Persistent hyperplastic primary vitreous

Dr Jeremy Jones and Dr Yuranga Weerakkody et al. Persistenthyperplasticprimaryvitreous(PHPV) , (also known as persistentfetal vasculature ) refers to a rare congenital developmental malformation of the eye.

Clinicalpresentation
Clinically, this condition usually manifests as unilateral or bilateral leucocoria. Patient's may also have poor vision, small eye (microphthalmia) and strabismus.

Pathology
It arises due to a failure of normal regression of the embryonic hyaloid vascular system. In the normal situation the primary vitreous forms around 7 th week of gestation life and starts involuting around 20 th week and nearly always disappears at the time of birth. Persistent fetal vasculature in PHPV can lead to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts.

Subtypes
PHPV can be divided into either anterior(ventral) or posterior(dorsal) types with most patients with PHPV having a combination of these 3.

Associations
PHPV can occur on its own or association with various other conditions (particularly) when bilateral, these include 9

Norrie disease Warburg syndrome retinal dysplasia

Radiographicfeatures
From an imaging standpoint, only the features of posterior PHPV are well known 3.

General
In posterior PHPV, the globe is small and can contain contains retinal detachments.

Ocularultrasound
An echogenic band maybe seen in the posterior segment of the globe extending from posterior surface of the lens to the optic nerve head. On colour Doppler, arterial blood flow was may be seen within this band.

CT
The CT appearance can be quite variable 9 and the described spectrum of CT findings includes 1

soft-tissue replacement (infiltration) of the vitreous body retrolental soft tissue along the Cloquet canal - fine linear structure extending from the head of the optic nerve to the posterior surface of the lens 3

absence of abnormal calcification within the orbit

microphthalmus retrohyaloid layered blood hypervascularity of the vitreous humor - after contrast administration, the vitreal abnormalities may enhance, which is believed to reflect a persistent hypervascular vitreous retinal detachments can be hyperdense on CT

Differentialdiagnosis

clinically it is one of the more important and frequent conditions that can mimic aretinoblastoma at ultrasound the main differential is of retinal detachment

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