OUTER RETINAL TUBULATION IN BIETTI CRYSTALLINE DYSTROPHY ASSOCIATED WITH THE RETINAL PIGMENT EPITHELIUM ATROPHY.

PURPOSE: To determine the prognostic value of outer retinal tubulation (ORT) in the eyes of a Chinese cohort with Bietti crystalline dystrophy (BCD). METHODS: This retrospective, multicenter cohort study enrolled 42 patients with clinically and genetically diagnosed BCD. Eighty eyes with good-quality images of spectral domain optical coherence tomography were included. Demographic details and clinical data were collected. The characteristics of ORT, including prevalence, location, and morphologic characteristics were analyzed. RESULTS: Forty-two patients with BCD harbored potentially CYP4V2 disease-causing mutations. The mutation spectrum comprised 17 unique variants, 9 of which were novel. Fifty-two of these 80 eyes demonstrated evidence of ORT. The incidence of ORT is significantly higher in Stage 2 than other stages ( P < 0.001). ORT was mainly bilateral and located at the margin of the atrophic area of retinal pigment epithelium (RPE), and dynamically changed with the progressive RPE atrophy. The process of RPE atrophy was slower in eyes with ORT ( P = 0.017), with significantly longer intact RPE width in Stage 3 ( P = 0.024). Eyes with ORT had slower vision loss than eyes without ORT ( P = 0.044). CONCLUSION: ORT may be a sign of the onset of RPE atrophy in early-stage BCD and may suggest less risk of rapid progression in late-stage BCD.

Effect of interactive, multimedia-based home-initiated education on preoperative anxiety inchildren and their parents: a single-center randomized controlled trial.

BACKGROUND: Anesthesiologists need to appreciate the impact of preoperative anxiety in children. The present study aimed to explore whether interactive multimedia-based home-initiated interventions could effectively relieve preoperative anxiety in pediatric patients. METHODS: In this prospective study, we compared preoperative anxiety between two groups of children aged 4-9 years. Children in the control group received a question-and-answer (Q&A) introduction, and children in the intervention group received multimedia-based home-initiated preoperative education using comic booklets, videos, and coloring game books. Differences in anxiety between the two groups were evaluated by the modified Yale Preoperative Anxiety Scale-Short Form (mYPAS-SF) at four time points: in the ophthalmology outpatient clinic before intervention as the baseline (T0); in the preoperative waiting area (T1); at the time of separating from their parents and moving to the operating room (T2); and at the time of anesthesia induction (T3). Parental anxiety was assessed by the Self-rating Anxiety Scale (SAS) and Visual Analog Scale (VAS) at T0 and T2. Other related information was collected by questionnaire. RESULTS: Eighty-four children who underwent pediatric strabismus in our center between November 2020 and July 2021 were included in this study. An intention-to-treat (ITT) analysis was performed on data from 78 enrolled children. Children in the intervention group exhibited lower m-YPAS-SF scores at T1, T2, and T3 than those in the control group (all p < 0.001). By using a mixed-effect model with repeated measurement (MMRM) after adjusting the m-YPAS score at T0 as a covariate, the interventional effect in terms of themYPAS-SF score was also significant over time (p < 0.001). The percentage of children with perfect induction compliance (ICC = 0) in the intervention group was significantly higher than that in the control group [18.4% vs. 7.5%], and poor induction compliance (ICC>4) was lower (2.6% vs. 17.5%, p = 0.048). The mean parental VAS score at T2 in the intervention group was significantly lower than that in the control group (p = 0.021). CONCLUSIONS: Interactive multimedia-based home-initiated intervention could reduce preoperative anxiety in children and improve the quality of anesthesia induction based on ICC scores, which may in turn impose a positive impact on parental anxiety.

Permanent Visual Impairment due to Delayed Diagnosis of Shunt Malfunction in Case of Lack of Typical Features of Increased Intracranial Pressure and Unchanged Ventricular Size.

OBJECTIVE: We present the clinical features, imaging, and management of 5 cases of visual impairment as the primary manifestation ventriculoperitoneal (V-P) shunt malfunction. METHODS: We retrospectively reviewed the medical records of 126 patients of V-P shunt malfunction in Shanghai Children's Medical Center between 2015 and 2020. Medical records including all hospital admissions were reviewed and follow-up data were collected. RESULTS: Five children (3.97%) had visual impairment as the primary manifestation of V-P shunt malfunction, with a mild or no headache. Four broken distal shunt catheters and one proximal catheter blockage were confirmed intraoperatively and cured by surgery. None of the patients had a definite improvement in ophthalmic examinations after 4-52 months of follow-up. CONCLUSION: Visual impairment as the primary manifestation of V-P shunt malfunction was uncommon and could be easily missed or misdiagnosed as the only problem for lack of typical features of increased intracranial pressure and unchanged ventricular size. Earlier definitive diagnosis and surgical intervention could prevent a further development of the visual loss caused by V-P shunt malfunction.

Axial length/corneal radius of curvature ratio and refractive development evaluation in 3- to 4-year-old children: the Shanghai Pudong Eye Study.

AIM: To measure and analysis axial length (AL)/corneal radius of curvature (CRC) ratio and other refractive parameters, provide a medical reference range for refractive development evaluation and earlier visual impairment screening of 3 to 4y kindergarten students. METHODS: Between April and June 2017, a total of 4350 participants aged 3- to 4-year-old (8700 eyes) from 10 cluster random sampling kindergartens in Shanghai, Pudong District were involved. According to the measurement and analysis of the unaided visual acuity (VA), AL, CRC, AL/CRC ratio, astigmatism and other refractive parameters, the data distribution and reference range were obtained. RESULTS: Uncorrected VA of examined children was 0.23±0.08 (logMAR, mean±SD) [95% confidence interval (CI) range ≤0.36]; AL was 22.10±0.79 mm (95%CI 20.55-23.65); CRC was 7.86±0.26 mm (95%CI, 7.35-8.37); AL/CRC ratio was 2.81±0.12 (95%CI, 2.57-3.05). The median of astigmatism was -0.5 D, a total of 56.3% had astigmatism <-0.50 D, 85.3%<-1.00 D, 6.7%>-1.50 D; 71% were astigmatism with the rule. Eye-specific analyses were conducted. Statistical difference of VA was in right and left eyes. There were no significant differences between two eyes of AL, CRC, AL/CRC ratio and astigmatism (P>0.05). CONCLUSION: VA and AL/CRC ratio reference could be used to assess refractive development in children and screening uncorrected refractive errors or amblyopia. Astigmatism needs to be considered in the diagnosis.