Persistent Hyperplastic Primary Vitreous : Case Report

Introduction : 

PHPV is a pathologic entity resulting from abnormal persistence of the fetal fibrovascular primitive stroma i.e hyaloid system of the eye, which should disappear by the time of birth. The primary vitreous forms around the seventh week of life and begins involuting by 20 weeks. Persistence and hypertrophy of these vessels can result in PHPV in the anterior and/or posterior chambers. It can give rise to leukocoria, retinal detachment and subretinal hemorrhage.  Submitted by Dr Anand Shrikant Gajakos,  Consultant Radiologist , Solapur                                                                            

Case Report :

A male child of 10 years, non-consanguineous parents, presented with  right sided small  eye since last 8 years. Neurological examination showed leukocoria and absent  vision of the right eye.                                                                                                                                                      B Scan examination of the bilateral eye was done. Left eye was normal clinically and on Ultrasound imaging..

Observations:

Anterior chamber of the right eye was shallow due to anterior displacement of the lens.  An echogenic  tubular structure extending from the retrolental area upto the  optic disc representing a persistent hyaloid canal containing hyaloid  artery  was noted.  Complete retinal detachment.  Doppler flow examination of the hyaloid artery shows a continous low resistance biphasic forward flow.

The above mentioned findings in the right eyeball were consistent with persistent hyperplastic primary vitreous .

References :

1. Reese AB. Persistent hyperplastic primary vitreous. Am J Ophthalmol 1955; 40: 317-31.

2. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous {PHPV}. Am J Ophthalmol 1997;124:587-626.

3. Castillo M, Wallace DK, Mukherji SK. Persistent hyperplastic primary vitreous involving the anterior eye. Am J Neuroradiol 1997;18:1526-8.

4. Kaste SC, Jenkins JJ 3rd, Meyer D, et al. Persistent hyperplastic primary vitreous of the eye: imaging findings with pathologic correlation. Am J Roentgenol 1994;162: 437-440.

5. Barkovich AJ. Pediatric neuroimaging. Philadelphia: Lippincott-Raven, 1996, pp 412-6.

6.. Glasier CM, Broodsky MC, Leihser Jr RE, et al. High resolution ultrasound with Doppler: a diagnostic adjunct in orbital and ocular lesions in children. Pediatr. Radiol 1992; 22:174-8.