Case Series of Pediatric Sickle Retinopathy By Sensitive Clinical Imaging Technologies Suggests Frequent Early Pathology
| Autor: | Nancy S. Green, Pahl A. Daniel, Jonathan S. Chang, Royce W.S. Chen, Elana Smilow, Maureen Licursi, Monica Bhatia, Margaret T. Lee, Courtney Briamonte |
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| Rok vydání: | 2016 |
| Předmět: |
Pathology
medicine.medical_specialty Retina Plexus Slit lamp genetic structures business.industry Immunology Visual impairment Cell Biology Hematology medicine.disease Biochemistry eye diseases Peripheral medicine.anatomical_structure Medical imaging medicine sense organs medicine.symptom business Perfusion Retinopathy |
| Zdroj: | Blood. 128:3672-3672 |
| ISSN: | 1528-0020 0006-4971 |
| Popis: | Introduction: Sickle cell retinopathy (SCR) reflects disease-related vascular injury. Previous studies of pediatric SCR using standard imaging approaches reported retinopathy in Results: Eight children (mean age 16.3 years; 5 HbSS, 2 HbSC,1 HbS-Beta thalassemia0) were evaluated. Three were on hydroxyurea therapy, 1 on chronic transfusion, 1 on both and 3 on no therapy. No patient had vision disturbance at the time of examination. Biomicroscopic examination revealed sunburst lesions in the mid-peripheral retina in 3 patients and subtle vascular changes in the far periphery of all patients. In contrast, UWFA detected extensive capillary non- perfusion of the peripheral retina with abnormal vascular loops (Goldberg Stage I) in all 8 patients; 3 patients had peripheral arteriovenous anastomoses (Goldberg Stage II) that were undetectable by biomicroscopy. No patient had Goldberg Stages III-V. SD-OCT demonstrated thinning of the temporal macula in 2 patients, and OCT-A of both patients revealed flow voids in both the superficial and deep retinal capillary plexus in the area of thinned retina. Both the SD- OCT and OCT-A abnormalities were undetectable with standard biomicroscopy. Conclusions: All 8 children with SCD in this case series demonstrated evidence of SCR using the combination of standard biomicroscopy, UWFA, SD-OCT, and OCT-A imaging. Findings from these sensitive imaging modalities suggest that pediatric SCR is more prevalent than previously suspected. Additional patients and age-equivalent controls will be included in this report to validate the specificity of findings to SCD. Larger cross-sectional and prospective analyses using sensitive retina imaging are needed to confirm our findings and identify their significance for detecting pathology. If confirmed, these approaches may enhance early screening for patients at risk of vision-threatening consequences of SCR. Supported by the Irving Institute CTSA at CUMC and by 2T35HL007616 (DP). Retinal findings in pediatric sickle cell disease patients SD-OCT: Spectral-domain optical coherence tomography; OCT-A: Optical coherence tomography angiography; UWFA: Ultra-widefield fluorescein angiography. Retinal findings in pediatric sickle cell disease patients. / SD-OCT: Spectral-domain optical coherence tomography; OCT-A: Optical coherence tomography angiography; UWFA: Ultra-widefield fluorescein angiography. Disclosures No relevant conflicts of interest to declare. |
| Databáze: | OpenAIRE |
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