Abstract
Abstract: :
Purpose: The Moebius–syndrome is characterized by an inborn disturbance of the horizontal eye–movements in combination with variable paresis of the facial nerve. In most cases facial paresis is symmetrical on both sides leading to the pathognomonical puppet–like face and to open eyes even in crying babies. Due to the aplasia of the abducens nucleus there is abducens paralysis additional to horizontal gaze paresis. Since vertical eye–movements are intact, this syndrome leads to the clinical picture of bilateral abducent nerve paralysis with crossed–eye convergence. Methods: Five patients with Moebius–syndrome have been presented to our clinic. All Patients showed not only a variable degree of Moebius–syndrome but also different combinations of associated malformations. In an infant aged one month there was additional bilateral ptosis, additional Poland–syndrome, severe hearing loss and a cranio–facial asymmetry with retrognatia. Another infant aged 10 months, additionally presented a ptosis of the right eye which covered the pupil. It also showed the misdirection jaw–winking syndrome (Marcus–Gunn). One other patient suffered from a defect of the ventricle septum of the heart. Two siblings suffered from a combined Moebius–syndrome with dysmorphia syndrome, congenital muscular dystrophy and defects of the vertebral column. Results: Treatment of Moebius–syndrome included regular controls and a regular application of artificial tear drops to avoid keratopathy. Esotropia was corrected by recession of the medialis rectus muscles. Due to the lid closure deficiency in N.VII–paresis in our 10 months old patient, surgical treatment of the ptosis was not possible and patching was performed to avoid amblyopia. Conclusions: Moebius–syndrome is often associated with other syndromes as for example the Poland–syndrome. Two of the presented children showed a ptosis in one or both eyes. The combination of these symptoms has a significant influence on the further proceeding. In case of facial paralysis and simultaneous congenital ptosis the benefits of a surgical correction of the ptosis have to be estimated against the possible complications of lagophthalmia and secondary keratopathy.
Keywords: strabismus • strabismus • strabismus