The impact of perinatal brain injury on retinal nerve fiber layer thickness and optic nerve head parameters of premature children.

PURPOSE: This study aims to evaluate the impact of birth weight (BW), gestational age (GA), retinopathy of prematurity (ROP), and perinatal brain injury (PBI) on optic nerve head (ONH) parameters and nerve fiber layer thickness (RNFLT) in preterm children. METHODS: ONH parameters and RNFLT were examined prospectively in 5-15-year-old preterm and full-term children with RTVue-100 OCT (Optovue, USA). The parameters of the two groups were compared and possible influences of BW, GA, ROP, and PBI analyzed in preterm children. RESULTS: In total, 51 full-term and 55 preterm children were included. The mean age was 9.98 ± 3.4 years in full-term and 10.0 ± 2.5 years in preterm children. The mean GA in preterm children was 29.6 ± 3.8 weeks with a BW of 1523 ± 732 g. RNFLT was significantly lower in preterm than in full-term children in all but temporal quadrants. Cup area, volume, cup/disc area ratio, and horizontal cup/disc ratio (CDR) were significantly larger and rim area significantly thinner in preterm children. GA was positively correlated with superior, nasal, and overall RNFLT and negatively correlated with cup area, volume, and horizontal CDR. ROP stage correlated negatively with superior and nasal RNFLT. PBI was the only significant predicting factor for RNFL thinning in all but temporal quadrant in multiple regression analysis. Preterm children with PBI had a significantly larger optic cup (CDR 0.70 ± 0.33 vs. 0.37 ± 0.27) and thinner optic rim. CONCLUSION: PBI correlated strongest with RNFL thinning, a thinner optic rim, and a larger optic cup in preterm children and should be evaluated in each patient to prevent incorrect diagnosis like glaucoma.

On the Current Care Situation and Treatment of Ocular Mucous Membrane Pemphigoid in Germany. / Zur Versorgungssituation und konservativen Therapie des okulären vernarbenden Schleimhautpemphigoids in Deutschland.

BACKGROUND: Ocular involvement in mucous membrane pemphigoid (MMP) is relatively rare, with a prevalence of 25 cases per million population, equating to approx. 2,100 patients throughout Germany. Diagnosis can be difficult - especially in cases of isolated ocular involvement - and treatment can be complex and lengthy. Immunosuppressants or immunomodulatory drugs are often used. Due to the complexity of diagnosis and treatment, MMP patients are usually referred to specialized centers. The aim of this project was to evaluate the current care situation of patients with ocular MMP in Germany. METHODS: A paper-based survey was designed and sent to all university eye clinics and other specialized centers in Germany in April 2020. The survey asked about the existence of a specialized outpatient service, the total annual number of patients with MMP, the annual number of newly diagnosed patients, any interdisciplinary collaboration for diagnostic or therapeutic purposes, as well as the local and systemic therapy used. RESULTS: Of a total of 44 clinics, 28 (64%) responded, reporting a total average of 27 ± 42 (0 - 200) patients and 3.6 ± 2.2 (0 - 10) new cases per year. This corresponds to a total of 741 patients. Only nine (32%) of the responding clinics offer specialized MMP clinics. 93% of the centers collaborate with the local dermatology department. 79% perform serological and histological diagnostics in-house. About half of the centers (n = 16) apply a standardized treatment regime. Systemic glucocorticoids (66.7%) are most commonly used, followed by mycophenolate mofetil and dapsone (57.1%), rituximab (33.3%), azathioprine and cyclophosphamide (28.6%), as well as methotrexate (19.0%). The least frequently used treatment is intravenous immunoglobulin (14.3%). CONCLUSION: This survey of German ophthalmology departments obtained data from about one third of the estimated total cohort of all patients with MMP in Germany. These are presumed to be exclusively patients with at least one ocular involvement. The complex care of these patients is usually provided in collaboration with a dermatologist and with the use of systemic anti-inflammatory medication. Currently, an ophthalmological MMP register is being established to better record the epidemiology and care situation of this rare disease in Germany and to improve it in the long term.

Successful Treatment of a Pediatric Neurotrophic Keratopathy With Cenegermin.

PURPOSE: We present a challenging case of a 9-year-old patient with refractory neurotrophic keratopathy (NK) who was successfully treated with cenegermin eye drops. METHODS: A 7-year-old boy developed an ocular infection after a visit to a public swimming pool. After having been unsuccessfully treated for 2 years with antibiotics, steroids, and artificial tears, the patient presented to our clinic with a therapy-refractory NK. We initiated treatment with autologous serum eye drops that showed only weak response. Therefore, treatment with cenegermin eye drops was started. RESULTS: After cenegermin therapy, a complete restoration of the corneal surface and an increase in visual acuity were achieved. CONCLUSIONS: The use of cenegermin is effective in treating pediatric NK.

Revisiting the Safety of the Corneal Collagen Crosslinking Procedure: Evaluation of the Effect of Ultraviolet A Radiation on Retinal Function and Structure.

PURPOSE: To evaluate the effect of ultraviolet A radiation on retinal function and structure after the corneal collagen crosslinking procedure. METHODS: In the present prospective, single-center cohort study 17 eyes of 17 patients who underwent collagen crosslinking (Dresden protocol) for the treatment of corneal ectasia were examined preoperatively and at 2 and 6 weeks postoperatively with multifocal electroretinogram and optical coherence tomography. The P1 amplitude density (P1-AD) of the central retinal response was documented in 5 concentric rings, with ring 1 (R1) representing the foveal response and rings 2 to 5 (R2-R5) corresponding to successive annuli of stimulation. Visual acuity, corneal densitometry at the 0 to 2 mm zone and at the 2 to 6 mm annulus, and macular thickness (MT; central, 1 mm, 3 mm, and 5 mm zones) were recorded. RESULTS: The preoperative values of P1-AD were R1 = 109.96 ± 28.96, R2 = 49.8 ± 14.46, R3 = 29.85 ± 8.9, R4 = 19.33 ± 6.3, and R5 = 16.39 ± 5.48 nV/deg. At 2 weeks, these values were R1 = 77.54 ± 24.47, R2 = 36.55 ± 12.53, R3 = 21.53 ± 7.71, R4 = 15.3 ± 6.13, and R5 = 13.32 ± 5.48, showing a significant reduction for R1-R4 (P ≤ 0.026). The 6-week P1-AD was R1 = 99.8 ± 31.23, R2 = 40.67 ± 16.39, R3 = 24.98 ± 7.13, R4 = 16.35 ± 4.84, and R5 = 13.76 ± 3.98, showing no significant differences compared with preoperative values (P ≥ 0.054). Corneal densitometry increased significantly at 2 weeks (P < 0.001) and remained increased at 6 weeks (P < 0.001), showing statistically insignificant correlations with 2-week P1-AD (P ≥ 0.553). The central MT increased (P = 0.787), whereas MT at 1 mm (P = 0.444), 3 mm (P = 0.039), and 5 mm (P < 0.001) zones decreased. CONCLUSIONS: The return of P1-AD to preoperative (physiologic) values at 6 weeks confirms the safety standards of the duration and intensity of ultraviolet A radiation, as proposed by the Dresden protocol. However, temporary postoperative retinal dysfunction cannot be excluded.

Therapy-resistant dry itchy eyes.

An 8 years old male presented to our clinic with dry eye symptomes. Different therapiy attemps were made in the last few months and did not lead to any improvement. Examining this patient revealed multiple signs of vitamin A deficiency, which could confirmed by laboratory examination. The initial substitution of vitamin A led to a fast rehabilitation and a following nutrition consulting kept the patient symptom-free over 6 month follow up. Vitamin A deficiency -although rare in the developed countries- is an importent differential diagnosis of the dry eye especially in children. Vitamin A deficiency not only causes ocular manifistaion, but also general symptoms. Dietary change and initial subtitution is the key element for a fast and sustaining improvement.

Comparison of Optic Disc Parameters in Hyperopic and Emmetropic Eyes of Healthy Children with HRT and OCT.

PURPOSE: To investigate, through Heidelberg retina tomography II (HRT II) and RTVue-100 optical coherence tomography (OCT), normal parameters of the optic nerve head (ONH) in highly hyperopic children, and compare these with a group of children with low hyperopia and emmetropia, as fundus examination of highly hyperopic children often shows crowding of the optic disc, which may be difficult to distinguish from mild optic disc swelling. PATIENTS AND METHODS: ONH of the higher hyperopic eye was examined with HRT II and the peripapillary nerve fiber layer (RNFL) with RTVue-100, in 6 to 15-year-old full-term children with normal visual acuity and spherical equivalent ≥ + 3.0 D (hyperopic group) and < + 3.0 to - 1.0 D (emmetropic group). RESULTS: Thirty highly hyperopic children and 33 emmetropic controls had a mean spherical equivalent of + 5.1 ± 1.5 D and a mean age of 8.4 ± 2.8 years, and + 1.0 ± 1.2 D and 9.6 ± 2.6 years, respectively. There was no significant difference in OCT-assessed RNFL thickness between the two groups. Compared to the emmetropic group, following HRT parameters were significantly smaller in the hyperopic group: Disc area (1.41 vs. 1.68 mm2, p = 0.023), cup area (0.19 vs. 0.37 mm2, p = 0.0001), cup volume (0.03 vs. 0.08 mm3, p = 0.02), cup/disc area (0.13 vs. 0.20, p = 0.006), linear cup/disc (0.33 vs. 0.42, p = 0.004), and mean cup depth (0.13 vs. 0.17 mm, p = 0.019). CONCLUSION: In children, ONH of highly hyperopic eyes are smaller than those of emmetropic eyes. RNFL thickness and neural rim volume are similar to emmetropic eyes. They can therefore appear more crowded.

Electroretinogram and visual field changes in a case of birdshot chorioretinopathy.

PURPOSE: This case report explores the role of the visual field perimetry and electroretinogram (ERG) in cases of non-infectious uveitis in the assessment and monitoring of retinal function and response to treatment. METHODS: A 59-year-old Caucasian female presenting with bilateral posterior uveitis newly diagnosed as birdshot chorioretinopathy (BSCR) presenting with bilateral decrease in visual acuity and cystoid macular edema, as well as a paracentral scotoma in the right eye. The diagnosis and follow-up of the case was done using visual field perimetry, optical coherence tomography (OCT), and ERG. RESULTS: Baseline ERG showed a marked decrease in the amplitudes of the scotopic and photopic responses and a delay of peak times mainly in the right eye. Mycophenolate mofetil at 2 g/day and oral prednisolone at 1 g/kg/day were administered with gradual tapering of the corticosteroids. After 5 months, there was a noticeable improvement in the visual acuity, macular edema in OCT, and an obvious increase in the amplitudes of the ERG associated with a decrease in peak times, particularly in the 30 Hz photopic 3.0 Flicker of the right eye. CONCLUSION: This case report highlights the importance of the peak time and wave amplitudes of the 30 Hz photopic DA 3.0 Flicker as being a sensitive parameter in the diagnosis and follow-up of BSCR.