Wadim Bowl

Wadim Bowl; Knut Stieger; Magdalena Bokun; Silke Schweinfurth; Kerstin Holve; Monika Andrassi-Darida; Birgit Lorenz

PURPOSE To compare retinal layer thicknesses in preterm and term-born children using spectral-domain optical coherence tomography (SD-OCT) and to correlate structure with retinal function. METHODS We performed SD-OCT single and volume scans in the foveal region of premature children aged 6 to 13 years without ROP (no-ROP, n = 100) and with spontaneously regressed ROP (sr-ROP, n = 50) documented with wide-angle digital imaging during routine screening for acute ROP, and 30 age-matched term-born children. Retinal layer segmentation and analysis was performed with custom-made software in single and volume-scans using an Early Treatment of Diabetic Retinopathy Study grid-based method, and compared to light increment sensitivity (LIS) data obtained with a microperimeter at eccentricity points of 0°, 2.8°, and 8°, as previously described. RESULTS Overall, seven children had to be excluded due to poor image quality (n = 1 no-ROP; n = 2 sr-ROP; n = 4 term). Total retina, ganglion cell + inner plexiform layer (GCL+) and outer nuclear layer + external limiting membrane (ONL+) thickness at the foveal center in no-ROP and sr-ROP were significantly higher compared with term children. Gestational age (GA) and birth weight (BW) were inversely correlated with these layer thicknesses. Rod and cone outer segment length did not differ in either group. The ratio of ONL+ to the whole retina at 0° correlated significantly with reduced LIS. CONCLUSIONS Increased thicknesses of the entire retina or specific layers at the fovea did not correlate with functional loss; but a thinner ONL in retinae without foveal pit did. This reduced ONL+ ratio is potentially caused by a reduced foveal cone density and may be the first morphologic functional correlate in prematurity and ROP.

Markus N. Preising; C. Pasquay; Christoph Friedburg; Wadim Bowl; M. Jäger; Monika Andrassi-Darida; Birgit Lorenz

BACKGROUND Autosomal recessive bestrophinopathy (ARB) is associated with mutations in BEST1. ARB is rarely diagnosed compared to BEST1-associated autosomal dominant (a. d.) juvenile vitelliform macular degeneration (Morbus Best, VMD). This is not only due to its low prevalence, but also to the phenotypic appearance. This paper describes typical features in two patients and discusses novel findings using improved ophthalmological diagnostic tools. MATERIAL AND METHODS Two unrelated boys with reduced visual acuity as well as five further relatives underwent a comprehensive ophthalmological examination including electroretinography (ERG) and electrooculography (EOG) according to ISCEV standard, fundus autofluorescence (FAF) and spectral-domain optic coherence tomography (SD‑OCT). BEST1 was screened for mutations based on the clinical diagnosis. RESULTS Visual acuity ranged between 0.2 and 0.5 in the patients. Multifocal yellowish paramacular and peripheral lesions were visible in the fundus correlating with spots of increased FAF. The lesions correlated with thickening of the RPE layer. Especially in the inner nuclear layer hyporeflective areas were visible, reminiscent of retinoschisis but without changes of FAF. In both patients the ganzfeld ERG was within the normal range and the mfERG presented obvious reductions of amplitudes in the central area. The EOG did not show a light peak. Goldmann perimetry was normal for isopters III/4e and I/4e. The fundus controlled perimetry revealed a central sensitivity loss. Molecular genetic analysis identified four (two novel) mutations in BEST1, in the compound heterozygous state in both patients. The screened relatives carried one of the mutations in the heterozygous state and were ophthalmologically unremarkable apart from age-related changes. CONCLUSION ARB is a rare disease, presenting with obvious differences to a.d. Mobus Best. The phenotype can easily be identified by the extramacular multifocal yellowish lesions with increased FAF and accompanied by early loss of visual acuity. Specific diagnostic tests like OCT, FAF recordings and electrophysiology support the diagnosis. Molecular genetic screening confirms the diagnosis and the autosomal recessive inheritance.

Georg Maier; Peter Barth; Georg Pisinger; Wadim Bowl; Monika Andrassi-Darida; Birgit Lorenz; Justus-Liebig-University Giessen

Retinopathy of prematurity (ROP) is an eye disease that affects prematurely-born babies. It may even lead to blindness in serious cases. Among others, ROP can be recognized by evaluating the tortuosity of retinal vessels. An automatic detection and evaluation of tortuous vessels can support ophthalmologists when judging retinal images. There already exist various methods for computing vessel tortuosity. However, they do not always cover the medical properties and requirements. Thus, we propose an efficient measure, which is based to the total curvature and the number of curvature sign changes (twists) of the vessel. In order to achieve a stable detection of twists we represent the extracted vessels by curves composed of circular arcs and line segments: The vessels center pixels are approximated by smoothly joined segments of constant curvature. For any maximum tolerance we obtain the minimum number of segments. This way vessels are represented by preferably long curve segments of constant curvature. Together with the proposed measure this representation enables an effective and stable method for evaluating vessel tortuosity. Its performance is examined by means of both synthetic data and vessels of real retinal images.

Wadim Bowl; Robert Knobloch; Silke Schweinfurth; Kerstin Holve; Knut Stieger; Birgit Lorenz

Purpose: To correlate visual field assessment with saccadic vector optokinetic perimetry (SVOP) in children with ganglion cell loss due to anterior pathway pathologies resulting in hemianopic visual field defects measured with optical coherence tomography (OCT). Methods: 5 young (aged 3-6 years) and 5 adult patients with hemianopia, 10 healthy preschoolers (mean age 4.4 years), and 10 healthy adults (mean age 25.3 years) were tested with SVOP and OCT (focusing on the ganglion cell layer, GCL+). In adults, visual field testing was also performed with static and fundus-controlled perimetry. Results: OCT allowed precise structure analysis and showed a vertical border with GCL+ loss on the hemianopic side in children and adults compared to controls. SVOP showed visual field defects on the hemianopic side in peripheral regions and inadequate results at the parafoveal positions in both groups. In contrast, static and fundus-controlled perimetry showed a clear border in foveal and parafoveal regions. Conclusions: All children underwent SVOP with minimal restrictions, allowing functional evaluation of peripheral visual field positions. Parafoveal positions showed multiple false-positive results. The function-structure relationship is measurable even in young children by using the GCL+ analysis. This combination of novel child-friendly techniques allows collecting objectively measured values and simplifies diagnosis and follow-up in treatment.

Wadim Bowl; Marianne Bowl; Silke Schweinfurth; Kerstin Holve; Monika Andrassi-Darida; Knut Stieger; Birgit Lorenz

Aim: Comparison of choroidal thickness (CT) and foveal morphology as seen with swept-source optical coherence tomography (SS-OCT) in children with a history of treated or spontaneously regressed retinopathy of prematurity (tROP or srROP) to assess the impact on best-corrected visual acuity (BCVA). Methods: CT was measured by SS-OCT (DRI-OCT Triton; Topcon, USA) single scans of a 6-mm diameter around the fovea in 17 children with tROP or srROP (4–7 years of age) and compared to 25 controls (age-matched children and adults). The disproportion of the outer nuclear layer and inner retinal layers at the fovea (i.e., the ONL+/IRL ratio) as a measure of macular developmental arrest (MDA) was manually analyzed. BCVA was tested with ETDRS letter charts and correlated with the morphology. Results: CT was significantly thinner in children with tROP and srROP compared to term-born healthy children (nKids) at all measurement marks (p < 0.001), and mostly affected in the subfoveal area. tROP showed the lowest CT. CT allowed no direct conclusion about ONL+/IRL, but correlated positively with BCVA. Conclusions: Reduced CT in children with a history of ROP is linked to ROP severity. These findings overlap with the degree of MDA. CT appears to be involved in ROP, but MDA showed a higher impact on the BCVA of the examined cohort.

Wadim Bowl; Birgit Lorenz; Knut Stieger; Silke Schweinfurth; Kerstin Holve; Christoph Friedburg; Monika Andrassi-Darida

Aim To correlate light increment sensitivity (LIS) and visual acuity (VA) with birth weight (BW), gestational age (GA) and stage of acute retinopathy of prematurity (ROP) (STG) in premature children at school age. Methods 180 children (150 former prematures and 30 age-matched term-born children) were enrolled at age 6–13 years. Former prematures were categorised by the results of the initial ROP screening based on digital wide-field fundus imaging: absence of ROP (n=100) and spontaneously resolved ROP (n=50). The latter group was further subdivided according to their STG (Stg 1; Stg 2; Stg 3). Both groups were categorised into sectors by BW (<1000 g; 1000–1500 g; >1500 g), and GA (≤28 weeks; >28<32 weeks; ≥32 weeks). VA was assessed with Early Treatment of Diabetic Retinopathy Study letters, LIS was measured at 0°, 2.8° and 8° in the visual field (Microperimeter MP1, Nidek Technologies), and spherical equivalent refraction assessed with a Nidek autorefractor (Nidek, Italy). Results Central and pericentral LIS (0° and 2.8°) and VA were significantly lower in all groups and sectors compared with term-born controls except for BW >1500 g for LIS and GA >28 to <32 W for VA. No significant differences were found for LIS at 8° in all groups. No correlation was found between LIS and VA on an individual basis. Conclusions Low BW, GA and increasing severity of spontaneously resolving ROP were associated with significantly decreased central visual function. In addition to VA, LIS measurement further describes foveal function and is a unique parameter to assess parafoveal function.

Alexander Ehnes; Yaroslava Wenner; Christoph Friedburg; Markus N. Preising; Wadim Bowl; Walter Sekundo; Erdmuthe Meyer zu Bexten; Knut Stieger; Birgit Lorenz

Wadim Bowl; Monika Andrassi-Darida; Kerstin Holve; Silke Schweinfurth; Robert Knobloch; Birgit Lorenz

Wadim Bowl; Knut Stieger; Birgit Lorenz

Wadim Bowl; Marianne Bowl; Silke Schweinfurth; Kerstin Holve; Robert Knobloch; Knut Stieger; Monika Andrassi-Darida; Birgit Lorenz