1. Annals of Otology, Rhinology & Laryngology
1–6
© The Author(s) 2015
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DOI: 10.1177/0003489415582255
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Original Article
Introduction
Acquired subglottic stenosis (AS) may be attributable to
a multitude of etiologies, including systemic inflamma-
tory conditions, trauma, prolonged intubation, or prior
tracheostomy. In contrast, idiopathic subglottic and tra-
cheal stenosis (ISTS) has no attributable cause and is a
diagnosis of exclusion after other etiologies have been
ruled out. Although it has been suggested to be associated
with gastroesophageal reflux disease (GERD), hormonal
influences, respiratory tract infection, cough, and autoim-
mune disease, there is limited research to delineate a clear
etiology; its pathogenesis is solely speculative and likely
multifactorial.1-9
ISTS is a rare, slowly progressive inflammatory condi-
tion affecting the subglottis and first 2 tracheal rings.8,10
It is
predominately diagnosed in otherwise healthy women in the
third to fifth decade of life.5,8,11
Symptomatically, patients
present with dyspnea on exertion, shortness of breath,
hoarseness, stridor, or chronic cough. Frequently, patients
are treated for asthma without benefit prior to diagnosis.1,4,8
In a prior series, 33% to 37% of subglottic stenosis patients
werepreviouslymisdiagnosedwithasthma.12,13
Identification
of the stenosis can be made via flexible laryngoscopy, surgi-
cal endoscopy, or imaging studies. Initial treatment may be
conservative, including observation.5
Surgical treatment
typically consists of serial incision and dilations, mucosal
rotational flaps, and/or tracheal resection and reanastomo-
sis.3,5,8,10-15
Adjuvant therapies may include mitomycin C
582255AORXXX10.1177/0003489415582255Annals of Otology, Rhinology & LaryngologyGnagi et al
research-article2015
1
Department of Otolaryngology–Head and Neck Surgery, Mayo Clinic
Arizona, Phoenix, Arizona, USA
2
Inside Story, Sydney, NSW, Australia
Corresponding Author:
David G. Lott, Department of Otolaryngology–Head and Neck Surgery,
Mayo Clinic Arizona, 5777 East Mayo Boulevard, Phoenix, AZ 85054,
USA.
Email: lott.david@mayo.edu
Idiopathic Subglottic and Tracheal
Stenosis: A Survey of the
Patient Experience
Sharon H. Gnagi, MD1
, Brittany E. Howard, MD1
,
Catherine Anderson, BA, PGDip, QPMR2
, and David G. Lott, MD1
Abstract
Objectives: To report and compare patients’ experiences with acquired subglottic stenosis (AS) versus idiopathic
subglottic and tracheal stenosis (ISTS).
Methods: A survey was made available to patients with AS and ISTS. Results were analyzed for inter- and intragroup
differences using a 2-tailed t test.
Results: The study included 160 survey participants (AS n = 28; ISTS n = 132), with a predominance of female participants
(82% AS, 98% ISTS). Acid reflux was the most prevalent comorbidity across groups (42%-43%). A significant difference
in time to diagnosis was found between groups, with 32% of AS patients diagnosed within 3 months of symptom onset,
compared to 2% with ISTS. A diagnosis delay greater than 18 months occurred for 58% of ISTS patients. There was no
difference in treatment approach, with the most common treatment being balloon dilation, followed by laser dilation.
Tracheal resection was performed in 36% of patients in both groups. Patient satisfaction with surgical outcomes was
significantly higher after tracheal resection (76%) compared to other treatment modalities (39%).
Conclusions: ISTS remains a diagnostic challenge as highlighted by the delay in diagnosis compared to AS. There appears
to be no historical or symptomatic factors specific to ISTS. Additionally, patients report increased satisfaction and symptom
resolution after tracheal resection.
Keywords
idiopathic subglottic stenosis, laryngotracheal stenosis, patient experience

2. 2 Annals of Otology, Rhinology & Laryngology
application,8,12,15-17
intralesional steroid injection,8,12,13
cryo-
therapy,18
proton pump inhibitors,1,8,12,19
trimethoprim-sulfa-
methoxazole,12
or postoperative inhaled steroid use.12
Airway stenosis can be a significantly debilitating disease.
Patients often find themselves living life from breath to
breath, wondering when they will need to undergo their next
treatment. Studies on the disease to this point have been from
the physician/scientist perspective and have not been defini-
tive. We postulated that a survey of patients with airway ste-
nosis might discover new points that could further guide
investigation or treatment. This article reviews the patients’
perspectives of their disease process and experiences with
treatment. The primary intent is to present a patient-centered
survey designed by a patient to seek commonalities in the
largest multinational cohort ever studied for this disease. The
manuscript is not meant to define causative factors for idio-
pathic subglottic stenosis but rather to elucidate any trends
that can be further investigated and provide clinicians with
the patients’ perspectives of their disease.
Methods
A survey was made available to patients internationally with
either acquired or idiopathic subglottic stenosis via the
Internet. Treating clinicians and institutions were purposefully
not involved in the administration or collection of the survey
other than to notify patients it was available in an attempt not
to bias the results. The goal of the survey was to provide phy-
sicians with the patient’s perspective of this condition as well
as contribute to the body of knowledge of this disease. This
research was Institutional Review Board exempt.
The survey was developed by a patient with subglottic
stenosis and encompassed questions regarding demograph-
ics, symptomatology, diagnosis, associated conditions,
treatment, and response to treatment. Given the various
backgrounds and cultures of the study subjects, questions
were created to allow for a broad range of answers so any
potential association would not be missed. Results were ana-
lyzed for inter- and intragroup differences using a 2-tailed t
test. A P value <.05 was considered statistically significant.
Results
Demographics
A total of 160 people participated in the study. The ISTS
group consisted of 132 people. There were 28 people in the
AS group. More than 95% of the participants completed the
survey via referral from various Internet-based support
groups, with less than 5% completing the survey as a result
of a referral from a treating clinician. Most participants
were from North America (83%) but included Europe
(13%), Australia/Oceania (3%), and South America (1%).
The ISTS group was comprised of 98% women and 2%
men. The AS group included 82% women and 18% men.
The most common age at diagnosis for ISTS was between
41 and 50 (28%), followed by 21 to 30 (26%). The most
common age at diagnosis for the AS patients was between
31 and 40 (32%) (Table 1).
Associated Symptoms
Fluctuations in difficulty breathing are not consistent across
patients. Twenty-one percent state that the morning is
worse. However, 37% describe no change in respiratory
symptoms over the course of a day. Coughing and mucus is
the most common associated symptom, with 91% com-
plaining of this.
Comorbid Conditions
Allowing for a write-in response in the survey, no comorbid
conditions or familial diseases were significantly more
prominent in either group (Figure 1). These were defined as
Table 1. Demographics for Patients With Acquired Subglottic Stenosis (AS) and Idiopathic Subglottic And Tracheal Stenosis (ISTS).
Gender (%)
Male Female
ISTS 2 98
AS 18 82
Age at Survey (%)
Under 20 21-30 31-40 41-50 51-60 61-70 Over 70
ISTS 1 14 19 33 23 9 1
AS 0 11 25 18 25 11 4
Age at Diagnosis (%)
Under 20 21-30 31-40 41-50 51-60 61-70 Over 70
ISTS 2 26 23 28 18 2 1
AS 7 4 32 29 7 7 0

3. Gnagi et al 3
either patient perception of symptoms or clinical diagnosis.
Acid reflux was the most common comorbid condition in
each group but was not significantly different between the
groups (43% ISTS, 42% AS). The majority of patients did
not have allergies or intolerances (63% ISTS, 57% AS).
Specific allergy differences did not achieve significance
(Figure 2).
Hormonal Alteration
Overall, more ISTS patients have had some sort of hormone
therapy and have used oral contraceptives more frequently
than AS (Figure 3). However, once the number of males in
the AS group were taken into account, there was no signifi-
cant difference. There was no difference between the type
of hormone replacement or length of treatment.
Diagnosis
One in 3 patients (32%) with AS were diagnosed within 3
months of symptom onset, compared to 2% in the ISTS
group (P < .05). A majority of the ISTS patients (58%) were
not diagnosed for more than 18 months, with 45% not diag-
nosed for more than 2 years after symptom onset (Figure 4).
Treatment
Otolaryngology physicians are by far the most common
physicians treating this disorder for both groups (61% ISTS,
67% AS). Thoracic surgeons managed 26% of ISTS and
23% of AS patients in this survey. Balloon dilation is the
most common treatment, followed by laser dilation in both
groups (Figure 5). Tracheal resection was performed in
36% of both ISTS and AS patients. More AS patients (21%)
had received no treatment at the time of the survey com-
pared to the ISTS group (6%).
0 10 20 30 40 50
Thyroid condition
Heart condition
Diabetes
Hypercholesterolemia
Hiatal hernia
Acid Reflux
None
Other
Percentage of Patients
Idiopathic Subglottic Stenosis
Acquired Subglottic Stenosis
Figure 1. Comorbid conditions.
0 10 20 30 40 50 60 70
Dairy intolerance
Gluten intolerance
Lactose intolerance
Soy intolerance
Dairy allergy
Egg allergy
Other
Unsure
No
Yes
Percentage of Patients
Idiopathic Subglottic Stenosis
Acquired Subglottic Stenosis
Figure 2. Allergies/intolerances.
0 10 20 30 40 50 60 70
Oral Contraceptive
Hormone Replacement Therapy
Estrogen for Postmenopausal
Therapy
Contraceptive Skin Implant
None
Other
Percentage of Patients
Idiopathic Subglottic Stenosis
Acquired Subglottic Stenosis
Figure 3. Hormonal alteration.

4. 4 Annals of Otology, Rhinology & Laryngology
Tracheal Resection Outcomes
Within the ISTS group, 34 patients had a tracheal resection.
Following resection, 47% of ISTS patients had complete
resolution of respiratory symptoms at the time of the survey
(Figure 6). Length of time after resection was not taken into
account. An additional 29% classified their breathing as
mostly normal. Therefore, 76% of patients classified their
breathing as essentially normal following resection. When
compared to ISTS patients who have not had a resection,
this demonstrated a significant improvement in respiratory
symptoms (resection 76%, no resection 39%). This benefit
decreases as exercise becomes more strenuous. Forty-seven
percent of patients classify themselves as essentially normal
with strenuous exercise after resection.
Discussion
The management of ISTS can be frustrating for both
patients and physicians. Compounding this frustration is
the lack of significant research findings that elucidate any
potential etiologies. This survey was created to seek com-
monalities in the largest multinational cohort ever studied
for this disease. The intent of the study was not to define
causative factors for idiopathic subglottic stenosis but
rather to collect a broad, inclusive experience from a large
and diverse patient cohort. As such, specificity was sacri-
ficed for inclusivity. These findings may then be used to
further investigate potential trends, provide clinicians with
insight into the patient’s perspective, or council new
patients based on shared experiences with others suffering
from this disease.
The survey method of data collection subjects the data in
this study to voluntary and nonresponse biases. Patient self-
reporting may lead to inconsistencies in terminology and
misunderstanding of diagnoses or treatment modalities.
Idiopathic cases may be overrepresented secondary to undi-
agnosed conditions, such as limited Wegener’s granuloma-
tosis. In addition, all respondents were at different stages of
treatment and follow-up, potentially misrepresenting the
efficacy of various treatments. Similarly, the methodology
of treatments, degree of stenosis at time of treatment, and
adjuvant therapies may not have been accurately reported,
thus further complicating the interpretation of treatment
response. The multinational nature of this patient cohort
may cause further misrepresentations of the reported data
due to cultural and medical management differences.
Despite these potential inconsistencies, the data presented
from this survey do contribute to the fund of knowledge of
this rare and minimally studied disease and did reveal some
trends that may warrant further investigation.
Demographically, this survey is consistent with prior
studies that demonstrate a female predominance among
patients with ISTS in a similar age group. Interestingly, the
AS group had a higher proportion of females than what is
typically reported. We postulate that this is likely secondary
to the higher female involvement in online support groups
for these conditions.
0
10
20
30
40
50
60
PercentageofPatients
Acquired Subglottic Stenosis
Idiopathic Subglottic Stenosis
Figure 5. Treatments received.
0 to 1 No to slight stridor; mostly to completely normal breathing
2 to 3 More to regular stridor and cough
4 to 5 Regular to constant stridor and cough
0
20
40
60
80
100
0 to 1 2 to 3 4 to 5
PercentageofPatients
Self Rating
Resection
No Resection
Figure 6. Response to treatment.
0
10
20
30
40
50
0-3 mo 4-6 mo 7-12 mo 12-18 mo 18-24 mo Over 2 yrs
PercentageofPatients Acquired Subglottic Stenosis
Idiopathic Subglottic Stenosis
Figure 4. Time to diagnosis.

5. Gnagi et al 5
The higher female predominance in the ISTS group
continues to question the possible hormonal role or estro-
gen effect in the etiology of ISTS. Although prior studies
have failed to demonstrate estrogen receptors in stenotic
lesions,8,10,11
the role of estrogen in wound healing draws
attention to its questionable contribution in this dis-
ease.8,20
In this survey, both acquired and idiopathic
patients were equally likely to have taken hormones, sug-
gesting that supplemental hormone usage does not play a
role in ISTS. However, the specifics of hormonal altera-
tion were not addressed and may be an area to further
investigate. It is important to highlight that this study
only asked about supplemental hormone usage, and the
fact that there was not a significant difference between
the ISTS and the AS groups does not exclude the role of
other hormonal or autoimmune causes. Fourteen percent
of patients stated that their physician had tested their hor-
mone levels in relation to their ISTS. Perhaps this shows
an increased awareness of the need for further investiga-
tion in this regard.
There was no statistically significant difference in his-
torical or symptomatic factors specific to ISTS when com-
pared to AS, including other medical conditions, allergies,
or intolerances. Both groups had a similar incidence of self-
reported or diagnosed GERD, which may suggest a poten-
tial role in the development of both ISTS and AS. However,
secondary to its equal presence in both disease states, it
decreases the likelihood that it is an inciting factor specific
to ISTS.
The nonspecific presentation of symptoms without a his-
tory of possible airway trauma likely explains the delay in
diagnosis of over 18 months for the majority of ISTS
patients, in comparison to a less than 3-month diagnosis in
a third of AS patients. This finding is consistent with prior
reports showing duration of symptoms ranging from an
average of 10 to 31 months prior to diagnosis.4,5,11
In fact,
Loutsidis et al21
proposed an average duration of symptoms
of 2 years as useful criteria in diagnosis. This highlights the
importance of ruling out subglottic stenosis in any middle-
aged woman with complaints of wheezing, chronic cough,
dyspnea on exertion, or shortness of breath.
Most patients were treated with either serial dilations
or tracheal resection. When treatment is necessary, dila-
tion is convenient, may be performed in an outpatient set-
ting, and avoids a skin incision. However, dilation is
typically associated with short-term improvement and a
recurrence rate of at least 60% in 5 years.12
An average of
approximately 1 to 2 procedures per patient per year has
been previously reported.13
Given the need for multiple
anesthetics and repeated dilation procedures, a tracheal
resection should be a serious and possibly earlier consid-
eration in patients presenting with ISTS. While open
resection and reanastamosis has a higher risk, longer hos-
pitalization, and longer recovery, it has been shown to be
a more definitive treatment with excellent long-term
results.22-24
In our study, when compared to patients
treated with dilations, patients receiving tracheal resec-
tion reported significantly improved breathing. Seventy-
six percent of resection patients rated their breathing as
mostly normal or better.
Conclusion
ISTS remains a diagnostic and therapeutic challenge for
physicians as highlighted by a significant delay in diagno-
sis. It is an important consideration in the differential diag-
nosis of chronic cough or dyspnea, especially in young to
middle-aged women. Little is known about the etiology of
ISTS, and this study did not reveal any statistically signifi-
cant differences between historical or symptomatic factors
specific to ISTS or AS. Etiologic factors previously pro-
posed, including GERD and hormonal influences, have not
been proven, and further research in this field is needed to
elucidate causative factors and potential targeted therapies.
Additionally, patients report increased satisfaction and
symptom resolution after tracheal resection when com-
pared to serial dilations. Therefore, it might be prudent to
make resection an earlier consideration in ISTS patients as
they often require multiple operative procedures over their
lifetimes.
Authors’ Note
This was a poster presentation at the American Bronchoesoph-
agological Association at the Combined Otolaryngology Spring
Meeting April 11, 2013, in Orlando, Florida.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
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