Möbius Syndrome

Abstract

Möbius syndrome is classically defined as combined congenital bilateral facial and abducens nerve palsies, although it may also be associated with a myriad of other craniofacial, musculoskeletal, cardiothoracic, endocrinologic, and developmental disorders. The problem that most patients complain about, however, is the inability to smile and close their lips while eating. Although the etiology of this syndrome is still unknown, scientific support has been growing for the hypothesis that it is due to an embryological disruption of subclavian artery development. The treatment of choice for facial reanimation in these patients is a neurovascular free muscle transfer, ideally using the gracilis muscle with direct repair of the gracilis muscle's motor nerve to the masseteric branch of the trigeminal nerve. If the masseteric nerve is unavailable, a partial hypoglossal or accessory nerve may be used. These operations, enhanced by the effects of cerebral plasticity, may allow Möbius patients to reach their goals of satisfactory spontaneous smiles.

Congenital facial palsy and emotion processing: The case of Moebius syndrome

“…Graefe, 19 is a rare congenital disorder which presents altered development of facial (VII) and abducens (VI) CNs, resulting in "unior bi-lateral, nonprogressive facial weakness and limited abduction of the eye(s)". [20][21][22][23][24][25][26][27][28][29] It can also be associated with other CN palsies (affecting XII, X, IX, III, VIII, V, IV and XI CN in order of decreasing frequency), [30][31][32] orofacial malformations (epicanthic folds, micrognathia), limb defects (such as club feet and missing or underdeveloped fingers or hands), musculoskeletal abnormalities and hypoglossia (weakness or malformation of the tongue). 27,[32][33][34][35][36] Functional anomalies of face and mouth implicate lack of facial expression, difficulties in speaking, eating, sucking and swallowing.…”

“…27,[32][33][34][35][36] Functional anomalies of face and mouth implicate lack of facial expression, difficulties in speaking, eating, sucking and swallowing. 32 Some MBS individuals retain residual lower facial muscle activity, possibly because of aberrant innervation from other CNs. 36 A few radiologic and neuroimaging studies also showed brainstem and cerebellum abnormalities in Moebius subjects.…”

“…26,50 In support of this hypothesis, findings from radiological imaging, including ultrasonography, computed tomography and magnetic resonance imaging in individuals with MBS showed calcifications and hypoplasia of the brainstem. 32,53 However, given that not all MBS patients show signs of CN nuclei hypoplasia, 54 this seems a poor demonstration to endorse the vascular event.…”

Congenital facial nerve palsy: Single center study

“…All five children have also a palsy of the abducens nerve, a typical symptom of Moebius syndrome. In 60% of those Moebius cases, it is combined with Poland sequence which is defined as congenital absence of the pectoral head with ipsilateral hand deformity ( 22 ).…”

Powering the Gracilis for Facial Reanimation

“…2 In the modern era, an increasingly common option for dynamic lower facial reanimation in chronic facial paralysis is free gracilis muscle transfer. Since its initial description by Harii et al 3 in 1976 using the deep temporal nerve to power the transplanted muscle, various donor nerves have been proposed, including a cross-facial nerve graft (CFNG), 4 hypoglossal or spinal accessory nerves, 5 masseteric nerve (MN), 6 and most recently, using both the masseteric and a CFNG. 7 Recommendations from experienced centers regarding the optimal donor nerve have been mixed.…”