This Article Résumé de cet Article Full Text Full Text (PDF) Submit a scholarly reply Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ames, W. A. Articles by McCaul, C. Search for Related Content PubMed PubMed Citation Articles by Ames, W. A. Articles by McCaul, C.

Anesthetic management of children with Moebius sequence

[Anesthésie chez des enfants atteints du syndrome de Moebius]

Warwick A. Ames, MBBS FRCA^*, Tal M. Shichor, MD, Melanie Speakman, MBBS MRCP FRCA, Ronald M. Zuker, MD FRCS(C) FACS FAAP and Conan McCaul, MB BCH BAO FFARCSI

^* From the Division of Pediatric Anesthesiology, Duke University Medical Center, Durham, North Carolina, USA;
the Departments of Anesthesia, and
Plastic Surgery, Hospital for Sick Children, Toronto, Ontario, Canada; and
the Departments of Anesthesia and Intensive Care,Regional Hospital, Waterford, Ireland.

Address corrrespondence to: Dr. Warwick A. Ames, Division of Pediatric Anesthesiology, DUMC, Box 3094, Durham, North Carolina 27710, USA. Fax: 919-681 8357; E-mail: wads{at}doctors.org.uk

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 co-exist. 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.