Leukocoria: Clinical and Neuroradiological Findings

Galluzzi, Paolo; Hadijstilianou, Theodora; Venturi, Carlo

doi:10.1177/197140090401700109

Cited by 2 publications

(2 citation statements)

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“…The validated "Retinoblastoma Imaging Atlas" was used as a basis for scoring the images [34], supplemented with imaging features suitable for Coats' Disease and PFV/retinal dysplasia. Prior selection of these additional features was based on review of the literature [6,15,[17][18][19][20][21]23] or expert radiologists' experience (a full predefined imaging feature list including illustrations is available in Figure S1). Although laterality is an important factor for differentiation between diseases in clinical practice, it was not considered in this MR imaging feature list because it can be reliably assessed through clinical examination and ultrasonography.…”

Section: Mr Imaging Assessmentmentioning

confidence: 99%

“…The smaller size of the affected eye (compared with the contralateral) on MR imaging ranges from PFV eyes (smallest, regularly microphtalmia with >2SD below mean size for age), to Coats' disease eyes (smaller than retinoblastoma eyes, but no microphtalmia), to retinoblastoma (mean size slightly smaller than healthy eyes) [15,16]. Hyperintensity (on T1, T2 and FLAIR images) of subretinal exudate has been described in advanced Coats' disease [17][18][19]. MR imaging of PFV shows intra-ocular hemorrhage, lens deformations, vitreous membranes from the optic nerve (residual fetal hyaloid canal), and retinal detachment in a T-/funnel-shape or Y-shape (sometimes called 'Martini-Glass-Sign') [20,21].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

MR Imaging Features to Differentiate Retinoblastoma from Coats’ Disease and Persistent Fetal Vasculature

Jansen

Bloeme

Brisse

et al. 2020

Cancers

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Retinoblastoma mimickers, or pseudoretinoblastoma, are conditions that show similarities with the pediatric cancer retinoblastoma. However, false-positive retinoblastoma diagnosis can cause mistreatment, while false-negative diagnosis can cause life-threatening treatment delay. The purpose of this study is to identify the MR imaging features that best differentiate between retinoblastoma and the most common pseudoretinoblastoma diagnoses: Coats’ disease and persistent fetal vasculature (PFV). Here, six expert radiologists performed retrospective assessments (blinded for diagnosis) of MR images of patients with a final diagnosis based on histopathology or clinical follow-up. Associations between 20 predefined imaging features and diagnosis were assessed with exact tests corrected for multiple hypothesis testing. Sixty-six patients were included, of which 33 (50%) were retinoblastoma and 33 (50%) pseudoretinoblastoma patients. A larger eye size, vitreous seeding, and sharp-V-shaped retinal detachment were almost exclusively found in retinoblastoma (p < 0.001–0.022, specificity 93–97%). Features that were almost exclusively found in pseudoretinoblastoma included smaller eye size, ciliary/lens deformations, optic nerve atrophy, a central stalk between optic disc and lens, Y-shaped retinal detachment, and absence of calcifications (p < 0.001–0.022, specificity 91–100%). Additionally, three newly identified imaging features were exclusively present in pseudoretinoblastoma: intraretinal macrocysts (p < 0.001, 38% [9/24] in Coats’ disease and 20% [2/10] in PFV), contrast enhancement outside the solid lesion (p < 0.001, 30% [7/23] in Coats’ disease and 57% [4/7] in PFV), and enhancing subfoveal nodules (38% [9/24] in Coats’ disease). An assessment strategy was proposed for MR imaging differentiation between retinoblastoma and pseudoretinoblastoma, including three newly identified differentiating MR imaging features.

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