Working…
ClinicalTrials.gov
ClinicalTrials.gov Menu

Airway Management of Pediatric Patients With Klippel-Feil Syndrome

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT03741790
Recruitment Status : Active, not recruiting
First Posted : November 15, 2018
Last Update Posted : November 10, 2021
Sponsor:
Information provided by (Responsible Party):
Patcharee Sriswasdi, Boston Children's Hospital

Tracking Information
First Submitted Date August 29, 2018
First Posted Date November 15, 2018
Last Update Posted Date November 10, 2021
Actual Study Start Date November 1, 2018
Actual Primary Completion Date June 30, 2019   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures
 (submitted: November 13, 2018)
Prevalence of difficult airway cases among KFS is defined by using the ASA task force definition. [ Time Frame: We will collect data on a day of surgery from patient's arrival into their induction and intubation period, and through their postoperative period upto 1 week. ]
The difficult airway is defined as "The clinical situation in which a conventionally trained anesthesiologist experiences difficulty with mask ventilation, difficulty with tracheal intubation, or both".
Original Primary Outcome Measures Same as current
Change History
Current Secondary Outcome Measures
 (submitted: November 13, 2018)
Specific anatomical abnormalities of KFS patients which related to difficult airway [ Time Frame: We will collect data on a day of surgery from patient's arrival into their induction and intubation period, and through their postoperative period upto 1 week ]
Specific anatomical abnormalities of KFS patients who had difficult airway is define by radiological classification based on the location of fusion; Type 1 patients have a single congenitally fused cervical segment; Type 2 patients show multiple non-contiguous, congenitally fused cervical segments; and Type 3 patients show multiple contiguous, congenitally fused cervical segments.
Original Secondary Outcome Measures Same as current
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title Airway Management of Pediatric Patients With Klippel-Feil Syndrome
Official Title Airway Management of Pediatric Patients With Klippel-Feil Syndrome
Brief Summary

Klippel-Feil syndrome (KFS) was first described in 1912 by Klippel and Feil as a classic triad are comprised of a short neck, a low posterior hairline and restricted motion of the neck. This disease is considered as one of the congenital causes of difficult airway with the incidence of 1:42,000 live births.

The current research findings suggested that the difficulties of airway management for KFS increases with age. In pediatric patients, the airway of those patients can be managed without difficulties. For adults, the fiberoptic-assisted intubation is also suggested.

The purpose of this study is to review the airway management of pediatric patients with KFS to provide recommendation of airway management for these patients. A retrospective electronic chart review will be conducted by using Boston Children's Hospital (BCH) database, which identified patients with KFS who had undergone general anesthesia from June 2012 to June 2018.

Detailed Description

Klippel-Feil syndrome (KFS) was first described in 1912 by Klippel and Feil as a classic triad are comprised of a short neck, a low posterior hairline and restricted motion of the neck due to fused cervical vertebrae with the incidence of 1:42,000 live births1,2. The etiology is presumed to involve in PAX gene family mutation defect and Notch signaling pathway. KFS patients are classified into 3 types based on the location of fusion; Type 1 patients have a single congenitally fused cervical segment; Type 2 patients show multiple non-contiguous, congenitally fused cervical segments; and Type 3 patients show multiple contiguous, congenitally fused cervical segments. In 1974, Hensinger et al studied 50 KFS patients aged 4 to 34 years and their associated anomalies. The findings revealed less than half had the classic triad of findings, more than half had scoliosis and a third had renal anomalies. All patients were at risk of having other serious anomalies such as Sprengel's deformity, hearing impairment, synkinesia and congenital heart disease.

The current literature focused on isolated case reports suggested an awake fiberoptic intubation as a safest option to secure airway in adult patient with KFS. For pediatric patients with KFS, their airway management could be challenging, there is no literature report describing unsuccessful or difficult mask ventilation or LMA insertion. Bakan et al reported an overview of direct laryngoscopy for tracheal intubation in KFS patients aged 26 days to 16 years old, 18 of 25 cases were successfully intubated by direct laryngoscope (DL). From 13 literatures reviewed by Bakan et al, there is no report of an unsuccessful DL in children with KFS younger than 4 years. Despite the formidable appearance for airway management, recent pediatric data encourage the anesthesia providers to perform DL instead of fiberoptic intubation. However, a successful DL event in anesthesia history record does not guarantee an ease of next DL event because the airway of KFS patients are progressively worse over time.

The investigators propose a retrospective electronic chart review of patients with KFS who had undergone general anesthesia in Boston Children's Hospital from June 2012 to June 2018. The purpose of this study is to review the airway management techniques of pediatric patients with KFS and provide recommendation of airway management for these patients. A retrospective electronic chart review will be conducted by using Boston Children's Hospital (BCH) database, which identified patients with KFS who had undergone general anesthesia from June 2012 to June 2018.

The investigators hope to provide specific anatomical abnormalities and age of pediatric patients with KFS to suggest they are at risk of difficult airway. Finally, the investigators hope this information can be used to suggest a proper choice of airway management for specific type and age group of KFS patients.

Study Type Observational
Study Design Observational Model: Cohort
Time Perspective: Retrospective
Target Follow-Up Duration Not Provided
Biospecimen Not Provided
Sampling Method Non-Probability Sample
Study Population All KFS patients who scheduled for surgery at BCH from June 2012 to June 2018.
Condition Klippel-Feil Syndrome
Intervention
  • Procedure: Ventilation
    The difficult mask ventilation is defined as A) It is not possible for unassisted anesthesiologist to maintain the SpO2 > 90% using 100% oxygen and positive pressure mask ventilation in a patient whose SpO2 > 90% before anesthetic intervention; and/or B) It is not possible for unassisted anesthesiologist to prevent or reverse signs of inadequate ventilation during positive mask ventilation.
  • Procedure: Intubation
    The difficult endotracheal intubation is defined as " It is not possible to visualize any portion of the vocal cords with conventional laryngoscopy" or when proper insertion of the endotracheal tube with conventional laryngoscopy requires more than three attempts or more than ten minutes.
Study Groups/Cohorts Not Provided
Publications *

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status Active, not recruiting
Estimated Enrollment
 (submitted: November 13, 2018)
300
Original Estimated Enrollment Same as current
Estimated Study Completion Date December 31, 2021
Actual Primary Completion Date June 30, 2019   (Final data collection date for primary outcome measure)
Eligibility Criteria

Inclusion Criteria:

  • All KFS patients who scheduled for surgery at BCH from June 2012 to June 2018.

Exclusion Criteria:

  • Patients with incomplete or absent of medical records on AIMS such as ventilation and intubation technique are not clearly document.
Sex/Gender
Sexes Eligible for Study: All
Ages Child, Adult, Older Adult
Accepts Healthy Volunteers Yes
Contacts Contact information is only displayed when the study is recruiting subjects
Listed Location Countries United States
Removed Location Countries  
 
Administrative Information
NCT Number NCT03741790
Other Study ID Numbers IRB-P00029640
Has Data Monitoring Committee No
U.S. FDA-regulated Product
Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
IPD Sharing Statement
Plan to Share IPD: Undecided
Plan Description: We might share individual participant data if it allows maximum use of the data.
Responsible Party Patcharee Sriswasdi, Boston Children's Hospital
Study Sponsor Boston Children's Hospital
Collaborators Not Provided
Investigators
Principal Investigator: Patcharee Sriswasdi, MD, MPH Boston Children's Hospital
PRS Account Boston Children's Hospital
Verification Date November 2021