What is Persistent Hyperplastic Primary Vitreous (PHPV)?
A congenital developmental anomaly caused by failure of regression of the embryonic primary vitreous and hyaloid vasculature.
What are other names for Persistent Hyperplastic Primary Vitreous (PHPV)?
Persistent fetal vasculature (PFV) or Persistent tunica vasculosa lentis.
Embryologic cause of PHPV?
Failure of the primary vitreous and hyaloid artery system to involute between the 7th month of gestation and early postnatal life.
Most common laterality of PHPV?
Unilateral in ~90% of cases.
Typical clinical presentation of PHPV in infants?
Leukocoria, microphthalmia, cataract, strabismus, or nystagmus.
Classic ultrasound finding in PHPV?
Echogenic retrolental membrane or stalk extending from posterior lens capsule to optic disc, with absent calcification.
Classic CT finding in PHPV?
Small globe (microphthalmia) with a retrolental soft tissue mass; no calcification (helpful to differentiate from retinoblastoma).
Classic MRI finding in PHPV?
Retrolental soft tissue mass, usually T1/T2 hypointense, sometimes with flow voids from persistent vessels; possible hyaloid stalk to optic nerve.
What is the ‘martini glass’ sign in PHPV?
On MRI, a funnel-shaped retrolental mass (the ‘glass’) with a central stalk to the optic nerve (the ‘stem’).
Does PHPV typically show calcification?
No, absence of calcification is a key feature distinguishing PHPV from retinoblastoma.
How does PHPV affect globe size?
Associated with microphthalmia and shallow anterior chamber.
What vascular finding may be present in PHPV?
Persistent hyaloid artery or tunica vasculosa lentis may be seen as enhancing tubular structures on MRI.
Anterior vs posterior PHPV—what’s the difference?
Anterior: cataract, retrolental membrane, shallow AC, glaucoma. Posterior: stalk to optic disc, retinal folds, dysplasia, tractional detachment.
Complications of PHPV?
Cataract, glaucoma, vitreous hemorrhage, retinal detachment, phthisis bulbi.
What is a common systemic association with PHPV?
Trisomy 13, Norrie disease, Warburg syndrome (rare).
What is the main differential diagnosis of PHPV in infants with leukocoria?
Retinoblastoma (most important), Coats disease, retinopathy of prematurity, congenital cataract, toxocariasis.
Key imaging feature differentiating retinoblastoma from PHPV?
Retinoblastoma usually has calcifications; PHPV does not.
How to differentiate Coats disease from PHPV?
Coats disease shows retinal telangiectasia, exudation, subretinal fluid; PHPV has retrolental stalk and microphthalmia.
How to differentiate retinopathy of prematurity from PHPV?
ROP is usually bilateral and occurs in premature infants with oxygen therapy; PHPV is usually unilateral and not related to prematurity.
Pathology of PHPV?
Fibrovascular tissue behind the lens composed of persistent hyaloid artery remnants and mesenchymal proliferation.
Histologic contents of PHPV retrolental mass?
Fibrovascular connective tissue with glial cells, hyaloid vessels, and collagen.
Which ocular abnormality is strongly associated with PHPV?
Congenital cataract.
What surgical treatment options exist for PHPV?
Lensectomy, vitrectomy to remove fibrovascular stalk, management of complications (e.g., glaucoma).
Prognosis of PHPV?
Generally poor visual prognosis, especially if posterior component present; anterior PHPV alone may allow some vision if treated early.
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Congenital, Coats disease66
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COLOBOMA80
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ORBITAL DERMOID AND EPIDERMOID70
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PHPV65
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ORBITAL NF170
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IDIOPATHIC ORBITAL INFLAMMATION, PSEUDO TUMOR70
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ORBITAL CELLULITIS70
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Sub-periosteal abscess65
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OPTIC NEURITIS70
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Thyroid-Associated Orbitopathy70
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LACRIMAL GLAND MIXED TUMOR70
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BENIGN ORBITAL TUMOR GENERAL16
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OPTIC PATHWAY GLIOMA71
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OPTIC NERVE SHEET MENINGIOMA81
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CONGENITAL INFANTILE HAEMANGIOMA63
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RB55
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OCUALR MELANOMA77
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Orbital_Lymphoproliferative_Lesions70
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LACRIMAL GLAND CARCINOMA70
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Orbital Langerhans Cell Histiocytosis52
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ORBITAL CAVERNOUS VENOUS MALFORMATION70
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LYMPHATIC MALFORMATION70
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Intra-ocular calcification CT78
