Summary for the Clinician

Typical clinical signs of congenital glaucoma occurring in the first 2 years of life:

• History of epiphora, photophobia and blepharospasm during the first months of life

• Corneal opacities, tears of the Descemet membrane (Haab striae), an overstretched superior limbal region and large corneal diameters

• In contrast to management in adult glaucoma, several specifications have to be considered in the diagnostic work-up:

• Abnormally increased ocular axial length (in comparison to the normal age-correlated growth curve) is a common feature of congenital glaucoma. However, if onset of glaucoma occurs after 3 years of age the typical stretching of the globe is usually absent

• Despite manifest congenital glaucoma the intraocular pressure may be normal under deep general anesthesia. Several sources of error may confuse intraocular pressure measurements in buphthalmic eyes

• Glaucomatous excavation of the optic disc is usually present, but evaluation of the optic disc is often impossible owing to corneal opacities. Glaucomatous excavation can be reversible in very young children following IOP control

• Visual field testing is usually impossible in children younger than 6-8 years. Optic disc morphometry can be performed even in younger children from 4 years of age

• Skiascopy, or another method of determining the objective ocular refraction, should be performed regularly, because refractive problems such as ametropia and ani-sometropia are very common in buph-thalmic eyes and may also contribute to amblyopia

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