Natural history of presumed congenital Brown syndrome.

OBJECTIVE: To evaluate the stability of the ocular alignment in patients with presumed congenital Brown syndrome. DESIGN: A retrospective review of patients with Brown syndrome with an emphasis on nonsurgical cases. Follow-up of at least 1 year was required for inclusion in the study. SETTING: Patients were selected for this study from the pediatric ophthalmology services at the Ivey Institute of Ophthalmology, London, Ontario, and the Children's Hospital of Eastern Ontario, Ottawa. PATIENTS: A cohort of 71 patients with presumed congenital Brown syndrome. Two cases were bilateral. Eleven cases were excluded because of insufficient length of follow-up, leaving 60 patients with an average follow-up of 46 months. All patients were assessed and followed up by a pediatric ophthalmologist. RESULTS: Of 38 patients who had no hypotropia in primary position at presentation, only two (5%) patients experienced a worsening with the development of a small vertical strabismus during the follow-up period. Six (10%) of the entire group of 60 patients experienced a complete spontaneous resolution of the deficiency in elevation at 4, 6, 8, 10, 12, and 15 years of age. CONCLUSION: Among patients with congenital Brown syndrome, those who are orthotropic in the primary position tend to remain stable or improve over time without surgical intervention.

Auditory function in Duane's retraction syndrome.

We obtained audiograms and auditory brainstem responses from 44 patients with Duane's retraction syndrome to assess the incidence and nature of hearing deficit. Of 44 patients, seven (15.9%) had evidence of hearing impairment. Three (6.8%) subjects had a temporary conductive hearing loss because of middle ear fluid, and another patient had hearing loss from Crouzon's disease. The remaining three (6.8%) patients demonstrated sensorineural hearing deficit. This hearing impairment was attributed to a cochlear lesion and not to a pontine lesion. We believe that the frequency of sensorineural hearing loss in these patients warrants hearing screening programs similar to those used for infants in neonatal intensive care units.

Late results of superior oblique muscle tenotomy in true Brown's syndrome.

We re-assessed nine patients (aged 2 to 28 years) in whom true Brown's syndrome had been diagnosed and superior oblique muscle tenotomy performed. The postoperative period ranged from three to 12 years. The final results were divided into three groups: (1) Patients with partial residual Brown's syndrome postoperatively who gradually improved spontaneously over several years, with one exception. (2) Patients who had minimal or moderate superior oblique muscle paresis, secondary overaction of the homolateral inferior oblique muscle, and excellent binocular single vision postoperatively who improved spontaneously. In Groups 1 and 2, the study showed that the long-term results are better than those immediately after surgery, and the surgeon should not be too hasty in reoperating. (3) Patients with marked superior oblique muscle paresis and secondary homolateral inferior oblique muscle overaction postoperatively did not improve spontaneously and required a weakening procedure on the homolateral inferior oblique muscle.

Topical 2.5% and 5% phenylephrine: comparison of effects on heart rate and blood pressure.

We compared the effects of 2.5% and 5% concentrations of topical ocular phenylephrine hydrochloride on blood pressure and heart rate in 200 adult patients undergoing extracapsular cataract surgery under either general or neuroleptic anesthesia. Although there was little initial change in mean systolic or diastolic blood pressure in either group, once anesthesia was induced there was a rise of 1 to 5 mm Hg in the lower-dose group, compared with 17 to 26 mm Hg in the higher-dose group. A slight decrease in mean heart rate of 5 to 6 beats/min was noted immediately after the instillation of either the 2.5% or 5% solution. The results suggest that lower doses of topical phenylephrine are safer than higher doses when used preoperatively. This may be particularly significant in hypertensive patients, who have higher baseline blood pressure.

Factors associated with binocular single vision in microtropia/monofixation syndrome.

We reviewed the charts of 398 patients with microtropia/monofixation syndrome to determine what factors influence the level of binocularity attained. Most patients (82%) exhibited some degree of stereoacuity. Patients with a later age at presentation of the initial deviation were more likely to exhibit stereoacuity (p less than 0.001). Patients with smaller initial deviations were also more likely to exhibit stereoacuity (p = 0.016), as were those with smaller manifest deviations on final testing (p less than 0.001). Patients with amblyopia on final testing were less likely to demonstrate stereoacuity (p = 0.0001). Generally, the more intervention required in the form of optical or surgical correction, the poorer the level of binocularity.

Comparison of 0.5% cyclopentolate plus 0.5% tropicamide and 1% cyclopentolate alone for mydriasis of dark irides.

We studied the mydriatic and cycloplegic effect of a single dose of a combination of 0.5% cyclopentolate hydrochloride and 0.5% tropicamide in 20 healthy subjects with dark brown irides. The mean change in dilatation with the combination drop was 3.5 mm, compared with 0.6 mm with 1% cyclopentolate alone (p less than 0.001). In 92% of 26 eyes studied the difference in refraction between the combination drop and 1% atropine was +1.00 dioptre or less. The combination drop safely provided satisfactory mydriasis and cycloplegia in 20 minutes, allowing for rapid and accurate examination of these patients.

Ocular and auditory toxicity of long-term, high-dose subcutaneous deferoxamine therapy.

There have been few reported ocular side effects of parenterally administered deferoxamine when used for the treatment of transfusional iron overload or acute iron poisoning. No auditory side effects have previously been reported. We describe two siblings with beta-thalassemia major who, while receiving daily subcutaneous infusions of deferoxamine, experienced visual loss secondary to optic neuropathy and sensorineural hearing loss. After discontinuation of the drug one sibling showed almost complete reversal of the optic neuropathy, but the other had a permanent unilateral visual loss. Both had a permanent hearing loss but benefited from hearing aids. The mechanism of these complications is presently unknown. Patients receiving deferoxamine should be closely monitored for ocular and auditory side effects. When such effects are detected the drug should be discontinued and the patient observed for improvement. When improvement has stabilized, therapy should be restarted at a reduced dosage.

The 20-prism-dioptre base-out test: an indicator of peripheral binocularity.

OBJECTIVE: To assess the ability of the 20-prism-dioptre base-out test to detect the presence of peripheral fusion in children too young to undergo formal sensory testing. DESIGN: Case series. SETTING: Pediatric ophthalmology service at the Children's Hospital of Western Ontario, London. PATIENTS: Eighty orthophoric patients (mean age at presentation 19 months [range 4 to 50 months]) with a family history of strabismus or with a diagnosis of pseudostrabismus, blocked tear duct or chalazion, followed for an average of 36 (range 6 to 129) months, and 41 patients with surgically corrected infantile esotropia (mean age at testing with the 20-PD base-out test 21 months [range 9 to 50 months]), followed for an average of 53 (range 14 to 117) months. INTERVENTIONS: Twenty-PD base-out test and red-green Worth four-dot test at near (0.3 m). OUTCOME MEASURE: Presence of peripheral fusion. RESULTS: The 20-PD base-out test had a positive predictive value of 100% in detecting peripheral fusion in the orthophoric patients and 93% (95% confidence interval [CI] 82% to 100%) in the patients with surgically corrected infantile esotropia. CONCLUSIONS: Although the 20-PD base-out test had a negative predictive value of 21% (95% CI 0% to 43%), a positive response remains a useful indicator of peripheral binocular single vision in children too young to undergo more formal sensory testing.

Pseudophakic bullous keratopathy. A clinical-pathologic analysis.

Of 226 patients who had an intraocular lens implanted over a three year period, 6.2% developed bullous keratopathy compared with 2.2% of 90 patients who had a standard cataract extraction performed by the same surgeon. An additional 4.0% of the lens implant patients developed peripheral or intermittent corneal edema. The factors related to the higher incidence of this complication in lens implant patients included corneal touch during implantation, prolonged postoperative iritis and, in cases of mild or peripheral corneal edema without bullous keratopathy, recurrent lens dislocation. The most consistent pathologic findings in 10 corneas that required keratoplasty were epithelial edema and loss of corneal endothelium. Means to prevent this complication are discussed.

Duane's retraction syndrome: southwestern Ontario experience.

We describe the clinical ocular and medical manifestations in 71 patients with Duane's retraction syndrome. We compare our results to those of recent studies, with special focus on the prevalence of anisometropia and amblyopia, found in 14% and 17% respectively.

Vertical congenital ocular motor apraxia.

The authors describe a case of vertical congenital ocular motor apraxia (COMA). The pathways of vertical saccades and pursuits are briefly outlined, and a possible cause of vertical COMA is suggested. A neuropathological correlate is needed to confirm the cause of both vertical and horizontal ocular motor apraxia.

Treatment of blepharospasm and hemifacial spasm with botulinum A toxin: a Canadian multicentre study.

Botulinum A exotoxin was recently approved for use in Canada. We describe the efficacy of botulinum toxin in the management of 235 patients with blepharospasm (mean age 64.3 years) and 130 patients with hemifacial spasm (mean age 60.4 years) treated at three Canadian ophthalmologic centres between 1984 and 1989. A total of 98% of the patients with blepharospasm and 100% of the patients with hemifacial spasm had significant relief of their symptoms; however, 11% of the former and 2% of the latter did not respond to the usual starting concentrations of the drug and needed stronger dosages for relief. The duration of relief varied widely in both groups. Up to 7% of patients had ineffective treatments but responded to subsequent injections. Analysis of variance and linear trend statistics showed that there were no changes in the mean duration of relief over the first several treatments for individual patients in either group. Side effects were transient and included ptosis, exposure keratitis, epiphora and strabismus.

Changes in astigmatism after surgery for congenital ptosis.

To determine the effect of ptosis surgery on the astigmatic refractive error in pediatric patients, we assessed 88 eyes postoperatively at 3, 6, and 12 months. Thirty-six eyes of unilateral cases were used as controls. There was an overall increase in average astigmatic refractive error of the study group by 0.30 diopters, while the control group decreased 0.15 D. Thirty-six percent of study eyes changed by more than 0.75 D. Results were similar for both fascia lata slings and levator resections. Those patients older than 4 years showed an increase in average cylinder (0.50 D), while those younger than 4 years decreased their average cylinder (0.20 D). Careful monitoring with postoperative refractions is required in pediatric ptosis patients.

Ocular alkali burn associated with automobile air-bag activation.

Alkali burns of the eye can result in permanent visual impairment and are therefore potentially devastating. Immedicate diagnosis and treatment are essential to a good prognosis. The authors report the case of a 52-year-old woman who suffered alkali keratitis as the result of the activation of an automobile air bag. This type of injury will be seen more frequently as more cars are equipped with air bags and should be suspected in drivers and passengers involved in accidents in which air bags have been activated.

Iris lymphoma in a pediatric cardiac transplant recipient: clinicopathologic findings.

PURPOSE: To report the unusual presentation of a confirmed iris lymphoma in the setting of posttransplant lymphoproliferative disorder. DESIGN: Case report with clinicopathologic findings. METHODS: The medical record was reviewed for clinical manifestations, course of disease, and pathologic and radiologic findings. RESULTS: A 4-year-old girl who had undergone a cardiac transplant at 3 months of age was found to have unilateral iris lesions associated with granulomatous uveitis. Her condition was unresponsive to local and systemic steroids, so she underwent an iris biopsy that was found to contain a B cell-derived monoclonal lymphoproliferation. Treatment with systemic chemotherapy including cyclophosphamide and prednisone resulted in a transient, partial response. Localized radiotherapy induced total regression of the iris lesions. She continues to be observed and has been free of recurrence or metastasis for the past 12 months. CONCLUSIONS: Because of the increased use of organ transplantation, eye care specialists should consider the diagnosis of intraocular lymphoma when confronted with uveitis-like syndrome unresponsive to topical steroid therapy. Although there are numerous treatment options for intraocular lymphoma, radiation therapy was found to be effective in the treatment of this case.