Visual status of patient with syndrome of Moebius

Abstract

PurposeTo analyze functional state of visual status in a patient with Moebius syndrome during 16 years of follow‐up.MethodsRetrospective analysis of clinical course of Moebius syndrome in a patient in the age 0.5 ‐ 16 years.ResultsParents appealed to the Institute because of unusual state of the child (lack of facial expressions and eye mobility). After the ophthalmologic and neurological examination, a diagnosis was made: Moebius syndrome. Underdevelopment of VII, IX, XII pairs of cranial nerves. Myopic astigmatism. At 10 years old facial asymmetry, exotropy with vertical component, positive traction test on both sides and lagophthalmus 3 mm at downgaze were noted. Best corrected visual acuity of both eyes is 0.2. Perimetry both eyes: concentric narrowing up to 40º from temporal and nasal sides, up to 20º from above and from below. Optic discs are pale, there are pigmentless sections of retinal dystrophy on the periphery and near the optic discs. Pleoptoortoptic treatment was carried out. As a result of regular courses of electrostimulation of extraocular muscles, by the age of 16, a tendency to insignificant motility of the eyes upwards appeared. Convergence was absent. Best corrected visual acuity decreased to 0.1 in both eyes. Significant help in diagnosis was provided by computer pupillography [Bushuyeva NN et al., 2003], which accurately records and measures pupil area. The initial size of the pupil area was: on the right eye 17.9 sq mm, and on the left eye 13.1 sq mm. After the flash, the pupil of the left eye narrowed to 3.7 sq mm and the right eye ‐ to 15.2 sq mm.ConclusionsPupillography allows evaluating vegetative nervous system status in Moebius syndrome patients. Electrostimulation of extraocular muscles allows slightly improving ocular motility of these patients. Obtained data needs further investigations on larger number of patients