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Year : 2019  |  Volume : 67  |  Issue : 3  |  Page : 406

Bilateral spontaneous lens capsule rupture in Alport's syndrome

Guru Nanak Eye Centre, Maulana Azad, Medical College, New Delhi, India

Date of Web Publication18-Feb-2019

Correspondence Address:
Dr. Monica Lohchab
B-448 Sector-1 Rohini, New Delhi - 110 085
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1089_18

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How to cite this article:
Lohchab M, Arora R. Bilateral spontaneous lens capsule rupture in Alport's syndrome. Indian J Ophthalmol 2019;67:406

How to cite this URL:
Lohchab M, Arora R. Bilateral spontaneous lens capsule rupture in Alport's syndrome. Indian J Ophthalmol [serial online] 2019 [cited 2020 Jul 5];67:406. Available from: http://www.ijo.in/text.asp?2019/67/3/406/252396

A 9-year-old male child presented with sudden painless nontraumatic loss of vision in both the eyes. Slit lamp biomicroscopy showed ruptured anterior capsule with cataractous matter in the anterior chamber [Figure 1]. The ruptured lens appeared like volcanoes on anterior segment OCT [Figure 2]. Systemic evaluation revealed proteinuria and sensorineural hearing loss. A diagnosis of Alport's syndrome was thus made. Anterior lenticonus, hearing loss, and proteinuria were detected in younger sibling also. Alport's syndrome is a genetic condition with progressive renal dysfunction, ocular abnormalities, and hearing loss.[1] Anterior lenticonus is an ocular finding in Alport's syndrome, and spontaneous perforation of anterior capsule has been reported, although bilateral perforations are rare.[2],[3] The rupture of anterior capsule occurs due to thinness and presence of multiple disruptions which can be demonstrated on electron microscopy.[4] Surgical intervention may be required if the refractive error cannot be managed conservatively or if spontaneous perforation develops like in our case.[5] Our patient underwent lens aspiration with intraocular lens in both the eyes. The postoperative best-corrected visual acuity improved to 6/18 in the right eye and 6/12 in both the eyes. Hearing aids were prescribed along with angiotensin-converting enzyme inhibitors for proteinuria. The patient's condition has remained stable over a 2-year follow-up period. Albeit rare, bilateral spontaneous anterior capsule rupture can be a presenting feature of Alport's syndrome.
Figure 1: Ruptured anterior lens capsule with cataractous lens matter protruding in anterior chamber on slit lamp biomicroscope: (a) right eye, magnification ×15; (b) left eye, magnification ×12

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Figure 2: Appearance on anterior segment optical coherence tomography: (a) right eye and (b) left eye

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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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Colville DJ, Savige J. Alport syndrome. A review of the ocular manifestations. Ophthalmic Genet 1997;18:161-73.  Back to cited text no. 1
Trifonova K, Jordanoff G, Stoyanov V, Slaveykov K. Spontaneous anterior lens capsule rupture of a patient with Alport syndrome – A case report. Open Access Maced J Med Sci 2017;5:974-7.  Back to cited text no. 2
Gupta A, Ramesh Babu K, Srinivasan R, Mohanty D. Clear lens extraction in Alport syndrome with combined anterior and posterior lenticonus or ruptured anterior lens capsule. J Cataract Refract Surg 2011;37:2075-8.  Back to cited text no. 3
Streeten BW, Robinson MR, Wallace R, Jones DB. Lens capsule abnormalities in Alport's syndrome. Arch Ophthalmol 1987;105:1693-7.  Back to cited text no. 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. 5


  [Figure 1], [Figure 2]


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