Magnification requirement after photodynamic therapy with verteporfin for subfoveal predominantly classic choroidal neovascularization due to age-related macular degeneration.

PURPOSE: To prospectively assess the magnification requirement after repeat photodynamic therapy (PDT) with verteporfin in patients with predominantly classic subfoveal choroidal neovascularization (CNV) due to age-related macular degeneration (AMD). METHODS: A total of 103 patients were treated for the first time with PDT between November 1999 and September 2002. These patients were followed up at 3-month intervals for a minimum of 12 months. In addition to the usual investigations undertaken during PDT therapy, the magnification requirement was determined, under standardized conditions, using the SZB test developed by the Swiss Central Association for Blindness. RESULTS: A stable lesion with a stable magnification requirement was achieved in 86 (83.5%) patients; these patients were followed up for 24.8 months (range 12 to 36 months). At the time of the last follow-up examination, the magnification requirement compared with baseline was < 3 log units higher in 46 patients (53.5%) and > or = 3 log units higher in the remaining 40 patients (46.5%). Seventy-four (86%) of these 86 patients had a magnification requirement of < or = 8x. Stability was not achieved in 17 (16.5%) patients; up to the last examination these patients had been followed up for 12 to 30 months (mean 20.8). At the time of the most recent examination, 7 (41.2%) patients had a higher magnification requirement of < 3 log units while 10 (58.8%) had changed by > or = 3 log units. Sixteen patients (94%) had a magnification requirement of < or = 8x. CONCLUSIONS: PDT with verteporfin helps achieve stability without severe impairment in reading ability in most patients with predominantly classic subfoveal CNV due to AMD.

[Reading ability after photodynamic therapy (PDT) for age-related macular degeneration (AMD) and for high myopia]. / Lesefähigkeit nach photodynamischer Therapie (PDT) für altersabhängige Makuladegeneration (AMD) und für hohe Myopie.

BACKGROUND: A prospective uncontrolled follow-up of changes in reading ability after PDT with Verteporfin in patients with predominantly classic subfoveal choroidal neovascularization (CNV) due to AMD or high myopia was carried out. PATIENTS AND METHODS: A follow-up time of at least 6 months is documented for 48 patients with AMD and for 22 patients with high myopia. In addition to the usual clinical parameters the need for magnification was measured using standardized reading charts provided by the SZB.A need for magnification higher than 3.2-fold was defined as a severe loss in reading ability.PDT's were repeated every 3 months according to the usual guidelines. RESULTS: Before treatment, the mean reading acuity for patients with AMD was between 20/200 and 20/100. The average need for magnification was between 2.5-fold and 3.2-fold.Reading acuity, need for magnification and the percentage of patients with a need for magnification of 3.2-fold or less remained stable over a 6-12 months follow-up period. High myopic patients initially had a mean reading acuity of 20/200 and a need for magnification of 2.5-fold. After 9 months follow-up, reading acuity showed a mean improvement from 0.2 up to 0.3 ( p<0.05). There was also a trend towards improvement in the need for magnification and the percentage of patients with a need for magnification of 3.2-fold or less improved significantly from 68% to 78% ( p<0.05). CONCLUSION: Reading ability could be stabilized with PDT for predominantly classic subfoveal CNV in patients with high myopia or AMD for at least 9-12 months. The risk of developing a severe loss in reading ability (need for magnification >3.2-fold) was significantly reduced in high myopia and was stable over time in AMD.

[Radiotherapy for choroidal neovascularization (CNV) in age-related macular degeneration (AMD)]. / Radiotherapie von choroidalen Neovaskularisationen (CNV) bei altersabhängiger Makuladegeneration (AMD).

AIM OF THE STUDY: Several pilot studies have indicated, that radiation therapy might have a beneficial effect on the course of CNV in AMD. This controlled study was aimed at the question, whether such treatment might halt progression of neovascular AMD and whether a low or a high radiation dose should be applied. METHODS AND PATIENTS: Enclosed were patients aged > or = 60 and < or = 85 and eyes with a VA of > or = 0.1 and < or = 0.6, revealing a juxta/subfoveal CNV either of the occult or the classic type. Treatment was performed with a linear accelerator at fractions of 2 Gy up to a total dose of 10 Gy or 36 Gy. 95 eyes had completed follow-up of > or = 12 < or = 24 months. RESULTS: Among eyes with occult CNV 8 received 36 Gy, 16 were treated with 10 Gy and 21 were in the control group. Mean visual loss was 3.5 lines after 12 months and 5 lines after 24 months with no difference between irradiated eyes and those in the control group. In the groups with classic CNV 8 eyes were treated with 36 Gy, 27 eyes received 10 Gy and 15 eyes were in the control group. Mean visual loss after 6 months was 2.2 lines in eyes of both groups treated with radiation and 5.7 lines in the control group. This was statistically significant (p < 0.05). VA was < 0.1 after 12 (24) months of follow-up in 50 (75)% of the cases with 36 Gy, in 48 (83)% with 10 Gy and in 60 (83)% of the controls. These results were statistically significant after 12 months only. DISCUSSION AND CONCLUSIONS: The natural course of occult CNV could not be improved by irradiation with 10 or 36 Gy. In eyes with classic CNV a VA of > or = 0.1 was maintained significantly more often in irradiated eyes than in those of the control group. Treatment with 36 Gy however was associated with an unacceptable incidence of radiation retinopathy.

[Radiotherapy for choroidal neovascularization in age-related macular degeneration. A pilot study using low- versus high-dose photon bean radiation]. / Radiotherapie von subretinalen Neovaskularisationsmembranen bei altersabhängiger Makuladegeneration (AMD). Niedrig- versus hochdosierte Photonenbestrahlung.

PURPOSE: Several pilot studies have indicated that low-dose radiation therapy might have a beneficial effect on the course of choroidal neovascularization (CNV) in age-related macular degeneration (AMD). This study aimed to ascertain whether such treatment might halt the progression of neovascular AMD and whether a low or a high radiation dose should be applied. PATIENTS: The patients comprised some randomized to 0 vs 10 vs 36 Gy of radiation and (after a change of the study protocol became necessary) others who participated in a prospective, controlled non-randomized pilot study. Enclosed were eyes with visual acuity of > or = 0.1 and < or = 0.6 revealing a juxta-subfoveal CNV either of the occult type (type 1) or the classic type (isolated or as part of a predominantly occult lesion). RESULTS: Eyes treated with 10 Gy for occult CNV (n = 12) were subject to severe visual loss in 41.6% of the cases compared to 38.5% in the control group (n = 13) at 12 months of follow-up. For eyes treated with 10 Gy because of classic CNV, the corresponding figures were 33% (n = 18) and 57% (n = 14) respectively. At 18 months of follow-up, the percentages were 63% and 75% respectively. Fluorescein angiographic growth of classic and occult CNV could not be halted by 10 Gy, while a temporary growth retardation was observed in cases irradiated with 36 Gy. CONCLUSION: In the study presented, the natural course of occult CNV could not be improved by irradiation with 10 or 36 Gy. In cases of classic CNV, low-dose irradiation with 10 Gy postponed severe visual loss by a maximum of 18 months. A positive treatment effect was also observed in cases irradiated with 36 Gy; however, a 25% incidence of radiation retinopathy seems unacceptable.

Pneumocystis carinii infection of the conjunctiva in a patient with acquired immune deficiency syndrome.

BACKGROUND: Ocular disease, especially the development of choroidal lesions, is a known extrapulmonary manifestation of Pneumocystis carinii infection in the acquired immune deficiency syndrome (AIDS). To our knowledge, conjunctival involvement due to P. carinii has not been described previously. METHODS: The authors describe a 33-year-old homosexual male with AIDS in whom a large placoid, white lesion developed involving the tarsal conjunctiva of the right upper lid. Conjunctival malignancy was suspected and biopsies and swabs were taken. At this time, the patient had been receiving monthly aerosolized pentamidine prophylaxis for 18 months, and there was neither a history of pneumonia nor any clinical signs of disseminated infection due to P. carinii. RESULTS: Histopathologic examination results of the conjunctival biopsy specimen showed a necrotic, frothy tissue surrounded by activated fibroblasts. Within this material, Gomori methenamine silver stains showed numerous round and cup-like cysts of P. carinii, confirming the diagnosis that had already been obtained by an indirect fluorescent-antibody stain of a conjunctival smear specimen. CONCLUSIONS: The presence of white placoid conjunctival lesions in a patient with AIDS may indicate an infection due to P. carinii. Conjunctival disease due to P. carinii widens the spectrum of AIDS-associated ophthalmic pneumocystosis.

Wagner vitreoretinal degeneration. Follow-up of the original pedigree.

PURPOSE: Wagner disease belongs to a heterogeneous group of hereditary vitreoretinal degenerations. The authors have observed complications of this disorder that have not been reported before and therefore re-examined Wagner's original pedigree to further delineate the spectrum of the associated findings and its prognosis. METHODS: Sixty members of the family agreed to be examined. All had complete clinical eye examinations, 40 had dark adaptation studies as well as single-flash and Ganzfeld rod and cone electroretinography. Fluorescein angiograms were performed in selected patients. RESULTS: Twenty-eight family members were affected. The most consistent finding was an empty vitreous cavity with avascular strands or veils. Chorioretinal atrophy and cataract increased with the patients' age and had occurred in all patients older than 45 years of age. Four patients had a history of a rhegmatogenous retinal detachment in one eye at a median age of 20 years. The authors observed peripheral tractional retinal detachments in 55% of eyes among patients older than 45 years. Glaucoma was present in ten eyes (18%), four of which showed neovascular glaucoma. Of all patients, 63% showed elevated rod and cone thresholds on dark adaptation, and 87% showed subnormal b-wave amplitudes of the rod- and of the cone system on the electroretinography. CONCLUSIONS: Clinical expressivity of Wagner disease varies from unaffected carriers to bilateral blindness. Rhegmatogenous retinal detachment is observed infrequently, whereas peripheral traction retinal detachment, chorioretinal atrophy, and cataracts are present in most of the elderly affected individuals. Progression of the chorioretinal pathology is paralleled by electrophysiologic abnormalities.

Treatment with insulin-like growth factor I alters capillary permeability in skin and retina.

Treatment with insulin-like growth factor I (IGF-I) is accompanied by mild generalized and reversible edema. These changes may be due to increased capillary permeability. Therefore, we studied the effects of subcutaneous IGF-I treatment in healthy subjects on capillary permeability of the skin and the retina. Eight healthy subjects were treated with saline or recombinant human IGF-I (rhIGF-I) (10 micrograms.kg-1.h-1 s.c.) in a randomized crossover fashion. Transcapillary diffusion of sodium fluorescein (NaF) was quantitated by video densitometry in the skin in all subjects on the 4th treatment day. In seven subjects, plasma penetration of NaF at the blood-retinal barrier was investigated using vitreous fluorometry (VF) on days 3 and 5. Fluorescent light intensities of the skin and plasma penetration determined by VF were significantly higher during the IGF-I treatment as compared with those during the control situation. In conclusion, IGF-I treatment at the above dose is accompanied by increased transcapillary diffusion of NaF in skin and in retinal vessels.

Genetic linkage of Wagner disease and erosive vitreoretinopathy to chromosome 5q13-14.

BACKGROUND: Wagner disease and erosive vitreoretinopathy are potentially blinding autosomal dominant diseases that share some similarities with Stickler syndrome. However, both disorders have associated retinal pigment epithelial changes, poor night vision, visual field defects, and abnormal electroretinographic findings, which are not found in families with COL2A1-associated Stickler syndrome. In addition, rhegmatogenous retinal detachments are uncommon in Wagner disease but occur in approximately 50% of patients with either Stickler syndrome or erosive vitreoretinopathy. OBJECTIVES: To identify the chromosomal location of the genes involved in Wagner disease and erosive vitreoretinopathy and to distinguish these conditions genetically from Stickler syndrome. METHODS: Fifteen affected members of a family affected with erosive vitreoretinopathy and 24 affected descendants of the pedigree described by Wagner were genotyped with a set of short tandem repeat polymorphisms distributed across the genome. RESULTS: Significant linkage was observed in each family between the disease phenotype and markers that map to chromosome 5q13-14. The highest lod score for the family affected with erosive vitreoretinopathy was 4.2 and was obtained with marker GATA3H06 (theta = 0). The highest lod score for the family affected with Wagner disease was 5.8 and was obtained with marker D5S815 (theta = 0). A candidate gene (cartilage link protein) that is known to lie near the linked interval was screened for mutations, but none was found in either family. CONCLUSIONS: These data suggest that erosive vitreoretinopathy and Wagner disease are allelic disorders and demonstrate that they are genetically distinct from COL2A1-associated Stickler syndrome.

[Results of conventional detachment surgery. I. Study planning, patients and vision results]. / Ergebnisse nach konventioneller Ablatiochirurgie. I. Studienplanung, Patienten und Visusergebnisse.

A consecutive series of patients with retinal detachment were examined. All patients were treated primarily with buckling procedure. Patients with redetachment, bilateral detachment, vitrectomy or history of strabismus or amblyopia were excluded. Of these 155 patients, 117 could be examined. 97% of patients without macula detachment are able to keep or improve their visual acuity. 85% of those patients with macula detachment showed an improvement of visual acuity. The most important prognostic factor for the visual outcome is the duration of macula detachment. In cases of more than four days duration a visual acuity of 20/40 or more could not be expected.

[Results of conventional retinal detachment surgery. II. Changes in refraction]. / Ergebnisse nach konventioneller Ablatiochirurgie. II. Refraktionsänderungen.

In a comparative study we examined the refractive changes in 117 patients following conventional buckling retinal detachment surgery. Of these, 54 had a segmental scleral buckle, 18, an encircling buckle, and 45, a combination of both methods. Comprising about 2D, the changes in spherical equivalent were most marked in the patients who had combined operations. A correlation exists between buckle height and extent of refractive change (p = 0.0297).

[Results of conventional retinal detachment surgery. III. Orthoptic results]. / Ergebnisse nach konventioneller Ablatiochirurgie. III. Orthoptische Resultate.

In a comparative study we examined orthoptic side effects in 117 patients following conventional retinal detachment surgery. Of these, 54 had had a segmental scleral buckle, 18 a simple encircling band and 45 encircling band combined with a radial buckle. Persistent diplopia was found in 6% of cases following segmental buckles, in 11% following simple encircling procedures and in 20% following combined operations with encircling bands and segmental buckles. The incidence of diplopia was more than 30% in cases with high encircling buckles.

[Spontaneous regression of an acquired arterial macroaneurysm of the retina]. / Spontanregression bei erworbenem arteriellen Makroaneurysma der Retina.

We report on a case of partially occluded macroaneurysm with central circinate retinopathy which showed an impressive, nearly complete, spontaneous regression after one year. Complications of macroaneurysms and indications to therapy are discussed.

[Retinal and vitreal metastasis of a small cell bronchus cancer]. / Retinale und vitreale Metastase eines kleinzelligen Bronchuskarizinoms.

The rare form of retinal metastasis with infiltration of the vitreous is presented in terms of a 47-year-old female patient suffering from an oat cell bronchogenic carcinoma with remote metastasis. Clinical appearance, differential diagnosis, diagnostic evaluation, histology and evolution are discussed.

[Risk of metastasis in retinoblastoma]. / Das Metastasierungsrisiko beim Retinoblastom.

580 patients with retinoblastoma were analysed retrospectively. Median follow up was 5.5 years. 41 patients developed metastases within 5 years. Clinical and histopathological risk factors were analysed by univariate tests with regard to the occurrence of metastases. Significant variables were then reevaluated using Cox proportional hazards method. Four factors correlated independently with the development of metastases: optic nerve invasion with involvement of the resection line, optic nerve invasion without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The regression coefficient beta of the Cox-model was used for a score classification with groups of high, medium and low metastatic risk. The 5-year incidence of metastases was 68%, 43% and 4% respectively.

[Healed (?) amoebic keratitis]. / Geheilte (?) Amöbenkeratitis.

Acanthamoeba keratitis was seen in two contact-lens wearers. In both cases, this finding was verified by microbiological examination of conjunctival swabs and of the lens solution. One patient had perforating keratoplasty. While neither vital amoebae nor cysts could be detected in the host corneal explant, immunohistochemical examination revealed fluorescence-positive fragments which probably correspond to incomplete cycstic walls.

Risk factors for metastases in patients with retinoblastoma.

The study is based upon a review of data from 583 consecutive patients with retinoblastoma over the years 1956 to 1986. Mean follow-up was 8 years, and median was 5.5 years. In 41 patients, metastases developed within 5 years. The influence of clinical and histopathologic risk factors on the occurrence of metastases was first analyzed by univariate tests. Significant variables were then reevaluated using the Cox proportional hazards method. Four factors were found to be independently associated with the development of metastases: optic nerve invasion with and without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The 5-year metastatic risks associated with these factors were 67%, 13%, 8%, and 4%, respectively. The relative risk estimate, calculated from the Cox model, was used for a score classification with groups of low, medium, and high metastatic risk. The 5-year incidence of metastases was 4%, 43%, and 68%, respectively.

Long-term treatment effects in patients with bilateral retinoblastoma: ocular and mid-facial findings.

A total of 99 patients with bilateral retinoblastoma who had been treated between 1965 and 1982 were reexamined in April 1988 to study the late effects of treatment. Their median age at the follow-up visit in 1988 was 16 years (range, 6-27 years), and the median follow-up was 15 years (range, 6-26 years). All patients underwent a full eye examination, morphometric measurements of the mid-face and genetic counselling. Each eye or orbit and the corresponding side of the patient's mid-face were evaluated separately, resulting in 198 data sets from 99 individuals. Subjects were divided into four treatment groups according to whether photo- and cryo-coagulation, enucleation, radiation therapy or various combinations thereof were used. In all, 81 eyes had a visual acuity of greater than 0.4 (in 23 of these, however, only with low-vision aids). Within a dose range of 36-51 Gy, the location of the tumor (36%) or cataract (15%) were the main factors responsible for poor visual acuity, whereas radiation retinopathy and/or optic neuropathy occurred in only three cases. Cataracts were more frequently observed following orthovoltage as compared with megavoltage therapy (P = 0.012). A total of 72 eyes had been enucleated and had not received radiation therapy at any time. Cosmetic results (as measured by several parameters) in these cases were significantly better then those in 28 subjects who underwent combined radiation therapy and enucleation. As defined by various subjective as well as objective findings, mid-facial hypoplasia occurred significantly more often following orthovoltage as compared with megavoltage therapy.