Ocular Lesion

Ocular Lesion
Kristine M. Mosier, DMD, PhD
DIFFERENTIAL DIAGNOSIS
Common
  • Retinoblastoma
  • Persistent Hyperplastic Primary Vitreous
  • Retinopathy of Prematurity
  • Congenital Cataract
Less Common
  • Coats Disease
  • Coloboma
  • Toxocariasis, Orbit
  • Congenital Microphthalmos
Rare but Important
  • Retinal Astrocytoma
  • Norrie Disease
  • Walker-Warburg Syndrome
ESSENTIAL INFORMATION
Helpful Clues for Common Diagnoses
  • Retinoblastoma
    • Key facts: Malignant tumor arising from neuroectodermal cells of retina
      • Classified as primitive neuroectodermal tumor (PNET)
      • Most common intraocular tumor of childhood
      • Most common cause of leukocoria
      • Rare trilateral or quadrilateral form involves bilateral globes + pineal, or pineal + suprasellar tumor
    • Imaging
      • Unilateral (70-75%)
      • CT: Punctate or speckled calcified intraocular mass
      • MR: T1 mildly hyperintense, T2 moderately hypointense (cf. vitreous); moderate to marked heterogeneous enhancement
  • Persistent Hyperplastic Primary Vitreous
    • Key facts: Congenital lesion due to incomplete regression of embryonic vitreous and blood supply
      • 2nd most common cause of leukocoria
    • Imaging
      • Hyperdense or hyperintense small globe
      • No calcification
      • Retrolental enhancing soft tissue, classically with “martini glass” shape
      • Associated retinal detachments common
  • Retinopathy of Prematurity
    • Key facts: Occurs due to prolonged exposure to supplemental oxygen in premature infants
      • Premature birth interrupts normal vasculogenesis image incomplete vascularization of retina image hypoxia image abnormal neovascularization
    • Imaging
      • Usually bilateral
      • Hyperdense globe ± abnormal retrolental soft tissue
      • Early: Microphthalmia
      • Advanced: Vitreal calcification
  • Congenital Cataract
    • Key facts: Lens opacification
      • Most are sporadic and unilateral
      • ˜ 20% familial
      • ˜ 17% associated with systemic disease or syndrome: Trisomy 21, craniofacial syndromes, diabetes, etc.
    • Imaging
      • Small, hypodense lens
      • Lens may assume spherical shape (spherophakia), differentiating from acquired cataract
Helpful Clues for Less Common Diagnoses
  • Coats Disease
    • Key facts: Primary retinal vascular anomaly with retinal telangiectasis and exudative retinal detachment
      • Unilateral in 80-90% of patients
      • Most patients male
    • Imaging
      • Advanced stages may appear to obliterate vitreous
      • CT: Mild diffusely and homogeneously hyperdense vitreous without calcification
      • MR: Retinal detachment with nonenhancing T1 and T2 hyperintense subretinal exudate
  • Coloboma
    • Key facts: Defect in ocular tissue involving structures of embryonic cleft
      • Bilateral > unilateral
    • Imaging
      • Focal defect of posterior globe with outpouching of vitreous
      • Defect at optic nerve head insertion with funnel-shaped excavation
  • Toxocariasis, Orbit
    • Key facts: Eosinophilic granuloma caused by infection of larval nematode Toxocara cani or cati
    • Imaging
      • CT: Diffuse hyperdensity in vitreous ± discrete mass; no calcification
      • MR: Enhancing, variable T1 hypo/isointense, T2 iso/hyperintense retrolental or vitreous mass
  • Congenital Microphthalmos
    • Key facts: Corneal diameter of < 11 mm and anteroposterior diameter of globe < 20 mm at birth
      • Isolated
      • Or associated with craniofacial anomalies, coloboma, persistent hyperplastic primary vitreous, retinopathy of prematurity
    • Imaging
      • Overall volume and dimension of globe < unaffected side or comparison normal
      • Simple microphthalmia: Structurally normal small eye or complex with associated findings, as above
Helpful Clues for Rare Diagnoses
Aug 10, 2016 | Posted by in PEDIATRICS | Comments Off on Ocular Lesion

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