Hereditary Posterior Polar Cataract with Report of a Pedigree

K HEREDITARY, developmental, posterior polar cataract has been encountered in a North Country family and its descendants over five generations. It shows two unique features: progression and anticipation. Although several types of hereditary cataract have already been fully reported in the literature, only one satisfactory pedigree relating to posterior polar cataract has previously been recorded (Ziegler and Griscom, 1915). In the series of cases described in that pedigree, neither of these two features was demonstrated. Case Reports In the family reported here, all except three of those affected in generations I1-V were seen personally. The following cases demonstrate the clinical features of the condition: Case 1 (IV, 11), female, aged 29, was the first patient seen, and the hereditary basis of the cataract was established by tracing her relatives. She had had good vision as a child, but it had begun to deteriorate noticeably when she was aged about 24. When first seen, at the age of 26, the visual acuity in the right eye was 6/24 and in the left 6/60 corrected. Posterior polar cataracts were noted, and that in the left eye operated on successfully. Three years later, she presented asking for the right eye to be dealt with. On ex-amination, the vision had deteriorated to " counting fingers ", and there was present a peculiar discoid posterior polar cataract, with fluffy white extensions through the posterior cortex towards the equator. There were also a few anterior cortical punctate opacities (Fig. 1 a, b, opposite). This eye was successfully operated on also. There were no other ocular abnormalities. Her daughter (V, 15), aged 3+ years, had no cataract. Case 2 (IV, 7, elder sister of Case 1), female, aged 33, was said to have had cataracts since she was aged about 10. The vision in both eyes, especially the left, had deterorated over the past 3 years, and was worse in bright light. On examination, the visual acuity in the right eye was 6/9 and in the left 6/60 corrected. Both eyes showed a discoid posterior polar opacity, together with fluffy white extension in the posterior cortex running towards the equator. In addition, there was a small opacity in the anterior cortex of each eye (Fig. 2 a, b, and c, opposite). Received for publication March 10, 1955.