Incidence and risk factors of retinopathy of prematurity in three neonatal intensive care units in Palestine.

BACKGROUND: Severe Retinopathy of Prematurity (ROP) is a serious vasoproliferative disorder that can affect extremely premature infants. It continues to be one of the most important preventable causes of blindness in children. Our study is aimed at finding the incidence of ROP and its association with some risk factors in Palestine. METHODS: From the 1st of January 2016 to 31st December 2016, a total number of 115 infants who met the criteria for ROP screening in three neonatal intensive care units were included in the study. The medical records of infants were reviewed retrospectively and multiple factors that may be associated with the development of ROP were collected manually. RESULTS: The incidence of ROP and severe type 1 ROP that require treatment was 23.5 and 11.3% respectively. After conducting univariate analysis of risk factors, statistically significant risk factors affecting the development of ROP in our study were: low gestational age, low birth weight, type of multiple gestation, the presence of affected sibling, low level of Hemoglobin at birth, respiratory distress syndrome, low Hemoglobin level, blood transfusion and days on oxygen supplements with either mechanical, non-mechanical methods or both combined. High bilirubin levels were found as a protective factor against the development of ROP. However, when a multivariate analysis was performed, only low gestational age, total days on oxygen supplement and high bilirubin levels were significant regarding the development of ROP. CONCLUSION: The incidence of ROP is considered a relatively low percentage compared to neighboring countries that have higher levels of human development index. Statistically significant risk factors need to be considered when clinicians deal with premature infants.

Detailed Clinical, Ophthalmic, and Genetic Characterization of ADGRV1-Associated Usher Syndrome.

PURPOSE: To present the clinical characteristics, retinal features, natural history, and genetics of ADGRV1-Usher syndrome (USH). DESIGN: Multicenter international retrospective cohort study. METHODS: Clinical notes, hearing loss history, multimodal retinal imaging, and molecular diagnosis were reviewed. Thirty patients (28 families) with USH type 2 and disease-causing variants in ADGRV1 were identified. Visual function, retinal imaging, and genetics were evaluated and correlated, with retinal features also compared with those of the commonest cause of USH type 2, USH2A-USH. RESULTS: The mean age at the first visit was 38.6 ± 12.0 years (range: 19-74 years), and the mean follow-up time was 9.0 ± 7.7 years. Hearing loss was reported in the first decade of life by all patients, 3 (10%) described progressive loss, and 93% had moderate-severe impairment. Visual symptom onset was at 17.0 ± 7.7 years of age (range: 6-32 years), with 13 patients noticing problems before the age of 16. At baseline, 90% of patients had no or mild visual impairment. The most frequent retinal features were a hyperautofluorescent ring at the posterior pole (70%), perimacular patches of decreased autofluorescence (59%), and mild-moderate peripheral bone-spicule-like deposits (63%). Twenty-six (53%) variants were previously unreported, 19 families (68%) had double-null genotypes, and 9 were not-double-null. Longitudinal analysis showed significant differences between baseline and follow-up central macular thickness (-1.25 µm/y), outer nuclear layer thickness (-1.19 µm/y), and ellipsoid zone width (-40.9 µm/y). The rate of visual acuity decline was 0.02 LogMAR (1 letter)/y, and the rate of constriction of the hyperautofluorescent ring was 0.23 mm2/y. CONCLUSIONS: ADGRV1-USH is characterized by early-onset, usually non-progressive, mild-to-severe hearing loss and generally good central vision until late adulthood. Perimacular atrophic patches and relatively retained ellipsoid zone and central macular thickness in later adulthood are more often seen in ADGRV1-USH than in USH2A-USH.