Persistent fetal vasculature

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Persistent fetal vasculature
Synonyms	Persistent hyperplastic primary vitreous (PHPV)
Pronounce	N/A
Specialty	N/A
Symptoms	Leukocoria, strabismus, nystagmus, microphthalmia
Complications	Cataract, glaucoma, retinal detachment
Onset	Congenital
Duration	Lifelong
Types	N/A
Causes	Failure of the fetal hyaloid vascular system to regress
Risks	Premature birth, intrauterine growth restriction
Diagnosis	Ophthalmoscopy, ultrasound, MRI
Differential diagnosis	Retinoblastoma, Coats' disease, retinopathy of prematurity
Prevention	N/A
Treatment	Surgery, vitrectomy, cataract extraction
Medication	N/A
Prognosis	Variable, depends on severity and treatment
Frequency	Rare
Deaths	N/A

Persistent fetal vasculature (PFV), also known as persistent hyperplastic primary vitreous (PHPV), is a congenital developmental anomaly of the eye. It occurs when the fetal hyaloid vascular system, which normally regresses before birth, fails to involute. This condition can lead to various ocular abnormalities and visual impairment.

Pathophysiology

During normal fetal development, the hyaloid artery supplies blood to the developing lens and vitreous body. This artery typically regresses and disappears before birth. In PFV, this regression does not occur, leading to the persistence of the hyaloid artery and its associated structures. The persistent vasculature can cause traction on the retina, leading to retinal detachment, and can also result in cataract formation, microphthalmia, and other structural abnormalities.

Clinical Presentation

PFV can present in various forms, ranging from mild to severe. Common clinical features include:

• Leukocoria (white pupillary reflex)
• Strabismus (misalignment of the eyes)
• Nystagmus (involuntary eye movement)
• Microphthalmia (abnormally small eye)
• Cataract (clouding of the lens)
• Glaucoma (increased intraocular pressure)

Diagnosis

Diagnosis of PFV is primarily clinical, based on the characteristic appearance of the eye. Imaging studies such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) can be used to confirm the diagnosis and assess the extent of the anomaly. Ophthalmoscopy and fundus photography are also useful in visualizing the persistent vasculature and associated retinal changes.

Treatment

The treatment of PFV depends on the severity of the condition and the associated ocular abnormalities. Options include:

• Surgical intervention to remove the persistent vasculature and address associated complications such as cataracts and retinal detachment.
• Laser therapy to ablate the persistent vessels.
• Medical management for associated conditions like glaucoma.

Early intervention is crucial to prevent further visual impairment and to optimize visual outcomes.

Prognosis

The prognosis for PFV varies depending on the severity of the condition and the success of treatment. Early detection and appropriate management can improve visual outcomes, but severe cases may result in significant visual impairment or blindness.

See also

• Hyaloid artery
• Retinal detachment
• Cataract
• Microphthalmia
• Glaucoma
• Leukocoria
• Strabismus
• Nystagmus