Prevalence and Characteristics of Cytomegalovirus Ocular Disease in Children: A Multi-Center Study

Carmel L Mercado,1,2 Colin P Froines,3 Eric D Gaier,4– 7 Qinyun Wang,8 Maanasa Indaram,8 Michael J Wan,9 Ankoor S Shah,4– 6 Euna B Koo10 1Department of Ophthalmology, University of Washington, Seattle, WA, USA; 2Department of Ophthalmology, Seattle Children’s Hospital, Seattle, WA, USA; 3School of Medicine, University of Washington, Seattle, WA, USA; 4Department of Ophthalmology, Boston Children’s Hospital, Boston, MA, USA; 5Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, MA, USA; 6Department of Ophthalmology, Harvard Medical School, Boston, MA, USA; 7Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, MA, USA; 8Department of Ophthalmology, University of California San Francisco, San Francisco, CA, USA; 9Department of Ophthalmology and Vision Sciences, SickKids, Toronto, ON, Canada; 10Department of Ophthalmology, Stanford University School of Medicine, Palo Alto, CA, USACorrespondence: Euna B Koo, Department of Ophthalmology, Stanford University School of Medicine, 2452 Watson Ct, Palo Alto, CA, 94303, USA, Tel +1 650-723-6995, Email eunakoo@stanford.eduPurpose: The objective of this study was to identify the prevalence of CMV ocular disease in children and to identify associated risk factors for ocular involvement.Design: Retrospective multicenter, cross-sectional study.Methods: Setting: Hospitalized patients screened for CMV viremia by PCR between 2005 and 2018 at four pediatric referral centers. Participants: Seven-hundred and ninety-three children showed CMV viremia (> 135 copies/mL by polymerase chain reaction; PCR). Main Outcomes and Measures: (1) Occurrence of ophthalmologic examination. (2) Presence of CMV ocular disease, defined as retinitis, vasculitis, hemorrhage, optic nerve atrophy, or anterior uveitis in the setting of CMV viremia without other identifiable causes.Results: A total of 296/793 (37%) underwent ophthalmologic examination following CMV viremia. A total of23/296 patients (8%) had ocular symptoms prompting evaluation while the rest had eye exams for baseline screening unrelated to CMV viremia. Of these, 13 cases (4% of those with an eye exam) with ocular disease were identified (three congenital CMV, five severe combined immunodeficiency disorder (SCID) status post-stem cell transplantation, three hematologic malignancy status post-stem cell transplantation for two of them, one Evans syndrome status post-stem cell transplantation, and one medulloblastoma status post-bone marrow transplantation). No patients with solid organ transplantation developed CMV ocular disease in our cohort.Conclusion: CMV ocular disease was a rare occurrence in this cohort without an identifiable pattern across sub-groups. Excluding the three congenital CMV cases, nine out of ten patients with CMV ocular disease were status post-stem cell transplantation. We provide integrated screening guidelines based on the best available evidence for this rare condition.Keywords: cytomegalovirus, retinitis, CMV, screening guidelines, pediatrics