Anterior mesenchymal dysgenesis of the eye. Congenital hereditary endothelial dystrophy and congenital glaucoma.
The present report describes a patient with congenital glaucoma and iris hypoplasia who underwent repeated surgery to control the glaucoma. Despite these procedures, the patient's corneas opacified progressively, and bilateral penetrating keratoplasties were performed. Histopathological studies, including transmission and scanning electro microscopy of the corneal buttons, demonstrated specific changes of Descemet's membrane and the endothelium typical of congenital hereditary endothelial dystrophy (CHED). CHED has usually been considered to be a specific clinical entity that is not combined with congenital glaucoma or other significant abnormalities of the anterior segment of the eye. The present case indicates that these conditions may be combined, which is of importance when dealing with infants with congenital glaucoma and corneal opacities.
Pedersen
O O
OO
Ophthalmology, Department King Fahd Hospital, King Faisal University, Dammam, Saudi Arabia.
Rushood
A
A
Olsen
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EG
D000867:Anterior Chamber / Q000002:abnormalities*; D003317:Corneal Dystrophies, Hereditary / Q000235:genetics*; D004728:Endothelium, Corneal / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D005901:Glaucoma / Q000151:congenital* / Q000235:genetics / Q000601:surgery; D006801:Humans; D007223:Infant