Cornea (Wills Eye Institute Atlas Series)

118 4 Ectatic Conditions of the Cornea

KERATOGLOBUS K eratoglobus is an extremely rare, bilateral condition of severe uniform peripheral or diffuse corneal thinning. It usually presents at or shortly after birth. Etiology ● Unknown Symptoms ● Poor vision, occasionally pain due to ● Total corneal thinning with maximal thinning in the midperiphery, resulting in protrusion of the entire cornea ( Fig. 4-3 ). ● The cornea can be very thin. ● Normal corneal diameter; very deep anterior chamber ● Acute hydrops may occur in advanced cases. hydrops Signs

● Patient may develop a corneal perforation from minimal trauma because of the severe corneal thinning. Associated Problems ● A syndrome comprising blue sclera, hyperextensible joints, dental and hearing abnormalities ● Ehlers–Danlos type VI ● Rubinstein–Taybi syndrome Treatment ● Mild and moderate cases: spectacles, which improve vision and provide some protection against trauma ● Severe cases: Some patients do well with a scleral lens. ● Surgical treatment is problematic. A large tectonic lamellar graft followed many months later by a smaller penetrating graft is an option. Prognosis ● Fair. Surgical treatment has a low success rate.

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