Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis

Alexander Gelbard, Donald T. Donovan, Julina Ongkasuwan, S. A R Nouraei, Guri Sandhu, Michael S. Benninger, Paul C. Bryson, Robert R. Lorenz, William S. Tierney, Alexander T. Hillel, Shekhar K. Gadkaree, David G. Lott, Eric S. Edell, Dale C. Ekbom, Jan L. Kasperbauer, Fabien Maldonado, Joshua Schindler, Marshall E. Smith, James J. Daniero, C. Gaelyn Garrett & 5 others James L. Netterville, Otis B. Rickman, Robert J. Sinard, Christopher T. Wootten, David O. Francis

Research output: Contribution to journalArticle

29 Citations (Scopus)

Abstract

Objectives/Hypothesis 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. Study Design Medical record abstraction. Methods 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. Results 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 (chi2 = 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. Conclusion 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.

Original languageEnglish (US)
Pages (from-to)1390-1396
Number of pages7
JournalLaryngoscope
Volume126
Issue number6
DOIs
StatePublished - Jun 1 2016

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Pathologic Constriction
Confidence Intervals
Recurrence
Tracheostomy
Therapeutics
Airway Obstruction
Natural History
Medical Records
Comorbidity

Keywords

  • comparative effectiveness
  • cricotracheal
  • dilation
  • Idiopathic subglottic stenosis
  • tracheostomy

ASJC Scopus subject areas

  • Otorhinolaryngology

Cite this

Gelbard, A., Donovan, D. T., Ongkasuwan, J., Nouraei, S. A. R., Sandhu, G., Benninger, M. S., ... Francis, D. O. (2016). Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis. Laryngoscope, 126(6), 1390-1396. https://doi.org/10.1002/lary.25708

Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis. / Gelbard, Alexander; Donovan, Donald T.; Ongkasuwan, Julina; Nouraei, S. A R; Sandhu, Guri; Benninger, Michael S.; Bryson, Paul C.; Lorenz, Robert R.; Tierney, William S.; Hillel, Alexander T.; Gadkaree, Shekhar K.; Lott, David G.; Edell, Eric S.; Ekbom, Dale C.; Kasperbauer, Jan L.; Maldonado, Fabien; Schindler, Joshua; Smith, Marshall E.; Daniero, James J.; Garrett, C. Gaelyn; Netterville, James L.; Rickman, Otis B.; Sinard, Robert J.; Wootten, Christopher T.; Francis, David O.

In: Laryngoscope, Vol. 126, No. 6, 01.06.2016, p. 1390-1396.

Research output: Contribution to journalArticle

Gelbard, A, Donovan, DT, Ongkasuwan, J, Nouraei, SAR, Sandhu, G, Benninger, MS, Bryson, PC, Lorenz, RR, Tierney, WS, Hillel, AT, Gadkaree, SK, Lott, DG, Edell, ES, Ekbom, DC, Kasperbauer, JL, Maldonado, F, Schindler, J, Smith, ME, Daniero, JJ, Garrett, CG, Netterville, JL, Rickman, OB, Sinard, RJ, Wootten, CT & Francis, DO 2016, 'Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis', Laryngoscope, vol. 126, no. 6, pp. 1390-1396. https://doi.org/10.1002/lary.25708
Gelbard A, Donovan DT, Ongkasuwan J, Nouraei SAR, Sandhu G, Benninger MS et al. Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis. Laryngoscope. 2016 Jun 1;126(6):1390-1396. https://doi.org/10.1002/lary.25708
Gelbard, Alexander ; Donovan, Donald T. ; Ongkasuwan, Julina ; Nouraei, S. A R ; Sandhu, Guri ; Benninger, Michael S. ; Bryson, Paul C. ; Lorenz, Robert R. ; Tierney, William S. ; Hillel, Alexander T. ; Gadkaree, Shekhar K. ; Lott, David G. ; Edell, Eric S. ; Ekbom, Dale C. ; Kasperbauer, Jan L. ; Maldonado, Fabien ; Schindler, Joshua ; Smith, Marshall E. ; Daniero, James J. ; Garrett, C. Gaelyn ; Netterville, James L. ; Rickman, Otis B. ; Sinard, Robert J. ; Wootten, Christopher T. ; Francis, David O. / Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis. In: Laryngoscope. 2016 ; Vol. 126, No. 6. pp. 1390-1396.
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abstract = "Objectives/Hypothesis 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. Study Design Medical record abstraction. Methods 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. Results 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 (chi2 = 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. Conclusion 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.",
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T1 - Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis

AU - Gelbard, Alexander

AU - Donovan, Donald T.

AU - Ongkasuwan, Julina

AU - Nouraei, S. A R

AU - Sandhu, Guri

AU - Benninger, Michael S.

AU - Bryson, Paul C.

AU - Lorenz, Robert R.

AU - Tierney, William S.

AU - Hillel, Alexander T.

AU - Gadkaree, Shekhar K.

AU - Lott, David G.

AU - Edell, Eric S.

AU - Ekbom, Dale C.

AU - Kasperbauer, Jan L.

AU - Maldonado, Fabien

AU - Schindler, Joshua

AU - Smith, Marshall E.

AU - Daniero, James J.

AU - Garrett, C. Gaelyn

AU - Netterville, James L.

AU - Rickman, Otis B.

AU - Sinard, Robert J.

AU - Wootten, Christopher T.

AU - Francis, David O.

PY - 2016/6/1

Y1 - 2016/6/1

N2 - Objectives/Hypothesis 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. Study Design Medical record abstraction. Methods 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. Results 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 (chi2 = 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. Conclusion 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.

AB - Objectives/Hypothesis 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. Study Design Medical record abstraction. Methods 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. Results 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 (chi2 = 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. Conclusion 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.

KW - comparative effectiveness

KW - cricotracheal

KW - dilation

KW - Idiopathic subglottic stenosis

KW - tracheostomy

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