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Lee, Jonghyun, MD, PhD*,†; El-Dairi, Mays A., MD*; Tran-Viet, Du, BS*; Mangalesh, Shwetha, MD*; Dandridge, Alexandria*; Jiramongkolchai, Kim, MD*; Viehland, Christian, MS; Toth, Cynthia A., MD*,‡

doi: 10.1097/IAE.0000000000001930
Original Study

Purpose: To explore vitreoretinal pathologies and their longitudinal changes visible on handheld optical coherence tomography (OCT) of young children with familial exudative vitreoretinopathy.

Methods: The authors retrospectively analyzed handheld OCT images for vitreoretinal interface and retinal abnormalities and optic nerve head (ONH) elevation.

Results: From 26 eyes of 16 children (mean age 32 months) with familial exudative vitreoretinopathy, 10 had ONH dragging on photographs, and in these, handheld OCT revealed temporal and anterior retinal displacement, prominent vitreopapillary adhesion or traction, and retinal nerve fiber layer thickening at ONH margins with adjacent retinal elevation. Despite a nearly normal photographic appearance, handheld OCT revealed ONH elevation with vitreopapillary traction (6/16 eyes), ONH edema (1/16 eye), and retinal vascular protrusion (5/16 eyes). Handheld OCT–visualized vitreous abnormalities (18/26 eyes) were more prevalent at higher stages of disease. Handheld OCT–visualized elevation of ONH and the retina worsened over time in nine eyes and improved in 5/6 eyes after vitrectomy.

Conclusion: Handheld OCT can detect early ONH, retinal, and vitreous changes in eyes with familial exudative vitreoretinopathy. Contraction of strongly adherent vitreous in young patients with familial exudative vitreoretinopathy appears to cause characteristic ONH dragging and tractional complications without partial posterior vitreous detachment. Vitreopapillary dragging may be visible only on OCT and may progress in the absence of obvious retinal change on conventional examination.

In addition to retinal traction by the peripheral fibrovascular membrane, strong anteroposterior traction by contraction of strongly adherent vitreous is a contributing factor during the progression of familial exudative vitreoretinopathy in infants.

*Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina;

Department of Ophthalmology, Inje University, Ilsan Paik Hospital, Goyang, Korea; and

Department of Biomedical Engineering, Duke University, Durham, North Carolina.

Reprint requests: Cynthia A. Toth, MD, Department of Ophthalmology, Duke University Medical Center, 2351 Erwin Road, Box 3802, Durham, NC 27710; e-mail:

Supported by Research Year of Inje University in 2015; The Hartwell Foundation; The Andrew Family Charitable Foundation; Grant Number R01 EY025009, R01 EY023039 from National Institute of Health (NIH); Grant Number P30 EY001583 from the National Eye Institute (NEI); Grant Number 1UL1RR024128-01 from the National Center for Research Resources (NCRR). Its contents are solely the responsibility of the authors and do not necessarily represent the official view of Inje University, NIH, NEI, or NCRR. The sponsors or funding organizations had no role in the design or conduct of this research.

C. A. Toth receives royalties through her university from Alcon and had previous research support from Bioptigen and Genentech. She also has unlicensed patents pending in OCT imaging and analysis. The remaining authors have no financial/conflicting interests to disclose.

© 2019 by Ophthalmic Communications Society, Inc.