[Persistent and hyperplastic primary vitreous syndrome. Clinical and therapeutic aspects]

Persistent hyperplastic primary vitreous (PHPV) is a rare developmental malformation of the eye. This anomaly is usually unilateral and unassociated with other disease. PHPV may have clinical variations: an anterior and/or a posterior one. Diagnosis and treatment are exposed in our paper.

We study 38 eyes in 34 patients. All patients had an anterior form of PHPV. Nine of them associated a posterior form. Eighteen eyes underwent surgery, the mean postoperative follow-up is 4.7 years. Treatment is discussed, depending on the initial form of PHPV. We report the evolution of the surgical eyes and the untreated eyes.

Slit lamp examination and echographic findings (A-scan and B-scan) support the diagnosis. In some cases computed tomography or RMN can be useful. If the cataract is mild in the anterior form of PHPV, treatment of amblyopia and frequent clinical examination can be sufficient. If the cataract is dense, a lensectomy must to be performed. The surgical technique can be difficult. For the eyes with posterior PHPV, the surgical treatment is to be avoided, because of high risk of retinal detachment.

PHPV may have clinical variations, mostly with cataract as major sign. In purely anterior presentation, in absence of marked microphthalmos, lensectomy can be useful.