Faculty Bibliography

Violent shaking of young infants has a variety of ocular and systemic sequelae, including retinal and subdural hemorrhages, seizures, coma, and death. The syndrome can be difficult to recognize because of the lack of external signs. The retinal examination and computed tomographic findings are absolutely essential for making the diagnosis, but very little information is available concerning this disorder in the ophthalmology literature. This paper presents the findings of a case of shaken baby syndrome and discusses the challenges in diagnosis

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

Spasmus nutans, usually believed to be a benign entity, is a disorder of young children consisting of nystagmus, head nodding and an anomalous head position of unknown etiology. We present a patient with spasmus nutans who proved to have a large arachnoid cyst. When the cyst was shunted the nystagmus lessened. Recent studies suggest that the head nodding and anomalous head position are adaptive mechanisms that compensate for the nystagmus and not pathological in themselves. Therefore, spasmus nutans is not a triad of findings, but an acquired nystagmus with associated adaptive responses. Because spasmus nutans implies a benign entity composed of three requisite components, we feel it is an inaccurate term that should be abandoned. A better designation would be acquired nystagmus of infancy, which would not imply a benign etiology

Abnormalities discussed in previous papers on myotonic dystrophy suggested that there are no defects in the sympathetic nervous system, and the pupillary abnormalities were probably of peripheral origin. The pupils of two patients with myotonic dystrophy were examined and were determined to have findings suggesting a lack of preganglionic sympathetic stimulation, which has never been reported before. Other findings of Horner's syndrome such as anhidrosis and lack of sympathetic stimulation to the Muller's muscle were not present