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Abstract #19289 Published in IGR 9-3

Coexistent congenital hereditary endothelial dystrophy and congenital glaucoma

Ramamurthy B; Sachdeva V; Mandal AK; Vemuganti GK; Garg P; Sangwan VS
Cornea 2007; 26: 647-649


PURPOSE: To retrospectively evaluate the coexistence of congenital glaucoma with congenital hereditary endothelial dystrophy. METHODS: Ten infants presented to our hospital with diffuse corneal edema and bilaterally elevated intraocular pressure (IOP). These patients were diagnosed with congenital glaucoma. All patients underwent trabeculotomy with trabeculectomy for control of IOP. Although IOP was normalized in all patients, corneal edema persisted. These patients underwent penetrating keratoplasty, and the buttons were subjected to histopathologic examination. RESULTS: The corneal grafts remained clear in all patients. The histopathologic examination of the excised corneal buttons showed diffuse stromal edema, loss of the endothelial cell layer, and thickening of the posterior nonbanded portion of the Descemet membrane, suggestive of congenital hereditary endothelial dystrophy. CONCLUSIONS: Congenital hereditary endothelial dystrophy may coexist with congenital glaucoma. This combination should be suspected where persistent and total corneal opacification fails to resolve after normalization of IOP.

Dr. B. Ramamurthy, L.V. Prasad Eye Institute, L. V. Prasad Marg, Banjara Hills, Hyderabad 500034, India. bsrmurthy@lvpei.org


Classification:

9.1.1 Congenital glaucoma, Buphthalmos (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)
9.4.2.5 Other (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.2 Glaucomas associated with disorders of the cornea, conjunctiva, sclera)



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