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Airway Management of Pediatric Patients With Klippel-Feil Syndrome

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ClinicalTrials.gov Identifier: NCT03741790
Recruitment Status : Active, not recruiting
First Posted : November 15, 2018
Last Update Posted : February 15, 2019
Sponsor:
Information provided by (Responsible Party):
Patcharee Sriswasdi, Boston Children’s Hospital

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.


Condition or disease Intervention/treatment
Klippel-Feil Syndrome Procedure: Ventilation Procedure: Intubation

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.


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Study Type : Observational
Estimated Enrollment : 300 participants
Observational Model: Cohort
Time Perspective: Retrospective
Official Title: Airway Management of Pediatric Patients With Klippel-Feil Syndrome
Actual Study Start Date : November 1, 2018
Estimated Primary Completion Date : June 30, 2019
Estimated Study Completion Date : August 30, 2019

Resource links provided by the National Library of Medicine



Intervention Details:
  • 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.


Primary Outcome Measures :
  1. 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".


Secondary Outcome Measures :
  1. 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.



Information from the National Library of Medicine

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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
All KFS patients who scheduled for surgery at BCH from June 2012 to June 2018.
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.

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03741790


Locations
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United States, Massachusetts
Boston children's hospital
Boston, Massachusetts, United States, 02115
Sponsors and Collaborators
Boston Children’s Hospital
Investigators
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Principal Investigator: Patcharee Sriswasdi, MD, MPH Boston Children’s Hospital

Publications:
Raj D, Luginbuehl I. Managing the difficult airway in the syndromic child. Continuing Education in Anaesthesia Critical Care & Pain. 2015;15(1):7-13.

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Responsible Party: Patcharee Sriswasdi, Attending staff, Boston Children’s Hospital
ClinicalTrials.gov Identifier: NCT03741790     History of Changes
Other Study ID Numbers: IRB-P00029640
First Posted: November 15, 2018    Key Record Dates
Last Update Posted: February 15, 2019
Last Verified: February 2019
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: Undecided
Plan Description: We might share individual participant data if it allows maximum use of the data.

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Patcharee Sriswasdi, Boston Children’s Hospital:
airway
intubation

Additional relevant MeSH terms:
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Syndrome
Klippel-Feil Syndrome
Disease
Pathologic Processes
Dysostoses
Bone Diseases, Developmental
Bone Diseases
Musculoskeletal Diseases
Musculoskeletal Abnormalities
Congenital Abnormalities