'Shadow sign' in congenital hypertrophy of the retinal pigment epithelium of young myopic pigmented patients.

PURPOSE Congenital hypertrophy of the retinal pigment epithelium (CHRPE) may simulate choroidal melanoma in certain cases. We report unique clinical features we have observed in cases of CHRPE in young myopic pigmented patients.METHODS Patients who were referred for evaluation of a suspicious choroidal lesion and found to have a CHRPE lesion with the clinical appearance of lesion elevation and a subretinal fluid-like 'shadow sign' were included. Patient and lesion characteristics were tabulated. Available images, including fundus photography, ultrasonography, optical coherence tomography (OCT), and fluorescein angiography (FA) were reviewed.ResultsSix patients were included. The 'shadow sign' was anterior to the CHRPE lesion in all cases. The mean age of the patients was 27.3 years. The ethnicities of the patients were Chinese (n=1), Hispanic (n=3), or African-American (n=2). Five of six patients were myopic.CONCLUSIONS Although most CHRPE lesions appear flat on ophthalmoscopy, lesions in young myopic patients of pigmented ethnicities may appear elevated with a 'shadow sign' due to 'dark without pressure.' This new finding may be related to the vitreoretinal interface in young myopic pigmented patients and must be distinguished from true subretinal fluid and lesion thickness, which are often observed in choroidal melanoma.

Earlier therapeutic effects associated with high dose (2.0 mg) Ranibizumab for treatment of vascularized pigment epithelial detachments in age-related macular degeneration.

SUMMARY STATEMENT: Intravitreal high dose (2 mg) ranibizumab may lead to quicker resolution of choroidal neovascularization (CNV) and associated retinal pigment epithelial detachment in eyes with exudative age-related macular degeneration, although it may possibly correlate with RPE tears in certain cases. PURPOSE: This prospective study compared the outcomes of 0.5 vs 2.0 mg intravitreal ranibizumab injections (RI) for treating vascularized pigment epithelial detachment (vPED) due to age-related macular degeneration. METHODS: Patients with vPED were randomized to receive 2.0 vs 0.5 mg RI monthly for 12 months or for 4 months and then repeated on a pro-re nata basis. Optical coherence tomography, fundus photography, and fluorescein and indocyanine-green angiography were obtained at baseline and subsequent specific intervals. Outcome measures were best-corrected standardized visual acuities, central 1-mm thickness, surface area (SA), greatest linear diameter (GLD), heights (PED and CNV), and amount of subretinal fluid (SRF) and cystoid macular edema (CME). RESULTS: Both groups yielded reductions of the central 1-mm thickness, PED and CNV SA and PED height and GLD, SRF, and CME. Vision improvement and reduction in SRF and PED height occurred earlier for eyes receiving the 2.0 mg dose. Cataract progression was similar but RPE tears developed more often with the 2.0 mg dose. CONCLUSIONS: There were similar visual and anatomical outcomes at the end of the study; however, the higher dose yielded more rapid reductions and more complete resolution of the PED, although there was possible increased tendency for an RPE tear with the higher dose.

Immediate intraocular pressure changes following intravitreal injections of triamcinolone, pegaptanib, and bevacizumab.

PURPOSE: To assess the intraocular pressure (IOP) changes, within the first 30 min after intravitreal injection of 0.1 ml (4 mg) triamcinolone, 0.09 ml (0.3 mg) pegaptanib, and 0.05 ml (1.25 mg) bevacizumab. METHODS: Records of patients who received intravitreal triamcinolone, pegaptanib, and bevacizumab and who had their IOP measured post-injection were reviewed. RESULTS: A total of 212 injections were performed (76 bevacizumab in 63 patients, 42 triamcinolone in 41 patients, 94 pegaptanib in 74 patients). At 10 min, over 87% of eyes receiving each drug had an IOP of less than 35 mmHg. Three of the 42 eyes receiving intravitreal triamcinolone were treated with IOP-lowering drops for pressures of 44, 46, and 60 mmHg. No patients treated with intravitreal bevacizumab or pegaptanib received IOP-lowering drops. The number of eyes in each injection group that had an IOP rise >10 mmHg within 30 min after injection was 27.6% of eyes receiving bevacizumab, 33.3% of eyes receiving triamcinolone, and 36.2% of eyes receiving pegaptanib. At 10 min, eyes with glaucoma were less likely to have an IOP<35 mmHg, but this difference became less marked with time. CONCLUSION: In our series, most patients receiving intravitreal injections did not require IOP-lowering drops after injection, and none required a paracentesis.

Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report.

OBJECTIVE: To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement. METHODS: A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma. RESULTS: Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23%. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52%, eyes 19%, brain and eyes 12%, and systemic 2%. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p = 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months. CONCLUSION: This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.

Outcomes following 25-gauge vitrectomies.

PURPOSE: Twenty-five gauge vitrectomy surgery offers potential advantages over standard 20-gauge vitrectomy surgery, but the short- and long-term post-operative complications, such as cataract formation, are still being evaluated. This study quantifies the outcomes seen following 25-gauge vitrectomies. METHODS: This is a retrospective, consecutive, non-comparative case series of 25-gauge vitrectomies performed between January 2002 and August 2004. Cases without at least 3 months of follow-up and previous vitrectomies were excluded. Analyses were performed with t-test and Kaplan-Meier curves. RESULTS: Seventy-one cases met inclusion criteria. The mean age of the patients was 65 years old (SD 11 years). A variety of surgical indications were included. A statistically significant difference was seen between the mean preoperative visual acuity (20/100) and the mean visual acuity at the 3-month post-operative visit (20/60; P<0.0001). A Kaplan-Meier curve established that for all cases 63.4% of eyes required cataract extraction at 1 year. Total mean follow-up time was 8.6+/-5.5 months. CONCLUSIONS: Statistically significant improvement was seen in mean vision by 3 months following 25-gauge vitrectomy. Cataract formation after 25-gauge vitrectomies remains an important consideration.

The use of 31-gauge needles and syringes for intraocular injections.

PURPOSE: To describe the use of disposable 31-gauge needles for intraocular injections. METHODS: Description of the technique and photographs of the needles. RESULTS: Patients feel the injection less and a smaller postinjection bleb is noted. CONCLUSION: Consideration should be given to the use of disposable 31-gauge needles or syringes for intraocular injections.

Imaging of hydrogel episcleral buckle fragmentation as a late complication after retinal reattachment surgery.

Hydrogel encircling bands were introduced in the early 1980s as a product that was superior to bands composed of silicone rubber or silicone sponge for the surgical treatment of retinal detachment. Late complications consisting of orbital swelling and diplopia requiring band removal began to be reported in the early 1990s. Pathologic studies of these expanded fragments of hydrogel material after removal showed in vivo hydrolysis with foreign body reaction and dystrophic calcification. We report the imaging findings in five patients in whom this late complication developed. Hydrogel fragmentation has a characteristic imaging appearance consisting of a circumferential orbital mass associated with rim enhancement. This appearance should prompt inquiries regarding previous scleral buckle procedures with hydrogel bands. Familiarity with this appearance will avoid misinterpretation and unwarranted biopsy before band removal.

Submacular surgery complicated by a choroidal neovascular membrane at the retinotomy site.

PURPOSE: We report a case of a submacular surgery complicated by a choroidal neovascular membrane at the retinotomy site. METHOD: The patient underwent submacular surgery for the removal of an idiopathic choroidal neovascular membrane. RESULT: Three weeks after surgery, the patient developed a choroidal neovascular membrane at the retinotomy site. CONCLUSION: A choroidal neovascular membrane can occur at a retinotomy site even without the use of endolaser or endodiathermy. The optimal placement of a retinotomy to prevent or minimize symptomatic visual loss from choroidal neovascular membrane requires further study.

Orbital lesions in the blue rubber bleb nevus syndrome.

PURPOSE: To identify ophthalmologic manifestations of the blue rubber bleb nevus syndrome, a rare cutaneovisceral hemangiomatosis. METHODS: The authors report two patients with a diagnosis of blue rubber bleb nevus syndrome with orbital hemangiomas. RESULTS: In one patient, the orbital lesion presented with signs and symptoms similar to an orbital varix and in the other with lid ecchymosis from an eyelid lesion. CONCLUSION: Patients with the blue rubber bleb nevus syndrome may have vascular orbital lesions associated with intermittent proptosis. Ophthalmologists should be familiar with the syndrome and its life-threatening complication of gastrointestinal hemorrhage.

Causes of uveitis in the general practice of ophthalmology. UCLA Community-Based Uveitis Study Group.

PURPOSE: Most uveitis case series have come from tertiary care centers, and the relative frequencies of disorders they report may reflect referral bias. We sought information about the types of uveitis encountered in the general practice of ophthalmology. METHODS: We prospectively examined 213 consecutive cases of general uveitis, defined as intraocular inflammation other than cytomegalovirus retinopathy, seen by a group of community-based comprehensive ophthalmologists. This group of cases was compared with 213 consecutive cases of general uveitis examined by a uveitis specialist at a university referral center in the same community. All cases were categorized by anatomic site of inflammation and disease course, and, if possible, they were assigned a specific diagnosis. Cases of cytomegalovirus retinopathy and masquerade syndrome seen during the same intervals were recorded separately. RESULTS: The distribution of general uveitis cases by anatomic site of disease was significantly different between the community-based practices (anterior, 90.6%; intermediate, 1.4%; posterior 4.7%; panuveitis, 1.4%) and the university referral practice (anterior, 60.6%; intermediate, 12.2%; posterior, 14.6%; panuveitis, 9.4%; P < .00005). A cause or clinical syndrome could be assigned to 47.4% of cases in the community-based practices, and to 57.8% of cases in the university referral practice (P = .03). HLA-B27-associated anterior uveitis, cytomegalovirus retinopathy, and toxoplasmic retinochoroiditis were among the five most common forms of uveitis in both practice settings. CONCLUSION: The relative frequencies with which various forms of uveitis are seen in a tertiary referral center do not necessarily reflect the experience of ophthalmologists from the community in which the center is located. Anterior uveitis and disorders of sudden onset constitute a greater proportion of cases seen by community-based comprehensive ophthalmologists.

Acetazolamide but not timolol lowers aqueous humor flow in sleeping humans.

The effect of timolol, acetazolamide, and the combination of the two drugs on the rate of aqueous formation in 18 healthy human subjects was measured during the day and at night in a placebo-controlled, double-masked, randomized study. In the absence of any drugs, aqueous flow during the day was 2.61 +/- 0.82 (mean +/- SD) microliters/min and at night, 1.08 +/- 0.59, a 59% lower flow rate when compared with the daytime value (P < 0.0001). When compared with these aforementioned control values, timolol alone reduced the rate of aqueous flow by 39% (P < 0.0001) in awake subjects but had no statistically significant effect on the flow rate in sleeping subjects (P = 0.33). Acetazolamide alone reduced aqueous flow during the day by 21% compared with the control flow rate (P = 0.02) and at night by 24% below the nocturnal flow rate in the sleeping eye (P = 0.04). The combination of the two drugs reduced flow during the day by an additional 13% (P = 0.024) compared with the flow rate achieved by timolol alone, and by an additional 32% (P < 0.0001) compared with the flow rate reduction attained by acetazolamide alone. There was no statistically significant difference in the nocturnal flow rates achieved by acetazolamide alone or in combination with timolol (P = 0.37). These data confirm previous studies demonstrating the effect of timolol, acetazolamide, and sleep on the rate of aqueous humor formation. Unlike a previous study, it was found that acetazolamide lowers the aqueous flow below the already low nocturnal flow rate that occurs spontaneously in the sleeping eye.(ABSTRACT TRUNCATED AT 250 WORDS)

A study of aqueous humor formation in patients with cystic fibrosis.

The circadian pattern of aqueous formation and the effect of timolol on aqueous flow was studied in 12 patients with cystic fibrosis. Cystic fibrosis is a disease characterized by a defect in a chloride channel-associated regulatory protein found in epithelial cells. Improper regulation of these chloride channels, causes abnormal composition of exocrine secretions, including respiratory tract, gastrointestinal tract, exocrine pancreas, and sweat glands. Ocular findings previously reported include abnormal endothelial cell permeability, decreased tear secretion, and abnormal tear composition. In this study, aqueous humor flow was measured by fluorophotometry. No statistically significant difference was found when flow rates measured during the morning, during the afternoon, at night, and after topical timolol treatment were compared to normal values. The conclusion is that the beta adrenergically regulated chloride selective channels defective in patients in cystic fibrosis do not play a major role in the formation of aqueous humor or they are not regulated by the cystic fibrosis transmembrane conductance regulator (CFTR).