Moebius Syndrome

Moebius syndrome (MBS) is a rare congenital cranial nerve disorder characterized by unilateral, bilateral symmetrical, or asymmetrical facial (VII) and abducens (VI) nerve palsies. Genetics and rhombencephalon vascular disturbances from intrauterine environmental exposures have been attributed to its development. It can present with various orofacial abnormalities. Although the diagnosis is purely clinical, certain characteristic features are present in the brain's images. With no cure, it is essential to devise management on a personalized basis...

OBJECTIVES: This study will list the most common comorbidities of congenital facial nerve palsy and how to detect and treat them, with special attention for ENT-problems such as hearing loss. Congenital facial nerve palsy is a very rare entity but in UZ Brussels hospital there was a follow-up of 16 children in the last 30 years. METHODS: Literature review has been done, combined with thorough research of our own series of 16 children with congenital facial nerve palsy...

No abstract text is available yet for this article.

BACKGROUND: Möbius (Moebius) and Poland's syndromes are two rare congenital syndromes characterized by non-progressive bilateral (and often asymmetric) dysfunction of the 6th and 7th cranial nerves and hypoplasia of the pectoral muscles associated with chest wall and upper limb anomalies respectively. Manifest simultaneously as Poland-Möbius (Poland-Moebius) syndrome, debate continues as to whether this is a distinct nosological entity or represents phenotypic variation as part of a spectrum of disorders of rhomboencephalic development...

A seven-year-old boy with Moebius syndrome and bilateral hip dysplasia underwent left-sided adductor lengthening, bilateral proximal femur varus derotational osteotomies, and internal fixation with proximal femur blade plates, and left-sided Dega pelvic osteotomy. Postoperatively, he was immobilized in a Petrie cast. A month later, the child presented with bilateral proximal femur blade plate implant failure. Simultaneous bilateral proximal femur implant failure in a child, to our knowledge, has not yet been reported...

Moebius Syndrome is a rare multifactorial condition defined by congenital complete or partial VII and VIth cranial nerves palsy and other physical abnormalities. We present the case of a 3 months old infant with Moebius sequence and breathing and eating difficulties, managed by tracheostomy and laryngoplasty.

Moebius syndrome is characterized by congenital complete or partial paralysis of the facial nerve and is often associated with orofacial and limb malformations. It is a rare syndrome that affects the sixth and seventh cranial nerves. Facial paralysis results in abnormal abduction of one or both eyes and facial paralysis or weakness. Moebius syndrome is an uncommon condition and only a few hundred cases have been reported in the literature. A seven-year-old girl with Moebius syndrome is featured in this report...

Although acute respiratory infections or diseases such as asthma commonly cause respiratory distress in a pediatric patient, neuromuscular disorders must be considered as a possible etiology in patients with significant hypotonia, neurological deficits, and gross developmental delay. We present a case where a patient's lack of response to initial asthma exacerbation therapy led to a reconsideration of the original diagnosis and adaptation of the management plan. Our patient presented with a rare combination of two congenital disorders that cause hypotonia: Prader-Willi syndrome and Moebius syndrome...

BACKGROUND: Moebius syndrome is a rare congenital disorder characterized by non-progressive palsy of the abducens (VI) and facial (VII) cranial nerves. Its common features include dysfunctions associated with other cranial nerves, orofacial abnormalities, skeletal muscle hypotonia, and other systemic disorders of differing severities. There are several concerns in the perioperative management of patients with Moebius syndrome. CASE PRESENTATION: We present a report on the management of general anesthesia of a 14-year-old male patient with Moebius syndrome who was scheduled for mandibular cystectomy...

Associated congenital anomalies may be observed in cases with achondroplasia. The prevalence reported in the literature and the types of co-occurring congenital anomalies are variable between the reported studies. The aim of this study was to establish the prevalence and to describe the associated anomalies in cases with achondroplasia. This study included 25 cases ascertained from our registry of congenital anomalies including all terminations of pregnancy, stillbirths and live births between 1979 and 2007 in 387,067 consecutive births (the prevalence of achondroplasia was 6...

Influential theoretical models argue that an internal simulation mechanism (motor or sensorimotor simulation) supports the recognition of facial expressions. However, despite numerous converging sources of evidence, recent studies testing patients with congenital facial palsy (i.e. Moebius syndrome) seem to refute these theoretical models. However, these results do not consider the principles of neuroplasticity and degeneracy that could support the involvement of an alternative neural processing pathway in these patients...

Moebius syndrome (MBS) is a congenital neurologic disorder that causes cranio-facial abnormalities. It involves paralysis of the VI and VII cranial nerves and causes bilateral or unilateral facial paralysis, eye movement disorder, and deformation of the upper and lower limbs. The orofacial dysfunctions include microstomia, micrognathia, hypotonic mimetic and lip muscles, dental enamel hypoplasia, tongue deformity, open bite or deep overbite, maxillary hypoplasia, high arched palate, mandibular hyperplasia or features indicating mandibular hypoplasia...

Moebius syndrome is a rare congenital facial palsy that can generate serious emotional repercussions, mainly due to the inability to smile. Currently, no treatment is available; however, surgery can restore muscle function. This case report introduces a new technique for the treatment of Moebius syndrome with zygomatic muscle neurotization using nerve grafts and end-to-side neurorrhaphies, in a 3-year-old girl diagnosed with bilateral Moebius syndrome who was unable to smile on the left side. After 4 years, the patient presented with full smile restoration on the left side, with right and left independent movements and complete symmetry...

Rehabilitation after free gracilis muscle transfer (smile surgery, SS) is crucial for a functional recovery of the smiling skill, mitigating social and psychological problems resulting from facial paralysis. We compared two post-SS rehabilitation treatments: the traditional based on teeth clenching exercises and the FIT-SAT (facial imitation and synergistic activity treatment). FIT-SAT, based on observation/imitation therapy and on hand-mouth motor synergies would facilitate neuronal activity in the facial motor cortex avoiding unwanted contractions of the jaw, implementing muscle control...

Temporal dynamics of behavior, particularly facial expressions, are fundamental for communication between individuals from very early in development. Facial expression processing has been widely demonstrated to involve embodied simulative processes mediated by the motor system. Such processes may be impaired in patients with congenital facial palsy, including those affected by Moebius syndrome (MBS). The aims of this study were to investigate (a) the role of motor mechanisms in the processing of dynamic facial expression timing by testing patients affected by congenital facial palsy and (b) age-dependent effects on such processing...

No abstract text is available yet for this article.

Space missions pose threats to the health of the astronauts due to long-term exposure to galactic cosmic rays and solar particle events comprised predominantly of medium to high energy protons, energetic helium ions, and energetic high atomic number particles (HZEs). While the tissue-specific effects of radiation have been studied extensively, the changes in exosomal miRNA expression levels in response to acute radiation exposure have not been assessed. Extracellular vesicles (EVs) originate from the host cells and contain nucleic acid and proteins that can modify the physiology of the receiving cells via the transfer of genomic, proteomic, and lipids cargo...

BACKGROUND: Facial paralysis is a frequent disabling entity that causes a negative impact on the cosmetic, functional, social, psychological and economic aspects of the patient. Surgical treatment aims to restore the patient to her previous life with the fewest possible sequelae. OBJECTIVE: Describe the experience of surgical management and propose a treatment algorithm. METHOD: A retrospective study was carried out from 2017 to 2019 of the records of patients with facial nerve involvement...

PURPOSE: To describe outcomes after treatment of Moebius syndrome (MBS) esotropia by adjustable bilateral medial rectus recession (BMR) with and without augmented superior rectus transposition (SRT). DESIGN: Retrospective case series. METHODS: Patients meeting 2014 diagnostic criteria for MBS and treated at Boston Children's Hospital between 2003 and 2019 were identified via billing records and chart review. Visual acuity, sensorimotor evaluations, strabismus procedures, and other clinical features were recorded...

INTRODUCTION: Splenogonadal fusion is a rare congenital anomaly of unknown etiology caused by an abnormal fusion of the splenic tissue and the gonadal tissue. CASE REPORT: 2-year-old patient with paralysis of the 6th, 7th, and 9th cranial nerves, tent-shaped mouth, cleft palate, right pectoralis major hypoplasia, disruptive defect of the right upper limb, and a mass located at the left inguinal region. At inguinal hernia repair surgery, a processus vaginalis with non-reducible content was observed...