[Ophthalmologic symptomatology in children with periventricular leucomalation].

Ophthalmologic symptoms were analyzed in 76 children with periventricular leucomalation (PL). Clinical or functional ophthalmic disorders were detected in 100% of patients. Changes in the optic nerve disc were diagnosed in 93.4% of patients. The syndrome of dilated excavation (SDE), i.e. dilation and cupping of the optic nerve disc combined in all cases with affection of postgenicular visual paths (of optic radiation and/or striatal cortex) verified by neuroradiology, was most frequently (80.3%) encountered in children with PL. Presumably, the progression of SDE is associated with transsynaptic retrograde degeneration conditioned by the hypoxic-ischemic affection of the CNS in the projection of postgenicular visual paths at pre- and perinatal stages. SDE is an important criterion in the diagnosis of lesions of postgenicular visual paths in babies.

[Bullous form of X-linked congenital retinoschisis in infants]. / Bulleznaia forma X-stseplennogo vrozhdennogo retinoshizisa u detei rannego vozrasta.

Clinical manifestations and course of bullous X-linked congenital retinoschisis were studied in 10 infants aged 6-18 months. Visual function was evaluated by registration of electroretinogram and visual evoked potentials. The patients were followed up for 7.65 +/- 2.51 years. Giant retinal cysts were observed in 40% patients with X-linked retinoschisis aged under 3 years. Collapse (spontaneous or after laser coagulation of the retina) of retinal cysts with formation of demarcation pigmented lines was observed in 85% children. Twenty percent patients developed hemophthalmia which was completely resorbed within 4-12 months. At the age of 6-12 years visual acuity was 0.36 +/- 0.23. The prognosis of the condition is favorable, and therefore surgical treatment or laser coagulation are not recommended in infants with bullous retinoschisis, on condition that no negative changes in the disease course are observed.