Is PHPV rare?

Though the exact prevalence remains unknown, PHPV is considered to be not a very rare disease. The condition is usually unilateral and isolated (without associated systemic findings) in normal full-term infants.

What causes PHPV?

Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a congenital anomaly of the eye that results from failure of the embryological, primary vitreous, and the hyaloid vasculature to regress.

How common is Pfv?

HOW COMMON IS PFV? Many people have mild signs of these embryonic vessels. For the most part these are not harmful and can be seen by an eye doctor, but do not cause symptoms or problems. More severe variants of PFV are quite rare.

Is PHPV hereditary?

Most cases of PHPV are sporadic, but it can be inherited as an autosomal dominant or recessive trait. Inherited PHPV also occurs in several breeds of dogs and cats. In a limited number of cases, Norrie disease and FZD4 genes are found to be mutated in unilateral and bilateral PHPV.

Does PHPV cause blindness?

Though the exact prevalence remains unknown, PHPV is not considered to be a very rare disease. A study on childhood blindness and visual loss in the United States showed that PHPV accounts for about 5% of all cases of blindness[4].

What is persistent hyaloid?

Persistent hyaloid artery represents a remnant of the hyaloid vascular system. This occasionally runs from the disc to the lens; more commonly an anterior remnant gives a Mittendorf dot on the posterior lens surface or a posterior remnant gives a Bergmeister’s papilla over the disc.

Does strabismus cause leukocoria?

[10] It is non-heritable and commonly causes unilateral involvement. Leukocoria results from a complete pupillary membrane, cataract, fibrovascular sheath attached to the posterior lens capsule, strabismus, and a rarely cloudy cornea due to glaucoma.

What is Pfv in ophthalmology?

Persistent Fetal Vasculature (PFV), previously known as Persistent Hyperplastic Primary Vitreous (PHPV), is a failure of the regression of a component of fetal vessels within the eye. It is sometimes described as a “benign mimic of retinoblastoma,” being the second most common cause of infantile leukocoria.

Does PHPV cause white pupillary reflex?

Leukocoria (also white pupillary reflex) is an abnormal white reflection from the retina of the eye. Leukocoria resembles eyeshine, but leukocoria can also occur in animals that lack eyeshine because their retina lacks a tapetum lucidum….Leukocoria.

Leucocoria
Specialty	Ophthalmology, pediatrics

When does the hyaloid artery regress?

It branches to form a network of capillaries, the tunica vasculosa lentis, which is prominent at about 9 weeks’ gestation [10, 11]. In healthy fetuses, gradual regression of the hyaloid artery starts at 18 weeks, and by 29 weeks, the hyaloid artery disappears and leaves Cloquet’s canal [12, 13].

What does the hyaloid canal do?

It is formed by an invagination of the hyaloid membrane, which encloses the vitreous body. In the fetus, the hyaloid canal contains a prolongation of the central artery of the retina, the hyaloid artery, which supplies blood to the developing lens.

What is persistent hyperplastic primary vitreous?

Persistent hyperplastic primary vitreous. Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a congenital anomaly of the eye that results from failure of the embryological, primary vitreous, and the hyaloid vasculature to regress.

What is the pathophysiology of primary primary vitreous (PHPV)?

Normally, the primary vitreous forms around the 7 th week of gestation and starts involuting around 20 th week and has nearly always disappeared by birth. Persistent fetal vasculature in PHPV can lead to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts.

What are the treatment options for ocular leukocoria?

The management of common ocular conditions causing leukocoria is as follows: Retinoblastoma: Chemoreduction followed by adjuvant consolidative treatment (laser photocoagulation, thermotherapy, cryotherapy, chemotherapy) has replaced external beam radiotherapy as the primary mode of treatment for intraocular retinoblastoma. [21]

Which conditions are included in the differential diagnoses of posterior vitreoretinopathy (PV)?

When the eye in consideration has total retinal detachment and retrolental fibrous tissues suggestive of posterior PFV syndrome, familial exudative vitreoretinopathy, incontinentia pigmenti, ocular toxocariasis, and retinopathy of prematurity should be especially considered.