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[Four cases of persistent hyperplastic primary vitreous]
1Department of Ophthalmology, Nagoya City University Medical School, Aichi-ken, Japan.
Nippon Ganka Gakkai Zasshi
|November 22, 1997
Summary
Persistent hyperplastic primary vitreous (PHPV) can present with diverse ocular findings and is often associated with other systemic and ocular anomalies. Comprehensive examination is crucial for patients diagnosed with PHPV.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Medical Genetics
Background:
- Persistent hyperplastic primary vitreous (PHPV) is a congenital developmental anomaly of the eye.
- It results from the failure of the primary vitreous to regress during fetal development.
- PHPV can lead to significant visual impairment if not diagnosed and managed promptly.
Observation:
- This study reviewed four cases of PHPV diagnosed at Nagoya City University Hospital.
- Cases presented with unilateral or bilateral involvement, affecting infants aged 3-8 months.
- Clinical observations included leukocoria, corneal opacity, retinal folds, and ciliary process elongation.
Findings:
- Magnetic resonance imaging (MRI) revealed total retinal detachment in some cases.
- Associated ocular anomalies included microphthalmia, posterior embryotoxon, sclerocornea, and iris hypoplasia.
- Systemic anomalies observed were arachnoidal cyst, syndactyly, microcephalus, heart defects, pulmonary atresia, and asplenia.
Implications:
- The findings highlight the significant association between PHPV and other ocular and systemic abnormalities.
- Early and thorough examination is essential for identifying associated conditions, particularly those related to neural crest cell disorders.
- Prompt diagnosis and management can potentially improve visual outcomes and address co-occurring systemic issues.