Cataracts and riboflavin deficiency.

Lenticular reduced glutathione, diminished in all forms fo human cataract, requires flavin adenine dinucleotide as a coenzyme for glutathione reductase. Deficiency of riboflavin, a precursor of flavin adenine dinucleotide, has been believed by some to be associated with cataract formation. We evaluated the riboflavin nutritional status of healthy young adults, presenile and senile cataract patients, and young and older patients with clear lenses. We found no evidence of an association between riboflavin deficiency and early cataract formation, either idiopathic or secondary. Older cataract patients had more riboflavin deficiency. An absence of riboflavin deficiency was found in our older patients with clear lenses. The degree of riboflavin deficiency encountered in the general population does not appear to be cataractogenic.

Perineural optic nerve changes in endocrine orbitopathy.

A-scan ultrasonographic measurements of the differences between nerve and dural diameters of the retrobulbar optic nerve were made in 55 eyes of 30 patients with endocrine orbitopathy. This measurement was found to be enlarged in all cases. Extraocular muscle thickness measurements were also made ultrasonically in these eyes, and the degree of intervaginal distension was found to increase with increasing extraocular muscle involvement. These findings were consistent whether the patients were hyperthyroid, hypothyroid, or euthyroid clinically, and the findings occurred in the absence of any detectable functional optic nerve compromise. The cause of this subdural perineural enlargement remains unknown.

Proptosis secondary to subdural hematoma.

A subdural hematoma extending into the orbit through a fracture of the sphenoid bone appeared as unilateral proptosis with no neurologic abnormalities. Repeated percutaneous needle aspirations of the orbital portion of this mass provided only temporary relief of proptosis, but decompressed the intracranial portion of the hematoma adequately for prevention of neurologic manifestations. Ultrasonography correctly diagnosed the nature of the lesion and identified the bony dehiscence. Computed tomographic (CT) evaluation suggesting that a tumor might be present led to the performance of curative neurosurgery. Even when seemingly trivial, head trauma must be considered along with congenital, developmental, and neoplastic causes as a potential cause of intracranial cyst formation that can produce proptosis.

Effect of corticosteroids on cataract formation.

While the cataractogenic effects of corticosteroids are beyond dispute, disagreement exists concerning effects of total dose, intensity of dose, and duration of administration on cataract formation. We studied 106 adult male patients matched for age, race, and socioeconomic status. We compared posterior subcapsular (PSC) cataract formation among those with (39) and without (67) a history of systemic corticosteroid therapy. Difference in incidence of PSC opacities among patients with and without a history of corticosteroid therapy was statistically significant. However, no statistically significant correlation was found between PSC opacities and total steroid dose, weekly dose (intensity), duration of dose, or age of patient. Our findings agree with some recent literature that suggests the most important factor in steroid-induced PSC cataract formation may be variability in individual susceptibility to side effects of corticosteroids. We suggest abandoning the concept of a "safe" dose.

Presenile cataract formation and decreased activity of galactosemic enzymes.

One hundred forty-seven patients, 144 with advanced cataract formation, had determinations of erythrocyte galactokinase and galactose-1-phosphate uridyl transferase performed. Significant reduction (more than 2 SDs) of one of these enzymes was found in 47.4% of patients 50 years old or less with presenile "idiopathic" bilateral cataracts, 7.1% of other patients with cataracts aged 50 years or less, and 3.8% of patients with cataracts aged 51 years or more. The differences between the group with presenile idiopathic cataracts and the other groups were statistically highly significant (P less than .001). Patients with reduced activity of galactokinase or galactose-1-phosphate uridyl transferase (presumed heterozygotes) compose about 1% of the general population, appear to be more susceptible to idiopathic presenile cataract formation, and may be more prone to secondary cataract formation after a variety of lenticular insults. Dietary restriction of milk and milk products may prevent or delay cataract formation in these individuals.

Hereditary pigmented paravenous retinochoroidal atrophy.

A father and son with pigmented paravenous retinochoroidal atrophy demonstrated the classical fundus appearance of bilateral sharply circumscribed patches of retinochoroidal atrophy and pigmentation along the retinal veins. Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. Electrophysiologic findings in our patients included elevated dark-adaptation thresholds, slightly reduced electroretinographic amplitudes, slightly prolonged a- and b-wave implicit times, and (in Case 1) abnormal electro-oculographic ratios. These findings indicate a diffuse tapetoretinal degeneration, and suggest that pigmented paravenous retinochoroidal atrophy is another incomplete form of retinitis pigmentosa.

Bilateral choroidal osteomas associated with fatal systemic illness.

An 11-year-old black boy complained of intermittent occipital headaches with nausea and projectile vomiting. Previous skin and lung biopsy specimens were interpreted as histiocytosis X. Cranial computed tomographic scanning disclosed a mass lesion in the region of the choroid plexus of the left lateral ventricle. This was surgically removed but proved nondiagnostic despite extensive histologic examination. An ophthalmologic evaluation showed discrete, elevated, yellow-white choroidal tumors in both maculas. The ophthalmoscopic appearance, as well as ultrasonography and computed tomography, led to the diagnosis of choroidal osteomas.

Effect of age on Arden grating acuity.

Arden grating test scores were obtained for 100 normal subjects under standardised testing conditions. While previously published ranges of interocular conguity were corroborated, a significant increase in test scores with increasing age was noted. This effect of age has not been previously reported. Physiological considerations suggest that such age dependency might have been expected. It is suggested that ranges of normal values be established for each of several age groups.

Comparison of Snellen acuity, VER acuity, and Arden grating scores in macular and optic nerve diseases.

Patients with various macular and optic nerve abnormalities underwent Snellen acuity, transient VER acuity, and Arden grating testing. Snellen acuity was the coarsest of the 3 evaluations, generally falling after Arden scores and VER acuity had already undergone significant degradation. The Arden gratings appeared to be the most sensitive of the 3 tests, equalling VER performance in optic nerve diseases and surpassing it in macular diseases. Variations in results between the different tests are generally understandable if one considers the functions tested by each and the anatomical derangements caused by the diseases in question. The Arden grating test appears to be an excellent and sensitive screening test for central visual disturbances.

Vitelliform macular lesions.

Two patients with vitelliform macular lesions, normal EOG Arden ratios, and no family history of Best's vitelliform dystrophy are presented. Their findings are compared with those in 10 similar reported cases, and a common, nongenetic aetiology is suggested for all. The several names given to this identical fundus picture by different authors are examined and are seen to be inappropriate. A common, descriptive terminology for all such lesions is suggested.

Macular cysts and holes.

The ocular findings in an eight-year-old Negro male with unilateral axial myopia and bilateral macular cysts and holes are presented. Normal psychophysical, electrophysiologic, and angiographic findings were noted despite obvious anatomic changes. The constellation of observed findings in this patient appears to be unique.

Asymmetric retinitis pigmentosa, luetic retinopathy and the question of unilateral retinitis pigmentosa.

A seropositive patient with the fundus appearance of monocular luetic retinopathy is presented. Electrophysiologically, this patient shows a late-onset form of progressive tapetoretinal degeneration (retinitis pigmentosa) presenting in an extremely asymmetric manner. The differential diagnosis of luetic and other inflammatory retinopathies vs. tapetoretinal degenerations is briefly discussed. Features distinguishing unilateral retinitis pigmentosa from bilateral tapetoretinal degenerations are reviewed, and the existence of unilateral retinitis pigmentosa as a genetic entity is questioned. Prolongation of ERG- a- and b-wave implicit times is characteristic of the tapetoretinal degenerations, and normal implicit times in the fellow eye are suggested as an additional criterion for the diagnosis of unilateral retinitis pigmentosa.

Arden grating test in evaluating "early" posterior subcapsular cataracts.

Patients with "early" posterior subcapsular (PSC) cataracts often complain of severe visual impairment, despite maintenance of relatively good Snellen acuity. The Arden grating test of contrast sensitivity provides a simple method of evaluating visual function in a manner more nearly approximating the real visual world. This test often reveals profound loss of contrast sensitivity in patients with early PSC cataracts who maintain relatively good acuity (20/40 or better) on Snellen's test. Examination of 122 such eyes revealed wide variations in contrast sensitivity, with Arden scores showing better correlation with subjective complaints than with Snellen acuity levels. While Snellen's test measures only one end of the spatial modulation transfer function (MTF), the Arden test plates provide an overall picture of MTF and help us to better understand the effect of PSC cataracts on visual function.

Neural and dural optic nerve measurements with A-scan ultrasonography.

Ultrasonography has been shown to be of great value in studying drusen, cupping, and elevation of the optic disk, as well as mass lesions of the orbital optic nerve. We have found A-scan ultrasonography to be an accurate and simple method of measuring both the diameter of the retrobulbar optic nerve and the size of the subdural perineural space. We have found the intervaginal space of the retrobulbar optic nerve to be uniformly enlarged in papilledema, optic nerve lesions, and endocrine orbitopathy.

Recurrent hemorrhage in traumatic hyphema.

A retrospective review of hospital records reveals that over 90% of recurrent bleeds in traumatic hyphema occur at night. It is postulated that rapid eye movement (REM) sleep, with its displays of saccadic virtuosity, is the major reason for the nocturnal prevalence of such recurrent hyphemas. Theoretical considerations suggesting a more rational therapy of traumatic hyphema are presented and clinical trials are suggested.

Ultrasonic diagnosis of posterior lens rupture.

A case of ultrasonically-diagnosed posterior lens capsule rupture, confirmed at surgery, is presented. The A-scan and contact B-scan findings enabling the diagnosis to be made are presented. The ability to make such a preoperative determination allows for the planning of more rational therapy, and should help improve the final visual results in such cases.

Bilateral oculodermal melanocytosis.

A case of bilateral oculodermal melanocytosis is presented in a Negro man. It is suggested that the term "nervus of Ota" be superseded by the more accurate and descriptive terms of "dermal melanocytosis," "ocular melanocytosis," and "oculodermal melanocytosis."

Blue field entoptoscopy and VER in preoperative cataract evaluation.

Blue field entoptoscopy (BFE) has been described as an effective method for evaluating macular function in patients with dense cataracts. We evaluated 150 preoperative cataract patients with BFE, and found the commercially-available instrument (MIRA BFE 100) to be accurate in patients with 20/400 or better preoperative acuity. There was a high percentage of false negative BFE results in patients with dense cataracts, and light flash VER testing was found to correlate better with attainable postoperative acuity in these patients.

Alpha-chymotrypsin glaucoma.

The postoperative course of 141 eyes in 115 patients undergoing uncomplicated intracapsular cataract extraction was reviewed. Alpha-chymotrypsin as used in our institution did not increase the percentage of eyes developing significant postoperative ocular hypertension. Smaller amounts of enzyme and a shorter waiting period than have heretofore been advocated are adequate for clinically effective zonulysis and appear to be protective against "enzyme glaucoma." Indeed, we found that among non-diabetics, those receiving enzyme experienced a lower incidence of postoperative ocular hypertension than did those not receiving enzyme. The one factor in our series related to an increased percentage of postoperative hypertensive responses was diabetes mellitus.

Ultrasonographic demonstration of patency of subarachnoid-orbital shunt.

Contact B scan photographs of a patent surgically created subarachnoid-orbital shunt are presented. Clinical evidence of cessation of shunt function corresponded with disappearance of demonstrable fluid drainage by ultrasonography.