Rare and Interesting Case Report of Bilateral Congenital Corneal Dystrophy with Associated Left Eye Anterior Staphyloma

Corneal dystrophy is a very rare cong entity with probable deposition of some unknown material in corneal stroma of an unknown etiology with strong heredo-familial tendency a better modality of defining corneal dystrophy is as follows lesions in the cornea of an unknown etiology which may manifest either at birth or at 1st or 2nd decade of life. May remain stationary or progressive has got a strong heredo familial tendency. Clinically a bilateral central symmetrical corneal opacity with impaired corneal sensations and absence of deep vascularisation is corneal dystrophy unless proved otherwise the other associated cong anamolies with bil cong dystrophy are very rare they are in the form of

a) Cong glaucoma

b) Keratoconus

c) Ant staphyloma

d) Cong absence of desmets membrane

e) Ched

f) Deafness known as cogans syndrome

g) Kc sicca

h) Medulated optic nerve fibers

i) Cong ptosis

j) Conjuctival xerosis

Cong glaucoma; Keratoconus; Ant Staphyloma; Desmets Membrane; Ched; Deafness; Cogans Syndrome; Kc Sicca; Medulated Optic Nerve Fibers; Cong Ptosis; Conjuctival Xerosis; Micocephaly; Icthyosis; Staphyloma; Ultrasonub; Dystrophy; Keratoplasty; Spina Bi-fida; Retina; Cong Anamolies; Corneal Stroma

A first married cousin first pregnancy in the first trimester of pregnancy ultrasound revealed a male fotus with spina bi-fida and microcephaly so the pregnancy was terminated 2nd pregnancy Ft delivered normal female child after l s c s alive 9 years of age no associated cong disorders 3rd f t male child delivered after l s c s died after 3 days due to generalized icthyosis 4th Ft female child delivered after l s s c had bil cong corneal dystrophy with left eye associated ant staphyloma with no other associated abnormalities seen at 24 hours of age IOP measured with tono pen was normal b scan ultrasound showed attached retina [1].

This baby at age of 3 months under went right eye modified keratoplasty and at 3 years of age left eye cosmotic kerato prosthesis was done.

It is very important to encourage parents of such patients who are very much depressed.

The condition in the presented case report was considered to be Capsular contraction syndrome. IOL exchange with a scleral suturefixated IOL was the most suitable treatment in our case resulting in improved visual acuity and low rate of complications postsurgery. Lately, with increase in the reports of the bag dislocation of the IOL, it’s important to consider the predisposing factors before the cataract surgery for better post-surgical outcomes. Special monitoring must be done while using phacoemulsification technique towards capsule polishing and zonular damage to avoid long term complications in cataract surgeries.

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