Two Cases of Congenital Hereditary Stromal Dystrophy of the Cornea.

Park, Mi Ra; Kim, Man Soo

Case Report

Journal of the Korean Ophthalmological Society 1998;39(9):2182-2186.
Published online September 1, 1998.

Two Cases of Congenital Hereditary Stromal Dystrophy of the Cornea.

Mi Ra Park, Man Soo Kim

Department of Ophthalmology, Kangnam St. Mary's Hospital, The Catholic University of Medical College, Seoul, Korea.

선천 유전 각막간질이영양증 2예

박미라(Mi Ra Park),김만수(Man Soo Kim)

Abstract

Congenital hereditary stromal dystrophy of the cornea is a rare hereditary disease which is inherited in autosomal dominant fashion. This disorder is not related to abnormal endothelial cell architecture and function. It presents as bilateral, congenital opacifications of the cornea and has unique histopathologic changes of the stroma such as alternating patterns of densely packed lamellae and loose lamellae. We experienced a family with congental corneal opacifications and performed penetrating keratoplasties in 2 patients. The obtained corneal buttons were examined by light and electron microscopy. The characteristic changes of stromal lamellae were consistent with a diagnosis of congenital hereditary stromal dystrophy of the cornea.

Key Words: Alternating patterns;Autosomal dominant;Changes of stromal lamellae;Congenital hereditary stromal dystrophy