Persistent hyperplastic primary vitreous: diagnosis, treatment and results

Area of Science:

• Ophthalmology
• Pediatric Ophthalmology
• Surgical Innovation

Background:

• Persistent hyperplastic primary vitreous (PHPV) is a congenital eye condition that can lead to severe vision impairment.
• Untreated PHPV can result in devastating complications such as glaucoma and phthisis bulbi.
• Early diagnosis and intervention are crucial for managing PHPV and preserving ocular health.

Observation:

• A significant percentage of patients with anterior PHPV achieved 20/200 or better vision (71.4%) with treatment.
• Successful visual outcomes (20/70 or better) were exclusively observed in eyes with purely anterior PHPV.
• Bilaterality of PHPV presented a very poor prognosis for vision, with affected patients having only light perception or hand motion vision.

Findings:

• Surgical intervention, including lensectomy and membrane removal, is vital for preventing secondary glaucoma and phthisis.
• The extent of the PHPV membrane is the most critical prognostic factor for visual outcomes.
• While saving the eye is achievable, obtaining useful vision is highly dependent on the anterior-only presentation of PHPV.

Implications:

• Families of infants with anterior PHPV can be counseled about the good prognosis for useful vision with surgical and amblyopia therapies.
• For posterior or combined anterior-posterior PHPV, the focus of treatment should be on preventing eye loss, with visual recovery being less likely.
• Further research into managing complex PHPV cases and improving binocular vision outcomes is warranted.