Bilateral idiopathic Brown's syndrome with delayed onset in the second eye.

PURPOSE: We describe 6 cases of a previously unreported variation of bilateral Brown's syndrome that presented in congenital form in one eye and developed later in the fellow eye with no underlying cause. METHODS: We reviewed the clinical records of 6 patients from 6 separate practices to determine whether there were any common clinical features on presentation or in their clinical courses. RESULTS: All 6 patients were diagnosed with unilateral congenital Brown's syndrome at the first ophthalmologic assessment but showed no evidence of the syndrome in the fellow eye. In 5 cases the contralateral syndrome developed in the second eye after surgery was performed on the first eye, and in 1 case it developed before any surgery was done. The ages at onset of the syndrome in the second eye ranged from 2 to 8 years. None of the children had any evidence of systemic illness or local orbital disease to explain an acquired Brown's syndrome. CONCLUSION: To our knowledge, this is the first reported series of cases of bilateral Brown's syndrome that manifested sequentially in the eyes with no known causes for an acquired syndrome in the second eye. This finding supports the premise that congenital and acquired Brown's syndrome are on a continuum with a common pathophysiology of restriction of free movement of the superior oblique tendon in the trochlea.

Focal therapy in the management of retinoblastoma: when to start and when to stop.

PURPOSE: To describe the treatment effects and side effects of different modalities of focal treatment used for retinoblastoma. METHODS: Green (532-nm) laser, continuous wave Nd:YAG (1064-nm) laser, transpupillary or transcleral (810-nm) laser, and cryotherapy were used in the treatment of 46 eyes in 35 patients affected with retinoblastoma. The number of treatment sessions and the amount of energy applied were recorded in an attempt to determine the amount of energy required to adequately treat tumors of various sizes. In addition, we have attempted to determine when treatment becomes overtreatment and is likely to lead to complications. RESULTS: The treatment endpoint for laser in this study was calcific, gliotic, or flat scars. Small tumors (<2 mm in height, <4 DD) were successfully treated in 3 or fewer sessions of 532-nm laser. Anterior small tumors were successfully treated with transcleral 810-nm laser or cryotherapy. Medium tumors, between 2.0 and 4.0 mm in thickness, required 2 to 9 treatments to achieve a good response and often required the addition of chemotherapy to reduce the size of the tumor before or during laser treatment. Large tumors required chemotherapy combined with many laser treatments for complete control. Complications associated with excessive laser were vitreous condensation with traction, vitreous hemorrhage, retinal detachment, tumor break, cataract formation, and iris burns. CONCLUSION: Laser treatment alone for small tumors, and combined with cryotherapy and chemotherapy for larger tumors, is effective in the treatment of retinoblastoma. Complications of focal therapy can most often be avoided by using the minimal effective laser power.

Visual prognosis of Coats' disease.

PURPOSE: Our purpose was to determine the visual prognosis of retinal telangiectasia (Coats' disease). METHODS: We performed a retrospective review of 35 patients with Coats' disease seen at the Hospital for Sick Children, Toronto, Canada between 1987 and 1996. Ten patients were excluded because of incomplete records. Treatment modalities consisted of no treatment, cryotherapy with and without 532 nm laser through the indirect ophthalmoscope, and enucleation. Visual outcome was determined where possible. RESULTS: Median follow-up was 4.5 years. Deterioration in visual acuity was associated with the presence of greater than 5 clock hours of involved retina and retinal detachment at diagnosis. Final visual acuity did not correlate with age of onset of disease. No eye treated with cryotherapy progressed to retinal detachment. CONCLUSIONS: Aggressive treatment of Coats' disease with cryotherapy with or without 532 nm laser, before retinal detachment, is likely to stabilize vision and decrease the risk of future total retinal detachment.

Chemotherapy with focal therapy can cure intraocular retinoblastoma without radiotherapy.

BACKGROUND: External beam radiotherapy is standard treatment for medium and large, or visually threatening, intraocular retinoblastoma but markedly increases the risk of cosmetic deformities and second malignant neoplasms in children with germline RB1 mutations. Large tumors and those with vitreous seeds do poorly despite radiotherapy. Chemotherapy traditionally is ineffective for intraocular retinoblastoma, perhaps because many retinoblastomas overexpress the multidrug resistance protein, P-glycoprotein. OBJECTIVE: To avoid radiotherapy in the management of intraocular retinoblastoma by using chemotherapy and focal therapy. INTERVENTIONS: We shrank retinoblastomas in 40 eyes of 31 patients that conventionally should be enucleated or receive radiotherapy by using chemotherapy (ie, vincristine-teniposide, 8 eyes; additional carboplatin, 32 eyes) combined with the administration of cyclosporine, a known multidrug-resistance-reversal agent. We then consolidated these responses to chemotherapy by subsequent 532- and 1064-nm laser therapy and cryotherapy. RESULTS: At the median follow-up of 2 2/3 [corrected] years (range, 1/10-4 3/4 years), the results of treatment were excellent. The actuarial relapse-free rate was 89% in patients not previously treated (91% for 28 eyes) and 67% in patients treated after relapse from previous therapy (70% for 12 eyes). For the eyes with the worst prognosis (ie, vitreous seeds), the relapse-free rate was 88%, better than previously reported. Cyclosporine is nontoxic and did not enhance the expected toxic effects of chemotherapy. Most eyes required laser therapy, cryotherapy, or both for consolidation of tumor control. CONCLUSIONS: This pilot study suggests that most retinoblastomas are curable by combining chemotherapy with cyclosporine therapy, laser therapy, and cryotherapy, without requiring external beam radiotherapy. We propose a randomized trial to clarify the role of cyclosporine.

Combining cyclosporin with chemotherapy controls intraocular retinoblastoma without requiring radiation.

Chemotherapy without radiation has not controlled most intraocular retinoblastoma, perhaps because of the common high expression of multidrug resistance P-glycoprotein that we found in retinoblastoma. Cyclosporin blocks P-glycoprotein-induced efflux of vincristine and teniposide in vitro, and possibly modulates responses to carboplatin. To avoid eye irradiation in bilateral retinoblastoma patients with RB1 germline mutations, which incurs a high second malignancy rate, we added cyclosporin A to a vincristine-teniposide-carboplatin protocol and consolidated chemotherapy responses with focal therapy. We scored patients requiring irradiation, enucleation, or focal ablation of central vision as failures. In 21 study patients, the overall relapse-free rate at a median follow-up of 3.3 years was 76%, with a rate of 92% for newly diagnosed and 50% for previously treated, relapsed retinoblastoma. Our results for the most unfavorable tumors with vitreous seeds (86% at 3.5 years) are better than published success rates of irradiation for similar tumors, or irradiation with the same chemotherapy without cyclosporin (45% at 2. 6 years). These results also exceeded our historic success rate with similar chemotherapy without cyclosporin, focal therapy, and/or radiation in 19 equivalently poor-risk patients (relapse-free rate 37% at a median follow-up of 5.6 years, P = 0.032), 16 of whom were previously untreated (relapse-free rate also 37%, P = 0.012). A better outcome occurred with higher cyclosporin blood levels and projected tissue exposure. Cyclosporin did not enhance the usual chemotoxicity. This clinical study suggests that cyclosporin improves the long-term response of retinoblastoma to chemotherapy, possibly by more than one mechanism.

Visual function after optic neuritis: a preliminary study.

We assessed visual function after recovery from optic neuritis in 15 consecutive patients using five objective tests: colour vision testing with Ishihara colour plates and with D15 colour desaturated spots, Humphrey automated perimetry, contrast acuity testing with Regan letter charts and testing of visual evoked response (VER). Recovery of visual function was not found to be dependent on presenting Snellen visual acuity or treatment with oral steroids. The most sensitive measures of residual visual deficit were mean defect on automated perimetry, low-contrast acuity and VER.