The Sturge - Weber - Krabbe syndrome (part I). Ocular features. Glaucoma manifestation
Introduction. The Sturge - Weber - Krabbe syndrome (SWS) belongs to a group of disorders known as phakomatoses that are characterized by hamartomas of a brain, skin and eye. Glaucoma detected in 30-70% of the patients with SWS, which can manifest at the time of newborn and later - in childhood and even in adults.Aim. To analyze the features of ophthalmic changes and glaucoma manifestations of the patients with SWS.Materials and methods. Under supervision were 19 children from 2 months to 17 years (under the age of 1 year - there were 8 children) with SWS. A complex ophthalmological examination was performed.Results and discussion. SWS syndrome characterized by facial port-wine stain (PWS), involving the I and II branch of the trigeminal nerve, which in all children and teenagers were detected. Usually we diagnosed unilateral glaucoma in SWS - 13 children, bilateral - 6 patients. At the same time bilateral glaucoma was more often observed in infants.Congenital glaucoma was detected in 15 children at 18 eyes (47.4%). "Eearly oncet" (congenital) form - up to 43.8%, "late oncet" in childhood and adults - up to 55% which is indicate a bimodal type of glaucoma development in patients with SWS.About 65.8% of SWS patients show pathologic ocular changes, involving the eyelid, bulbar conjunctiva, sclera, episclera, anterior chamber, iris, ciliary body, choroid. Hemangiomas of the choroid we detected in 33% of cases.Conclusion. Considering bimodal type of glaucoma development all patients with SWS need follow-up supervision.