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Abstract #85230 Published in IGR 21-1
Case Series of Brittle Cornea Syndrome
Eleiwa T; Raheem M; Patel NA; Berrocal AM; Grajewski A; Abou Shousha MCase reports in ophthalmological medicine 2020; 2020: 4381273
PURPOSE: This case series demonstrate diagnostic features, treatment options, and challenges for Brittle Cornea Syndrome. Three cases presented with bluish sclera and extremely thin cornea. Genetic workup was performed and confirmed the diagnosis of Brittle Cornea Syndrome, a rare autosomal recessive disorder characterized by corneal thinning and blue sclera. Case 1 was a 4-year-old boy who developed cataract and glaucoma after undergoing right tectonic penetrating keratoplasty (PK) secondary to a spontaneous corneal rupture. Glaucoma was controlled medically. Later, the kid underwent right transcorneal lensectomy and vitrectomy with synechiolysis. After 6 weeks, he sustained graft dehiscence that was repaired using onlay patch graft. Case 2 was a 7-year-old boy who underwent PK in the right eye, then a pericardial patch graft in the left eye following spontaneous corneal rupture. Glaucoma in both eyes was controlled medically. Case 3 was the 2-year-old sister of the 2 case. She had a pachymetry of 238 m OD and 254 m OD and 254 . CONCLUSIONS: Long-term follow-up of children diagnosed with Brittle Cornea Syndrome is paramount to minimize the morbidity of corneal rupture and late-onset extraocular conditions.
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Classification:
9.4.2.5 Other (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.2 Glaucomas associated with disorders of the cornea, conjunctiva, sclera)
9.1.2 Juvenile glaucoma (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)