What are the treatment options for Persistent Hyperplastic Primary Vitreous (PHPV)?

Treatment of Persistent Hyperplastic Primary Vitreous (PHPV)

Early surgical intervention with lensectomy and anterior vitrectomy is the primary treatment for anterior PHPV with dense cataract, while posterior PHPV should generally be managed conservatively due to high surgical risks and poor visual outcomes. 1, 2, 3

Treatment Algorithm Based on PHPV Presentation

Anterior PHPV (Isolated)

Mild cataract:

• Conservative management with aggressive amblyopia treatment and frequent clinical monitoring is sufficient 1
• This approach avoids surgical risks in eyes where some visual potential remains 1

Dense cataract:

• Lensectomy with anterior vitrectomy should be performed as the minimal surgical treatment 3
• Early intervention is critical to prevent progressive pathologic changes including recurrent intraocular hemorrhage, secondary glaucoma, and eventual phthisis 4, 5
• Surgical timing should prioritize amblyopia treatment windows, as early surgery prevents orbital underdevelopment from prolonged visual deprivation 3
• Anterior placement of surgical incisions is recommended to avoid intraoperative retinal complications in microphthalmic eyes 5

Expected outcomes:

• Only eyes with purely anterior PHPV without posterior segment involvement achieve good visual results 3
• Best visual outcomes range from 20/60 in successful cases 2
• Reoperation rate is approximately 32% for complications including membrane reproliferation, glaucoma, vitreous hemorrhage, retinal detachment, or strabismus 2

Posterior PHPV (Isolated or Combined)

Surgical treatment should be avoided due to:

• High risk of retinal detachment 1
• Poor visual outcomes despite anatomically successful surgery, secondary to underlying retinal dysplasia and abnormalities 3
• Direct retinal insertion into pars plicata and thick vitreous gel creating significant intraoperative complications 5

Conservative management approach:

• Clinical observation with serial examinations 1
• Monitor for progressive complications requiring intervention 5
• Preoperative visual evoked potential (VEP) testing can help identify cases with no visual potential where surgery should be withheld 2

Combined Anterior and Posterior PHPV (Most Common Presentation)

• This represents 71% of PHPV cases 2
• Treatment decisions must weigh anterior segment salvageability against posterior segment limitations 2
• Lensectomy with anterior vitrectomy may be performed for the anterior component, but visual expectations must be tempered by posterior involvement 2
• Some eyes (17%) can maintain functional Snellen visual acuity despite posterior PHPV with retinal dysplasia, making selective surgical intervention reasonable 2

Critical Preoperative Considerations

Diagnostic workup:

• Slit lamp examination and echography (A-scan and B-scan) are essential for diagnosis and surgical planning 1
• CT or MRI can be useful in selected cases 1
• VEP testing aids in determining surgical candidacy, particularly when posterior involvement is present 2
• Unrecordable VEP indicates severe disease where surgery should be withheld 2

Surgical contraindications:

• Marked microphthalmos 1
• Unrecordable VEP 2
• Extensive posterior segment involvement with severe retinal dysplasia 2, 3

Common Pitfalls and How to Avoid Them

Most common cause of surgical failure:

• Inadequate assessment of posterior segment involvement leads to unrealistic visual expectations 3
• Posterior incision placement in microphthalmic eyes causes intraoperative retinal complications 5

Progressive deterioration without treatment:

• Untreated eyes show progressive deterioration in the majority of cases (8 of 9 eyes in one series) 5
• Without intervention, PHPV produces recurrent hemorrhage, glaucoma, and may require enucleation 4

Differential diagnosis awareness:

• PHPV is a documented source of leukocoria, but clinicians must exclude retinoblastoma, congenital cataract, Norrie's disease, retinopathy of prematurity, retinal detachment, and Coat's disease 4

Long-term Management

• Follow-up ranges from months to years, with mean postoperative follow-up of 4.7 years in surgical series 1
• Approximately one-third of patients require reoperation for complications 2
• Five of 30 surgically treated eyes achieved useful vision in one long-term series 3
• No enucleations were required in properly selected surgical candidates with appropriate follow-up 3