Spironolactone in the treatment of central serous chorioretinopathy - a case series.

BACKGROUND: The pathogenesis of central serous chorioretinopathy (CSC) is still poorly understood. An animal model of CSC proved that the mineralocorticoid receptor [1] of the choroid also plays a role in CSC. Since there is still no evidence-based therapy for non-self-limiting CSC, this case series evaluates the effect of oral spironolactone in CSC patients. METHODS: In this interventional, uncontrolled, prospective case series, we present 18 consecutive CSC patients. Patients were treated with spironolactone 25 mg twice daily (Spironolacton AL® 50 mg, ALIUD PHARMA) for up to 12 weeks. Follow-up examinations with BCVA, OCT, and EDI-OCT were performed at 1, 2, and 3 months after starting the treatment. Main outcome measure was a change of subretinal fluid (SRF) (in micrometers) measured by optical coherence tomography. Secondary outcome was a change in central retinal thickness (CRT) (in micrometers) measured by OCT and a change in BCVA. RESULTS: The subretinal fluid (SRF; mean) decreased from 219 µm (baseline) to 100 µm (visit 3) (difference 119 µm). Total central retinal thickness (CRT; mean) decreased from 405 µm before treatment (baseline) to 287 µm after treatment (difference 118 µm). The BCVA (in logMAR; mean) increased from 0.32 at baseline to 0.20 at visit 3. CONCLUSION: Our case series could confirm a positive influence of spironolactone on the course CSC. Longer follow-up with a larger number of cases could provide more data about the long-term efficiency, recurrences, and safety of this well-tolerated and non-invasive treatment option of CSC.

[The role of light in the developement of RPE degeneration in AMD and potential cytoprotection of minocycline]. / Altersbedingte Makuladegeneration: die Rolle von Licht bei der Entstehung degenerativer Veränderungen im menschlichen RPE und möglicher Zell-Schutz durch Minocyclin.

BACKGROUND: Light-induced oxidative stress is an suggested reason for retinal pigment epithelium (RPE) degeneration in age-related macular degeneration (AMD). This study investigates the influence of light on intracellular reactive oxygen species (ROS) and apoptosis in the human RPE and potential cytoprotective effects of the tetracycline antibiotic minocycline. METHODS: Primary human RPE cells were either pre- or post-incubated with minocycline and then exposed to white light or oxidative stress (600 µM, H(2)O(2)). Then viability, induction of intracellular reactive oxygen species (ROS), apoptosis and cell death was determined. Expression of apoptotic BAX and anti-apoptotic Bcl-2 protein and their mRNA were determined by RT-PCR and Western blot analysis. RESULTS: Both light exposure and oxidative stress decreased RPE cell viability and Bcl-2 expression and increased intracellular ROS, apoptotic cell death, and BAX expression. Minocycline reduced these effects under certain conditions. CONCLUSIONS: This study demonstrates that minocycline effectively protects human RPE cells against oxidative damage. However, in the light of minocycline's photosensitising properties its potential role in AMD treatment needs further evaluation.

Sorafenib protects human optic nerve head astrocytes from light-induced overexpression of vascular endothelial growth factor, platelet-derived growth factor, and placenta growth factor.

OBJECTIVES: Growth factors, such as vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), and placenta growth factor (PlGF) are key players in the development of diabetic retinopathy, age-related macular degeneration, and other retinal neovascular diseases. Glial cells provide a significant source of retinal growth factor production under physiologic and pathologic conditions. Cumulative light exposure has been linked to increased retinal growth factor expression. Previous reports indicate that sorafenib, an oral multikinase inhibitor, might have a beneficial effect on retinal neovascularization. This study was designed to investigate the effects of sorafenib on light-induced overexpression of growth factors in human retinal glial cells. METHODS: Primary human optic nerve head astrocytes (ONHAs) were exposed to white light and incubated with sorafenib. Viability, expression, and secretion of VEGF-A, PDGF-BB, and PlGF and their mRNA were determined by reverse transcription-polymerase chain reaction, immunohistochemistry, and enzyme-linked immunosorbent assay. RESULTS: Light exposure decreased cell viability and increased VEGF-A, PDGF-BB, and PlGF expression and secretion. These light-induced effects were significantly reduced when cells were treated with sorafenib at a concentration of 1 microg/ml. CONCLUSION: Sorafenib significantly reduced light-induced overexpression of VEGF-A, PDGF-BB, and PlGF in primary human ONHAs. Sorafenib has promising properties as a potential supportive treatment for retinal neovascularization.

[Retinal pigment epithelial tears after intravitreal injection of bevacizumab for AMD. Frequency and progress]. / Retinale Pigmentepithelrisse nach intravitrealem Bevacizumab bei AMD. Häufigkeit und Verlauf.

PURPOSE: Intravitreal injection of the antibody bevacizumab is unofficially becoming more and more the "standard of care" in the treatment of neovascular AMD. After initial concerns about possible systemic adverse events of the drug, intravitreal injection has as yet shown a very good safety profile. Due to the common application of this VEGF inhibitor it is of great importance to report complications that may be related to the use of bevacizumab. In this scope we present a series of patients with predominantly serous detachment of the retinal pigment epithelium (PED), who developed a tear (rip) in the retinal pigment epithelium (RRPE) after intravitreal application of bevacizumab. METHODS: Our data are based on a prospective, consecutive, interventional case series of 420 patients with neovascular AMD. These patients received at least 1 intravitreal application of 1.25 mg bevacizumab within the period of 1 year. Follow-up examinations were every 4-6 weeks. Visits were documented with best corrected visual acuity according to the ETDRS standard, biomicroscopy of the retina, intraocular pressure measurement, evaluation of central retinal thickness, fluorescein angiography and fundus photography. RESULTS: Of 420 patients, 74 were classified as having predominantly serous PED. In the further course 13 out of 74 patients developed RRPE. Patients who had an intact subfoveal RPE, gained vision scores of 1.4+/-8.3 ETDRS letters (span width -15 to 14) despite RRPE or had stable Snellen vision of 0.0+/-0.1 logMar. In contrast patients with no subfoveal RPE due to RRPE showed loss of vision of -6.2+/-7.2 ETDRS letters (span width -15 to 1). CONCLUSION: This case series describes RRPE as a novel complication of intravitreal anti-VEGF therapy with bevacizumab. However, it seems that this complication is limited to the entity of predominantly serous PED. These patients should therefore be informed about the risk of RRPE before initiating anti-VEGF therapy with bevacizumab, although the reverse conclusion to generally exclude patients with PED from anti-VEGF therapy is not justifiable due to therapeutic efficiency and associated gain of vision.

Long-term results after drainage of premacular subhyaloid hemorrhage into the vitreous with a pulsed Nd:YAG laser.

OBJECTIVE: To investigate the effects of drainage of premacular subhyaloid hemorrhage into the vitreous with an Nd:YAG laser in a large series of patients with long-term follow-up. METHODS: A retrospective review was conducted on 21 eyes with a circumscribed premacular subhyaloid hemorrhage of various causes. These eyes were treated with a pulsed Nd:YAG laser to drain the entrapped blood into the vitreous. The period of review ranged from 12 to 32 months (mean, 22 months). RESULTS: In 16 eyes, visual acuity improved within 1 month. Four eyes had persistent, dense, nonclearing vitreous opacity for at least 3 months and finally required vitrectomy. One clotted hemorrhage did not drain into the vitreous. Final visual outcome was determined by the underlying diagnosis, such as Valsalva retinopathy (7 eyes), diabetic retinopathy (7 eyes), branch retinal vein occlusion (4 eyes), and retinal macroaneurysm, Terson syndrome, or blood dyscrasia (1 eye each). Eyes with Valsalva retinopathy fared the best. Complications included a macular hole in 1 eye and a retinal detachment from a retinal break in a myopic patient. CONCLUSIONS: Drainage of premacular subhyaloid hemorrhage into the vitreous with an Nd:YAG laser is a viable treatment alternative for eyes with recent bleeding. However, a macular hole and a retinal detachment were observed as complications. Thus, to establish Nd:YAG laser treatment as a routine procedure, the risks and benefits have to be weighed in a randomized trial and compared with those of deferral of treatment or primary vitrectomy.

Indocyanine green selectively stains the internal limiting membrane.

PURPOSE: To demonstrate whether indocyanine green stains the inner limiting membrane of the retina or residual vitreous cortex. METHODS: We report on the intraoperative staining patterns of the vitreomacular interface in 10 eyes of 10 consecutive patients who underwent vitrectomy with indocyanine green staining for macular hole formation and diffuse diabetic macular edema. RESULTS: In five eyes of five patients with macular holes, indocyanine green staining of the macula after posterior vitreous detachment resulted in an immediate visibility of a discernable membrane that was not previously seen. In five eyes of five patients with diffuse diabetic macular edema and adherent cortical vitreous, indocyanine green failed to stain the vitreomacular interface. After peeling off the residual vitreous cortex, however, a discernable membrane could be identified using indocyanine green dye again. Light and transmission electron microscopy revealed the inner limiting membrane as the membrane that had been stained and removed in all specimens. CONCLUSION: Indocyanine green selectively stains the inner limiting membrane. Staining of the vitreomacular interface using indocyanine green as a vital dye enables the surgeon to distinguish between the residual vitreous cortex and the inner limiting membrane, and it allows safer and easier removal of the inner limiting membrane.

Indocyanine green-assisted peeling of the internal limiting membrane may cause retinal damage.

PURPOSE: To demonstrate possible retinal damage caused by indocyanine green dye for staining of the internal limiting membrane in surgery for idiopathic macular hole. METHODS: Consecutive interventional case series. We report on the ultrastructural findings of the internal limiting membrane in 10 eyes of 10 patients. RESULTS: All specimens revealed not only the internal limiting membrane, but also some small amounts of retinal elements, such as the plasma membrane of Müller cells and other undetermined structures. This indicates a cleavage plane not exactly at the inner undulating aspect of the internal limiting membrane but within the innermost retinal layers. CONCLUSION: Dilutions of indocyanine green as recommended in the literature may alter the structure of the retina to some degree. Possible factors responsible for this inadvertent action may include (1) concentration, (2) osmolarity pH, (3) time of tissue contact, and (4) mechanical factors from more forceful traction during peeling. Although functional consequences of these findings remain unclear as yet, factors that may induce damage to the innermost retina should be elucidated.

Central retinal artery occlusion in association with an aneurysm of the internal carotid artery.

PURPOSE: To report an unusual case of central retinal artery occlusion in a 41-year-old woman. DESIGN: Interventional case report. METHODS: A 41-year-old woman presented with sudden and painless decrease of visual acuity of the left eye. Indirect ophthalmoscopy revealed the typical fundus findings of a central retinal artery occlusion. The fovea was spared by a cilioretinal arteriole. RESULTS: Cerebral angiography showed an aneurysm of the left internal carotid artery, measuring approximately 1.7 x 1.5 x 1.7 cm. The aneurysm was located in close proximity to the ophthalmic branch. CONCLUSION: Aneurysm of the intracranial arteries should be included in the differential diagnosis of central retinal artery occlusion in young patients.

Photocoagulation of choroidal neovascular membranes with a diode laser.

Nine eyes with parafoveal choroidal neovascular membranes due to age-related macular degeneration or angioid streaks were treated with a diode laser and were followed up to 40 weeks (mean 26 weeks). Angiographically proved closure of the membrane was achieved in seven eyes. Four lesions needed a second treatment for growth of subretinal neovascular tissue. Post-treatment visual acuity ranged from 6/9 to 6/60. Two eyes developed subfoveal membranes resulting in poor visual acuity. The morphology of the diode laser lesions differed from that of the argon green laser and was more similar to that of the krypton laser, producing a 'black hole' on the fluorescein positive print. In one particular eye fluorescein angiography revealed subfoveal choroidal non-perfusion next to the site of the diode lesion suggesting choroidal vascular closure.

Diode laser photocoagulation for diabetic macular oedema.

AIMS: This study aimed to investigate whether diode laser irradiation, which is poorly absorbed by haemoglobin, can induce closure of leaking retinal microvascular lesions in the treatment of diabetic macular oedema. METHODS: Thirty three eyes with clinically significant diabetic macular oedema were treated with a diode laser. Fundus evaluation before and after treatment included visual acuity, stereoscopic biomicroscopy, colour photographs, and fluorescein angiography. RESULTS: At a mean period of review of 6 months macular oedema had completely or partially resolved in 27 eyes. Visual acuity improved in three, deteriorated in one, and was unchanged in 29 eyes. CONCLUSION: Preliminary data suggest that diode laser therapy induces closure of leaking retinal microaneurysms and is effective in the treatment of diabetic macular oedema.

Central corneal thickness determined with optical coherence tomography in various types of glaucoma.

AIMS: To evaluate central corneal thickness determined by optical coherence tomography (OCT) in various types of glaucoma, and its influence on intraocular pressure (IOP) measurement. METHODS: Central corneal thickness (CCT) was determined by using OCT in 167 subjects (167 eyes). 20 had primary open angle glaucoma (POAG), 42 had low tension glaucoma (LTG), 22 had ocular hypertension (OHT), 10 had primary angle closure glaucoma (AC), 24 had pseudoexfoliation glaucoma (PEX), 13 had pigmentary glaucoma (PIG), and 36 were normal. RESULTS: CCT was significantly higher in ocular hypertensive subjects (593 (SD 35) microm, p <0.0001) than in the controls (530 (32) microm), whereas patients with LTG (482 (28) microm, p < 0. 0001), PEX (493 (33) microm, p <0.0001), and POAG (512 (30) microm, p <0.05) showed significantly lower readings. There was no statistically significant difference between the controls and patients with PIG (510 (39) microm) and AC (539 (37) microm). CONCLUSIONS: Because of thinner CCT in patients with LTG, PEX, and POAG this may result in underestimation of IOP, whereas thicker corneas may lead to an overestimation of IOP in subjects with OH. By determining CCT with OCT, a new and precise technique to measure CCT, this study emphasises the need for a combined measurement of IOP and CCT in order to obtain exact IOP readings.

Macular hole size as a prognostic factor in macular hole surgery.

BACKGROUND/AIM: In 1991 there was a series of successful closures of a macular hole after vitrectomy and membrane peeling. Today this technique has become a standard procedure. The aim of this study was to evaluate the role of optical coherence tomography in diagnosing and staging, as well as in predicting, the functional and anatomical outcome after macular hole surgery. METHOD: In a prospective study 94 consecutive patients (20 male, 74 female) with a mean age of 67.6 (SD 6.0) years and a macular hole stage II (n = 8), III (n = 72), and IV (n = 14) according to the classification by Gass were examined with optical coherence tomography (OCT) before pars plana vitrectomy. Macular hole diameters were determined at the level of the retinal pigment epithelium (base diameter) and at the minimal extent of the hole (minimum diameter). Calculated hole form factor (HFF) was correlated with the postoperative anatomical success rate and best corrected visual acuity. The duration of symptoms was correlated with base and minimum diameter of the macular hole. RESULTS: In eyes without anatomical closure of the macular hole after one surgical approach (13/94) the base diameter (p1) and the minimum diameter (p2) were significantly larger than in cases with immediate postsurgical closure (p1 = 0.003; p2 = 0.028). There was a significant negative correlation between both the base and the minimum diameter of the hole and the postoperative visual function (p1 = 0.016; p2 = 0.002). In all patients with HFF >0.9 the macular hole was closed following one surgical procedure, whereas in eyes with HFF <0.5 anatomical success rate was 67%. Better postoperative visual outcome correlated with higher HFF (p = 0.050). There was no significant correlation between the duration of symptoms and base or minimum diameters (p1 = 0.053; p2 = 0.164), respectively. CONCLUSION: Preoperative measurement of macular hole size with OCT can provide a prognostic factor for postoperative visual outcome and anatomical success rate of macular hole surgery. The duration of symptoms did not correlate with the diameters measured. Base and minimum diameters especially seem to be of predictive value in macular hole surgery.

Clinical comparison of semiconductor diode versus neodymium: YAG non-contact cyclo photocoagulation.

AIMS: The advent of diode lasers has allowed their use in transscleral cyclo photocoagulation for refractory glaucoma. A trial was performed to compare the ocular hypotensive and inflammatory effects of cyclo photocoagulation using a continuous wave diode (810 nm) and a free running neodymium:yttrium aluminium garnet (Nd:YAG) laser (1064 nm). METHODS: Forty patients with refractory glaucoma were randomised to receive either diode or Nd:YAG therapy. The intraocular pressure (IOP) and inflammatory response to treatment were monitored over 3 months. RESULTS: There was no significant laser related difference in the effect on IOP after one treatment. There was, however, a difference in effect in retreatments with the IOP lowering effect significantly less, but equally sustained in diode retreatment patients. Severe postoperative complications such as hyphaema or fibrinous anterior uveitis only occurred in the Nd:YAG group. CONCLUSION: The degree and duration of the ocular hypotensive response to cyclo photocoagulation appears to be related to the available power output of the system used, and the extent of tissue damage.

Central corneal thickness measurement with a retinal optical coherence tomography device versus standard ultrasonic pachymetry.

PURPOSE: To demonstrate the capability of a standard, commercially available optical coherence tomography device (originally designed to measure retinal thickness) to image the human cornea in vivo and to measure central corneal thickness (CCT) in normal and edematous corneas. The intrapatient precision and interpatient variability of this novel application was compared to standard ultrasonic pachymetry. Also, the correlation of both methods was investigated. METHODS: CCT measurements using optical coherence tomography (OCT) and ultrasonic pachymetry (PACH) were obtained in 36 normal eyes and 16 eyes with corneal edema. RESULTS: Direct in vivo imaging of the cornea and measurements of CCT by OCT could be performed in all eyes. In normal subjects, CCT(OCT) was 530+/-32 microm and CCT(PACH) was 581+/-34 microm. The two methods showed a highly significant correlation with a standardized regression coefficient of 0.988. The difference between both methods was constant over the range of CCT (mean difference, 49.4+/-5.9 microm). The mean intrapatient SD of CCT measurements was 4.90 microm and 4.96 microm for OCT and PACH, respectively. In patients with corneal edema, mean CCT(OCT) was 601+/-59 microm, and mean CCT(PACH) was 667+/-68 microm. The difference between the two techniques increased slightly with increasing corneal edema (mean difference, 66.9+/-10.9 microm). CONCLUSION: Imaging of the human cornea can be performed by a standard retinal OCT device, and OCT measurement of CCT shows excellent correlation to values obtained by PACH, giving similar readings separated by a constant difference. In corneal edema, the difference between the two methods is additionally increased, but continues to demonstrate excellent consistency.

[Comparative use of diode and argon laser for panretinal photocoagulation in diabetic retinopathy]. / Vergleichende Anwendung von Dioden- und Argonlaser zur panretinalen Photokoagulation bei diabetischer Retinopathie.

The recently introduced semiconductor diode laser is portable, more compact, and cheaper to maintain than other laser systems. It is unclear whether its wavelength characteristics in the near infrared (810 nm) lead to different clinical results of retinal photocoagulation. In a prospective study ten diabetics (3 type I and 7 type II) ranging in age from 26 to 72 years with bilateral proliferative or severe nonproliferative diabetic retinopathy and visual acuity better than 6/18 in both eyes underwent panretinal photocoagulation. One eye was treated with the diode laser, the fellow eye with argon green (514 nm). Follow-up was documented by best-corrected visual acuity, fundus photography and fluorescein angiography. Mean duration of follow-up was 12 months. In neither group was there a significant difference in the response of retinopathy and neovascularization to the treatment, or in the course of visual acuity. Fluorescein angiography revealed the more profound effects of the diode laser in the choroid. Compared to argon laser treatment, patients found diode laser treatment more painful, but appreciated the absence of bright flashes during therapy. Photocoagulation for diabetic retinopathy using the diode laser was as effective as using the argon system in this initial pilot study.

[Iris neovascularization, increased intraocular pressure and vitreous hemorrhage as risk factors for invasion of the optic nerve and choroid in children with retinoblastoma]. / Irisneovaskularisationen, erhöhter Augeninnendruck und Glaskörperblutungen als Risikofaktoren für den Befall des Sehnerven und der Aderhaut bei Kindern mit Retinoblastom.

A review of 289 eyes enculeated for retinoblastoma to evaluate prognostic factors for involvement of the choroid or optic nerve revealed choroidal invasion in 67 eyes (22%) and optic nerve invasion in 84 eyes (27%) due to retinoblastoma. Intraocular pressure (IOP) > or = 22 mmHg was found in 85 eyes prior to enucleation. A total of 118 eyes (38% showed histopathologic evidence of the development of glaucoma: 90 of them with iris neovascularisation and 28 with angle closure configuration. Patients with elevated IOP, iris neovascularisation and angle closure configuration had a significantly higher risk of optic nerve (P < 0.005) and choroidal (P < 0.002) involvement. On the other hand, optic nerve invasion due to retinoblastoma was significantly more frequent in eyes with elevated IOP (P < 0.02) and iris neovascularisation or angle closure configuration (P < 0.01). Risk factors for choroidal invasion of retinoblastoma were raised IOP (P < 0.04) and iris neovascularisations or angle closure configuration (P < 0.01) (univariant analysis). Raised IOP, iris neovascularisations and vitreous haemorrhage remained significant in the multivariant analysis. This study emphases the importance of IOP, iris neovascularisation and angle closure configuration as risk factors for outspreading invasion due to retinoblastoma.

[Multifactorial analysis of therapeutic outcome of pseudophakic retinal detachment surgery]. / Multifactoririelle Analyse des Therapieerfolges des Pseudoaphakieablatio.

BACKGROUND: Pseudophakic retinal detachment is one of the most severe complications after cataract surgery and is a common cause of permanently reduced visual acuity. We evaluated parameters predicting reduced functional outcome by a model of stepwise regression analysis. PATIENTS AND METHODS: A series of 102 consecutive patients with pseudophakic retinal detachment were analyzed for various parameters regarding cataract surgery, retinal surgery, and retinal detachment features. First, univariate analysis determined the correlations with reduced functional outcome. Secondly, a stepwise regression model analyzed statistically significant variables for their predictive value of a reduced visual outcome. RESULTS: The overall reattachment rate was 99%. In 69% of the patients there was an improvement of more than two lines at the end of the follow-up period. The most predictive factors for reduced functional outcome were the need for a silicone oil tamponade and the visual acuity prior to retinal detachment surgery. When silicone oil tamponade was not needed, the requirement of more than two retinal surgeries was the most predictive factor for reduced visual outcome. CONCLUSION: In our series the strongest predictive factors for a reduced functional outcome were the necessity of silicone oil, reduced visual acuity at the time of retinal detachment, and the requirement of more than two retinal surgeries. These findings suggest that first-line procedures should not be necessarily minimally invasive measurements but rather procedures that result in a stably attached retina in the first instance without permanent silicone oil tamponade, even if this first operation consists of an extended pars plana vitrectomy.

[Imaging of vitreoretinal adhesions in the partner eye of patients with penetrating macular foramina in optical coherence tomography]. / Darstellung vitreoretinaler Adhärenzen in Partneraugen von Patienten mit durchgreifenden Makulaforamina in der optischen Kohärenztomographie.

BACKGROUND: Vitreoretinal adhesions play a crucial role in the development of a macular hole. To visualize vitreoretinal adhesion we used optical coherence tomography to investigate fellow eyes of patients with macular holes. METHODS: In a prospective study we scanned the retina in 188 patients with a macular hole stage III or IV (Gass classification). The foveal shape and vitreous were classified into grades. RESULTS: Of the 188 patients 45% showed no vitreous reflex, 45% a partial vitreous detachment with foveolar adhesions, and 10% a vitreous detachment with complete separation from the fovea. While eyes with normal foveolar shape displayed partial vitreous detachment in 33%, this figure rose to 66% in eyes with a macular hole stage I. CONCLUSION: Diffuse thickening of the fovea is followed by an intraretinal split and formation of a cyst. The shape of the foveolar adhesion suggests that continuing anteroposterior vitreal traction leads to a retinal break and formation of a full-thickness macular hole.

[North Carolina macular dystrophy. Hereditary macular disease with good functional prognosis]. / North-Carolina-Makuladystrophie. Hereditäre Makulaerkrankung mit guter funktioneller Prognose.

BACKGROUND: North Carolina macular dystrophy (NCMD) is a rare autosomal dominant maculopathy with highly variable expressivity. Genetic analysis of an American family consisting of 247 members out of which 96 were affected with NCMD allowed chromosomal assignment of the NCMD locus to 6q14-q16.2. Few families with NCMD are known in Europe, one of these is living in Germany. By routine investigation, a second family affected with NCMD was detected in Germany. As some authors still doubt the good prognosis of this disease, our results should be added to the experience of others. PATIENTS AND METHODS: In a total of 18 family members from three generations between the age of 2 and 65 years, clinical investigations and genetic analysis was carried out. Some individuals had additional examinations such as colour contrast sensitivity, EOG, ERG, and microperimetry. RESULTS: Ten of 18 family members turned out to be affected. All grades of NCMD were present with great variability. Visual acuity ranged from 0.32 to 1.0 and did not correlate to the grade of the disease or to the age of the person. In those patients who underwent microperimetry, central fixation was confirmed. Genetic linkage analysis further narrowed the region harbouring the NCMD locus and supported the assumption that the central areolar pigment epithelial dystrophy (CAPED) is an allelic disorder. CONCLUSION: Similar visual acuity in three generations of NCMD patients supports the observation that NCMD is not a progressive disorder. If geographic atrophy is found in a patient with good visual acuity, NCMD should be considered and genetic analysis should be carried out.

[Wide-field fundus autofluorescence in non-infectious posterior uveitis]. / Weitwinkelfundusautofluoreszenz bei nichtinfektiöser Uveitis posterior.

BACKGROUND: Posterior uveitis comprises a heterogeneous group of diseases with inflammatory alterations of the posterior fundus and is a common cause of visual impairment and blindness. The goal of this study was to evaluate the diagnostic value of wide-field fundus autofluorescence (FAF) in patients with non-infectious posterior uveitis and chorioretinal alterations. MATERIAL AND METHODS: In this study 73 eyes from 51 patients were included. Best-corrected visual acuity, wide-field color and FAF images achieved by a wide-field scanning laser opththalmoscope (SLO, Optomap P200Tx, Optos PLC, Dunfermline UK) and a full ophthalmological examination were obtained from each patient. A systematic analysis of chorioretinal alterations detected with FAF and color images was conducted followed by the evaluation of the diagnostic information of wide-field FAF compared to the clinical finding and wide-field color images. RESULTS: Of the 73 eyes included in the study 52 showed peripheral alterations. In 32 cases wide-field FAF images revealed a greater number and more extensive chorioretinal alterations than the corresponding wide-field color images of the posterior fundus. CONCLUSIONS: In this study wide-field FAF images showed more chorioretinal alterations than seen in funduscopy or in color SLO images. Therefore, wide-field FAF images offer important additional information for detection and documentation of peripheral and central chorioretinal alterations.