Persistent hyperplastic primary vitreous

Persistent hyperplastic primary vitreous (PHPV) is also referred to as persistent fetal vasculature (PFV) or persistent fetal vasculature syndrome (PFVS), OMIM 611308 (autosomal dominant PHPV) or OMIM 611311 (autosomal recessive PHPV).

It has previously been called persistent tunica vasculosa lentis, persistent posterior fetal fibrovascular sheath of the lens, congenital retinal septum, and ablatio falciformis congenita.


PHPV is a congenital anomaly of the eye that results following failure of embryological primary vitreous and hyaloid vasculature to regress. It is characterized by persistence of various portions of the primary vitreous (embryonic hyaloid vascular system) with hyperplasia of the associated embryonic connective tissue, and associated with microphthalmia, cataract and glaucoma.

PHPV was described by A. B. Reese in 1949. It belongs to the group of vitreoretinopathies.

PHPV is usually divided into three types:

  • Anterior PHPV: Anterior PHPV occurs when the remnant vascular stalk is seen attached to the back of the lens but no longer extends back to the optic nerve. The purely anterior PHPV is also known as persistent tunica vasculosa lentis and persistent posterior fetal fibrovascular sheath of the lens. This form is typically associated with cataract, glaucoma and retrolenticular membrane.
  • Posterior PHPV: In posterior PHPV the remnant vascular stalk is seen arising off the optic nerve but not reaching the lens, thus usually not causing cataract. Posterior PHPV may be associated with abnormal development of the retina, optic nerve and macula, vitreal stalk, vitreal membranes. The purely posterior presentation of PHPV is termed falciform retinal septum and ablatio falcicormis congentia.
  • A combination of anterior and posterior PHPV: A combination of anterior and posterior PHPV is the most commonly seen clinical presentation.