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Strabismus surgery in children with Möbius syndrome.

Möbius syndrome is a congenital disorder of facial diplegia associated with lateral gaze paralysis. Although palsy of the sixth and seventh cranial nerves is the minimum diagnostic finding for Möbius syndrome, neuropathologic evidence indicates that this is a more complex syndrome.(1) Clinically, it is characterized by a total absence of facial expression and severe esotropia. Other anomalies may be associated with this syndrome, especially other cranial nerve palsies and Poland syndrome. The etiology of this syndrome has not been clearly established. Brain stem necrosis resulting from a vascular deficiency has been offered as a possible pathogenetic explanation.(2) The strabismus in Möbius syndrome is congenital esotropia with bilateral limitation in abduction. Even though many reports have described the various features of Möbius syndrome, only a few articles have reported the results of strabismus surgery in children, including bimedial rectus muscle recession. (3-5) Some authors report that bilateral medial rectus muscle recession alone has been disappointing; therefore, a combination of a medial rectus muscle recession and a lateral rectus muscle resection was recommended for satisfactory results. (5-7) In more severe cases, muscle transposition was needed to ensure straight position of the eyes in primary gaze. (8-9)[1]

References

  1. Strabismus surgery in children with Möbius syndrome. Spierer, A., Barak, A. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus. (2000) [Pubmed]
 
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