Prenatal Sonographic Evidence Supporting an in Utero Developmental Etiology of Möbius Sequence

Sherer, David M.; Spafford, Patrick

doi:10.1055/s-2007-994578

Cited by 13 publications

(3 citation statements)

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“…43 A case by M. Sherer et al reported a normal amount of amniotic fluid and normal fetus swallowing at early gestation but with massive polyhydramnios during the latter part of pregnancy, along with micrognathia, absent mandibular movement, and a non-visualized stomach, which strongly points towards a late in-utero development of Moebius Syndrome. 44 Recently, Nguyen et al were able to use antenatal MRI imaging to demonstrate several features characteristic of Moebius syndrome, such as the fattened posterior aspect of the pons & medulla and tectal beaking, in a fetus who was later diagnosed with Moebius syndrome. 45 Genetic testing through amniocentesis may be done in cases of polyhydramnios and a family history of Moebius Syndrome to investigate further a pathogenic variant in the PLXND1 / REV3L / homeobox genes.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Moebius Syndrome: An Updated Review of Literature

Monawwer,

Ali,

Naeem

et al. 2023

Child Neurology Open

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Moebius Syndrome, is a rare, non-progressive congenital neuropathological syndrome characterized primarily by the underdevelopment of the facial (CN VII) and abducens nerve (CN VI). Other features of Moebius Syndrome include facial nerve paresis, ophthalmoplegias, orthodontic deficiencies (including crowded dentition, swollen and hyperplastic gingiva, dental calculus, etc.), musculoskeletal abnormalities, and impaired mental function. Due to the rarity of the disorder, very few case studies have been reported in the literature. This article summarizes the significant features of the disease according to commonalities in reported cases, along with several newly recognized features cited in recent literature. We have explored the different diagnostic criteria and the newly recognized imaging modalities that may be used. Understandably, the condition detrimentally affects a patient’s quality of life; thus, treatment measures have also been outlined. This study aims to provide updated literature on Moebius Syndrome MBS and improve understanding of the condition.

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Section: Etiology and Pathogenesismentioning

confidence: 99%

Moebius Syndrome: An Updated Review of Literature

Monawwer,

Ali,

Naeem

et al. 2023

Child Neurology Open

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“…46 Prenatal sonography, which may show decreased respiratory and swallowing movements, polyhydramnios, and related anomalies, may first alert the physician to the possible diagnosis of Möbius syndrome. 47,48 The differential diagnosis for Möbius syndrome includes other syndromes that combine cranial nerve palsies with craniofacial and limb abnormalities such as Hanhart syndrome, hypoglossia-hypodactyly syndrome, glossopalatine ankylosis, fascioscapulohumeral dystrophy, and Charlie M syndrome. 43,[49][50][51] ETIOLOGY The clinical complexity of Möbius with its confusing array of malformations continues to hinder scientists in their pursuit to discover its etiology.…”

Section: Diagnosismentioning

confidence: 99%

Möbius Syndrome

Singham

Manktelow²

2004

Seminars in Plastic Surgery

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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.

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“…Multiple congenitally missing teeth (both deciduous and permanent) have been reported, along with paralysis and hypoplasia of the tongue, absence of facial expression (7) and weakness of the palate (3, 9). Micrognathia is also associated with Mobius syndrome and is thought to be due to a neuromuscular deficiency in early movement of the mandible (10). Abramson et al.…”

Section: Review Of the Literaturementioning

confidence: 99%

Mobius syndrome: a dental hygiene case study and review of the literature

Sensat

2003

Int J Dental Hygiene

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Mobius syndrome, a rare, non-progressive, congenital neuromuscular disorder, presents with multiple dental and medical complications. Signs and symptoms of this condition include: congenital, bilateral or unilateral palsies of the facial and abducens cranial nerves (cardinal sign), and a broad scope of multisystem abnormalities, which may or may not include the following: opthalmoplegia externa, lingual palsy, clubfoot, branchial malformation, ptosis, mental retardation, and pectoralis muscle defect. From a dental standpoint, a number of anomalies may manifest, including: multiple congenitally missing teeth (both deciduous and permanent), micrognathia, paralysis and hypoplasia of the tongue, absence of facial expression and weakness of the palate. This article reviews the case study of a 40-year-old male client with Mobius syndrome, dental hygiene diagnosis (DHDx) and intervention, and the dental hygienists' responsibility and role in care.

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Prenatal Sonographic Evidence Supporting an in Utero Developmental Etiology of Möbius Sequence

Cited by 13 publications

References 0 publications

Moebius Syndrome: An Updated Review of Literature

Moebius Syndrome: An Updated Review of Literature

Möbius Syndrome

Mobius syndrome: a dental hygiene case study and review of the literature

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