What is Persistent Fetal Vasculature (PFV)?
Persistent Fetal Vasculature (PFV), formerly known as persistent hyperplastic primary vitreous (PHPV), is a congenital eye disorder resulting from the failure of the fetal intraocular vasculature to regress properly. This condition can lead to various ocular abnormalities and can significantly impact a child's vision.
How Common is PFV?
PFV is a rare condition. Its exact prevalence is not well-documented, but it is considered an uncommon cause of congenital blindness. Both unilateral and bilateral forms exist, with the unilateral form being more frequent.
What Causes PFV?
The exact
etiology of PFV is not fully understood. However, it is known to be a developmental anomaly where the normal regression of the embryonic hyaloid vascular system does not occur. This failure can lead to a variety of abnormalities within the eye, including the presence of a fibrovascular mass behind the lens.
What are the Symptoms and Clinical Features?
Clinical features of PFV can vary widely but often include a white pupillary reflex (leukocoria), microphthalmia (small eye),
cataracts, and elongated ciliary processes. In some cases, secondary glaucoma or retinal detachment may also occur. Visual impairment ranges from mild to severe, depending on the extent of the malformation.
How is PFV Diagnosed?
Diagnosis of PFV typically involves a thorough ophthalmologic examination. Imaging studies like
ultrasound and
MRI can also be used to assess the internal structures of the eye. Early diagnosis is crucial for managing the condition effectively and preventing further complications.
What are the Treatment Options?
Treatment for PFV depends on the severity and specific features of the condition. Surgical intervention is often required, especially if there is a significant structural abnormality like a cataract or retinal detachment. Procedures may include
lensectomy or vitrectomy to remove the abnormal tissue. In some cases, additional treatments such as laser therapy or cryotherapy may be employed.
What is the Prognosis?
The prognosis for children with PFV varies. Early intervention can improve outcomes, but the degree of visual recovery depends largely on the initial severity of the condition and the success of the treatment. Regular follow-ups with a pediatric ophthalmologist are essential to monitor the child's vision and manage any ongoing issues.
What is the Role of Genetic Counseling?
Although PFV is generally considered sporadic, there may be a genetic component in some cases. Genetic counseling can help families understand the condition, its potential implications, and any risks for future offspring. This information can be particularly valuable for planning and early intervention.
How Can Parents and Caregivers Support Children with PFV?
Support from parents and caregivers is crucial for managing PFV. Ensuring adherence to treatment plans and attending regular follow-up appointments are key components of care. Additionally, providing emotional and educational support can help children cope with any visual impairments and lead fulfilling lives.
