities in Alport syndrome. Korean J Ophthalmol 2005;19:84-9.  Back to cited text no. 11
    
12.
Carones F. Residual (astigmatism threshold and patient satisfaction with bifocal, trifocal and extended range of vision intraocular lenses IOLs). Open J Ophthalmol 2017;7:1-7.  Back to cited text no. 12
    


    Figures

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



 

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