A survey of practice in the anesthetic management of adolescent idiopathic scoliosis spine fusion by the North American Pediatric Spine Anesthesiologists Collaborative.

BACKGROUND: Adolescent Idiopathic Scoliosis (AIS) affects 2%-4% of the general pediatric population. While surgical correction remains one of the most common orthopedic procedures performed in pediatrics, limited consensus exists on the perioperative anesthetic management. AIMS: To examine the current state of anesthetic management of typical AIS spine fusions at institutions which have a dedicated pediatric orthopedic spine surgeon. METHODS: A web-based survey was sent to all members of the North American Pediatric Spine Anesthesiologists (NAPSA) Collaborative. This group included 34 anesthesiologists at 19 different institutions, each of whom has a Harms Study Group surgeon performing spine fusions at their hospital. RESULTS: Thirty-one of 34 (91.2%) anesthesiologists completed the survey, with a missing response rate from 0% to 16.1% depending on the question. Most anesthesia practices (77.4%; 95% confidence interval [CI], 67.7-93.4) do not have patients come for a preoperative visit prior to the day of surgery. Intravenous induction was the preferred method (74.2%; 95% CI 61.3-89.9), with the majority utilizing two peripheral IVs (93.5%; 95% CI 90.3-100) and an arterial line (100%; 95% CI 88.8-100). Paralytic administration for intubation and/or exposure was divided (51.6% rocuronium/vecuronium, 45.2% no paralytic, and 3.2% succinylcholine) amongst respondents. While tranexamic acid was consistently utilized for reducing blood loss, dosing regimens varied. When faced with neuromonitoring signal issues, 67.7% employ a formal protocol. Most anesthesiologists (93.5%; 95% CI 78.6-99.2) extubate immediately postoperatively with patients admitted to an inpatient floor bed (77.4%; 95% CI 67.7-93.3). CONCLUSION: Most anesthesiologists (87.1%; 95% CI 80.6-99.9) report the use of some form of an anesthesia-based protocol for AIS fusions, but our survey results show there is considerable variation in all aspects of perioperative care. Areas of agreement on management comprise the typical vascular access required, utilization of tranexamic acid, immediate extubation, and disposition to a floor bed. By recognizing the diversity of anesthetic care, we can develop areas of research and improve the perioperative management of AIS.

Severe Multilevel Tracheal Stenosis with Significant Twisting in a Patient with Spondylometaphyseal Dysplasia: A Case Report.

Spondylometaphyseal dysplasia (SMD) is a rare genetic disorder affecting skeletal growth and development presenting anesthesiologists with many perioperative challenges. We present a case of a patient found to have multilevel tracheal stenosis due to twisting and folding of his trachea. This was discovered on imaging during a research review of SMD cases at our institution. Structural and functional abnormalities of the trachea have not been reported in SMD. This is the first description of a patient with SMD with severe multilevel tracheal disease requiring tracheal reconstructive surgery.

Tracheal Narrowing and Its Impact on Anesthesia Care in Patients With Morquio A (Mucopolysaccharidosis Type IVA): An Observational Study.

BACKGROUND: Recently, tracheal narrowing has been recognized as a significant comorbid condition in patients with Morquio A, also known as mucopolysaccharidosis IVA. We studied a large cohort of patients with Morquio A to describe the extent of their tracheal narrowing and its relationship to airway management during anesthesia care. METHODS: This is an observational study, collecting data retrospectively, of a cohort of patients with Morquio A. Ninety-two patients with Morquio A syndrome were enrolled, among whom 44 patients had their airway evaluated by computed tomography angiography and had undergone an anesthetic within a year of the evaluation. Our hypothesis was that the tracheal narrowing as evaluated by computed tomography angiography increases with age in patients with Morquio A. The primary aim of the study was to examine the degree of tracheal narrowing in patients with Morquio A and describe the difficulties encountered during airway management, thus increasing awareness of both the tracheal narrowing and airway management difficulties in this patient population. In addition, the degree of tracheal narrowing was evaluated for its association with age or spirometry parameters using Spearman's rank correlation. Analysis of variance followed by the Bonferroni test was used to further examine the age-based differences in tracheal narrowing for the 3 age groups: 1 to 10 years, 11 to 20 years, and >21 years. RESULTS: Patient age showed a positive correlation with tracheal narrowing ( rs= 0.415; 95% confidence interval [95% CI], 0.138-0.691; P = .005) with older patients having greater narrowing of the trachea. Among spirometry parameters, FEF25%-75% showed an inverse correlation with tracheal narrowing as follows: FEF25%-75% versus tracheal narrowing: ( rs = -0.467; 95% CI, -0.877 to -0.057; P = .007). During anesthetic care, significant airway management difficulties were encountered, including cancelation of surgical procedures, awake intubation using flexible bronchoscope, and failed video laryngoscopy attempts. CONCLUSIONS: Clinically significant tracheal narrowing was present in patients with Morquio A, and the degree of such narrowing likely contributed to the difficulty with airway management during their anesthetic care. Tracheal narrowing worsens with age, but the progression appears to slow down after 20 years of age. In addition to tracheal narrowing, spirometry values of FEF25%-75% may be helpful in the overall evaluation of the airway in patients with Morquio A.

Transcranial electric motor evoked potential monitoring during scoliosis surgery in children with cerebral palsy and active seizure disorder: is it feasible and safe?

PURPOSE: Use of spinal cord monitoring in children with cerebral palsy (CP) and neuromuscular scoliosis is challenging. The previous reports suggest low success rates in the setting of CP, and it is unclear if transcranial electric motor evoked potentials (TcMEP) monitoring is contraindicated in patients with an active seizure disorder. The purpose of this study was to determine (1) are patients with CP able to be appropriately monitored with TcMEP? and (2) does TcMEP cause an increase in seizure activity? METHODS: This was an institutional review board-approved retrospective cohort study observing 304 patients from 2011 to 2020. Inclusion criteria included all patients with CP undergoing posterior spinal fusion during this time. Intraoperative data were examined for the ability to obtain monitoring and any intraoperative events. Patients were followed for 3 months postoperatively to determine any increase in seizure activity that could have been attributed to the TcMEP monitoring. RESULTS: Of the 304 patients who were observed, 21% (20.8%) were unable to be monitored due to lacking baseline signals from the extremities. Seventy-seven percent (77.5%) were successfully monitored with TcMEP. For these patients, no increased seizure activity was documented either intra- or postoperatively. CONCLUSION: A high percentage of children (77.5%) with CP were able to be successfully monitored with TcMEP during posterior spinal fusion. Furthermore, the concerns about increased seizure activity after TcMEP were not supported by the data from this cohort. Technical details of successful neuromonitoring in these patients are important and included increased stimulation voltage requirements and latency times. LEVEL OF EVIDENCE: III retrospective comparative study.

Metatropic dysplasia-a skeletal dysplasia with challenging airway and other anesthetic concerns.

BACKGROUND: Metatropic dysplasia is a rare form of skeletal dysplasia requiring multiple anesthetics for surgical and imaging procedures, most of which are orthopedic procedures. We provide centralized care to patients with skeletal dysplasia at our tertiary care pediatric hospital, and we were able to collect the largest number of metatropic dysplasia patients reported to date. AIM: The aim of this retrospective study was to describe and characterize the anesthetic difficulties in this high-risk population. METHODS: Medical charts of all patients with metatropic dysplasia were reviewed to collect data, including anesthetics performed, difficulties, and complications related to the anesthetic care, co-morbid conditions, and related events. RESULTS: Twenty-three patients with metatropic dysplasia underwent 188 anesthetics with 61% of the anesthetics having been administered for orthopedic procedures. Fourteen of 23 (60.8%) progressively became difficult to intubate over the course of their care, with 12 out of 14 having undergone cervical spine fusion. These 14 patients had a total of 133 procedures. Sixty procedures (45.1%) had an airway described as difficult. Glidescope was the difficult airway tool most commonly used (68%) with flexible fiberoptic scope used 12% and Miller or Macintosh blade used 18% of the time. In addition to the airway difficulties, spinal canal narrowing or stenosis was widely prevalent, and no neuraxial anesthetic was performed in any of our patients. CONCLUSION: Difficult airway is the most common co-morbid condition present in patients with metatropic dysplasia, especially if their cervical spine has been fused. Familiarity with the difficulties involving the airway and its management is critical in safe and successful management of anesthesia in this high-risk population.

Clinical relevance of echocardiogram in patients with cerebral palsy undergoing posterior spinal fusion.

BACKGROUND: Spinal deformity is one of the secondary musculoskeletal problems that occur with cerebral palsy (CP). Of the co morbidities associated with CP and spinal deformity, cardiac function is of theoretical concern. OBJECTIVE: The goal of our study was to determine the clinical relevance of routine preoperative cardiology evaluation via echocardiogram for patients with CP presenting for posterior spine fusion (PSF) surgery. METHODS: A retrospective chart review was performed of CP patients presenting for scoliosis surgery. The data collected for each patient included: age, sex, height, weight, Cobb angle, and medical history. All patients had a preoperative cardiac evaluation. RESULTS: Seventy-two patients were included. The mean age was 13.6 ± 3.4 years. Left ventricular systolic function was normal in all patients; the mean shortening fraction was 39.3 ± 6.2%. No patient had more than mild insufficiency of either the semilunar or atrioventricular valve. One patient was diagnosed with aortic root dilation as well as aortic valve insufficiency. All patients had PSF surgery without changes in anesthetic or surgical plans, and no patient experienced complications attributable to a cardiac origin. CONCLUSION: The results suggest that routine preoperative cardiology evaluation via echocardiogram for children with CP in the absence of clinical history or physical examination findings suggestive of cardiac disease is not necessary.

Major surgical procedures in children with cerebral palsy.

There are 3 surgical procedures that patients with cerebral palsy (CP) undergo that may be considered major procedures: femoral osteotomies combined with pelvic osteotomies, spine fusion, and intrathecal baclofen pump implant for the treatment of spasticity. Many complications are known to occur at a higher rate in this population, and some may be avoided with prior awareness of the preoperative pathophysiology of the patient with CP.

Anesthesia management of a patient with a laryngotracheo-esophageal cleft.

Laryngotracheo-esophageal cleft is a rare congenital anomaly that results from complete or partial failure of the development of the tracheoesophageal septum. The presenting symptoms include stridor, respiratory distress, and coughing or cyanotic episodes with feeding. There are four classifications for laryngeal clefts; the severity depends on the type present. We discuss the anesthesia management of a neonate with a Type IV cleft who presented for an emergency gastric division to prevent pulmonary aspiration and later returned for final repair of the defect.

Anesthetic management for pediatric spinal fusion: implications of advances in spinal cord monitoring.

Currently, the detection of emerging injury through intraoperative neurologic monitoring is the best way to prevent neurologic injury. This requires a team approach that includes the anesthesiologist, neurophysiologist, and surgeon. The monitoring modalities available for the patient must be considered in planning the anesthetic management. In addition, intraoperative care for the patient requires an ongoing attention to how the anesthetic drugs affect spinal cord monitoring.

Plastic bronchitis: a case report.

We report a case of perioperative management of a toddler with plastic bronchitis complicated by tracheal obstruction. We discuss our management of this case as well as the diverse group of patients who may present with this disease. We also reviewed the literature regarding medical management of cast bronchitis.

Multimodality monitoring of transcranial electric motor and somatosensory-evoked potentials during surgical correction of spinal deformity in patients with cerebral palsy and other neuromuscular disorders.

STUDY DESIGN: This prospective, descriptive study determined the reliability of transcranial electric motor and posterior tibial nerve somatosensory-evoked potentials in children with neuromuscular scoliosis. OBJECTIVE: To assess the applicability of transcranial electric motor and posterior tibial nerve somatosensory-evoked potentials during surgical correction of neuromuscular scoliosis, particularly with cerebral palsy-related deformity. SUMMARY OF BACKGROUND DATA: During corrective spinal surgery for neuromuscular scoliosis, intraoperative multimodality spinal cord monitoring is recommended. There exist conflicting, retrospective studies regarding the reliability of spinal cord monitoring in patients with neuromuscular scoliosis. METHODS: Transcranial electric motor potentials and posterior tibial nerve somatosensory-evoked potentials were monitored in all patients presenting for spinal fusion between 2000 and 2001. Anesthesia was standardized for all patients. RESULTS: There were 68 patients subdivided into two subject groups. Group I consisted of 39 patients with neuromuscular scoliosis associated with cerebral palsy, and Group II consisted of 29 children with neuromuscular scoliosis due to a disease process other than cerebral palsy. Five of the 68 patients had significant amplitude changes in 1 or both monitoring methods during surgery relative to baseline. Of these, one had permanent neurologic deficit despite standard intervention. Somatosensory-evoked potentials were monitored successfully in 82% of the cerebral palsy and 86% of the noncerebral palsy patients. Transcranial electric motor-evoked potentials, on the other hand, were monitorable in 63% of patients with mild or moderate degrees of cerebral palsy and 39% of those with severe involvement. Eighty-six percent of those with noncerebral palsy-related neuromuscular scoliosis had recordable motor-evoked potentials at baseline. CONCLUSION: Both transcranial electric motor and posterior tibial nerve somatosensory-evoked potentials can be monitored reliably in most patients with neuromuscular scoliosis. Those with severe cerebral palsy present the greatest challenge to successful neurophysiologic monitoring.