Cerebro-hepato-renal syndrome of Zellweger. Ocular histopathologic findings.

An infant who was the product of a full-term pregnancy was born with multiple congenital defects, including diffuse bilateral corneal opacification. He failed to thrive and died at the age of 4 days. Autopsy findings were consistent with those seen in the cerebro-hepato-renal syndrome. Histopathologic examination of both eyes, obtained at autopsy, demonstrated similar changes. The main pathologic findings in the anterior segment were bilateral, diffuse corneal edema associated with paracentral iridocorneal adhesions and focal attenuation of Descemet membrane. Both retinas disclosed a selective degeneration of the outer nuclear layers and photoreceptors, confined mainly to the macular region. Atrophy of the optic disc and nerve, with reactive gliosis and demyelination, were consistent with findings in the central nervous system described in this syndrome.

Orbital involvement in 'sinus' histiocytosis. A report of four cases.

Sinus histiocytosis is a newly recognized benign disease affecting mainly children and young adults and usually having a protracted clinical course that is relatively unaffected by therapy. This paper describes four additional patients who had orbital involvement initially and reviews the salient clinical and histopathologic features of this entity. The outstanding clinical feature is cervical lymphadenopathy. Associated findings include low-grade fever, anemia, leukocytosis, and elevated IgG levels. A small percentage of patients develop proptosis with palpable orbital tumors. Such patients may not have appreciable lymphadenopathy. Progressive proptosis may lead to exposure keratitis, corneal ulceration, endophthalmitis, and loss of the eye. Histopathologically, the lymph nodes and orbital mass show a proliferation of large histiocytes intermixed with a variable proportion of lymphocytes and plasma cells. Lymphocytes and occasionally other cells derived from the hematopoietic system are commonly seen within the cytoplasm of the histiocytes.

An automated visual acuity testing computer program using the Apple II system.

We developed an automated visual acuity testing program that uses an E optotype with surrounding confusion bars. The computer software program runs on Apple II equipment and a black-and-white monitor with a five-inch screen. The program is available in response box and joystick versions. The test is suitable for children older than 31/2 to 4 years of age and for adults. A t-test on the same floppy disk as the visual acuity programs is used to test the probability that the differences in test results are greater than chance. Visual acuities of 20 normal subjects were reduced by means of plus lenses. Test-retest acuity correlation coefficients were similar for letter charts and computer-generated E optotypes, suggesting approximately equal reliability under the test conditions employed. Visual acuities of 12 amblyopic eyes were obtained by a Ferris-type letter chart and computer-generated E optotypes. The correlation coefficient was +0.93, suggesting similar test results by these two methods.

A comparative study of grating and recognition visual acuity testing in children with anisometropic amblyopia without strabismus.

Bailey-Lovie-Ferris visual acuity charts and Teller visual acuity cards were used to compare recognition and grating visual acuity at near testing distances in 32 children with anisometropic amblyopia without strabismus. Appropriate optical corrections were worn. Test-retest intraobserver reliability was higher for letters (r =.95) than for gratings (r = .68). Using 20/30 visual acuity or better as the criterion for normal visual acuity, eight eyes with letter visual acuities ranging from 20/42 to 20/138 would have been inaccurately found to be normal by using the Teller visual acuity cards alone. Grating visual acuity measurements tended to be better than letter visual acuity; and, in general, they did not worsen proportionately with poorer letter visual acuity.

Hemangioendothelioma of frontal bone.

Radiographic studies disclosed that a 5-year-old girl with proptosis and inferior displacement of her left globe had a lytic lesion in the superior orbital rim. Tissue removed at the time of the initial biopsy was not sufficiently distinctive for a definitive diagnosis, but microscopic examination of residual tumor clearly established the diagnosis of hemangioendothelioma of the frontal bone. Electron microscopic studies demonstrated that the vascular structures, which resembled endothelium, contained many thin microfilaments and mitochondria and scattered dense bodies. Each vessel was surrounded by a prominent mantle of pericytes.

Pattern-reversal visual-evoked potentials in the diagnosis of amblyopia in children.

We assessed the potential clinical usefulness of pattern-reversal visual-evoked potentials in the diagnosis of amblyopia. Twenty-seven children with anisometropic amblyopia and four children without amblyopia participated. Estimates of visual acuity for each eye (Snellen visual acuity) were obtained by conventional psychometric methods. Visual-evoked potentials to reversing checks subtending 15 minutes of visual arc were also obtained. Visual-evoked potential testing and interpretation were done in a masked fashion. Ten of the 31 children were retested seven to 21 days after the first test to estimate reliability of the procedures. Of the 27 amblyopic children, 22 were correctly identified by the visual-evoked potential test alone. In four patients initial visual-evoked potential tests failed to identify the disparity in visual acuity between the eyes and retests in two of the four again had false-negative results. In one child initial visual-evoked potential testing incorrectly identified the amblyopic eye but repeat testing did identify it. Of the four children with symmetrically good vision, three were correctly identified as normal by the initial visual-evoked potential test. The other normal child was incorrectly identified by the visual-evoked potential test as having amblyopia.

Development of vision in infants and young children.

Behavioral improvement in the quality of infant vision parallels neuroanatomic and neurophysiologic brain development. Progressive refinement of acuity can be tracked by means of tests that employ fixation and following, optokinetic nystagmus, preferential viewing, and pattern-evoked visual responses. These methods of evaluating vision in the preverbal child differ from each other in several respects; their relative advantages and disadvantages are discussed.

Amblyopia: definition, classification, diagnosis, and management considerations for pediatricians, family physicians, and general practitioners.

Amblyopia is a reduction in the quality of central, corrected vision resulting from a disturbance in retinal image formation during the first decade of human life. Although the neurophysiology and neuropathology of amblyopia are now better understood than in the past, treatment has not changed significantly in more than 200 years. The earlier amblyopia is detected, the shorter the duration of treatment required to reduce its severity. The mainstay of treatment still consists of forcing the use of the amblyopic eye, most often by occlusion of the sound eye. Complications of treatment include occlusion amblyopia and induction of strabismus in straight-eyed patients.

Ophthalmia neonatorum.

Although many types of conjunctival infections in neonates are relatively inconsequential, chlamydial, gonococcal, and herpes simplex infections can cause permanent ocular damage and serious systemic complications. These specific causes are discussed along with silver nitrate conjunctivitis, staphylococcal infections, and nasolacrimal duct obstructions.

Ocular findings in pediatric systemic disease.

The many diseases and disorders that frequently have ophthalmologic implications in the pediatric age group are discussed.

Management of infantile esotropia.

The population of patients with infantile esotropia is etiologically and neuroanatomically heterogeneous, and major advances in management will probably require more accurate subgroup delineation. Amblyopia is relatively common in patients with infantile esotropia. It should always be suspected and, when found, should be vigorously treated. Present methods of amblyopia detection and evaluation in young children are still imperfect. Most clinical evidence suggests rather strongly that sensory and motor functions are more nearly normal if alignment (within 10 prism diopters of orthotropia) is attained within the first 2 years of life. Whether alignment obtained within the first year of life provides higher grades of binocular vision is less certain. Virtually all patients with infantile esotropia fail to develop normal binocular vision. Central scotomas are almost always identifiable, even in patients with optimal motor alignment and with the highest levels of binocular vision. Past surgical protocols have tended to produce an excess of under-corrections. More recent surgical protocols are more generous in terms of millimeters of surgery per prism diopter of deviation. Higher cure rates are now being reported. Patients who are successfully aligned early in life still need careful postoperative monitoring for amblyopia, nystagmus, inferior oblique overactions, dissociated vertical divergence, and accommodative esotropia. These patients require frequent follow-up visits until they reach the age of approximately 9 years.

Wegener's granulomatosis presenting as orbital pseudotumor in children.

The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. A review of the literature reveals that, to date, there are 21 well-documented cases of Wegener's granulomatosis occurring in children under the age of 16. Of these 21 cases, eight patients (39%) manifested ocular or orbital signs during the course of the disease. Four of these cases (50%) presented initially with idiopathic inflammatory pseudotumor. The diagnosis of Wegener's granulomatosis should be considered in children with orbital pseudotumor. Long-term follow-up may be necessary to exclude this possibility.

Practical management of amblyopia.

One hundred sixteen strabismus patients with amblyopia treated with initial full-time or part-time conventional occlusion of the preferred eye followed when necessary with maintenance occlusion up to a maximum of nine years were studied. Based on the need for maintenance occlusion, patients were divided into a primary occlusion group, composed of patients who did not require maintenance occlusion and a maintenance group occlusion group, composed of patients who did require maintenance occlusion. The main difference between the two groups of patients was the degree of amblyopia at the onset of occlusion therapy, being significantly greater in the maintenance occlusion group. The maintenance occlusion group showed a significantly higher incidence of visual acuity regression when evaluated at ages 12 and 13 years. Occlusion therapy administered to strabismus patients in the manner described in this study resulted in significant visual improvement with a regression in visual acuity of no more than an average of one line on the Snellen chart.

Results of combined surgery on the superior oblique and horizontal rectus muscles for A-pattern horizontal strabismus.

Results obtained in 32 patients with A-pattern horizontal strabismus and overacting superior oblique muscles treated with combined bilateral superior oblique tenotomies and bilateral symmetric surgery on the horizontal rectus muscles were analyzed retrospectively. The magnitude of reduction of the A-pattern correlated positively (r = 0.69) with the size of the preoperative A-pattern. Overall, 27 of the 32 patients (84%) had a satisfactory reduction in their A-pattern. Esodeviated patients received less correction in the primary position than exodeviated patients. Postoperative complications were minimal if the superior oblique tendon was hooked under direct visualization, and minimal dissection was performed on the surrounding tissue prior to transecting it near the nasal side of the superior rectus muscle.

Phlyctenular keratoconjunctivitis.

There is growing evidence that a variety of corneal disorders may be expressions of altered immune mechanisms. Phlyctenular keratoconjunctivitis is probably such a condition. Typically described as arising from hypersensitivity to tuberculin protein, other antigens clearly may participate, particularly staphylococcus products. When corneal involvement occurs, it need not be confined to the peripheral cornea. The symptoms of the process may be disproportionate to obvious findings and so exaggerated as to suggest a psychiatric disorder. Resultant visual deficits, if the disease is corneal, progressive, unrecognized, and untreated may be profound. Representative examples of this disease are cited. Immune mechanisms are reviewed. The importance of recognizing the characteristic sign and symptom complex is stressed. Appropriate diagnostic studies and treatment regimens are presented.

Antiemetic effectiveness of intraoperatively administered droperidol in pediatric strabismus outpatient surgery--preliminary report of a controlled study.

Low doses (0.05 mg/kg) of intravenously administered droperidol were given intraoperatively to randomly assigned pediatric strabismus patients in a controlled double-masked paradigm. No difference between control and treatment groups in the severity of vomiting was noted in the postanesthesia recovery room, but such a difference was probably present in the hospital rooms during the interval between room arrival and the meeting of hospital discharge criteria. Administration of the drug did not appear to produce somnolence sufficient to delay postoperative recovery.

Preschool visual acuity screening tests.

The purpose of the study was to evaluate the relative merits of two screening tests used for visual acuity assessment of preschool children. The tests that were compared were the Good-Lite Company versions of the E-Test and of the STYCAR (Screening Test for Young Children and Retardates). The former is the most popular method for evaluating central acuity in young children in this nation; the STYCAR is a relatively new letter-matching-test developed in England, where it is widely employed. The E-Test poses left-right orientation problems which are eliminated by the symmetrical letters H, T, O and V utilized in the Letter-Matching-Test. Both visual acuity tests were administered on two separate occasions by personnel from the Prevention of Blindness Society of Metropolitan Washington to 633 preschool children in Washington, D.C. By random selection, 150 of the children received the E-Test at both sessions, 162 children received the Letter-Matching-Test at both sessions, 160 chilt athe the second session, and 161 children received the Letter-Matching-Test at the first session and the E-Test at the second session. The author medically examined the eyes of 408 of the 633 children without knowledge of which test had been initially administered. Statistical analysis of the data obtained from the study indicated that the Letter-Matching-Test was significantly better in terms of testability rates, group and individual instruction time, and performance time. The E-Test was more reliable in terms of test-retest acuity scores and was also more valid in terms of agreement between pass-fail results obtained at the first screening session and two levels of pass-fail refraction criteria.