Anesthetic Management of a Patient with Moebius Syndrome

Krajcirik, William J.; Azar, Isaac; Opperman, Sheldon; Lear, Erwin

doi:10.1213/00000539-198503000-00013

Cited by 9 publications

(11 citation statements)

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“…Also of concern is the association of cardiomyopathy in disease processes associated with skeletal myopathy such as Duchenne muscular dystrophy. Although a literature review did not identify reports of an association of cardiomyopathy with Mfbius syndrome and preoperative echocardiography revealed normal function in our patient, there was a suggestion of myocardial involvement in the patient reported by Krajcirik et al [9] who was significantly older (13 years vs 10 months) than our patient. In addition, Ferguson [11] reported that 4 of the 19 patients in his series had associated congenital heart disease (2 with ventricular septal defect, 1 with patent ductus arteriosus, and 1 with dextrocardia), thereby further emphasizing the consideration of preoperative echocardiography in these patients.…”

Section: Discussioncontrasting

confidence: 81%

“…To date, there are only 3 published reports addressing the relevant anesthetic issues in patients with Mfbius syndrome [9][10][11]. Krajcirik et al [9] described the anesthetic care of a 13-year-old girl with Mfbius syndrome during a diagnostic fiberoptic bronchoscopy for persistent pneumonia.…”

Section: Introductionmentioning

confidence: 99%

“…Krajcirik et al [9] described the anesthetic care of a 13-year-old girl with Mfbius syndrome during a diagnostic fiberoptic bronchoscopy for persistent pneumonia.…”

Section: Introductionmentioning

confidence: 99%

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Anesthetic implications of Möbius syndrome

Gondipalli

Tobias

2006

Journal of Clinical Anesthesia

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Section: Discussioncontrasting

confidence: 81%

Section: Introductionmentioning

confidence: 99%

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Anesthetic implications of Möbius syndrome

Gondipalli

Tobias

2006

Journal of Clinical Anesthesia

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“…Patients are susceptible to recurrent aspirations and chronic lung disease. 6,7 Moebius sequence also has associated limb abnormalities including talipes, syndactyly, congenital limb amputations and rudimentary digits. 8 Cardiac defects are described 9 and intelligence is reported as either normal 10 or associated with a high incidence of developmental delay (as high as 75%).…”

Section: Discussionmentioning

confidence: 99%

“…Fourth, incomplete eyelid closure predisposes to perioperative corneal abrasions. 6 Fifth, central hypoventilation has been described, and caution with opioids is advocated. 20 Although many of our patients were given opioids during anesthesia only one episode of postoperative apnea was observed.…”

Section: Surgical Managementmentioning

confidence: 99%

Obstetrical and Pediatric Anesthesia Anesthetic management of children with Moebius sequence

Ames

Shichor

Speakman

et al. 2005

Can J Anesth/J Can Anesth

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Background: Moebius sequence is a rare congenital absence of the sixth and seventh cranial nerves, although there may be additional congenital cranial neuropathies. Developmental delay, cardiac and musculoskeletal abnormalities may also coexist. Oro-facial manifestations include bilateral facial nerve palsy resulting in a mask like facies, drooling, incomplete eye closure, and strabismus secondary to the extra-ocular muscle imbalance. This condition has multiple implications for anesthetic care. Methods:We reviewed 111 anesthesia records of 46 patients with Moebius sequence for anesthesia technique and related complications.Results: Facial nerve palsy was universally present and bilateral in 44 (93.6%) patients. Thirty-two (68%) had concomitant sixth nerve palsy. Oro-facial and limb abnormalities were present in 16 (35%) and 18 (39%) of patients respectively. Endotracheal intubation, when attempted, was easy in 76 of 106 cases. Tracheal intubation was consistently difficult in seven patients and intubation failure occurred in a single patient only. Statistically significant factors associated with difficult tracheal intubation included structural abnormalities of the mandible and palate and abnormalities of four cranial nerves (IX, X, XI, XII). Conclusion:We confirm that tracheal intubation may be difficult in patients with Moebius sequence. We identify disease features that might predict a difficult tracheal intubation and thus allow the anesthesiologist an opportunity to plan accordingly.

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Moebius syndrome: a review of the anaesthetic implications

Ferguson¹

1996

Pediatric Anesthesia

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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.

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Anesthetic Management of a Patient with Moebius Syndrome

Cited by 9 publications

References 0 publications

Anesthetic implications of Möbius syndrome

Anesthetic implications of Möbius syndrome

Obstetrical and Pediatric Anesthesia Anesthetic management of children with Moebius sequence

Moebius syndrome: a review of the anaesthetic implications

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