Response to ranibizumab therapy in neovascular AMD - an evaluation of good and bad responders.

BACKGROUND: Treatment of neovascular age-related macular degeneration (AMD) with Lucentis shows a broad spectrum regarding the course of visual acuity (VA). While some patients show a good response (increase in VA), others disclose much less promising results. PATIENTS AND METHODS: A retrospective data analysis of all eyes treated for neovascular AMD at the University Hospital of Zurich, Switzerland for at least 12 months was carried out. The courses of VA between the 90th (good responders, GR) and the 10th (bad responders, BR) percentiles were compared at 3, 12 and 24 months from baseline. An analysis regarding demographic data, lesion type and size as well as injection frequency and visits was done and predictive factors for GR and BR were evaluated. RESULTS: Marked differences in the course of VA between GR (n = 30) and BR (n = 30) are already observed 3 months from baseline. In GR the gains in VA after 3, 12 and 24 were 15.7 +/- 9 letters ETDRS, 25.3 +/- 7 and 14.0 +/- 14. BR showed a deterioration of 8.3 +/- 11 letters ETDRS after 3, 22.1 +/- 8 after 12 and 23.6 +/- 13 after 24 months. The gender distribution was equal with a higher percentage of female patients (64 % in BR and 66 % in GR). The baseline VA was statistically significantly lower in GR (45.7 +/- 10 vs. 55.4 +/- 11, p < 0.05) than in BR. No other significant differences in baseline data were found, and no predictor for group membership could be identified. CONCLUSIONS: Only the course of VA in the first three months seems to be of value for an estimation of the response to treatment. In the future the response to treatment in the early phase may influence the treatment algorithm and the injection frequency.

[Photodynamic therapy of AMD for the first (better sight) and the second (worse sight) eyes]. / Photodynamische Therapie bei altersbedingter Makuladegeneration am schlechteren und besseren Auge.

BACKGROUND: Photodynamic therapy (PDT) is the standard treatment procedure for many forms of exudative and/or neovascular AMD. Despite therapy, visual acuity often drops to low vision levels. The cost efficiency of treating the eye in which vision is worse is therefore the subject of some controversy. PATIENTS AND METHODS: A retrospective case-control study was conducted in all patients who were treated with PDT at the Universitätsspital Zürich between September 1999 and November 2004. Each patient's first (with worse vision) and second (with better vision) eyes were compared for situation on presentation and course during treatment. RESULTS: In 117/228 cases (51.3%) visual acuity of the treated eye was better than (or identical to) that of the fellow eye at presentation. Visual acuity before therapy was an average of 0.58+/-0.27 logMAR [Snellen: 0.26 (0.14-0.49)] in the eyes with better visual acuity and 0.69+/-0.4 logMAR [Snellen 0.20 (0.08-0.51)] in the fellow eyes (p=0.015). After therapy there was no significant difference between the patient groups in visual acuity or in the magnitude of any change in visual acuity, or in lesion size or change in lesion size. CONCLUSION: The outcome of PDT of a second eye (with better visual acuity) is not significantly better than the result obtained in the first eye (the one with worse visual acuity initially).

A comparison of imaging techniques for diagnosing drusen of the optic nerve head.

OBJECTIVE: To determine the best imaging procedure for diagnosing drusen of the optic nerve head. METHODS: We reviewed retrospectively the clinical records of 142 patients (261 eyes) with suspected drusen of the optic disc. The patients were referred to our hospital over a 7-year period and evaluated by B-scan echography, orbital computed tomographic (CT) scan, and/or preinjection control photography for detection of autofluorescence. The relative diagnostic yield of these imaging techniques was compared. RESULTS: Thirty-six of the 261 eyes were evaluated using all 3 imaging techniques, with drusen of the optic nerve head diagnosed in 21 eyes. Findings from B-scan echography were positive in all 21 eyes compared with 9 positive findings from the CT scans and 10 positive findings from the preinjection control photographs (P<.01 for B-scan echography vs both CT scan and preinjection control photography). In 82 eyes with suspected buried drusen of the optic nerve head, B-scan echography showed drusen in 39 eyes compared with 15 eyes in which drusen were shown using preinjection control photography (P<.001). In the whole series, no diagnosis of drusen was made by either preinjection control photography or CT scan and was missed on B-scan echography. CONCLUSIONS: Drusen of the optic nerve head are diagnosed most reliably using B-scan echography compared with both preinjection control photography and CT scans. Preinjection control photography should be performed mainly when confirmation of visible drusen of the optic disc is desired. Neuroimaging using CT is suitable to exclude diagnosis of an intracranial mass lesion and possibly to detect buried drusen of the optic nerve head at the same time.

[Intravitreal anti-VEGF therapy for capillary hemangioblastomas in von Hippel-Lindau disease]. / Intravitreale anti-VEGF-Therapie von retinalen kapillären Hämangioblastomen bei von-Hippel-Lindau-Erkrankung.

BACKGROUND: Up-regulated expression of the vascular endothelial growth factor (VEGF) in von Hippel-Lindau (VHL) disease has been postulated to induce retinal hemangioblastoma. Intravitreal injections of anti-VEGF drugs might provide a new therapeutic option in this condition. METHODS: In a single case decision a patient with active retinal hemangioblastomas due to VHL disease received repeated intravitreal injections of 0.5 mg ranibizumab. RESULTS: Subsequent to intravitreal anti-VEGF therapy, the signs of activity of the retinal hemangioblastomas slowly regressed. CONCLUSIONS: Intravitreal anti-VEGF therapy might, as monotherapy or as combination therapy, offer a new treatment option for retinal hemangioblastoma.

Intravitreal triamcinolone acetonide for serous retinal pigment epithelial detachments in exudative age-related macular degeneration.

BACKGROUND: Due to the high risk of RPE tears PDT is usually not performed in eyes with serous RPE detachments (sRPED). For this reason this subform of exudative AMD was so far untreatable. PATIENTS AND METHODS: We report on a prospective uncontrolled observational case series. 20 eyes of 20 patients with subfoveal sRPED demonstrated by OCT were treated between June 2005 and April 2006 with intravitreal triamcinolone acetonide (IVTA). In 15 cases there was a primary sRPED, in 5 cases it had developed after one or more sessions of photodynamic therapy with Visudyne. RESULTS: There was a trend for better average visual acuity in the group with primary sRPED from 0.73 logMAR (0.19 Snellen equivalent) at baseline (n = 15) to 0.68 logMAR (0.21 Snellen) after one month (n = 15) (p = 0.19) and to 0.60 logMAR (0.25 Snellen) after three months (n = 14) (p = 0.41). The maximal height of sRPED decreased to an average of 35.3 % after one month (n = 15) and increased again to 56.9 % after 3 months (n = 14). One patient was lost to follow-up. In the group of eyes with sRPED after PDT, one eye developed an RPE tear with severe vision loss two weeks after IVTA. In the remaining four eyes average visual acuity improved from 0.90 logMAR (0.13 Snellen) at baseline to 0.73 logMAR (0.19 Snellen) after one month and to 0.80 logMAR (0.16 Snellen) after 3 months. Complete resolution of sRPED was observed in 8/20 eyes (4/5 eyes with sRPED after PDT and 4/15 eyes with primary sRPED). CONCLUSIONS: IVTA seems to be a therapeutic option in otherwise untreatable eyes with sRPED.

[Vasoproliferative retinal tumors]. / Vasoproliferative retinale Tumore.

BACKGROUND: Vasoproliferative Tumors of the retina (VPTR) are benign tumors of unknown origin, occurring mostly in otherwise systemically healthy patients. These highly vascularised tumors are characterised by a pink to yellow colour on funduscopy and are usually situated in the inferior part of the retina. They are associated with intraretinal hemorrhages, intra- or subretinal exudates and hyperpigmentation of the retinal pigment epithelium. MATERIALS AND METHODS: We performed a retrospective case review of seven cases which have been diagnosed with VPTR between 2004 and 2006 in the Department of Ophthalmology, University Hospital Zurich. RESULTS: The median follow up was 8 months (range: 1-24 months). We found a presumed underlying cause for the VPTR in 6 out of 7 patients. 3 patients showed a long standing retinal detachment; in the other 3 there was an untreated chronic uveitis. In one case the tumour was surgically excised and histology was performed. CONCLUSIONS: VPTR of the ocular fundus are a distinct entity in the differential diagnosis of intraocular tumors. These benign lesions represent reactive gliovascular proliferations, with varying degrees of both gliosis and of vascular proliferation. VPTR can be idiopathic, or they develop after inflammatory, vascular, traumatic, dystrophic or degenerative ocular diseases, in particular, uveitis. The major differential diagnosis includes other vascular lesions or tumors of the ocular fundus like capillary retinal hemangiomas, Coat' Disease or malignancies. The natural course of VPTR appears to be variable. The therapy is based on tumor size, localisation and complications.

First clinical experience with anecortave acetate (Retaane).

BACKGROUND: Anecortave acetate is an angiostatic cortisene which is injected as a posterior juxtascleral depot and has been shown to be effective in the treatment of exudative age-related macular degeneration (AMD). The compound is not yet approved in Switzerland but can be used as "compassionate use" in individual cases. PATIENTS AND METHODS: An uncontrolled case series with standardised documentation of ETDRS visual acuity, near acuity, need for magnification and fluorescein angiography was performed. RESULTS: 22 eyes of 19 patients (8 male, 11 female, average age 78.8 years) were treated with a posterior juxtascleral depot injection (PJD) of 15 mg anecortave acetate. The mean change in visual acuity after 3 months in eyes treated with anecortave acetate was -2.6 ETDRS letters corresponding to 0.52 Snellen lines. 3/20 eyes gained more than 1 line. 11/20 eyes showed stable visual acuity (+/- 1 Snellen line, +/- 5 ETDRS letters). 5/20 eyes developed moderate vision loss (one to two Snellen lines, 6-10 ETDRS letters). 1/20 lost 18 ETDRS letters (> 3 Snellen lines). There were no moderate or severe adverse events. CONCLUSIONS: A PJD of 15 mg anecortave acetate is safe and well tolerated. In eyes with occult CNV without recent progression or with residual neovascular activity after photodynamic therapy anecortave acetate may be an alternative therapeutic option before considering intravitreal anti-VEGF agents due to the much less invasive character and lower risk profile.

[Does cataract surgery increase the risk of exudative age-related macular degeneration? Results from a large retrospective case-control study]. / Besteht ein Zusammenhang zwischen einer Kataraktoperation und nachfolgender exsudativer altersbedingter Makuladegeneration? Eine grosse retrospektive Fallkontrollstudie.

BACKGROUND: Many epidemiological studies indicate a positive correlation between cataract surgery and the subsequent progression of age-related macular degeneration (AMD). Such a correlation would have far-reaching consequences. However, in epidemiological studies it is difficult to determine the significance of a single risk factor, such as cataract surgery. PATIENTS AND METHODS: We performed a retrospective case-control study of patients with new onset exudative age-related macular degeneration to determine if cataract surgery was a predisposing factor. A total of 1496 eyes were included in the study: 984 cases with new onset of exudative AMD and 512 control eyes with early signs of age-related maculopathy. Lens status (phakic or pseudophakic) was determined for each eye. RESULTS: There was no significant difference in lens status between study and control group (227/984 [23.1 %] vs. 112/512 [21.8 %] pseudophakic, p = 0.6487; OR = 1.071; 95 % CI = 0.8284-1.384). In cases with bilateral pseudophakia (n = 64) no statistically significant difference of the interval between cataract surgery in either eye and the onset of exudative AMD in the study eye was found (225.9 +/- 170.4 vs. 209.9 +/- 158.2 weeks, p = 0.27). CONCLUSIONS: Our results provide evidence that cataract surgery is not a major risk factor for the development of exudative AMD.

Macular atrophy after combined intravitreal triamcinolone acetonide (IVTA) and photodynamic therapy (PDT) for retinal angiomatous proliferation (RAP).

BACKGROUND: Retinal angiomatous proliferation (RAP) is a variant of exudative AMD that appears to respond poorly to standard PDT. This pilot study explores the potential efficacy and safety of combined intravitreal triamcinolone acetonide (IVTA). PATIENTS AND METHODS: In a prospective interventional case series 10 eyes of 8 patients with RAP were treated with combined IVTA/PDT. RESULTS: After 3 months there was no evidence of significant exudative activity in 7/10 eyes treated with IVTA/PDT, three eyes required re-treatment. After 6 months 4/10 eyes were "dry" on fluorescein angiography, visual acuity was stable (loss of less than 3 Snellen lines) or had improved in 5/10 eyes. 5/10 eyes had lost 3 or more lines, due to central macular atrophy in 4/5 eyes and secondary to a progressive exudative lesion in 1/5 eyes. CONCLUSIONS: Intravitreal steroids combined with PDT appear to be a step ahead in the treatment of RAP. Our results, however, raise the question whether combined treatment may create more atrophy leading to vision loss in some cases.

Diagnosis of MALT lymphoma by conjunctival biopsy: a case report.

BACKGROUND: Most extranodular lymphatic tissue is found in the intestinal mucosa. Together with similarly structured lymphatic tissue at other locations it has been named mucosa-associated lymphatic tissue (MALT). Malignant transformation of such tissue to lymphoma is well known. Although MALT lymphoma has been described in tissue physiologically void of MALT, lymphoma manifestation in the conjunctiva is rare. METHODS: We report a case of a 47-year-old woman who was referred to our clinic for symptomatic treatment and evaluation of severe symptoms of dry eyes. She was thought to suffer from Sjögren's syndrome because of xerophthalmia and xerostomia, as well as massive bilateral swelling of the parotid gland. Ophthalmological examination revealed marked hyperplasia of the conjunctiva, of which a biopsy was taken. RESULTS: Histological and immunohistochemical examination of the conjunctival biopsy, together with analysis of gene rearrangement by Southern blot, led to the diagnosis of low-grade B-cell lymphoma of the MALT. CONCLUSION: The differential diagnosis of keratoconjunctivitis sicca presenting with conjunctival swelling of unknown origin should include lymphoma, especially since Sjögren's syndrome may be associated with malignant disorders of the lymphatic system. A biopsy of suspicious conjunctival changes can clarify a multisystem disease by providing a tissue diagnosis.