Surgical techniques for smile restoration in patients with Mobius syndrome

Abstract

Möbius syndrome is a congenital condition, the etiology when is not associated with misoprostol is not well defined. Signs and symptoms include difficulty swallowing, speech problems, drooling, strabismus, limitation of eye movement and more importantly, the facial blankness that these individuals have, result of the facial paralysis, due to atrophy of the cranial nerves that are involved in this condition. The ability to express emotions is affected and are considered “children without a smile.” There is currently no treatment to solvent the birth defects, the treatment options for reduce these alterations is the surgical option that has as main objective to restore muscle function through various techniques, used as required, the possibilities of applying them, is taking into consideration the outcome of the procedure to execute. Among the surgical techniques used mainly: the lengthening myoplasty of the temporal muscle,muscle transfers, cross-facial grafting, neurorrhaphy and nerve transposition, of which latter are the best performers, giving the patient a more natural, in as far as regards expression and function. Key words:Möbius syndrome, surgery, smile, facial nerve, muscle transfer, transfer nerve, temporalis muscle.

Sindrome De Moebius Em Paciente Com Fissura Labiopalatina: Relato De Caso

“…13 O paciente descrito nesse caso apresenta sinais característicos da entidade em discussão: ausência de mímica facial, déficit motor ocular, hipertelorismo, hipoacusia bilateral, pé direito torto, ausência de selamento labial, sialorreia, micrognatia, retrognatia, fissura palatina e miscrostomia, que corroboram com a literatura revisada. 1,2,3,5,8,11,12 Embora presente na minoria dos casos, o paciente descrito apresenta retardo no desenvolvimento cognitivo e psicomotor. 5,11 Para Gondipalli e Tobias (2006) a micrognatia pode estar relacionada à deficiência neuromuscular que limita os movimentos da mandíbula e reduz o estímulo para o seu crescimento.…”

“…1,2,3,5,7,8,9,12 Autores sugerem que a isquemia consequente pode causar agenesia, atrofia, hipoplasia ou displasia dos núcleos de nervos cranianos envolvidos na SM.…”

Facial Nerve Paralysis in Children

“…A congenital facial nerve paralysis, although other cranial nerves such as the Ⅲ, Ⅳ, Ⅴ, Ⅷ can be involved, is presented within the Möbius syndrome. The reported prevalence of this syndrome is about 1/150000 live births [9][10][11][12] . It is reported to be due to hypoplasia of the motor nuclei of the cranial nerves within the brainstem, probably due to a hypoxic-ischemic encephalopathy [10] .…”

“…The reported prevalence of this syndrome is about 1/150000 live births [9][10][11][12] . It is reported to be due to hypoplasia of the motor nuclei of the cranial nerves within the brainstem, probably due to a hypoxic-ischemic encephalopathy [10] . Those affected by Goldenhar syndrome (hemifacial microsomia, with a spectrum of congenital malformations involving the structures derived from the first and second branchial arch) can also present a congenital facial paralysis [11] .…”

“…Genetic causes of facial nerve paralysis includes hereditary myopathies, such as myotonic dystrophy and myasthenia. Also two loci responsible for isolated hereditary forms of facial paralysis (chromosome 3q21-22 and 10q21.3-22.1) have been identified [9,10,13] . Acquired facial paralysis can frequently be due to viral infections.…”