Persistent hyperplastic primary vitreous (PHPV)

Case contributed by Praveen Jha
Diagnosis probable

Presentation

Blindness, leukocoria on examination, excessive tears.

Patient Data

Age: 1 year
Gender: Female
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Low level echoes in the vitreous are seen with irregular echogenic soft tissue in retrolental area, with a central arterial flow.

This study is a stack
Axial
T2
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Sagittal
T2
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Coronal
T1
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Coronal
T2
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Coronal T1
C+ fat sat
This study is a stack
Sagittal T1
C+ fat sat
This study is a stack
Axial T1
C+ fat sat
Axial T1
C+ fat sat
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Mildly hyperintense signal vitreous is seen, with no appreciable post-contrast enhancement. A central hyaloid artery is seen extending from lens to retina, giving a characteristic 'wine-glass' appearance. Right lacrimal gland enlargement was also noted.

Axial
non-contrast
non-contrast
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Hyperdense vitreous, with no evidence of any calcification

Case Discussion

Persistent hyperplastic primary vitreous (PHPV) is a rare developmental disorder of childhood. It is one of the differentials of leukocoria, which should be ruled out. Characteristic presence of persistent hyaloid artery, and absence of contrast enhancement or calcification, differentiates it from other conditions like retinoblastoma or Coat's disease.

Lacrimal gland enlargement and PHPV does not seem to have a direct association, however does explain excessive tears in this patient.

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