Uveal involvement in children with hemifacial scleroderma

Background. Linear scleroderma of the head (LSH) is a devastating disease of childhood since it is associated with a deep deformation of the face and head tissues, neurological disorders, organ of vision injury, and prognosis-defining uveal tract involvement.

Aim. To establish the prevalence and features of the uveal tract lesion in children with LSH.

Materials and methods. Ten cases with uveal tract lesions were retrospectively reviewed in 110 children with LSH. A comprehensive clinical, laboratory, and instrumental examination was carried out, including magnetic resonance imaging of the brain, electroencephalography, and an ophthalmologist's examination, including viscometry, biomicroscopy, and ophthalmoscopy.

Results. In 9 patients, only the anterior segment of the eye was affected (iridocyclitis); 2 patients had the posterior segment involved, 1 had peripheral chorioretinitis, and 1 patient had a combination of iridocyclitis with central chorioretinitis. The lesion developed immediately after the LSH onset or 6–10 years after the onset of the disease, and half of the children had a recurrent course of the disease. In all children, changes in the uveal tract were detected on the side of the lesion of the scalp and face. In 3 out of 10 patients, iridocyclitis was bilateral. Simultaneously, epileptic syndrome and focal lesions of brain tissue were noted in 2 children with eye lesions. Persistent vision loss in the outcome of the disease was observed in 3 children.

Conclusion. Uveal tract involvement was detected in 0.9% of our group of examined children with LSH, mostly in the form of anterior iridocyclitis. The lesion of the uveal tract can develop both at the onset of scleroderma and after many years of illness, which makes it necessary to perform at least an annual routine examination of the eyes using basic ophthalmological techniques – visometry, biomicroscopy, and ophthalmoscopy. Also, an eye examination is necessary in case of disease recurrence.