Congenital hereditary stromal dystrophy of the cornea.
Cornea from patients in two separate pedigrees showing dominant inheritance of a congenital nonprogressvie 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.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000473:pathology* / Q000648:ultrastructure; D003317:Corneal Dystrophies, Hereditary / Q000151:congenital / Q000235:genetics* / Q000473:pathology; D016039:Corneal Transplantation; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009759:Nystagmus, Pathologic / Q000150:complications; D010375:Pedigree; D014184:Transplantation, Homologous; D014792:Visual Acuity