title: Facial animation in children with Möbius syndrome after segmental gracilis muscle transplant.
abstract: Möbius syndrome is a complex congenital anomaly involving multiple cranial nerves, including the abducens (VI) and facial (II) nerves, and often associated with limb anomalies. Muscle transplantation has been used to address the lack of facial animation, lack of lower lip support, and speech difficulties these patients experience. The purpose of this study was to investigate the results of bilateral, segmental gracilis muscle transplantation to the face using the facial vessels for revascularization and the motor nerve to the masseter for reinnervation. The outcome of the two-stage procedure was assessed in 10 consecutive children with Möbius syndrome by direct interview, speech assessment, and oral commissure movement. Preoperative data were collected from direct questioning, viewing of preoperative videotapes, notes from prior medical evaluations, and rehabilitation medicine and speech pathology assessments. All of the patients developed reinnervation and muscle movement. The children who described self-esteem to be an issue preoperatively reported a significant posttransplant improvement. The muscle transplants produced a smile with an average commissure excursion of 1.37 cm. The frequency and severity of drooling and drinking difficulties decreased postoperatively in the seven symptomatic children. Speech difficulties improved in all children. Specifically, of the six children with bilabial incompetence, three received complete correction and three had significant improvement. Despite the length and complexity of these procedures, complications were minimal. Muscle transplantation had positive effects in all problematic areas, with a high degree of patient satisfaction and improvement in drooling, drinking, speech, and facial animation. The surgical technique is described in detail and the advantages over regional muscle transfers are outlined. Segmental gracilis muscle transplantation innervated by the motor nerve to the masseter is an effective method of treating patients with Möbius syndrome.
authors.affiliation: The Hospital for Sick Children, and the Department of Surgery, University of Toronto, Ontario, Canada. ronald.zuker@sickkids.on.ca
authors.forename: R M
authors.initials: RM
authors.lastname: Zuker
authors.forename: C S
authors.initials: CS
authors.lastname: Goldberg
authors.forename: R T
authors.initials: RT
authors.lastname: Manktelow
mesh_terms: D000293:Adolescent; D001184:Articulation Disorders / Q000209:etiology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005149:Facial Expression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007805:Language Development Disorders / Q000209:etiology / Q000601:surgery; D008297:Male; D008866:Microsurgery / Q000379:methods*; D020331:Mobius Syndrome / Q000175:diagnosis / Q000601:surgery*; D018482:Muscle, Skeletal / Q000098:blood supply / Q000294:innervation / Q000637:transplantation*; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D013536:Suture Techniques; D016896:Treatment Outcome