Corneal clouding with increased acid mucopolysaccharide accumulation in Bowman's membrane

Two infants had bilateral congenital corneal clouding and abnormal acid mucopolysaccharide accumulation in a thickened Bowman's membrane. This unusual entity was not associated with acid mucopolysaccharide deposits in the skin and visceral tissues or with increased levels of acid mucopolysaccharide in the urine. The similarity and differences have been compared to the systemic mucopolysaccharidosis and macular corneal dystrophy.