Poland-Mobius syndrome associated with dextrocardia.

Bosch-Banyeras, J. M.; Zuasnabar, A; Puig, Albert Martinez; Català, Margarita; Cuatrecasas, J

doi:10.1136/jmg.21.1.70

Cited by 30 publications

(12 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The 15 infants with the Poland Anomaly showed that this phenotype of absence of the sternal and costal portions of the pectoralis major (Poland, ) occurred more often without an associated abnormality of the ipsilateral limb. The observed presence of Moebius syndrome of cranial nerve palsies and dextrocardia in two of the infants with the Poland Anomaly has been reported previously (Bosch‐Banyeras, Zuasnabar, Puig, Català, & Cuatrecasas, ).…”

Section: Discussionsupporting

confidence: 78%

Malformations attributed to the process of vascular disruption

Holmes

Westgate

Nasri

et al. 2018

Birth Defects Research

View full text Add to dashboard Cite

Malformations attributed to the process of vascular disruption were a distinctive entity, among the recognized etiologies. The timing of the causative event in the first trimester was established for infants with exposures to either the prostaglandin misoprostol or the prenatal diagnosis procedure chorionic villus sampling. One challenge is to identify the developmental steps in vascular disruption when no causative exposure can be identified.

show abstract

Section: Discussionsupporting

confidence: 78%

Malformations attributed to the process of vascular disruption

Holmes

Westgate

Nasri

et al. 2018

Birth Defects Research

View full text Add to dashboard Cite

show abstract

“…Cardiac anomalies are well described in Moebius syndrome (Carvella & Rogers 1978;Bosch-Banyeras et al 1984). Four of the 19 cases reviewed in this study had congenital cardiac disease.…”

Section: Discussionmentioning

confidence: 73%

“…These malformations include micrognathia, microstomia, tongue-tethering and premaxillary overgrowth (Jones 1988;Gorlin 1990). Other anomalies with anaesthetic implications which occur more frequently in Moebius syndrome include congenital cardiac disease (Carvella & Rogers 1978;Bosch-Banyeras et al 1984), cervical spine anomalies (Bouwes-Bavinck & Weaver 1986), peripheral neuropathies (Rubenstein et al 1975;Abid et al 1978) and pectoral muscle deficiencies (also known as Poland syndrome) (David 1972).…”

Section: Introductionmentioning

confidence: 99%

Moebius syndrome: a review of the anaesthetic implications

Ferguson¹

1996

Pediatric Anesthesia

View full text Add to dashboard Cite

Moebius syndrome is a rare congenital anomaly characterized by multiple cranial nerve palsies, orofacial malformations and limb anomalies. This study retrospectively reviewed the anaesthetic records of 19 children with Moebius syndrome who had anaesthesia at the Royal Alexandra Hospital for Children over a 15 year period and analysed the complications which occurred. Affected children most commonly present for anaesthesia for correction of strabismus, or for orthopaedic procedures to improve limb function. Despite the abnormal facies and drooling of saliva, these children are almost always of normal intelligence. Problems can arise during anaesthesia, with a high incidence of difficult or failed intubation. The use of a facemask and spontaneous breathing technique, where appropriate, seems to present no problems and maintaining an airway in this way appears to be safe. However, the potential for problems with aspiration of oral secretions should be remembered and the use of antisialogogue premedication is recommended. Affected children have a high incidence of other anomalies, including congenital cardiac disease, spinal anomalies, corneal abrasions and peripheral neuropathies and a careful preoperative assessment is essential.

show abstract

“…Although clubfeet are the most commonly associated anomaly, brachydactyly, syndactyly, ectrodactyly, acheiria, arthrogryposis, kyphosis, kypholordosis, absent or rudimentary fingers or toes, and rib defects can also be found in Möbius patients. 5,6,11,[13][14][15][16][17][18] Möbius syndrome may also be associated with Poland's anomaly, which is defined as congenital absence of the pectoral head with ipsilateral hand deformity. 14,16,19,[20][21][22] Klippel-Feil anomaly (fusion of the cervical vertebrae into a single short bone), Goldenhar anomaly (absent serratus anterior muscle), and hypoglossia-hypodactyly may also be detected.…”

Section: Definition and Clinical Featuresmentioning

confidence: 99%

“…5,6,11,[13][14][15][16][17][18] Möbius syndrome may also be associated with Poland's anomaly, which is defined as congenital absence of the pectoral head with ipsilateral hand deformity. 14,16,19,[20][21][22] Klippel-Feil anomaly (fusion of the cervical vertebrae into a single short bone), Goldenhar anomaly (absent serratus anterior muscle), and hypoglossia-hypodactyly may also be detected. 5,6,14,19,23,24 Associated myopathy and neuropathy have also been described.…”

Section: Definition and Clinical Featuresmentioning

confidence: 99%

Möbius Syndrome

Singham

Manktelow²

2004

Seminars in Plastic Surgery

View full text Add to dashboard Cite

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.

show abstract

Poland-Mobius syndrome associated with dextrocardia.

Cited by 30 publications

References 8 publications

Malformations attributed to the process of vascular disruption

Malformations attributed to the process of vascular disruption

Moebius syndrome: a review of the anaesthetic implications

Möbius Syndrome

Contact Info

Product

Resources

About