Overview
Persistent hyperplastic primary vitreous (PHPV) is a developmental anomaly characterized by the persistence of the tunica vasculosa lentis (lens stalk) into adulthood, leading to various ocular complications including pupillary obstruction and abnormal vascular anastomoses 123.Diagnosis
Clinical Features: Dense pupillary opacity, persistence of vascular structures, and potential anterior/posterior segment abnormalities 123.
Imaging: Ultrasonography and OCT may help visualize persistent vascular structures and lens abnormalities 3.
Differentiation: Challenging to distinguish from traumatic ocular injury in adults; careful history and imaging crucial 2.Management
Surgical Intervention: Pars plana vitrectomy is effective for clearing pupillary axis obstruction 12.
Technique: Closed-eye vitrectomy through a limbal approach can achieve a clear pupillary space 1.
Caution in Therapy: Surgical and amblyopia therapy may not be beneficial due to potential abnormal development of the macula and optic nerve 3.Special Populations
Adults: PHPV in adults is exceedingly rare; diagnosis requires meticulous differentiation from trauma 2.
Pediatrics: Abnormal iris vessels can indicate PHPV even with an opaque lens; careful monitoring for developmental issues 3.Key Recommendations
Consider pars plana vitrectomy for persistent pupillary obstruction in PHPV (Evidence: Moderate 12).
Exercise caution with surgical interventions due to potential underlying developmental abnormalities (Evidence: Weak 3).
Utilize imaging techniques to aid in diagnosis and differentiate from traumatic conditions (Evidence: Moderate 123).References
1 Stark WJ. Surgical management of persistent hyperplastic primary vitreous. Developments in ophthalmology 1981. link
2 Mason GI, Huamonte FU. PHPV in an adult managed by vitrectomy. Ophthalmic surgery 1979. link
3 Meisels HI, Goldberg MF. Vascular anastomoses between the iris and persistent hyperplastic primary vitreous. American journal of ophthalmology 1979. link90463-x)