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of the cornea, and a missing deep corneal stroma, Descemet’s membrane and endothelium centrally. Surgical treatment is generally unsuccessful and these eyes have a very poor prognosis (Figs. 4.8 and 4.9).
In aniridia the amount of missing iris tissue is variable. Even if most of the iris is absent, a
Fig. 4.8 Right eye of a baby with Peters anomaly showing dense central leucomas of the cornea due to misdevelopment of the inner structures with anterior synechiae and iris holes
Fig. 4.10 Aniridia with visible margins of the lens as golden reflex nasally and temporally. Usually there are small remnants of iris tissue in the chamber angle
small amount of iris tissue is always present, which is mostly torn forward closing the trabecular meshwork of the chamber angle leading to secondary angle closure glaucoma (Fig. 4.10). The gene map locus is on chromosome 11p13. In sporadic aniridia with large deletions of the chromosome the probability of developing a Wilms tumor is high.
All these dysgenetic disorders have a risk of blindness of about 50% due to glaucoma, despite many attempts to regulate the IOP.
Several other diseases of the eye may lead to a pediatric glaucoma: aphakia (especially when operated on before week 8 after birth), persistent primary hyperplastic vitreous, retinopathy of prematurity, ectopia of the lens, microspherophakia, microphthalmus, phacomatoses, inflammations, tumors, etc.
Fig. 4.9 Left eye of the baby in Fig. 4.8 with Peters anomaly showing dense central leucomas of the cornea due to misdevelopment of the inner structures with anterior synechiae and iris holes
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Diagnostik und Therapie. Z prakt Augenheilkd 32: 364–368
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