Obstetrical and Pediatric Anesthesia Anesthetic management of children with Moebius sequence

Abstract

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.

Consideraciones anestésicas en el síndrome de Moebius: a propósito de un caso

“…Rev Mex Anestesiol, 2020; 43 (1) www.medigraphic.org.mx discusión El síndrome de Moebius fue descrito en 1888 por Paul Julius Moebius. Su etiología no es clara, pero es producto de un desarrollo embrionario anormal (4) . La etiología más defendida es la disrupción de la morfogénesis de las arterias basilar o vertebral en un estadío crítico para la gestación.…”

“…Dicha alteración se traduce en una ausencia de desarrollo de los núcleos de los pares craneales VI y VII y la aparición de calcificaciones y aplanamiento de la base del cuarto ventrículo. Además de la exposición a ciertos medicamentos tales como benzodiacepinas, alcohol, cocaína y el recientemente relacionado misoprostol (1,4) . La mayoría de los pacientes no presentan relación genética, pero se ha documentado casos con transmisión autosómica.…”

Moebius Syndrome: Challenges of Airway Management

“…Ames et al 5 reviewed 111 anesthesia records of 46 patients with Moebius syndrome who underwent different surgical procedures. Face mask ventilation was easily employed in all patients, while in 76 cases (71.6% of all intubations) tracheal intubation was uneventful.…”

“…Moebius syndrome has multiple implications for anesthesia care, most notably that of airway management 5 , especially when it is associated with other rare clinical conditions such as trigeminal trophic syndrome (TTS) in our case. …”

Postoperative airway events of individuals with CHARGE syndrome

“…It is known that tracheal intubation may be difficult in syndromes that have similar anatomic anomalies to CHARGE, such as Moebius sequence [14]. Many cranial facial syndromes have preoperative, inhalation concerns; however, it is also the postoperative airway events that are of concern in CHARGE syndrome.…”