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CASE REPORT
Use of an onlay corneal lamellar graft for brittle cornea syndrome
  1. Kirithika Muthusamy,
  2. Stephen Tuft
  1. Moorfields Eye Hospital NHS Foundation Trust, London, UK
  1. Correspondence to Dr Kirithika Muthusamy, kirithika.muthusamy{at}moorfields.nhs.uk

Summary

Brittle cornea syndrome (BCS1 OMIM #229200, BCS2 #614170) is a rare autosomal recessive condition characterised by diffuse thinning and fragility of the cornea. Affected individuals are at risk of globe rupture and blindness after relatively minor eye trauma. We describe a 9-year-old girl with BCS1, already blind in one eye following trauma, who had a 14 mm diameter corneoscleral onlay graft to her contralateral eye to reduce gross irregular corneal astigmatism and potentially to reduce further risk from accidental injury. Although there was a significant initial improvement in the unaided visual acuity, there was subsequent visual loss from secondary glaucoma. In addition, despite the onlay graft, an acute corneal hydrops developed approximately 2 years following surgery, suggesting that in BCS1, corneal tissue degeneration or resorption continues despite external support. Finally, because secondary glaucoma is not a feature of BCS1, we speculate that the onlay graft may have reduced aqueous outflow by compression of the thinned sclera.

  • eye
  • genetics
  • anterior chamber
  • congenital disorders
  • paediatric surgery
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Footnotes

  • Contributors ST, KM: data collection, obtaining consent, writing and final approval.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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