Abnormalities of the Cornea

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Chapter 619 Abnormalities of the Cornea

Peters Anomaly

Peters anomaly is a central corneal opacity (leukoma) that is present at birth (Fig. 619-1). It is often associated with iridocorneal adhesions that extend from the iris collarette to the border of the corneal opacity. Approximately half of patients have other ocular abnormalities, which may include cataracts, glaucoma, and microcornea. As many as 80% of cases may be bilateral, and 60% are associated with systemic malformations that may affect any major organ system. Some investigators have divided Peters anomaly into 2 types: a mesodermal or neuroectodermal form (type I), which shows no associated lens changes, and a surface ectodermal form (type II), which does. Histologic findings include a focal absence of Desçemet membrane and corneal endothelium in the region of the opacity. Peters anomaly may be caused by incomplete migration and differentiation of the precursor cells of the central corneal endothelium and Desçemet membrane or a defective separation between the primitive lens and cornea during embryogenesis.

Interstitial Keratitis

This denotes inflammation of the corneal stroma. The most common cause is syphilis, interstitial keratitis being one of the characteristic late manifestations of congenital syphilis. The corneal changes in congenital syphilis occur in 2 phases. The acute phase presents between the ages of 5 and 10 yr, with an intense keratitis that may last for several months and causes a severe reduction in vision. The acute effects of syphilis are mainly due to the host immune response, such as mononuclear cell infiltrates, proliferative vascular changes, and occasionally granuloma formation. The deep inflammation produces pain, photophobia, tearing, circumcorneal injection, and corneal haze. The acute episode is followed by a chronic stage with significant regression in the corneal findings along with a parallel improvement in visual acuity. Although the corneal findings may regress with time, “ghost vessels,” which represent the previous vascular changes, and patchy corneal scarring remain and serve as permanent stigmata of the disease.

Cogan syndrome is a nonluetic interstitial keratitis associated with hearing loss and vestibular symptoms. Although its cause is unknown, a systemic vasculitis is suspected. Prompt treatment is required to avoid permanent hearing loss. Both the corneal changes and the auditory involvement may respond to the use of immunosuppressive agents.

Less frequently, interstitial keratitis is caused by other infectious diseases, such as tuberculosis or leprosy.

Corneal Manifestations of Systemic Disease

Several metabolic diseases produce distinctive corneal changes in childhood. Refractile polychromatic crystals are deposited throughout the cornea in cystinosis (Chapter 79.4). Corneal deposits producing various degrees of corneal haze also occur in certain types of mucopolysaccharidosis (MPS; Chapter 82), particularly MPS IH (Hurler), MPS IS (Scheie), MPS I H/S (Hurler-Scheie compound), MPS IV (Morquio), MPS VI (Maroteaux-Lamy), and sometimes MPS VII (Sly). Corneal deposits may develop in patients with GM1 (generalized) gangliosidosis (Chapter 80.4). In Fabry disease, fine opacities radiating in a whorl or fanlike pattern occur, and corneal changes can be important in identifying the carrier state (Chapter 80.4). A spraylike pattern of corneal opacities may also be seen in the Bloch-Sulzberger syndrome. In Wilson disease, the distinctive corneal sign is the Kayser-Fleischer ring, a golden brown ring in the peripheral cornea resulting from changes in Desçemet membrane (Chapter 349.2). Pigmented corneal rings may develop in neonates with cholestatic liver disease. Corneal changes may occur in autoimmune hypoparathyroidism and band keratopathy in patients with hypercalcemia. Transient keratitis may occur with rubeola and sometimes with rubella.

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