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Rare case of bilateral cong corneal dystrophy with rare associated cong left eye ant staphyloma

Gowhar Ahmad

Introduction: Cong corneal dystrophy is a kind of very rare corneal disorder whose exact cause is not known. However it is thought to be due to deposition of some unknown material. May be amyloid in the corneal stroma with strong heredofamalual tendency easily differentiated from corneal degeneration which is unilateral and of non-herdofamilial existence. Other way defining corneal dystrophy is lesions in the cornea of an unknown aetiology which may manifest either 1st or 2nd decade of life may remain stationery or progressive throughout the life has got strong herdofamil tendency. Any bilateral central symmetrical corneal opacity with absence of deep vascularisation and impaired corneal sensation is corneal dystrophy unless proved otherwise.
Case Report: A first cousin married couple in the first pregnancy during first trimester pregnancy ultrasound reviled an abnormal male foetus showing spina bifida and microcephaly the pregnancy was terminated.
2nd pregnancy: FT female baby delivered after, LSCS no history of exposure to oxygen or jaundice. Breast fed normal milestones no other cong anomalies the baby girl is normal alive healthy and 13 years of age studying in the 7th class.
3rd pregnancy: FT male delivered after LSCS died after 3 days due to Ichthyosis.
4th pregnancy: FT female delivered after LSCS has nil cong corneal dystrophy with left eye ant staphyloma. After 24 hours birth of child IOP with to open Vs was normal B scan ultrasound shows attached retina. At the age of 3 months R eye Keratoplasty was done at the 3 years of left eye which had ant staphyloma cosmetic kerstaptodth dis was done. Child is now about 6 years old she is very bright and intelligent 2nd is able to see well with her R-eye.