Persistent hyperplastic primary vitreous (PHPV) is a congenital eye disease of children, because its clinical manifestations are nonspecific, it is easy to be misdiagnosed as congenital cataract and the prognosis is poor.There has been more researches on PHPV clinical characteristics than its histopathological feature and immunochemistry.
This study was to investigate the clinical characteristics of PHPV and the pathological characteristics of retrolental membranes secondary to PHPV.
The clinical data of 6 PHPV eyes from 6 patients during May 2005 to December 2014 were retrospectively analyzed.All the PHPV eyes received a combinating procedure of cataract extraction and anterior vitrectomy.The retrolental membranes were collected during surgery for the histopathological examination by hematoxylin & eosin staining and periodic acid-Schiff (PAS) staining.Immunochemistry was used to detect the expression of Vimentin, smooth muscle actin (SMA), S-100, glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE), epithelial membrane antigen (EMA), CD34 and proliferating cell nuclear antigen (PCNA) in the retrolental membranes.
Monocular PHPV was found in 6 cases since born, with the main clinical manifestation of similar to congenital cataract.The ocular axial was shorter in 5 PHPV eyes than that of the contralateral eye by A-sonography, and 1 eye accompanied by eye nystagmus, strabismus and small cornea.The retrolental membranes were seen during the surgery in all cases, and the white band through the vitreous in 2 eyes.Pathological examination showed that the retrolental membrane was mainly composed of loose or myxoid connective tissue fibers, the capillaries and small veins.PAS positive tissue, residual transparent artery and differentiated adipocytes were exhibited in some specimens.Immunohistochemistry indicated that spindle-shape cells in the retrolental membranes presented the characteristics of mesenchymal cells, and some cells appeared the myofibroblast characteristics with the low proliferation activity.The development of the lens and the ciliary body was abnormal because of the stretching of retrolental membranes into the lens capsule in 1 PHPV eye.The follow-up was performed for 6-9 years in 4 eyes, with the best corrected visual acuity (BCVA) for 0.06, light perception, 0.05 and 0.07 respectively.The intraocular pressure was normal in all the eyes.
PHPV is usually misdiagnosed as congenital cataract and shows a poor postoperative BCVA.PHPV occurs mainly due to an incomplete regression of the embryonic vitreous and hyaloid vasculature and eventual abnormality of lens.