Diagnosis of neuronal ceroid lipofuscinosis by ultrastructural examination of peripheral blood lymphocytes.

A 7-year-old child with a history of seizures, psychomotor regression, and progressive visual loss was found to have juvenile type neuronal ceroid lipofuscinosis on the basis of characteristic ophthalmoscopic and electroretinographic findings. Although transmission electron micrographs of two separate skin biopsy specimens were normal, peripheral blood lymphocytes were vacuolated and demonstrated, by ultrastructural examination, characteristic "fingerprint profiles" confirming the diagnosis. Peripheral blood lymphocytes from the patient's mother, who had an acquired seizure disorder, demonstrated vacuolated lymphocytes with membranous formations and osmophilic granular bodies, as revealed by electron microscopy. Examination of peripheral blood lymphocytes can be a sensitive diagnostic test in suspected neuronal ceroid lipofuscinosis and may be useful in identifying carriers or family members in the early clinical stages of the disease.

Asymptomatic rhegmatogenous retinal detachments.

OBJECTIVE: To describe the clinical characteristics and risk of progression of asymptomatic rhegmatogenous retinal detachments. METHODS: We retrospectively reviewed the clinical records of 28 patients (31 eyes) with asymptomatic rhegmatogenous retinal detachments followed up without surgery for 0.5 to 12.1 years (mean, 3.4 years). Tractional tears were present in six eyes and atrophic holes in 25 eyes. In five patients, the asymptomatic retinal detachment was noted when the patient presented with a symptomatic retinal detachment in the fellow eye. RESULTS: Twenty-nine of the 31 eyes remained asymptomatic without progression of the retinal detachment. Two eyes progressed to a symptomatic retinal detachment 2.25 and 3.3 years after the initial examination, underwent a successful scleral buckling procedure, and maintained 20/20 visual acuity. CONCLUSIONS: Observation can be considered a reasonable option in the treatment of patients with asymptomatic retinal detachments. Chart documentation of the risks and benefits of observation and instruction of the patient on self-monitoring of the peripheral visual field are necessary in such patients.

Nonsurgical management of macular hemorrhage secondary to retinal artery macroaneurysms.

OBJECTIVE: To report visual acuity outcomes of nonsurgical management of macular hemorrhage secondary to retinal artery macroaneurysms. METHODS: Forty-one patients at multiple centers with macular hemorrhage secondary to retinal artery macroaneurysms managed with observation alone were reviewed. Time to clearance of macular hemorrhage, visual acuity at final follow-up, and presence or absence of macular pigmentary changes after absorption of the hemorrhage were recorded for each patient. RESULTS: On initial examination, visual acuity was 20/200 or worse in all except 4 patients (3 with 20/70, 1 with 20/80). At an average follow-up of 15. 7 months, a final visual acuity of 20/40 or better was achieved in 15 eyes (37%), between 20/50 and 20/100 in 12 (29%), and 20/200 or worse in 14 (34%). Macular pigmentary abnormalities were noted after clearance of the hemorrhage in 23 (56%) of 41 cases, and these eyes generally had worse visual acuity outcomes. CONCLUSIONS: In eyes with macular hemorrhage secondary to retinal artery macroaneurysms managed with observation alone, good visual acuity outcomes can often be achieved. Poorer visual acuity outcomes are associated with macular pigmentary changes after resorption of blood. Arch Ophthalmol. 2000;118:780-785

A model for predicting the site of paraxial retinal lesions secondary to "coaxial" operating microscope illumination systems.

We measured a 15 +/- 2-mm displacement of the illuminating element of the coaxial operating microscope from the true optical center of the microscope. We further calculated the displacement of the image of the illuminating element of the operating microscope on the retina of the simplified schematic eye and found that the "coaxial" operating microscope would focus the light between 1.3 and 1.8 mm superior to the fovea of the eye, depending on the theoretical refractive status of the simplified eye. Actual measurements on a simulated retina (35-mm camera) confirmed the calculated displacements. The deviation varied with eye tilt and microscope position. We also found that the maneuvers commonly used by anterior segment surgeons of tilting the microscope and rotating the eye downward tended to displace the image of the illuminating element closer to the fovea, increasing the chance for symptomatic retinal damage. A combination of such maneuvers may reduce the potential for clinically significant retinal phototoxicity by displacing the light further inferior to the fovea.

Management of endophthalmitis while preserving the uninvolved crystalline lens.

Twelve phakic eyes (11 patients) with culture-positive endophthalmitis were treated without removal of the uninvolved crystalline lens. Eight eyes were treated by pars plana vitrectomy and intraocular injection of antibiotics. Four eyes were treated with intraocular antibiotic injection alone. The clinical infection was successfully eradicated in all patients, including one patient treated with reinjection of antibiotics. During follow-up after successful treatment, eight eyes had progression of a preexisting lens opacification, two eyes had stable lens opacification, and two eyes maintained clear lenses. Six of 12 eyes achieved 20/80 or better visual acuity with an average follow-up time of 13 months. Six of eight eyes treated with pars plana vitrectomy and intraocular antibiotic injection achieved this level of visual acuity in contrast to none of four eyes treated with only intraocular antibiotic injection. These results indicated that endophthalmitis in phakic eyes can be successfully treated while preserving a clear, uninvolved crystalline lens by the use of pars plana vitrectomy and intraocular administration of appropriate antibiotics.

Choroidal nonperfusion in giant cell arteritis.

A 68-year-old man had visual loss secondary to isolated choroidal nonperfusion as a clinical manifestation of giant cell arteritis. Ophthalmoscopy disclosed scattered yellow-white lesions at the level of the retinal pigment epithelium in the posterior pole of the right eye. Intravenous fluorescein angiography demonstrated marked delay in choroidal filling of the macula in the right eye. There was no ophthalmoscopic or angiographic evidence of anterior ischemic optic neuropathy or central retinal artery occlusion. After approximately 72 hours of intravenous corticosteroid therapy, the patient's visual acuity improved and repeat intravenous fluorescein angiography showed normal choroidal circulation. Isolated choroidal ischemia is a potential cause of reversible visual loss in patients with giant cell arteritis.

The site of operating microscope light-induced injury on the human retina.

We determined the site of the focal illumination from the Zeiss OPMI-6 operating microscope on the retina of the phakic and aphakic human cadaver eye by directly observing the illuminating element image on the posterior scleral surface of the globe. With the eye straight ahead and the operating microscope level, the focal oval area of retinal illumination was located superior to the foveola in both the phakic and aphakic eye. Tilting the operating microscope 10 degrees toward the surgeon displaced the entire illuminating element image 0.50 mm below the foveola in the phakic eye and 0.25 mm below the foveola in the aphakic eye. Rotating the eye inferiorly 10 degrees displaced the entire illuminating element image 1.0 mm below the foveola in the phakic eye and 1.25 mm below the foveola in the aphakic eye. Centering the field of view superiorly (viewing the superior limbus) paradoxically displaced the illuminating element image inferiorly, resulting in central foveal illumination. Foveal light exposure was avoided in most eye positions by tilting the microscope at least 10 degrees toward the surgeon.

Gentamicin and other antibiotic toxicitiy.

Antibiotics have the potential to cause significant ocular toxicity when they gain access to the inside of the eye. The aminoglycosides, in particular gentamicin, are the most toxic of the antibiotics commonly used in ophthalmology. Extreme caution should be used when administering a periocular injection of aminoglycoside for treatment or prophylaxis of infection. Intraocular injection of aminoglycoside for gram-negative coverage in endophthalmitis management has been replaced in most cases by ceftazidime. Ceftazidime provides excellent coverage against gram-negative bacteria with less potential for retinal toxicity at therapeutic dosages. Experimental and clinical studies have shown that intraocular vancomycin is safe and effective treatment against gram-positive organisms causing endophthalmitis. A combination of ceftazidime and vancomycin provides broad-spectrum coverage for virtually all bacteria causing endophthalmitis and is the current intraocular treatment of choice.

Treatment outcomes in a 10-year study of endogenous fungal endophthalmitis.

BACKGROUND AND OBJECTIVE: To review prevalence of organisms, associated factors, and treatment outcomes from one medical center's 10-year experience managing culture-proven endogenous fungal endophthalmitis (EFE). PATIENTS AND METHODS: The authors retrospectively reviewed the microbiology and corresponding clinical records of patients diagnosed as having culture-proven EFE at the Bascom Palmer Eye Institute during a 10-year period. RESULTS: Culture-proven EFE occurred in 20 eyes of 18 patients. Candida species occurred in 17 of 20 eyes (85%), and Aspergillus species occurred in 3 of 20 eyes (15%). The most common association was long-term intravenous line placement, which was present in 12 patients (67%). Whereas 12 patients (67%) had a history of recent hospitalization, only 2 (11%) had a documented history of systemic fungal infection. After initial examination, only 2 patients had a systemic culture positive for a fungal organism (none had a positive blood culture). Treatment after initial examination included pars plana vitrectomy in 17 of the 20 eyes (85%), intravitreal amphotericin B in 19 eyes (95%), and systemic antifungal medication in 16 eyes of 15 patients. Thirteen of the 17 eyes (76%) with Candida endophthalmitis and 0 of 3 eyes with Aspergillus endophthalmitis achieved visual acuity of 20/400 or better. CONCLUSION: The most common cause of culture-proven EFE at the authors' institution is Candida species. The overall visual outcomes were more favorable for Candida cases than they were for Aspergillus cases. In the treatment of patients with marked vitreous infiltrates from EFE, pars plana vitrectomy, intravitreal amphotericin B injection, and administration of appropriate systemic antifungal medication (fluconazole for Candida) are generally recommended.