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* 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
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Abstract |
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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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Introduction |
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Methods |
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, individual consent from each patient/family was not obtained nor deemed necessary for the study. All patients with a clinical diagnosis of Moebius sequence were included in the study. The medical records of these patients were reviewed. The data base included pre-morbid features such as cranial nerve involvement, oro-facial abnormalities and cardiac anomalies. Each anesthetic record was reviewed to determine, in particular, the ease of tracheal intubation, and the anesthetic drugs used.
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Results |
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). The male-to-female ratio was 1:1.75, and age at the time of surgery ranged from one month to 18.5 yr (mean 8 yr). Phenotypical features are presented in Table II. Limb and cardiac lesions were seen in 39% and 13% respectively. Prematurity and developmental delay was observed in 17% and 13% respectively.
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Analgesia
Intra- and postoperative pain management included morphine [administered by bolus, infusion or patient-controlled analgesia (PCA)], fentanyl or meperidine PCA, codeine, and acetaminophen. Epidural analgesia was administered in one patient. There were no recorded complications related to postoperative pain management.
Recovery
All intubated patients were extubated in the operating room at the end of surgery. One patient was re-intubated due to apnea and was extubated successfully soon afterwards in the postanesthetic care unit. There were two cases of post-extubation stridor and two cases of postoperative pneumonia. Patients undergoing gracilis muscle transplantation were all managed postoperatively on a constant observation unit with pulse oximetry for the first 24 hr. No patient required respiratory support and there were no intensive care unit admissions and no deaths. Of 14 ambulatory procedures there were no unplanned hospital admissions. Only one surgical complication was noted; a hematoma at the flap implantation site that required surgical re-exploration.
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Discussion |
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The congenital absence or dysgenesis of other cranial nerves leads to specific clinical issues, including convergent strabismus, deafness, tongue atrophy, poor swallowing and an inadequate cough reflex. 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 described9 and intelligence is reported as either normal10 or associated with a high incidence of developmental delay (as high as 75%).11 We report a high incidence of prematurity (17%).
The diagnosis of Moebius sequence is essentially clinical, but radiological findings can support the diagnosis. Computed tomography scans are typically normal, whereas magnetic resonance imaging studies may show brainstem hypoplasia and demonstrate straightening of the floor of the fourth ventricle suggesting an absence of the facial colliculus (reflecting loss of the abducens and/or facial nerve nuclei).12,13
Pathogenesis
No single etiological factor has been definitively associated with Moebius sequence, nor is it certain whether the pathology is a result of dysgenesis or degeneration. The most accepted theory proposes a disruption, in utero, of the basilar or vertebral arteries at a critical and early stage in gestation14 by hypoxia, hyperthermia, infection, or drugs, in particular benzodiazepines,15 alcohol and cocaine.5 Misoprostol, a non-steriodal medication used for early non-surgical termination of pregnancies, has a strong association16 and is thought to be responsible for a suggested recent increased incidence of Moebius sequence.17 Finally, chorionic villus sampling in early gestation has a positive association.4
Surgical management
Children with Moebius sequence may present for surgical correction of strabismus, cleft palate and limb malformations as well as conditions unrelated to the sequence itself (Table I
). The most common surgical procedure performed at our hospital is a segmental gracilis muscle transplantation, wherein a segment of gracilis muscle is transplanted to the face and re-vascularized with the facial artery and vein. Motor innervation is achieved from a functioning cranial nerve, the aim being to generate facial expression and provide lower lip support to both reduce drooling and improve speech.18 The results of facial re-animation in one patient in this series can be seen in Figure 2.
Neurological examination is performed prior to surgery to identify the potential motor donors. Clearly not all cranial nerves are appropriate for use in re-implantation surgery because not all have a motor component. In general, if the facial palsy is unilateral, then the contra lateral facial nerve can be used.5 The masseter nerve, a motor branch of the trigeminal nerve, is favoured at our institution because it is seldom diseased, is accessible, and is extremely effective in providing muscle excursion. In our experience of segmental gracilis muscle transplantation, immediate postoperative complications were restricted to one hematoma that required re-exploration.
The preferred surgical timing is between the ages of four to five years, essentially before school age. Post-transplantation muscle function begins at six to seven weeks. Each side is corrected separately, about three months apart. In a study reporting the surgical results from our institution, drooling and fluid loss with drinking were eliminated. Speech was significantly improved in most children and a smile achieved in all. Improvement in self esteem and social interaction were reported.18
Anesthetic management
From this study, we report the anesthetic management of patients with Moebius sequence at our institution. Firstly mask ventilation was easy in all cases. Facemask anesthesia is a feasible technique where there is no need to intubate the trachea for surgery.19 However, it is of limited utility in our patient population where operative episodes are lengthy, and the airway is shared with the surgeon. Nasal intubation is preferred during gracilis muscle transplantation so that the surgeon can visualize and manipulate the mouth. 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.
Difficult intubation is the most noteworthy anesthetic challenge. In this, the largest series of patients with Moebius sequence yet reported, we encountered difficult tracheal intubation in 15.2% of patients and 28.3% of attempted intubations. 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. In our series, difficult intubation was more common in patients who had structural abnormalities of the mandible and palate, and where mouth opening was limited. This has important implications for clinical anesthesia practice, in that difficult intubation can be anticipated in patients with such abnormalities, and is thus predictable in most instances.
An interesting observation from this series is that four cranial neuropathies were associated with difficult intubation; the IX, X, XI and XII cranial nerves. In terms of embryology, it is not easy to attribute difficult airway management with the pharyngeal arch origins of these cranial nerves. It is also not obvious how the loss of any of these nerves could lead to anatomical variations sufficient to render a patient difficult to intubate.
Finally, Moebius sequence has extremely variable features. Our series shows that a wide variety of anesthetic techniques (Table III) and approaches to the airway (Figure 1) were used, and therefore no single technique can be advocated on the basis of this data. However, there are some consistent anesthetic considerations to be noted. First, limb abnormalities may restrict iv access sites (although this was not a problem in our series). Second, developmental delay, again although not common in our series, may be present and may limit patient cooperation, communication and the ability to use PCA devices. Third, cardiac disease may be present and should be identified and treated appropriately.9 Antibiotic prophylaxis may be required for certain cardiac lesions. 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. It is not clear whether this was central apnea, anesthetic-related, or a combination of both. The patient was re-intubated but extubated soon after, and had an otherwise uneventful postoperative course. Sixth, recurrent aspirations may lead to chronic lung disease. Tracheotomy, for airway toileting, may even be necessary. Despite the mean length of surgery (6.8 hr) and the potential for chronic lung disease, there were very few respiratory complications or any significant morbidity in our 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. Intubation was not attempted in all patients, and our series may not have been representative of all patients with Moebius sequence. Furthermore, patients presenting for facial reconstructive surgery (our largest patient group) may have more severe oro-facial abnormalities than those who do not require such intervention. Lastly, detection bias may be present as no standardized definition of ‘difficult intubation’ is utilized in our department. Rather, we chose to accept the written comments of the attending anesthesiologist and our interpretation of the steps required to secure the airway as de facto evidence of difficult tracheal intubation.
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Acknowledgments |
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Footnotes |
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References |
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