Darshan Kumar U. Kansara1, Sunil G. Biradar2
PURPOSE: Reporting a case of Peters’ type II with congenital heart defect a rare form of anterior segment dysgenesis in which abnormal cleavage of the anterior chamber occurs. It is characterized by central corneal opacification secondary to defects in a posterior corneal stroma, Descemet's membrane, and endothelium. Majority of cases are sporadic, bilateral, and associated with other systemic malformations.
MATERIAL AND METHOD: A18 year old female presented with a history of bilateral cloudy corneas and diminution of vision since early childhood. Visual acuity was RE 6/60, LE 6/60 with PH improvement in BE 6/36. Slit lamp examination showed BE micro cornea with central and paracentral macular grade opacity with lenticulo-irido-corneal adhesions. Iris was found to be fused with the posterior surface of the peripheral cornea with poor formation of the anterior chamber with coloboma of iris. Lens was cataractous. Fundus examination in the left eye showed no abnormality. She was diagnosed as Peters’ type II anomaly. Systemic examination revealed congenital heart defects. Echocardiography showed congenital bicuspid aortic valve, grade 3 aortic regurgitation.
RESULT: Patient require penetrating keratoplasty with combined procedure (Cataract extraction with trabeculectomy).
CONCLUSION: Early detection of the disease will help in early treatment, with good vision development to combat amblyopia.
