Gowhar Ahmad*

Professor, Department of Ophthalmology, Florence Hospital Multispeciality Center, Chanapora, India

*Corresponding Author: Gowhar Ahmad, Professor, Department of Ophthalmology, Florence Hospital Multispeciality Center, Chanapora, India.

Received: March 14, 2024; Published: March 21, 2024

Citation: Gowhar Ahmad. “Rare and Interesting Case of Bilateral Cong Corneal Dystrophy with Rare Associated Cong Left Eye Ant Staphyloma”. Acta Scientific Paediatrics 7.5 (2024): 29-30.

Abstract

Bilateral cong corneal dystrophy is a kind of rare ocular condition where their is deposition of some unknown substance may be kind of amyloid exact cause is not known however heredofamilial tendency plays an important part to be differentiated from corneal degeneration which is essentially unilateral of non heredofamilial status other way of classification of corneal dystrophy is lesions in the cornea of unknown etiology which may manifest either at birth or 2nd or 3rd decade of life has got heredofamilial tendency. Clinically bilateral central symmetrical corneal opacity with absence of deep vascularisation and impaired corneal sensations is corneal dystrophy unless proved otherwise we have certain types of corneal dystrophies like granular macular lattice arcus senalis and arcus Juvenile and many more.

Keywords: Cong Glaucoma; Keratoconus; Deafness

Bilateral Cong corneal dystrophy can be associated with other Cong ANAMOLIES like

• Cong glaucoma
• Keratoconus
• Ant staphyloma
• Cong absence of destmets membrane
• Ched
• Deafness
• Medullated optic nerve fibers
• Ks sicca
• Ptosis u lid
• Conjuctival xerosis
• CHED with progressive sensorineural deafness is known as Harboyan Syndrome

Case Report

A first cousin married couple in its first pregnancy during first trimester ultrasound showed an abnormal male foetus with microcephly and spina bifida so pregnancy was terminated 2^nd pregnancy a female child delivered after lscs normal no associated cong anomalies is hale and heart studying in 12^th class 3^rd pregnancy male child delivered after lscs died after 3 days due to cong ichthyosis 4^th pregnancy a feae child delivered after lscs had bil cong cor dystrophy with left eye ant staphyloma at one day of age neonates iop on ton open was normal and b scan ultrasound showed attached retina at 3 months of age r eye keratoplasty was done and at 3 years of age left eye cosmetic keratoprosthesis was done.

Discussion

Bilateral Cong corneal dystrophy is a kind of corneal blindness it is a matter of great concern destress not inky for child but for parents who see their new born child having bilateral blindness here is the role of treating paediatric ophthalmologist to boost parents in terms of encouragement patience which is the key to success.

That is what happened to this child who had successful keratoplasty at 3 month’s and of course nice oculoplasty surgery of left eye ant staphyloma in terms of cosmetic keratoprostheses at 3 years of age.

Conclusion

This child now 7 years old seeing very well with glasses eye studying in is standard her intelligence is more than the normal so none can conclude never loose hope in such cases patience parental counselling is jeybto success [1-4].

Bibliography

1. Bhat y R Sanoj K M. “Images in clinical practice s Sclerocirnea”. Indian Paediatrics (2006): 42.
2. Medin Pery H D and Cehera J D. “Cong corneal opacities”.
3. Desir J and Abramiwicz M. “Congenital hereditary endothelial dystrophy with progressive sensorineural deafness (Harboyan syndrome)”. Orphanet Journal of Rare Diseases 15 (2008): 3-28.
4. Waizenegger UR., et al. “Congenital familial cornea plana with ptosis, peripheral sclerocornea and conjunctival xerosis”. Klinische Monatsblätter für Augenheilkunde2 (1995):111-116.

Copyright: © 2024 Gowhar Ahmad. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.