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 [Fig. 1]. The ruptured lens appeared like volcanoes on anterior segment OCT [Fig. 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
Figure 2.
Appearance on anterior segment optical coherence tomography: (a) right eye and (b) left eye
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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 The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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