Anesthetic management of children with Moebius sequence.
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).


CONCLUSIONS
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.
Ames
Warwick A
WA
Division of Pediatric Anesthesiology, DUMC, Box 3094, Durham, North Carolina 27710, USA. wads@doctors.org.uk
Shichor
Tal M
TM
Speakman
Melanie
M
Zuker
Ronald M
RM
McCaul
Conan
C
D000010:Abducens Nerve / Q000002:abnormalities* / Q000601:surgery; D000015:Abnormalities, Multiple / Q000601:surgery*; D000293:Adolescent; D000758:Anesthesia; D002648:Child; D002675:Child, Preschool; D005154:Facial Nerve / Q000002:abnormalities* / Q000601:surgery; D005158:Facial Paralysis / Q000209:etiology; D005260:Female; D006801:Humans; D007223:Infant; D007442:Intubation, Intratracheal; D008297:Male; D012121:Respiration, Artificial; D012189:Retrospective Studies; D013577:Syndrome