Am J Otolaryngol xxx (xxxx) xxxx

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Am J Otolaryngol
journal homepage: www.elsevier.com/locate/amjoto

Differential diagnosis and management of adult-onset laryngomalacia

⁎
Grace M. Ferria, Yash Prakasha, Jessica R. Levib, Lauren F. Tracyb,
a
Boston University School of Medicine, 72 East Concord St, Boston, MA 02118, United States of America
b
Department of Otolaryngology - Head and Neck Surgery, Boston Medical Center, Boston University School of Medicine, 830 Harrison Ave, Boston, MA 02118, United
States of America

A R T I C LE I N FO A B S T R A C T

Keywords: Objective: Adult-onset laryngomalacia is a rare clinical entity that has been infrequently reported. This study
Laryngomalacia aims to evaluate the clinical presentation, diagnosis, and management of adult-onset laryngomalacia through
Stridor literature review and report of a case.
Epiglottic prolapse Methods: PubMed and Google Scholar databases were queried for articles published from 1960 to 2019 in-
Adult laryngomalacia
cluding only patients aged 18 years and older. Included keywords were: ‘laryngomalacia’, ‘adult laryngomalacia’,
Supraglottoplasty
‘acquired laryngomalacia’, ‘idiopathic laryngomalacia’, ‘laryngeal obstruction’, ‘floppy epiglottis’, ‘floppy epiglottis’, and
‘epiglottis prolapse’. Data extracted from literature included clinical presentation, diagnostic workup, surgical
management, and follow-up care.
Sources: PubMed and Google Scholar.
Results: A total of 21 articles reported 41 cases of adult-onset laryngomalacia. Within these cases, 5 etiologies
were identified: neurologic (n = 14), exercise-induced (n = 9), post-operative (n = 7), idiopathic (n = 7), and
age-related (n = 4) laryngomalacia. Anterior prolapse of arytenoids and aryepiglottic folds was the most
common laryngoscopic finding (n = 21), followed by posterior epiglottic prolapse (n = 20). Management in-
cluded supraglottoplasty (n = 14), epiglottidectomy (n = 8) or epiglottopexy (n = 2). Neurologic etiology
required tracheotomy more often than the other etiologies (n = 5, 36% vs. 15%). Three patients were managed
expectantly without surgical intervention and reported symptom resolution.
Conclusion: Adult laryngomalacia is a rare diagnosis comprising a spectrum of disease. This diagnosis may be
overlooked, but association with neurologic injury or trauma should encourage consideration. In comparison to
pediatric laryngomalacia, patients often require surgical intervention. Surgical decision is based on the direction
of supraglottic collapse, where supraglottoplasty and partial epiglottidectomy are effective interventions.
Level of evidence: N/A.

1. Introduction
Adult-onset laryngomalacia is an incompletely understood and
perhaps underdiagnosed clinical entity. The term laryngomalacia is most
frequently used in reference to pediatric patients; however, this con-
dition can affect adults in the absence of congenital anomaly.
During normal breathing, the epiglottis tilts anteriorly to enable
airflow into the larynx; in contrast, the supraglottic larynx in a patient
with laryngomalacia collapses on inspiration. This collapse decreases
airway diameter and can cause stridor and dyspnea. The differential
diagnosis for an adult presenting with stridor and dyspnea is broad,
encompassing conditions such as paradoxical vocal fold motion, lar-
yngeal stenosis, asthma, and the less commonly reported adult-onset
laryngomalacia. This study aims to assemble and analyze the reported
cases of adult laryngomalacia in an effort to improve understanding of
the clinical presentation, contributing factors, and treatment outcomes.
A case report of adult-onset laryngomalacia is also presented.
2. Methods
PubMed and Google Scholar searches were conducted with the
terms ‘laryngomalacia’, ‘adult laryngomalacia’, ‘acquired laryngomalacia’,
‘idiopathic laryngomalacia’, ‘laryngeal obstruction’, ‘floppy epiglottis’,
‘floppy epiglottis’, and ‘epiglottis prolapse’. Case reports or articles de-
scribing presentation and management of laryngomalacia in persons
aged 18 or above were included. No restrictions were applied to study

⁎
Corresponding author at: Boston Medical Center, Boston University School of Medicine, Department of Otolaryngology-Head and Neck Surgery, 800 Harrison
Avenue, BCD Building, 5th Floor, Boston, MA 02118, Unites States of America.
E-mail address: lauren.tracy@bmc.org (L.F. Tracy).

https://doi.org/10.1016/j.amjoto.2020.102469
Received 20 March 2020
0196-0709/ © 2020 Published by Elsevier Inc.

Please cite this article as: Grace M. Ferri, et al., Am J Otolaryngol, https://doi.org/10.1016/j.amjoto.2020.102469
G.M. Ferri, et al.
Fig. 1. Before (left) and after (right) supraglottoplasty during inspiration.
design, date of publication, or language. Articles were screened for the
following inclusion criterion: description of one or more adult patients
with laryngomalacia. Excluded reports included those describing a
clinical and pathophysiological presentation consistent with adult lar-
yngomalacia but without diagnosis. Title and abstract screening of
eligible publications was performed by three independent reviewers.
Data extracted included patient demographics, clinical presentation,
diagnostic modalities, surgical management, and post-operative out-
comes. Statistical analysis was performed using Microsoft Excel 2011
(Redmond, Washington). This study was exempt from Institutional
Board Review at Boston Medical Center.
3. Case report
A thirty-six-year-old female presented to laryngology clinic with
2–3 weeks of variable dyspnea, inspiratory stridor and throat irritation
worsened by inspiring or laying supine. Flexible laryngoscopy demon-
strated anteromedial prolapse of arytenoid cartilages and aryepiglottic
folds during inspiration (Fig. 1). Initial trial of respiratory retraining
therapy did not improve symptoms. CO2 laser supraglottoplasty was
then performed with symptom resolution. Six years after initial surgery,
the patient redeveloped stridor and shortness of breath. Repeat lar-
yngoscopy revealed recurrent inspiratory medial arytenoid collapse.
Treatment with respiratory retraining therapy resolved symptoms
without additional surgery.
4. Results
4.1. Patient demographics
Forty-one cases of adult laryngomalacia in 21 reports (26M:14F;
median 42 years old) were identified for review [1–19]. Most common
symptoms at presentation were inspiratory stridor (n = 26) and dys-
pnea (n = 11); other associated symptoms included dysphonia (n = 3),
throat irritation or cough (n = 2), and dysphagia (n = 2). Symptom
onset coincided with extubation in four patients and decannulation in
two patients. In all patients, diagnosis was made with awake fiberoptic
laryngoscopy demonstrating upper airway obstruction with prolapse of
supraglottic structures during inspiration.
4.2. Etiology
Adult-onset laryngomalacia was most commonly associated with
neurologic injury (n = 14). This etiology more often required
2
Am J Otolaryngol xxx (xxxx) xxxx
tracheotomy (n = 5, 36%) in comparison to other etiologies. Two pa-
tients had resolution of respiratory symptoms after initial neurologic
insult was managed.
Exercise-induced (n = 9) laryngomalacia was the second most fre-
quently described etiology. While 2/9 patients with exercise-induced
laryngomalacia experienced symptom resolution without surgery,
seven patients were treated surgically with CO2 laser epiglottidectomy
or supraglottoplasty. Among the 7 patients treated endoscopically,
symptoms resolved in 5 of 7; however, 2 of the 5 patients required
tracheotomy in addition to endoscopic laryngeal surgery.
Laryngomalacia following surgery or trauma, or post-operative
laryngomalacia, occurred in 7 patients. Of these cases, 4/7 patients
experienced delayed symptom onset 1.5 to 4 years after head and neck
surgery, including neck dissection and repair of laryngeal fracture. Six
of 7 patients reported symptom resolution following surgical manage-
ment with epiglottidectomy or supraglottoplasty. Less frequently re-
ported causes of adult laryngomalacia were idiopathic (n = 6) and age-
related (n = 4).
4.3. Assessment and treatment
Laryngoscopy detected anterior prolapse of arytenoids and ar-
yepiglottic folds (n = 21) and/or posterior epiglottic prolapse (n = 20).
Anterior prolapse of arytenoids and aryepiglottic folds was treated with
supraglottoplasty (n = 14), and posterior prolapse of the epiglottis was
managed with epiglottidectomy (n = 8) or epiglottopexy (n = 2).
Additional management strategies included tracheotomy (n = 10),
observation (n = 3) and total laryngectomy (n = 1) for severe, neu-
rologic-associated laryngomalacia.
5. Discussion
The diagnosis of adult-onset laryngomalacia requires first con-
sideration in the differential diagnosis and differentiation from similar
pathologic states. Awake fiberoptic laryngoscopy is the gold standard
for diagnosis and identifies the direction of supraglottic collapse, which
guides surgical management. The etiology of laryngomalacia does not
always dictate the direction of supraglottic collapse, but the majority of
patients with post-operative (71.4%) and neurologic (64.3%) lar-
yngomalacia demonstrated anteromedial arytenoid and aryepiglottic
prolapse. In contrast, the majority of patients with exercise-induced
(66.7%) and idiopathic laryngomalacia (85.7%) had posterior epiglottic
collapse.
Adult laryngomalacia of neurologic etiology is thought to be due to
G.M. Ferri, et al.
neuromuscular hypotonia after intracranial injury or neurologic dis-
ease. Cadaveric dissection performed by Belmont and Grundfast de-
termined that movement of the hyoglossus, digastric, palatophar-
yngeus, palatoglossus and intrinsic laryngeal muscles was responsible
for supraglottic support and dilation [20]. Weakening of the supporting
muscles of the larynx and pharynx may impair normal breathing in
neurologic etiology. Two cases of neurologic laryngomalacia resolved
after improvement of the initial neurologic insult. Therefore, clinicians
can consider primary treatment of neurologic injury if possible. Wiggs
et al. came to this conclusion in 1995 and advocated against surgical
intervention to correct laryngomalacia secondary to neurologic com-
promise.
Post-operative laryngomalacia resulted from intubation trauma,
laryngeal trauma or transcervical surgery. Woo et al. postulated that
post-operative epiglottic prolapse may result from damaging airway
support, compounded by a flow-related pull on the epiglottis [12]. Of
note is a case report by Kletzer et al., where a patient developed post-
operative laryngomalacia after surgery for bilateral vocal cord pa-
ralysis. Although supraglottoplasty improved stridor and symptoms, the
patient reported a decrease in vocal function attributable to the loss of
supraglottic tissue for “false fold phonation.” [6]
Exercise-induced laryngomalacia is characterized by dyspnea,
stridor and wheezing with exertion. Laryngoscopy during exercise is
necessary to distinguish exercise-induced laryngomalacia from asthma
or paradoxical vocal fold motion. Importantly, laryngomalacia and
asthma can coexist [4,15]. The severity of exercise needed to produce
symptoms is variable; therefore, diagnosis of exercise-induced lar-
yngomalacia requires modulation of physical intensity in accordance
with the stamina of the individual patient. Seven of the nine patients
underwent endoscopic surgery for exercise-induced laryngomalacia,
and recovered without requiring additional intervention. However, two
of those six patients underwent tracheotomy as well, which may be an
inappropriate intervention for a laryngomalacia that resolves with
termination of exercise.
Idiopathic laryngomalacia was associated with the following co-
morbidities: GERD, obstructive sleep apnea (OSA), or allergic rhinitis.
Although specific etiology could not be identified, GERD and allergic
rhinitis are speculated to contribute to supraglottic mucosal re-
dundancy and collapse. Management of idiopathic laryngomalacia in-
cluded surgery with medical treatment of comorbid disease to limit
recurrence.
Age-related laryngomalacia was reported in people over age 60
whose etiology could not be otherwise identified. The histology of age-
related laryngomalacia differs from that of other subcategories of lar-
yngomalacia and is characterized by a decreased fiber count in the
hyoepiglottic ligament [21]. Although the mechanism is unknown, the
presence of fewer fibers may result in a decrease in the tensile strength
of the hyoepiglottic ligament. The decrease in tension may limit the
ability of the epiglottis to 1) return to resting position after swallowing
and 2) induce supraglottic collapse.
This review summarizes the largest number of cases of adult-onset
laryngomalacia to date. However, this study is intrinsically limited by
the size of the patient population and the corresponding lack of pub-
lished reports in the literature. Data in this review is restricted by the
information presented within the assembled reports, which varies ac-
cording to the provider and country of practice. Moreover, case reports
are inherently biased in favor of rare presentations and cannot provide
generalizations about the patient population at large. Finally, failure to
recognize laryngomalacia in the adult population may have resulted in
misdiagnosis and the exclusion of a variety of cases from our review.
6. Conclusion
Adult laryngomalacia is a rare diagnosis comprising a wide
Am J Otolaryngol xxx (xxxx) xxxx
spectrum of disease. Although the diagnosis is frequently overlooked
within the adult population, association with neurologic injury, sur-
gery, exercise, comorbidity, or age should encourage consideration.
Adult patients more commonly require surgical intervention than pe-
diatric patients. Surgery is based on the direction of supraglottic col-
lapse, where partial epiglottidectomy and supraglottoplasty are effec-
tive interventions.
Declaration of competing interest
None.
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