Corneal Opacifications

Much attention is focused on the cornea in the diagnosis of congenital glaucoma. There are several typical signs that can be observed such as a stretched superior limbal region (Fig. 7.3), tears in the Descemet membrane, the so-called Haab striae (Fig. 7.4), and corneal opacifications (Fig. 7.5). Corneal opacities are frequent in infantile glaucoma, occurring in up to 75% of glaucomatous eyes [17]. Corneal clouding as an isolated ocular symptom (without Haab striae and limbal stretching) in infants is a suspect sign of a genetic (congenital hereditary endothelial dystrophy, Turner or Noonan syndrome)

Corneal Opague Girl

Fig. 7.3. Primary congenital glaucoma in 14-month-old girl showing massive distension and stretching of the superior limbus region

B-scan sonography - wherever available - should be obligatory in buphthalmic eyes with severe opacities of cornea, lens, or vitreous to rule out secondary glaucoma owing to retinoblastoma, other tumor-like lesions, or persistent primary hyperplastic vitreous. Although rare, these secondary glaucomas require different treatment strategies with their own prognosis.

Follow-up examination of buphthalmic eyes with opaque optical media should also include B-scan sonography, because of the relatively high incidence of retinal detachment following any kind of antiglaucomatous surgery.

Fig. 7.3. Primary congenital glaucoma in 14-month-old girl showing massive distension and stretching of the superior limbus region

Congenital Glaucoma Pictures Haabs
Fig. 7.4. Haab striae (endothelial tears) in a adult patient who had undergone glaucoma surgery for congenital glaucoma in his first year of life
Fig. 7.5. Iridocorneotrabeculodysgenesis with glaucoma in a 3-year-old girl

or metabolic (mucopolysaccharidoses, cysti-nosis) disease, sometimes imitating congenital glaucoma. Even where meticulous examination is not possible, such as in a crying or restless, awake child, the degree of corneal clarity should always be observed. Even the smallest corneal opacity can be symptomatic of a severe ophthal-mological disease such as congenital glaucoma or anterior-dysgenesis syndrome.

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