THE TARGET SIGN: A Near Infrared Feature and Multimodal Imaging in a Pluri-Ethnic Cohort with RDH5-Related Fundus Albipunctatus.

PURPOSE: Retinol dehydrogenase 5 (RDH5)-related fundus albipunctatus can present with phenotypic variability. Our purpose was to investigate new clinical characteristics and multimodal imaging findings in patients from different ethnic origins, carrying different mutations. METHODS: Multicenter international retrospective case series of 18 patients with genetically confirmed RDH5-related fundus albipunctatus. Patients' files were reviewed for fundus images, visual acuity, macular optical coherence tomography scans, near-infrared images, fundus autofluorescence, electroretinogram, and genetic mutations. Imaging and electroretinogram findings. RESULTS: All eyes (n = 36, 100%) showed small circular findings seen on near-infrared images, termed as the "target sign," correlating to the yellowish dots seen clinically and to the distinct hyperreflective linear lesions on optical coherence tomography at the level between external limiting membrane and retinal pigment epithelium. Perifoveal atrophy with foveal sparing was seen in 4 eyes of 2 patients (both RDH5-c.160C>T, p.R54X mutation). Fundus autofluorescence revealed small hyperautofluorescent dots (n = 16, 44.4%). Scotopic electroretinograms were significantly reduced in all cases with an electronegative pattern, 66.7% displayed cone dysfunction. CONCLUSION: Our results show distinct imaging findings present in all patients with fundus albipunctatus independent of ethnicity or genetic mutation. Our results can facilitate the current algorithm to diagnose RDH5-related fundus albipunctatus and allow for targeted genetic testing.

Hyperbaric oxygen treatment for non-arteritic central retinal artery occlusion retrospective comparative analysis from two tertiary medical centres.

OBJECTIVES: To compare the visual outcome of patients treated for non-arthritic central retinal artery occlusion (CRAO) in a medical centre that uses hyperbaric oxygen therapy (HBOT) as part of the standard of care (SOC) to medical centres that does not. METHODS: The study included data from two tertiary medical centres. The medical records of all patients diagnosed with non-arthritic CRAO without a patent cilioretinal artery between January 2010 and December 2018 in two tertiary medical centres were reviewed. RESULTS: One hundred and twenty-one patients were treated by HBOT and 23 patients received only SOC. In the HBOT group, best-corrected visual acuity (BCVA) improved from 2.89 ± 0.98 logMAR at presentation to 2.15 ± 1.07 logMAR upon the end of HBOT (P < 0.001), while the SOC group had no significant improvement, from 3.04 ± 0.82 logMAR at presentation to 2.80 ± 1.50 logMAR (P = 0.24). With adjustment for age, gender, and the duration of symptoms, final BCVA in the HBOT group was significantly better compared to the control group (P = 0.023). Rates of patients achieving vision of 20/200 or better were similar between groups (17.4% vs. 19.8%, P = 0.523). CONCLUSION: Utilizing HBOT as part of the SOC for CRAO improves the final visual outcome. HBOT is safe and can be implemented, if available, as part of SOC in all tertiary medical centres.

Postoperative supine position after primary Descemet-stripping automated endothelial keratoplasty reduces graft detachment rate.

OBJECTIVE: To characterize the association between prolonged supine postoperative positioning of patients undergoing Descemet-stripping automated endothelial keratoplasty (DSAEK) and graft dislocation rate. METHODS: In this retrospective cohort study, medical records of patients who underwent uncomplicated DSAEK surgery at Yitzhak Shamir Medical Center between 2010 and 2019 were reviewed. Nursing documentation of patients' adherence to supine positioning during the postoperative hospitalization period was collected. A patient was considered compliant if he or she was documented as cooperative with supine positioning throughout the first 24 hours. RESULTS: A total of 170 eyes of 138 patients were found eligible. Main indications for surgery were pseudophakic bullous keratopathy (50.6%), previous graft failure (25.9%), and Fuch's endothelial dystrophy (FED; 20.6%). Twelve surgeries were combined with cataract extraction. Postoperative graft detachment occurred in 26 eyes (15.3%) after an average period of 1 day (range, 0-20 days). Compliance with supine positioning was documented in 84.1% (n = 143 patients). Noncompliance rates during the first 24 hours in the detached and nondetached groups were 26.9% (n = 7) and 14.4% (n = 20), respectively; after adjustment for possible confounders, the odds ratio (OR) was 1.44 (p = 0.249). Graft dislocation was observed in 13.3% (19 of 143) and 25.9% (7 of 27) of cooperative and noncooperative patients, respectively (p = 0.17). Subanalysis of 120 eyes with either BPK or FED for which it was the first transplantation demonstrated a protective effect of supine positioning (OR 3.42, confidence interval 1.095-10.700; p = 0.034). Findings for both groups remained unchanged in multivariate analysis. CONCLUSIONS: We found a statistically significant protective effect of 24 hours of postoperative supine positioning against graft detachment after DSAEK in eyes with no prior transplantations.

Corneal Cross-Linking in Pediatric Patients: Evaluating Treated and Untreated Eyes-5-Year Follow-Up Results.

PURPOSE: To evaluate the long-term results of corneal collagen cross-linking (CXL) for treatment of pediatric keratoconus and the long-term outcomes of the fellow untreated keratoconic eye in patients younger than 18 years old. METHODS: A retrospective case analysis was performed on 88 eyes of 44 patients aged 18 years or younger, with keratoconus, who underwent CXL in at least 1 eye. Follow-up measurements, for the treated and untreated eye pair, taken up to 5 years after treatment, were compared with baseline values. Parameters included uncorrected distance visual acuity (UCDVA), best spectacle-corrected distance visual acuity (BCDVA), manifest refraction, pachymetry, and corneal topography and tomography. RESULTS: Mean age of patients was 15.6 ± 2.1 years. For the treated eyes, during all years of follow-up, UCDVA improved significantly (from 0.83 ± 0.30 to 0.72 ± 0.28 logMAR; P = 0.01). Improvement in BCDVA was not statistically significant (from 0.28 ± 0.19 to 0.23 ± 0.15 logMAR; P = 0.06). The manifest cylinder showed a significant reduction (from 5.8 ± 3.6 to 4.3 ± 2.5 diopters; P = 0.006). There was no significant change in maximum keratometry. Average keratometry and corneal thickness reduced significantly (P = 0.009 and P = 0.002, respectively). Five patients had very mild corneal haze after CXL. For the fellow untreated eyes-during 5 years of follow-up, UCDVA showed a slight decrease that was not statistically significant. BCDVA, average keratometry, and maximum keratometry remained stable. CONCLUSIONS: Our long-term follow-up study suggests that CXL is a safe procedure in the pediatric age, and there is no urgency in treating pediatric patients with keratoconus without proof of progression.

Modified treatment of distichiasis with direct tarsal strip excision without mucosal graft.

PURPOSE: To describe a new modified technique of direct tarsal excision for treatment of distichiasis. METHODS: Retrospective review of consecutive patients who underwent direct tarsal excision without grafting to treat distichiasis between December 2007 and November 2015. Gender, number of eyelids treated, follow-up time, and surgical outcome were recorded. The technique involved dividing the anterior and posterior lamella and excising a two mm tarsoconjunctival strip including the abnormal lash follicles, without suturing or mucosal graft. RESULTS: Seventeen eyelids of 12 patients (Fourteen upper eyelids and 3 lower eyelids) were studied. Sixteen eyelids had acquired distichiasis and one eyelid had congenital distichiasis. Mean age was 64.8 (SD 22.0; range, 25-86 years) Mean postoperative follow-up time was 53 months (SD = 31, range 8 - 104 months). During this period, no recurrence was documented. There were no complications. CONCLUSIONS: Direct tarsal excision without a graft is a safe, quick, and effective method for the treatment of distichiasis.

The relation of somatotypes and stress response to central serous chorioretinopathy.

PURPOSE: To investigate a possible relationship between central serous chorioretinopathy (CSC) and specific body types and compositions (somatotypes), and to examine the cortisol stress response among CSC patients of different somatotypes in comparison with healthy subjects. METHODS: Prospective case-control study. A group of 28 patients with a previous or current diagnosis of CSC was compared with a group of 26 healthy subjects. Anthropometric measurements were used to estimate somatotype ratings in all subjects. Serum cortisol was measured at rest and following a stress-inducing computerized test in order to estimate response to stress in both groups. The main outcome measures included somatotype categorization and the change in serum cortisol following stress in both groups. RESULTS: No significant difference in somatotype composition was found between the groups. There was no statistically significant difference between the groups in the elevation of cortisol following the stress-inducing test. The sample size was too small to exclude or find any significant difference between the different 13 subgroups of somatotype composition in the elevation of cortisol. CONCLUSIONS: Our study did not show a typical somatotype related to CSC. While previous studies showed higher cortisol values in CSC patients, we did not see a higher elevation in blood cortisol following a stress response in this group in comparison with healthy subjects.

Accuracy and feasibility of axial length measurements by a new optical low-coherence reflectometry-based device in eyes with posterior subcapsular cataract.

PURPOSE: To evaluate the feasibility and accuracy of measuring axial length (AL) by a new optical low-coherence reflectometry (OLCR)-based device in eyes with central posterior subcapsular cataract (PSC). SETTING: Department of Ophthalmology, Assaf-Harofeh Medical Center, Zerifin, Israel. DESIGN: Retrospective case series. METHODS: Consecutive cases of patients who had uneventful cataract surgery and whose preoperative AL measurements were not feasible with the partial coherence interferometry (PCI) device because of a central PSC were assessed. Preoperative AL was measured by the OLCR device and immersion ultrasound (US). Preoperative results were compared with the postoperative AL measurements obtained by the PCI. RESULTS: Twenty-seven patients (27 eyes) were enrolled in the study. The median difference between the OLCR and the PCI AL measurements (0.07 mm) was lower than the median difference between the US and the PCI AL measurements (0.13 mm) (P = .016). The ranges of the limits of agreement were 0.15 mm between OLCR and PCI, and 0.88 mm between US and PCI. The proportion of eyes with an AL difference of less than 0.1 mm was significantly higher between the OLCR and the PCI devices (24 eyes [88.9%]) than between the US and the PCI devices (9 eyes [33.3%]) (P = .001). CONCLUSIONS: The OLCR-based device successfully measured the preoperative AL in all eyes with central PSC for which preoperative PCI scans were not feasible. These measurements had a high level of agreement with the postoperative AL measurements obtained by the PCI device.