scholarly journals A congenital cranial dysinnervation disorder: Möbius’ syndrome

2017 ◽  
Vol 52 (3) ◽  
pp. 165-168 ◽  
Author(s):  
Hatice Mutlu Albayrak ◽  
Nuriye Tarakci ◽  
Hıseyin Altunhan ◽  
Rahmi Ors ◽  
Huseyin Caksen
2018 ◽  
Vol 9 (1) ◽  
pp. 102-107 ◽  
Author(s):  
Fady Sedarous ◽  
Toby Y.B. Chan ◽  
Inas Makar

Congenital cranial dysinnervation disorders, also known as CCDDs, are characterized by aberrant innervation to extraocular and facial muscles resulting in unusual forms of incomitant strabismus. Anomalous innervation to extraocular muscles can result in a wide variety of phenotypes causing various clinical conditions such as Duane syndrome, congenital fibrosis of the extraocular muscles, and Möbius syndrome. We report a case of bilateral dysinnervation disorder causing atypical ocular movements in both eyes as the patient changes fixation from one eye to the other and from right gaze to left gaze that fits with the wider diagnosis of CCDDs.


Neurosurgery ◽  
2010 ◽  
Vol 67 (3) ◽  
pp. 663-674 ◽  
Author(s):  
Kartik G. Krishnan ◽  
Gabriele Schackert ◽  
Volker Seifert

Abstract BACKGROUND The functions of the human face are not only of esthetic significance but also extend into metaphoric nuances of psychology. The loss of function of one or both facial nerves has a remarkable impact on patients' lives. OBJECTIVE To retrospectively analyze the functional outcomes of microneurovascular facial reanimation using masseteric innervation. METHODS Seventeen patients with irreparable facial paralysis resulting from benign lesions involving the facial nuclei (n = 14) or Möbius syndrome (n = 3) were treated with free muscle flaps for oral commissural reanimation using ipsilateral masseteric innervation and using temporalis muscle transfer for eyelid reanimation. Results were analyzed by the absolute commissural excursion and commissural excursion index and by a patient self-evaluation score. Presence of synkinesis was documented. Follow-up ranged from 8 to 48 months (mean, 26.4 months). RESULTS Normalization of the commissural excursion index was observed in 8 of 17 patients (47%), an improvement was seen in 7 of 17 (41%), and failure was observed in 2 of 17 (12%). The individual dynamics of absolute commissural excursion and commissural excursion index changes are presented. A natural smiling response was observed in 10 of 17 patients (59%) but not in the remaining 7 (41%). This response reflected the patient's ability to relay the natural emotion of smiling through the masseteric nerve. Patients' self-evaluation scores were a level higher than objective indices. CONCLUSIONS Innervation of free muscle flaps with the masseteric nerve for oral commissure reanimation might play an important role in patients with lesions of the facial nuclei (as in Möbius syndrome). Synkinesis persists for long periods after surgery. However, most of the patients learned to express their emotions by overcoming this phenomenon. Despite hypercorrection or inadequate correction, patients evaluated themselves favorably.


2017 ◽  
Vol 15 (3) ◽  
Author(s):  
Dr Anuradha Raj ◽  
Prof. R. C. Nagpal ◽  
Prof. Renu Dhasmana ◽  
Dr. Amit Maitreya

We describe the management of the congenital entropion with tarsal kink in a case of mobius syndrome. Mobius syndrome is extremely rare  congenital neurological disorder results from the underdevelopment of the sixth and seventh cranial nerves which causes  facial paralysis and inability to move the eyes from side to side.  A congenital malformation of the tarsus known as tarsal kink was the associated eyelid anomaly which caused upperlid entropion. A  two year old  girl presented with  both  eyelids entropion of left eye which was corrected by upper lid tarsotomy, marginal rotation and levator  suturing with tarsal plate and lower lid retractors were strengthened by their advancement to the tarsal plate.


Author(s):  
Jana Tabachová

The article focuses on the problematic of Möbius syndrome, a specific description of a child with this syndrome. This is a very rare syndrome. At the time of diagnosing this girl‘s syndrome, doctors in the Czech Republic had almost no experience and had no seen any other child with Möbius syndrome. So she got the intensive care specific for this syndrome just before her third birthday. The aim of our study was to obtain data in motor development, cognitive skills and abilities and especially to obtain information about speech development. In this article, we try to capture the main therapeutic approaches that led to the development of communication skills and promote the development of all language levels. 


2001 ◽  
Vol 15 (6) ◽  
pp. 47
Author(s):  
J Gordon Millichap
Keyword(s):  

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