Persistent hyperplastic primary vitreous

Persistent Hyperplastic Primary Vitreous (PHPV), also known as Persistent Fetal Vasculature (PFV), is a rare congenital anomaly of the eye that results from a failure of the primary vitreous and the hyaloid vascular system to regress during prenatal development. This condition can lead to various ocular abnormalities and visual impairment.

Etiology

The exact cause of PHPV is unknown, but it is believed to be due to a disruption in the normal regression of the hyaloid artery and primary vitreous during the prenatal period. This can be due to genetic factors, maternal infections, or exposure to certain drugs during pregnancy.

Clinical Features

PHPV can present with a variety of clinical features, depending on the severity and extent of the condition. These can include microphthalmia (small eye), leukocoria (white pupillary reflex), cataract, glaucoma, and retinal detachment. In severe cases, PHPV can lead to blindness.

Diagnosis

The diagnosis of PHPV is primarily based on clinical examination and imaging studies. Ultrasound and magnetic resonance imaging (MRI) can be used to visualize the persistent hyaloid artery and other ocular abnormalities.

Treatment

The treatment of PHPV is primarily surgical, aimed at removing the abnormal tissue and correcting any associated ocular abnormalities. This can include cataract extraction, vitrectomy, and retinal detachment repair. However, the prognosis for vision is often poor, especially in severe cases.

Epidemiology

PHPV is a rare condition, with an estimated incidence of 1 in 10,000 to 25,000 live births. It can occur in one or both eyes, and there is no known gender or racial predilection.

See Also

• Congenital anomalies of the eye
• Pediatric ophthalmology
• Vitreoretinal surgery