Persistent hyperplastic primary vitreous syndrome and features of congenital cataract surgery and aphakia correction

Persistent hyperplastic primary vitreous (PHPV) is a rare, predominantly unilateral congenital eye pathology associated with delayed reverse development of the hyaloid artery and embryonic vascular membrane of the lens and often co-occurs with congenital cataract and microphthalmos.

AIM: To develop optimal differentiated tactics for surgical treatment and correction of aphakia in congenital cataract extraction in children with PHPV.

MATERIAL AND METHODS: Fifty-two children (54 eyes) aged 3–10 months to 1 year and 8 months with unilateral (50 eyes, 92.6%) and bilateral (4 eyes, 7.4%) congenital cataract with PHPV were studied. Grade I microphthalmos was noted in 15 eyes; grade II microphthalmos in 9 eyes; a fibrous cord coming from the optic nerve disk in 49 eyes; a persistent vascular bag of the lens in 12 eyes; posterior synechiae in 9 eyes; a retrolental membrane with vessels and elongated ciliary processes fixed to it, occupying 1/8 to 1/2 of the area of the posterior chamber of the eye in 16 eyes; and a dislocation of the lens in 2 eyes. The children were comprehensively examined by biomicroscopy, ophthalmoscopy, biometrics, tonometry, keratorefractometry, B-scanning, ultrasound biomicroscopy, and color Doppler mapping.

RESULTS: The clinical picture of eyes with congenital cataract in three groups of children, united by the severity of clinical manifestations of PHPV, was analyzed. Differentiated microsurgical tactics for the removal of congenital cataracts and indications for implantation of intraocular lenses are described.

CONCLUSION: The clinical picture of PHPV in children with congenital cataracts is characterized by pronounced polymorphism, indicating the need for a differentiated approach in determining the optimal timing of surgery, surgical tactics, and method of aphakia correction.