Persistent hyperplastic primary vitreous

Case contributed by Dr Anand Shrikant Gajakos

Presentation

Leukocoria, right monocular blindness.

Patient Data

Age: 4 years, 3 months
Gender: Male
Ultrasound

The right eye globe is small in size.

A elongated thick echogenic band is seen in the posterior segment extending from the lens upto the optic nerve head.This echogenic band shows a small sized artery showing continuous biphasic blood flow on doppler.

The ciliary process is thick and echogenic.Lens is small, echogenic with irregular contour.

Case Discussion

Embryologically the primary vitreous forms around 7th week of gestational life and starts regressing at around 20-21 weeks of gestational life and is completely regressed at the time of birth.

Pathologically it is a condition in which the embryonic hyaloid artery fail to regress. Persistence of this leads to fibrosis resulting in cataract, retinal detachment, and ciliary process hypertrophy.

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Case information

rID: 36489
Case created: 25th Apr 2015
Last edited: 14th Nov 2015
System: Head & Neck
Inclusion in quiz mode: Included

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