7/27/2019 Radiologi Persistent Hyperplastic Primary Vitreous

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Persistent hyperplastic primary vitreousDr 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.

Clinical presentation

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.

Sub types

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.

CTThe 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

7/27/2019 Radiologi Persistent Hyperplastic Primary Vitreous

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• 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

Differential diagnosis

• 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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