Moebius syndrome: a review of the anaesthetic implications

Abstract

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.

Obstetrical and Pediatric Anesthesia Anesthetic management of children with Moebius sequence

“…This compares to an earlier series by Ferguson, which reported difficult intubation in 42% of patients with Moebius sequence. 19 The lower incidence encountered in our series is at least partially explained by the lower incidence of micrognathia and/or microstomia. These were present in 16/19 patients in Ferguson's series.…”

“…Last, cervical spine abnormalities have been previously documented in Moebius sequence but were not detected in any of our patients. 19 Limitations of this study We acknowledge that a major limitation of this study is that it is retrospective in nature, and that issues such as difficult tracheal intubation may have been under reported. It is also possible that a selection bias in this study may prevent an accurate estimate of the true prevalence of intubation failure in Moebius sequence.…”

“…Where tracheal intubation failure occurred, laryngeal mask airways were utilized in Ferguson's series, but were noted to be difficult to position. 19 Laryngeal mask airways were not commonly used in our patients. The reasons for this are uncertain but may include institutional philosophy and concerns related to the 'shared airway', aspiration risk and duration of surgery.…”

Airway Management in Neonates with Pierre Robin Syndrome along with Mobius Syndrome and Platoglossal Fusion

“…Postoperative use of opioid, sedative and anesthetic agents in th presence of central hypoventilation can predispose to grave respiratory consequences. In addition, postoperative pain assessment is difficult due to absence of facial expression because of the facial palsy [9,10,25,26].…”

“…Moebius syndrome is characterized by congenital palsy of the 6th and 7th cranial nerves, but may also be associated with palsies of other cranial nerves, including the 9th and 10th, creating dysfunction of the pharynx, dysphagia, feeding difficulties, retention of oral secretions and recurrent bouts of aspiration pneumonia [8][9][10][11]25], and can also be associated with central alveolar hypoventilation. Postoperative use of opioid, sedative and anesthetic agents in th presence of central hypoventilation can predispose to grave respiratory consequences.…”