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Iris Fixation via External Pentagram Suturing

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Bilateral primary hyperplastic persistent vitreous: report of two cases.

Neha Singh¹, Siddharth Agrawal¹, Pallavi Mishra¹

¹Department of Ophthalmology, King George Medical University, Lucknow, Uttar Pradesh, India.

GMS Ophthalmology Cases

|November 20, 2020

Summary

Bilateral primary hyperplastic persistent vitreous (PHPV) is rare, presenting with leukocoria. Diagnosis requires differentiating it from retinoblastoma using specific clinical and imaging methods.

Keywords:

B-scan PHPV Peters’ anomaly leucocoria

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Area of Science:

Ophthalmology
Pediatric Ophthalmology
Medical Genetics

Background:

Primary hyperplastic persistent vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital eye condition.
It typically manifests unilaterally with key signs including leukocoria, microphthalmos, and cataract.

Observation:

This report details two rare cases of bilateral PHPV.
One patient presented with concurrent Peters' anomaly, highlighting a complex clinical presentation.

Findings:

Bilateral PHPV closely mimics retinoblastoma, necessitating careful differential diagnosis.
Diagnostic tools such as examination under anesthesia, ultrasound B-scan, and aqueous lactate dehydrogenase (LDH) levels were crucial.

Implications:

Accurate diagnosis of bilateral PHPV is essential to avoid misidentification as retinoblastoma.
Understanding these diagnostic methods aids clinicians in managing this rare condition and distinguishing it from sight- and life-threatening diseases.