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[Persistence of the primordial vitreous body and buphthalmos]
P Cernea1, C Simionescu, I Bosun
1Clinique Ophtalmologique de Craiova.
Insights
Persistent hyperplastic primary vitreous (PHPV) is a congenital ocular anomaly. This case report details a 3.5-year-old infant with PHPV, bilateral lens dislocation, and other severe ocular malformations.
Area of Science:
- Ophthalmology
- Developmental Biology
- Pediatric Medicine
Background:
- Persistent hyperplastic primary vitreous (PHPV) results from abnormal regression of embryonic hyaloid vasculature.
- This condition can lead to significant visual impairment and structural ocular abnormalities.
Observation:
- A 3.5-year-old infant presented with PHPV, characterized by lens dislocation into the anterior chamber (left eye) and vitreous (right eye).
- The right eye exhibited secondary buphthalmos, microphthalmos, a vascularized whitish mass with hyaloid artery remnants, and vitreous hemorrhage.
- Microscopic examination revealed glial hyperplasia near the vitreous and a fibrovascular membrane with degenerative changes.
Findings:
- The study confirms PHPV in the left eye and bilateral crystalline lens dislocation.
- Multiple ocular malformations were identified, including secondary buphthalmos, microphthalmos, and vitreous abnormalities.
- Histopathology demonstrated fibrovascular membranes and glial hyperplasia, consistent with developmental anomalies.
Implications:
- This case highlights the complex spectrum of ocular malformations associated with PHPV.
- Early diagnosis and management are crucial for potentially preserving vision in affected infants.
- Understanding the embryological basis of PHPV aids in diagnosing and managing these rare pediatric eye conditions.
Abstract:
Persistence of the hyperplasic primordial vitreous body is determined by a deletion of embryonal development of the vitreous body and of the hyaloid vascular system. Infant aged 3.5 years presents persistence of primordial vitreous body with crystalline dislocation in the camera aquosa and secondary buphthalmos of the left eye and microphthalmos with dislocation of the crystalline in the vitreous body of the right eye. At the back of the right eye we noticed a whitish mass, richly vascularized with vestiges from the hyaloid artery, but the posterior half of the vitreous cavity is filled with microscopic blood; the fibrovascular membrane is made of conjunctive tissue set in parallel layers and vessels with macrolipophagic degeneration. Microscopic investigation of retina reveals glial hyperplasia zones in the neighbourhood of the vitreous body. In the present paper the authors show the persistence of the primordial vitreous body in the left eye and bilateral dislocation of the crystalline, revealing multiple ocular malformations.