Dohlman (1951) described a Swedish kindred in which 15 members had corneal endothelial dystrophy (cornea guttata) in association with anterior polar cataract; in all patients the central cornea was more severely affected than the periphery. In 3 others ... Dohlman (1951) described a Swedish kindred in which 15 members had corneal endothelial dystrophy (cornea guttata) in association with anterior polar cataract; in all patients the central cornea was more severely affected than the periphery. In 3 others at least 1 of the symptoms was observed. It appeared that a number of deceased members of the kindred probably had at least polar cataract. Clinically, polar cataract did not always seem to be evident at birth. It most often appeared between the ages of 3 and 10 years and became stationary after puberty. Cornea guttata did not seem to be present at birth but arose later and progressed slowly, resulting in a 'beaten-metal' appearance on retro-illumination. Dohlman (1951) commented that the changes were limited so sharply to the posterior of the cornea that hardly more than the endothelium and Descemet membrane could feasibly be involved. The corneal changes were mostly bilateral and roughly equally advanced in each eye; however, the severity of changes varied from patient to patient. The lesions were most advanced in the center of the cornea and decreased in severity toward the periphery, and in none of the patients were changes seen in other layers of the cornea. In the young, impairment of vision, which was sometimes severe, was due to polar cataract, but with advancing age, corneal deterioration contributed to the visual disability. Traboulsi and Weinberg (1989) observed an American family in which 12 members had both abnormalities. All affected individuals had excellent visual acuity. This family was descendant from a family that had immigrated to the United States in the 17th century from Ireland, where they had settled in the 13th century after moving from Scandinavia.