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June 1978

Congenital Hereditary Stromal Dystrophy of the Cornea

Author Affiliations

From the University Eye Clinic, Freiburg, West Germany (Drs Witschel and Grützner); the Registry of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC (Dr Fine); and the Washington Medical Center, Washington, DC (Dr McTigue).

Arch Ophthalmol. 1978;96(6):1043-1051. doi:10.1001/archopht.1978.03910050563015

• Corneas from patients in two separate pedigrees showing dominant inheritance of a congenital nonprogressive corneal clouding were examined by light and electron microscopy. The characteristic changes of abnormal corneal lamellae were widely and rather uniformly distributed throughout the stroma. The remaining corneal layers were normal. The findings are consistent with a diagnosis of congenital hereditary stromal dystrophy of the cornea, which differs greatly on both histologic and clinical grounds from the entity known as congenital hereditary endothelial dystrophy of the cornea.