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Disease homogeneity and treatment heterogeneity in idiopathic subglottic stenosis
Author(s) -
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 S.,
Smith Marshall E.,
Daniero James J.,
Garrett C. Gaelyn,
Netterville James L.,
Rickman Otis B.,
Sinard Robert J.,
Wootten Christopher T.,
Francis David O.
Publication year - 2016
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.25708
Subject(s) - medicine , confidence interval , natural history , subglottic stenosis , etiology , comorbidity , retrospective cohort study , disease , hazard ratio , surgery , airway
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 (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. 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. Level of Evidence 4. Laryngoscope , 126:1390–1396, 2016