Treatment of vernal keratoconjunctivitis a double-masked study.

Thirty-one children aged five to 14 years with severe vernal keratoconjunctivitis (VKC) were enrolled in a double-masked study evaluating the efficacy of three ophthalmic preparations: cromolyn sodium (Opticrom) 2%, artificial tears and fluorometholone 0.1% (FML). These drugs were coded respectively as A, B, C, and dispensed in similar bottles. Both eyes were treated, and drug assignment was made at random. Objective assessment of the disease activity was done under slit lamp by two ophthalmologists according to a five-point scale. The results were tabulated and the chi-square test performed. Decoding of the drugs was done only after completion of the statistical analysis. FML was found effective with a statistically significant difference from the other two drugs, both after the first week on the initially assigned treatment (p = 0.05) and on completion of the treatment period according to protocol (p = 0.005). Although several patients improved under cromolyn sodium, there was no statistically significant difference between cromolyn sodium 2% and artificial tears. All the preparations were well tolerated. One patient developed bilateral allergic blepharitis when treated with FML during the study. Another patient who responded well to FML developed posterior subcapsular cataract while continuing treatment with corticosteroids a few months after completion of the study.

Ocular and orbital complications following the treatment of retinoblastoma.

PURPOSE: To investigate the ocular and orbital complications observed in children treated for retinoblastoma. SUBJECTS AND METHODS: We retrospectively studied 73 children (39 boys, 34 girls) suffering from retinoblastoma. Thirty-six had bilateral tumor and 37 unilateral disease for a total of 109 eyes affected. The follow-up was 6-180 months (median 36 months). Enucleation was the most common initial treatment approach in the unilateral group, and radiotherapy (by external beam) was the most common initial therapy in the bilateral group. Cryotherapy, photocoagulation, brachytherapy and/or systemic chemotherapy were used as adjuvant treatments when necessary. Ocular complications were recorded at the follow-up examinations. RESULTS: Cataract developed in 20% of the irradiated eyes. The mean time from irradiation until development of cataract was 28.5 months (6-64 months). Radiation retinopathy developed in 12% and was first detected 11-72 months (mean 37 months) after irradiation therapy. Mild transient keratopathy was observed in all eyes undergoing irradiation, and xerophthalmia in one eye. Complications after enucleation included: marked discharge from the socket (11.0%), extrusion of the implant (9.6%), and contraction of the socket (3.0%). No complications were observed after cryotherapy or laser photocoagulation of the tumor. CONCLUSIONS: Ocular complications after treating children with retinoblastoma are common and may seriously affect the quality of life of children surviving the primary malignancy.

Relationships among visual acuity demands, convergence, and nystagmus in patients with manifest/latent nystagmus.

BACKGROUND: We investigated the role convergence plays in nystagmus dampening, in particular, relationships among visual acuity demands, convergence, and nystagmus. Previously we showed that subjects with idiopathic infantile nystagmus exhibit a range of responses to acuity targets, one of which is nystagmus blockage syndrome. We report herein eye movement responses to acuity targets of patients with manifest/latent nystagmus. METHODS: Fourteen patients, 11 with latent or manifest latent nystagmus and 3 with combined manifest latent with infantile nystagmus, were asked to indicate the direction of the gap in Landolt C optotypes while their eye movements were recorded. RESULTS: The tested patients exhibited various responses to acuity demands: (1) dampening of nystagmus with convergence (i.e., nystagmus blockage syndrome) (5/14 patients), (2) changes in vergence without nystagmus dampening (2 patients), (3) decrease of nystagmus without convergence (2 patients), and (4) little change in nystagmus or vergence (5 patients). In nystagmus blockage syndrome the amount of convergence increased with acuity demands in two of five patients and the convergence duration in four of five patients; nystagmus dampening increased with acuity demands in one of five patients and the blockage duration in four of five patients. CONCLUSIONS: Many, but not all, patients with manifest/latent nystagmus, similar to those with infantile nystagmus, used convergence to dampen their nystagmus. The convergence response tended to increase with acuity demands, but the amount of dampening was idiosyncratic and not predictably related to the measured convergence across patients.

Congenital periodic alternating nystagmus. Diagnosis and Management.

PURPOSE: The purpose of the study is to investigate diagnostic criteria and treatment methods for patients with congenital periodic alternating nystagmus (PAN). METHODS: A retrospective analysis was performed of clinical findings and serial eye movement recordings of patients with congenital PAN. Eighteen patients observed from 1983 through 1996 and diagnosed with congenital PAN are included. Five of these have ocular or oculocutaneous albinism. Nine of the 18 patients were treated. Three had Kestenbaum operations before referral to the authors, one was treated with baclofen, and five had large recessions of the four horizontal recti. The studied parameters included visual acuity (VA) and abnormal head posture (AHP); temporal aspects of PAN cycle, nystagmus waveforms, frequency, amplitude, and velocity; as well as mean foveation fraction, a mean percentage of the nystagmus cycle spent at retinal slip velocities less than 10 degrees per second. RESULTS: The authors diagnosed PAN in 9% of patients with congenital nystagmus, although most had not been diagnosed with PAN before referral, despite changing nystagmus. Sixteen patients had AHP, typically shifting. The PAN cycle was of variable duration, often with asymmetric right- and left-beating components. Although horizontal jerk nystagmus with accelerating slow phase was predominant, other waveforms were encountered in the active phase of PAN. In the quiet phase (close to null zone), similar, but less intense, oscillations than those in the active phase were characteristic. Half of the patients showed a combination of waveforms in both phases. Baclofen treatment was unsuccessful. Patients who had Kestenbaum procedures remained with AHP in the original or opposite direction, without change in nystagmus or VA. Large recessions of four horizontal recti proved uncomplicated. This treatment improved, at least for several years, AHP and VA and caused favorable changes in nystagmus parameters in all patients. Mean foveation fractions increased significantly after surgery. CONCLUSIONS: Congenital PAN often is underdiagnosed. Differing waveforms may indicate PAN. Evaluation of nystagmus, especially before surgery, for at least 3 minutes, preferably with eye movement recordings, is necessary to diagnose PAN and perhaps prevent Kestenbaum procedures, which seem inappropriate. Large horizontal recti recessions seem to provide safe and promising treatment.

The ocular manifestations of Weissenbacher-Zweymuller syndrome.

PURPOSE: Weissenbacher-Zweymuller syndrome (WZS) is an autosomal recessive disorder of delayed skeletal maturation. Its characteristic features include rhizomelic dwarfism with metaphyseal and vertebral changes. It has been challenged whether WZS is a part of the spectrum of Stickler syndrome. We report ocular findings in the largest ever-presented series of patients with WZS. METHODS: Patients underwent a paediatric examination, including assessment of growth and development, genetic work-up and X-ray of vertebra and long bones. All had a complete ophthalmic examination, cycloplegic refraction, and face and body photography. RESULTS: All patients had hypertelorism and protruding eyes. Four patients had refractive errors necessitating optical correction ranging from +3 to -8 D. Two patients had strabismus. None had vitreoretinal degeneration, glaucoma, or cataract. CONCLUSIONS: Ocular manifestations of WZS differ from those in Stickler syndrome, indicating that the two likely represent distinct clinical entities. Strabismus and various refractive errors often accompany WZS. An ophthalmologist should follow children with this disorder from an early age to prevent amblyopia.