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Medical & Surgical Ophthalmology Research

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

Gowhar Ahmad*

Department of ophthalmology, University of Jammu and Kashmir, India

*Corresponding author: Gowhar Ahmad, Department of ophthalmology, University of Jammu and Kashmir, India, Tel: 9419009850/9622983444; Email:gowhar.ahmad1948@gmail.com

Submission: January 29, 2018;Published: April 13, 2018

DOI: 10.31031/MSOR.2018.02.000527

ISSN 2578-0360
Volume2 Issue1

Introduction

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

Keywords:

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

Obstetric History

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].

Case Report

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.

Discussion

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

Conclusion

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.

References

  1. Bhat YR, Sanoj KM (2005) Images in clinical practices sclerocornea indian paediatrics. p. 42.
  2. Hand CK (1999) Harmondl kennedy sm fitzSimon js collum lm parfrey na localisation of the gene for autosomal recessive cong heeditary endothelial dystrophy ched2 to chrosome 20 by homozy gosity Mapping genomics 1(6): 111.
  3. (2003) Medine 4 miler mm butrus s hidayat a wei l l pontigo m corneoscleral transplantation in cong corneal staphyloma and peters. Anamoly Ophthalmic Genet 1(59): 83.
  4. Desir J, Abramowicz M (2008) Cong hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan Syndrome). Orphanet J Rare Dis 15(3): 28.
  5. Waizenegger UR, Kohnen T, Weidle EG, Schutte E (1995) Cong familial cornea plana with ptosis Peripheral sclerocornea and conjuctival xerosis klin monatsbl augenheilkd 7(2): 111.
  6. Medline Perry HD, Cameron JD. Cong Corneal Opacities.

© 2018 Gowhar Ahmad. This is an open access article distributed under the terms of the Creative Commons Attribution License , which permits unrestricted use, distribution, and build upon your work non-commercially.



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