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Definition: Pathologic entity caused by abnormal hypertrophy of the ocular hyaloid system.
Classic clue: Child presenting at or soon after birth with leukocoria, micropthalmia, fluid-fluid level, no calcifications, and persistent Cloquet’s canal is virtually pathognomonic.
In normal embryologic development, the primary vitreous is replaced by the adult secondary vitreous and does not normally hypertrophy or persist to infancy or childhood.
Persistent hyperplastic primary vitreous (PHPV) is the second most common cause of leukocoria after retinoblastoma and is a very important differential. ,
Microphthalmia.
Commonly causes immature cataract with lens opacity on computed tomography (CT).
More than 90% are unilateral.
Marked vitreal hyperintensity on T1, proton density, and T2 with conspicuous fluid-fluid level.
Microphthalmia.
No calcifications (as in retinoblastoma). ,
Increased vitreous attenuation. ,
Enhancement of abnormal intravitreal tissue (if contrast given).
Fluid-fluid levels from breakdown of recurrent hemorrhage.
Lens may be small and irregular.
Often shallow anterior chamber (type I anterior PHPV).
Optic nerve (ON) may be small. Computed Tomography
Less preferred, but may be first imaging study.
Radiation to orbit undesirable.
No calcifications (as in retinoblastoma). Magnetic Resonance Imaging Features
Characteristic hyperintense T2 signal differentiates this from retinoblastoma, which has hypointense T2 signal.
Microphthalmia.
Fluid-fluid level.
Hypointense to isointense thin triangular band extending from optic disc to lens.
Marked hyperintensity of vitreous on T1, proton density, and T2.
Related to proteinaceous fluid/methemoglobin (from hemorrhagic blood degradation products).
Avid enhancement of fibrovascular intravitreal mass.
Retinal detachments frequent:
Type A detachment from ON.
Type B detachment from point on wall eccentric to ON.
Exact findings depend on type:
Anterior, posterior, or combined.
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