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Brittle cornea syndrome: Integrating unique presentations and novel management options
Jyothi Balakrishnan1, Aafreen Bari1, Prafulla Kumar Maharana1
1Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Delhi, India.
Abstract:
Brittle cornea syndrome (BCS) is a connective tissue disorder with a vast majority of clinical presentations and prognostic outcomes. This case series highlights various ocular presentations, their responses to treatment strategies, and final visual outcomes. It was an observational case series at a tertiary eye care center. Three patients with BCS and different corneal pathologies were managed and studied. The first case presented with wound dehiscence, a shallow anterior chamber, and a disenclaved iris claw lens. He underwent intraocular lens (IOL) explantation and wound closure using cyanoacrylate glue and a bandage contact lens. The second case involved corneal ectasia with rupture and an intumescent cataract, along with lens-endothelial touch. He underwent lens aspiration with posterior chamber IOL implantation and epi-keratoplasty with a biosynthetic cornea. However, the biosynthetic cornea had to be removed due to a severe inflammatory reaction following the procedure. The patient was later rehabilitated with deep anterior lamellar keratoplasty. The third patient presented with bilateral tractional descemet membrane detachment resulting from multiple minor injuries. Refraction with prescription glasses was used for visual rehabilitation. In conclusion, the management of BCS requires individualized treatment strategies tailored to each patient's presentation and response.
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