Superior oblique myectomy and trochlear resection for superior oblique myokymia.

PURPOSE: To describe our experience with myectomy of the superior oblique muscle combined with resection of the trochlea for recurrent or primary superior oblique myokymia (SOM). DESIGN: Retrospective, interventional case series. METHODS: We performed superior oblique myectomy combined with resection of the trochlea in 3 patients with SOM in whom medical management had failed. In 2 patients, the symptoms of myokymia were recurrent after previous superior oblique tenectomy, and in 1 patient, our procedure was the first surgery. RESULTS: All 3 patients have experienced complete symptomatic relief from SOM with follow-up ranging from 1 to 22 years. Iatrogenic superior oblique palsy has been managed in each patient. Dysesthesia in the infratrochlear and supratrochlear regions was judged by each patient to be much less bothersome than the symptoms of SOM. CONCLUSIONS: We recommend myectomy of the superior oblique muscle combined with resection of the trochlea if symptoms of SOM recur after a prior superior oblique tenectomy. Based on this small series with long follow-up, the procedure also may be considered as the primary operation for SOM that fails medical management.

Genetic and clinical evaluation of juvenile retinoschisis.

Juvenile retinoschisis is a rare retinal dystrophy caused by RS1 gene mutations.(1) Clinical examinations and molecular testing definitively diagnosed juvenile retinoschisis in 2 male infants, one of whom had a novel mutation not previously reported in the United States. Genetic testing may be the simplest way to confirm this diagnosis in infants.

The effect of colored crowding bars on the HOTV visual acuity test in amblyopic patients.

BACKGROUND: Colored versus black crowding bars surrounding a black optotype may induce a greater level of interest in visual acuity testing in young children. To evaluate the adequacy of colored bars for a satisfactory crowding effect in children with amblyopia, we compared visual acuity measurements using black versus red, green, or yellow bars. METHODS: To choose the best color to compare with black, 53 patients with amblyopia were randomly assigned to 1 of 3 crowding bar colors. Subjects had the visual acuity of each eye measured with black HOTV optotypes and bars and then with black optotypes and either red, green, or yellow bars. In a second phase of the study red crowding bars were compared with black in a group of 48 different patients with amblyopia. RESULTS: The difference in logMAR visual acuity between amblyopic eyes tested with red versus black crowding bars was not statistically significant (mean visual acuities of -0.24 and -0.26, respectively; p = 0.315), but the difference was significant with either green (-0.30 and -0.34; p = 0.047) or yellow (-0.14 and -0.24; p = 0.0003). In the nonamblyopic eyes none of the colored crowding bars yielded significantly different visual acuity measurements compared with black. In the second phase of the study red crowding bars yielded an average of one letter worse visual acuity than black bars, but the difference was not statistically significant. CONCLUSIONS: Red crowding bars surrounding black optotypes are an acceptable alternative to black crowding bars for the measurement of visual acuity in amblyopic eyes.

Application of genetic approaches to ocular disease.

The human eye is a complex organ whose development requires extraordinary coordination of developmental processes. Multiple genes responsible for the proper development and maintenance of the vertebrate eye have been identified and shown to be involved in a variety of debilitating ocular conditions. Genetic diseases involving the eye represent a leading cause of blindness in children and adults. This article summarizes current genetic approaches and their application to studies of ocular disease.

Comparison of the HOTV and Lea symbols visual acuity tests in patients with amblyopia.

PURPOSE: To determine whether the Lea symbols visual acuity test, compared with the HOTV visual acuity test, overestimates visual acuity in patients with amblyopia. PATIENTS AND METHODS: Fifty-nine patients with amblyopia or a history of amblyopia treatment had visual acuity measurements in both eyes with the HOTV and Lea symbols tests. Crowding bars, separated by a width of 1 optotype, and the original pilot-testing protocol in the Amblyopia Treatment Study were used to quantify visual acuity. RESULTS: The patients' ages ranged from 4 to 35 years (mean age, 10.1 years). Visual acuity in the amblyopic eyes ranged from 20/20 to 20/250. The mean visual acuity difference (HOTV scores vs Lea symbols scores) was -0.056 logMAR units (P < .001), slightly more than half of a line. The negative value indicates that patients performed better on the HOTV test. In nonamblyopic eyes, the mean difference was -0.05 logMAR units (P < .001). Patients with strabismic amblyopia had a mean difference of -0.05 logMAR units (P = .08); those with anisometropic amblyopia had a mean difference of -0.07 logMAR units (P = .002). Patients 8 years and younger had a mean difference of -0.04 logMAR units (P = .05); those older than 8 years had a mean difference of -0.06 logMAR units (P = .002). CONCLUSIONS: In both amblyopic and nonamblyopic eyes, visual acuity measurements were better on HOTV testing compared with Lea symbols testing. The differences persisted regardless of patient age and the cause and severity of amblyopia. Overestimation of visual acuity by the Lea symbols test was not found.

Outcome of surgery for esotropia in children with down syndrome.

BACKGROUND AND PURPOSE: The outcome of surgery for esotropia in children with Down syndrome has not received significant attention. PATIENTS AND METHODS: We reviewed motor outcomes of esotropia surgery in all patients with Down syndrome in our practice from 1991-2001. RESULTS: Twenty-one patients had surgery for esotropia. The mean age at surgery was 55 months. The mean preoperative esotropia was 36(Δ). Three patients required a second surgery: two for residual esotropia and one for consecutive exotropia. A second operation was recommended for two additional patients: one with residual esotropia and one with consecutive exotropia. The mean length of follow-up was 39 months. At the last follow-up examination, fourteen patients had a deviation of 0-10(Δ) (thirteen esotropic and one exotropic). Five patients had a final deviation of 11-20(Δ) (four esotropic and one exotropic). One patient had a residual esotropia of 21-30(Δ) and one had greater than 30(Δ) of residual esotropia. CONCLUSION: The motor outcomes for esotropia surgery in children with Down syndrome were not as good as those in children with acquired esotropia but were comparable to those in children with infantile esotropia. More children had residual esotropia than consecutive exotropia, which may reflect a tendency toward conservative amounts of surgery or an underlying esotropic tendency in Down syndrome. Parents of children with Down syndrome may be counseled to expect good outcomes in the surgical management of esotropia.