Faculty Bibliography

We examined 12 patients with familial dysautonomia who had clear corneas to determine if there was any optic nerve involvement. The patients ranged in age from 6 to 34 years. Visual acuity ranged from 20/20 to 20/100. Nineteen eyes of 12 patients (79%) had abnormal (mean +/- 3 S.D.) pattern reversal visual-evoked potentials as compared to 50 control eyes (P100 = 98.8 msec; S.D., +/- 4.5 msec). P100 latency appeared to worsen with age. Exodeviation was present in seven patients (58%) and correlated with visual acuity and P100 latency. Ophthalmoscopic examination showed some degree of optic nerve pallor in all eyes

We prospectively studied 100 consecutive deaths at St. Vincent's Hospital and Medical Center in New York City for possible eye donation. Fifty-two patients were unsuitable mainly because of medical contraindications and age at death. Of the 48 suitable candidates, 21 pairs (44%) of eyes were obtained. Sixteen of the 42 eyes (38%) were used for transplantation. Projection of the number of corneas obtainable for transplant in New York City were calculated. The figures indicated that the three-month waiting list of the New York Eye-Bank for Sight Restoration, Inc., could be eradicated if hospitals were able to get seven donors from every 100 deaths suitable for donation, a rate only 16% of that realized in this study

We examined 32 patients with cystic fibrosis, paying special attention to optic nerve performance and pupillary function. Decreased visual acuity occurred in nine of 64 eyes. Three of 17 patients (18%) who used chloramphenicol had bilaterally delayed P100 waves of the visual-evoked response of greater than 3 standard deviations. This was not found in patients who did not use chloramphenicol. Contrast sensitivity in patients with cystic fibrosis was decreased at every spatial frequency when compared to healthy controls. This decrease was noted in patients who did and did not use chloramphenicol, suggesting that chloramphenicol is not the only cause of decreased contrast sensitivity in cystic fibrosis. With pharmacologic pupil testing we determined that patients with cystic fibrosis display a preganglionic oculosympathetic paresis that corresponded to the disease severity, as measured by the Shwachman score

We examined 55 clinic patients with biopsy-proven Hansen's disease and catalogued the ocular findings. Forty patients had lepromatous disease and 15 had tuberculoid disease. Visual acuity was 20/40 or better in 52 patients. Examination of pupillary size in all cases and after dilation with cocaine and hydroxyamphetamine in 15 cases showed decreased sympathetic innervation to the iris dilator muscle in seven cases. Ocular inflammation was uncommon: there was one case of active interstitial keratitis, three of episcleritis, and four of iritis. Although 41 patients had ocular problems attributable to Hansen's disease, the severity of the ocular complications was much less than reported in similar studies from other countries. We believe that the differences were the result of effective antimicrobial and anti-inflammatory therapy