Airway Management of Pediatric Patients With Klippel-Feil Syndrome

NCT03741790 | Active Not Recruiting

• 1 other identifier: IRB-P00029640
• Study Type: observational
• Target: 300
• Locations: 1 country
• Sites: 1

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.

Conditions

Klippel-Feil Syndrome

Keywords

airway; intubation

Outcome Measures

Primary Outcomes (1)

• Prevalence of difficult airway cases among KFS is defined by using the ASA task force definition.

  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".

  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.

Secondary Outcomes (1)

• Specific anatomical abnormalities of KFS patients which related to difficult airway

  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

Interventions

Ventilation PROCEDURE

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.

Intubation PROCEDURE

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.

Eligibility Criteria

• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

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

You may qualify if:

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

You may not qualify if:

• Patients with incomplete or absent of medical records on AIMS such as ventilation and intubation technique are not clearly document.

Sponsors & Collaborators

• Boston Children's Hospital: lead

Study Sites (1)

Boston children's hospital

Boston, Massachusetts, 02115, United States

Location

Related Publications (8)

• Hase Y, Kamekura N, Fujisawa T, Fukushima K. Repeated anesthetic management for a patient with Klippel-Feil syndrome. Anesth Prog. 2014 Fall;61(3):103-6. doi: 10.2344/0003-3006-61.3.103.

  PMID: 25191983 BACKGROUND

• Altay N, Yuce HH, Aydogan H, Dorterler ME. Airway management in newborn with Klippel-Feil syndrome. Braz J Anesthesiol. 2016 Sep-Oct;66(5):551-3. doi: 10.1016/j.bjane.2014.03.006. Epub 2014 Apr 29.

  PMID: 27591474 BACKGROUND

• Stallmer ML, Vanaharam V, Mashour GA. Congenital cervical spine fusion and airway management: a case series of Klippel-Feil syndrome. J Clin Anesth. 2008 Sep;20(6):447-51. doi: 10.1016/j.jclinane.2008.04.009.

  PMID: 18929286 BACKGROUND

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

  BACKGROUND

• Khawaja OM, Reed JT, Shaefi S, Chitilian HV, Sandberg WS. Crisis resource management of the airway in a patient with Klippel-Feil syndrome, congenital deafness, and aortic dissection. Anesth Analg. 2009 Apr;108(4):1220-5. doi: 10.1213/ane.0b013e3181957d9b.

  PMID: 19299791 BACKGROUND

• Samartzis DD, Herman J, Lubicky JP, Shen FH. Classification of congenitally fused cervical patterns in Klippel-Feil patients: epidemiology and role in the development of cervical spine-related symptoms. Spine (Phila Pa 1976). 2006 Oct 1;31(21):E798-804. doi: 10.1097/01.brs.0000239222.36505.46.

  PMID: 17023841 BACKGROUND

• Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am. 1974 Sep;56(6):1246-53.

  PMID: 4436358 BACKGROUND

• Bakan M, Umutoglu T, Zengin SU, Topuz U. The success of direct laryngoscopy in children with Klippel-Feil Syndrome. Minerva Anestesiol. 2015 Dec;81(12):1384-6. Epub 2015 Sep 18. No abstract available.

  PMID: 26381356 BACKGROUND

MeSH Terms

Conditions

Klippel-Feil Syndrome

Interventions

Ventilation; Intubation

Condition Hierarchy (Ancestors)

Dysostoses; Bone Diseases, Developmental; Bone Diseases; Musculoskeletal Diseases; Musculoskeletal Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Intervention Hierarchy (Ancestors)

Environment, Controlled; Environment; Environment and Public Health; Therapeutics; Investigative Techniques

Study Officials

• Patcharee Sriswasdi, MD, MPH

  Boston Children's Hospital

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Attending staff

Study Record Dates

• First Submitted: August 29, 2018
• First Posted: November 15, 2018
• Study Start: November 1, 2018
• Primary Completion: June 30, 2019
• Study Completion (Estimated): December 31, 2027
• Last Updated: April 22, 2026

Record last verified: 2026-04

Locations

US (1)