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Pediatric glaucoma associated with ocular anomalies 125

Peters anomaly

ETIOLOGY AND DIAGNOSIS

Peters anomaly consists of central corneal opacification associated with the lack of Descemet’s membrane presenting in the newborn infant (159, 160). Defects in the PAX6 gene lead to the development of Peters anomaly. Iris strands to the cornea are often noted and cataract commonly coexists. It may be unilateral or bilateral and generally occurs sporadically.

159

MANAGEMENT/TREATMENT AND PROGNOSIS

Many patients will require corneal transplantation for the corneal opacification. If glaucoma occurs, it generally requires surgical intervention. Approximately 50% of patients who undergo corneal transplantation develop glaucoma. The glaucoma in these patients is difficult to treat and often requires multiple surgeries. Glaucoma can increase the risk of corneal transplant failure. These issues have led to a debate regarding the benefit of corneal transplantation in children with unilateral disease.

160

159 Peters anomaly with cataract in the right

160 Mild Peters anomaly.Note

eye of a child with multiple congenital systemic

corneolenticular adhesions.

anomalies.Lensectomy was required,corneal

 

transplantation was not.The patient died of

 

cardiac complications unrelated to eye surgery.