title: Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis.
abstract: Idiopathic subglottic stenosis (iSGS) is a rare and potentially life-threatening disease marked by recurrent and progressive airway obstruction frequently requiring repeated surgery to stabilize the airway. Unknown etiology and low disease prevalence have limited the ability to characterize the natural history of iSGS and resulted in variability in surgical management. It is uncertain how this variation relates to clinical outcomes.
  
  
  
  Medical record abstraction.
  
  
  
  Utilizing an international, multi-institutional collaborative, we collected retrospective data on patient characteristics, treatment, and clinical outcomes. We investigated variation between and within open and endoscopic treatment approaches and assessed therapeutic outcomes; specifically, disease recurrence and need for tracheostomy at last follow-up.
  
  
  
  Strikingly, 479 iSGS patients across 10 participating centers were nearly exclusively female (98%, 95% confidence interval [CI], 96.1-99.6), Caucasian (95%, 95% CI, 92.2-98.8), and otherwise healthy (mean age-adjusted Charlson Comorbidity Index 1.5; 95% CI, 1.44-1.69). The patients presented at a mean age of 50 years (95% CI, 48.8-51.1). A total of 80.2% were managed endoscopically, whereas 19.8% underwent open reconstruction. Endoscopic surgery had a significantly higher rate of disease recurrence than the open approach (chi(2) = 4.09, P = 0.043). Tracheostomy was avoided in 97% of patients irrespective of surgical approach (95% CI, 94.5-99.8). Interestingly, there were outliers in rates of disease recurrence between centers using similar treatment approaches.
  
  
  
  Idiopathic subglottic stenosis patients are surprisingly homogeneous. The heterogeneity of treatment approaches and the observed outliers in disease recurrence rates between centers raises the potential for improved clinical outcomes through a detailed understanding of the processes of care.
  
  
  
  4. Laryngoscope, 126:1390-1396, 2016.
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: Alexander
authors.identifier: 0000-0003-0078-1305
authors.initials: A
authors.lastname: Gelbard
authors.affiliation: Baylor College of Medicine Dept. of Otolaryngology, Houston, Texas.
authors.forename: Donald T
authors.initials: DT
authors.lastname: Donovan
authors.affiliation: Baylor College of Medicine Dept. of Otolaryngology, Houston, Texas.
authors.forename: Julina
authors.initials: J
authors.lastname: Ongkasuwan
authors.affiliation: Imperial College Healthcare NHS Trust Dept. of Otolaryngology, London, United Kingdom.
authors.forename: S A R
authors.initials: SA
authors.lastname: Nouraei
authors.affiliation: Imperial College Healthcare NHS Trust Dept. of Otolaryngology, London, United Kingdom.
authors.forename: Guri
authors.initials: G
authors.lastname: Sandhu
authors.affiliation: Cleveland Clinic Dept. of Otolaryngology, Cleveland, Ohio.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Benninger
authors.affiliation: Cleveland Clinic Dept. of Otolaryngology, Cleveland, Ohio.
authors.forename: Paul C
authors.initials: PC
authors.lastname: Bryson
authors.affiliation: Cleveland Clinic Dept. of Otolaryngology, Cleveland, Ohio.
authors.forename: Robert R
authors.initials: RR
authors.lastname: Lorenz
authors.affiliation: Cleveland Clinic Dept. of Otolaryngology, Cleveland, Ohio.
authors.forename: William S
authors.initials: WS
authors.lastname: Tierney
authors.affiliation: Johns Hopkins University Dept. of Otolaryngology, Baltimore, Maryland.
authors.forename: Alexander T
authors.initials: AT
authors.lastname: Hillel
authors.affiliation: Johns Hopkins University Dept. of Otolaryngology, Baltimore, Maryland.
authors.forename: Shekhar K
authors.initials: SK
authors.lastname: Gadkaree
authors.affiliation: Mayo Clinic Dept. of Otolaryngology and Pulmonology, Rochester, Minnesota.
authors.forename: David G
authors.initials: DG
authors.lastname: Lott
authors.affiliation: Mayo Clinic Dept. of Otolaryngology and Pulmonology, Rochester, Minnesota.
authors.forename: Eric S
authors.initials: ES
authors.lastname: Edell
authors.affiliation: Mayo Clinic Dept. of Otolaryngology and Pulmonology, Rochester, Minnesota.
authors.forename: Dale C
authors.initials: DC
authors.lastname: Ekbom
authors.affiliation: Mayo Clinic Dept. of Otolaryngology and Pulmonology, Rochester, Minnesota.
authors.forename: Jan L
authors.initials: JL
authors.lastname: Kasperbauer
authors.affiliation: Mayo Clinic Dept. of Otolaryngology and Pulmonology, Rochester, Minnesota.
authors.forename: Fabien
authors.initials: F
authors.lastname: Maldonado
authors.affiliation: Oregon Health and Science University Dept. of Otolaryngology, Portland, Oregon.
authors.forename: Joshua S
authors.initials: JS
authors.lastname: Schindler
authors.affiliation: University of Utah School of Medicine Dept. of Otolaryngology, Salt Lake City, Utah.
authors.forename: Marshall E
authors.initials: ME
authors.lastname: Smith
authors.affiliation: University of Virginia Health System Dept. of Otolaryngology, Charlottesville, Virginia, U.S.A.
authors.forename: James J
authors.initials: JJ
authors.lastname: Daniero
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: C Gaelyn
authors.initials: CG
authors.lastname: Garrett
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: James L
authors.initials: JL
authors.lastname: Netterville
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: Otis B
authors.initials: OB
authors.lastname: Rickman
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Sinard
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: Christopher T
authors.initials: CT
authors.lastname: Wootten
authors.affiliation: Vanderbilt University Medical Center Dept. of Otolaryngology and Pulmonology, Nashville, Tennessee.
authors.forename: David O
authors.initials: DO
authors.lastname: Francis
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007828:Laryngoscopy / Q000379:methods / Q000706:statistics & numerical data*; D007829:Laryngostenosis / Q000150:complications / Q000473:pathology / Q000601:surgery*; D007830:Larynx / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D014139:Tracheostomy / Q000706:statistics & numerical data*; D016896:Treatment Outcome
keywords: Idiopathic subglottic stenosis; comparative effectiveness; cricotracheal; dilation; tracheostomy