Annals of Otology, Rhinology & Laryngology 121(1):57-60.

2012 Annals Publishing Company. All rights reserved.

Congenital Laryngomalacia: Symptom Duration and

Need for Surgical Intervention
Charles T. Wright, MD, MBA; Steven L. Goudy, MD

Objectives: We undertook to characterize the natural course and duration of stridor and other symptoms associated with
laryngomalacia and determine the need for surgical intervention.
Methods: A retrospective observational study was performed at a tertiary childrens hospital of 120 consecutive cases of
congenital laryngomalacia diagnosed and treated by the senior author between July 2005 and June 2009. The patients
symptoms, associated symptoms, and required interventions were recorded.
Results: In 115 cases that were managed without surgery, stridor resolved at a mean patient age of 7.6 months. Five pa-
tients (4.2%) required supraglottoplasty to resolve their airway obstruction. The patients who required surgery presented
emergently to the hospital 80% of the time, and at a younger mean age (45 days) than did patients who did not require
surgery (95 days; p = 0.13); these findings suggest the severe nature of their symptoms. Presenting symptoms of dyspnea
or accessory muscle use, feeding difficulties, apnea, cyanosis, oxygen desaturation, and failure to thrive were signifi-
cantly associated with the requirement for operative intervention (all p values less than 0.02). Nonoperative management
included placement of a nasogastric tube due to aspiration in 3 patients.
Conclusions: The stridor resolved at an average age of 7.6 months of age in patients with laryngomalacia managed with-
out surgery. A young age at presentation and emergent evaluation in the hospital are associated with a higher degree of
symptom severity and a higher rate of surgical intervention. Surgical intervention was necessary to treat laryngomalacia
in 4.2% of patients in this study population.
Key Words: laryngomalacia, outcome, stridor.

Introduction between 11.6% and 31% of patients will require sur-

Laryngomalacia is the most common congeni-
tal laryngeal anomaly. It is the number one cause
of stridor in infants and accounts for up to 75% of
all cases.1 The characteristic inspiratory stridor re-
sults when collapse of the supraglottic larynx dur-
ing inspiration leads to a narrowed airway and tur-
bulent airflow. Although various etiologic theories
exist,2-4 the exact cause of laryngomalacia remains
unknown.
Descriptions of the symptom course have evolved
since the first series of 18 patients was reported by
Sutherland and Lack5 in an 1897 Lancet article. Stri-
dor and other associated symptoms generally begin
at birth or within the first few weeks of life.2,3,6,7
Previous authors have observed a symptom course
that worsens initially, peaks, and enters a plateau
phase between 6 and 9 months, and then begins to
spontaneously resolve.1,5,8 Although variability ex-
ists in the reported length of time until resolution,
authors agree that most infants have mild symptoms
that follow a benign course and resolve within 12 to
24 months.2,3,6,7 However, the literature reports that
From the Department of OtolaryngologyHead and Neck Surgery, Vanderbilt University Medical Center, Nashville, Tennessee.
Correspondence: Steven L. Goudy, MD, 2200 Childrens Way, Doctors Office Tower, 7th Floor, Vanderbilt University Medical Cen-
ter, Nashville, TN 37232.
gical intervention for more severe disease.2,7,9,10 We
hypothesized that stridor resolves before 18 to 24
months of age and that fewer than 10% of patients
require surgical intervention.
Methods
After approval was given by the Vanderbilt Uni-
versity Institutional Review Board, data were retro-
spectively reviewed on all patients treated for con-
genital laryngomalacia consecutively between July
2005 and June 2009 at Vanderbilt University Chil-
drens Hospital. All cases of congenital laryngoma
lacia were included for review, and cases of ac-
quired, exercise-induced, or late-onset laryngoma
lacia were excluded.
All patient data were documented in a similar
fashion, including all patient demographics, consul-
tations, hospital admissions, medical treatment, and
the need for surgical intervention for laryngoma
lacia. Patients admitted to the hospital for concern
of an apparent life-threatening event (ALTE) were
monitored by telemetry, and patients found to have

57
58 Wright & Goudy, Congenital Laryngomalacia 58
Table 1. Laryngomalacia Patient Summary
Patients With
Patients With Surgical
Observation Intervention
(N = 115) (N = 5)
Sex
Male 69 (60%) 4 (80%)
Female 46 (40%) 1 (20%)
Mean age at onset of 9.9 (birth to 152) 2.3 (birth to 8)
symptoms (d)
Mean age at diagnosis (d) 95.4 (2 to 511) 45.0 (2 to 87)
Location of initial evaluation
by otolaryngologist
Clinic 86 (75%) 1 (20%)
Emergency department 29 (25%) 4 (80%)*
or inpatient
Flexible endoscopy findings
Arytenoid prolapse 102 (89%) 5 (100%)
Omega-shaped epiglottis 75 (65%) 5 (100%)
Shortened aryepiglottic folds 53 (46%) 4 (80%)
Two or more anatomic sites 82 (71%) 5 (100%)
of collapse
*p = 0.02 by Fishers exact test.
cyanotic events were considered surgical candi-
dates. After the diagnosis of laryngomalacia by flex-
ible laryngoscopy, the patients were seen at 1-month
and 3-month visits in the office. The site of supra-
glottic obstruction was recorded in all patients by
means of flexible laryngoscopy and according to the
proposed classification scheme of Olney et al.7 Rou-
tine airway endoscopy was not performed for isolat-
ed laryngomalacia unless the patient had significant
worsening of symptoms or developed new symp-
toms not related to the laryngomalacia (eg, recurrent
croup). Routine radiologic films to evaluate tracheal
or pulmonary problems were not obtained. Patients
who were managed without surgery were com-
pared to patients who required surgical intervention.
Those patients who were managed without surgery
used feeding strategies, including the use of higher-
calorie formulas with smaller volumes, side-lying
or prone positioning, and weekly weight checks by
their doctor. Stridor resolution was defined as an ab-
sence of stridor and retractions on examination by
the senior author (S.L.G.), in addition to a paren-
tal report of complete stridor resolution (both at rest
and during crying). All supraglottoplasty procedures
in this study were performed with a carbon dioxide
laser on a setting of 6 W in suprapulse mode to di-
vide the aryepiglottic folds and remove redundant
arytenoid mucosa bilaterally. Statistical compari-
sons were made with a 2 test, Wilcoxon rank test,
or Fishers exact test, as appropriate. All p values
of less than 0.05 were considered statistically sig-
nificant.
Table 2. Associated Conditions and
Presenting Symptoms
Patients With
Patients With Surgical
Observation Intervention
(N = 115) (N = 5) p*
Prematurity 13 (12%) 0
GERD or LPR, total 60 (52%) 4 (80%)
pH probe or impedance 17 (28%) 4 (100%) 0.009
probe
Surgical correction 0 2 (50%) <0.005
(Nissen/Thal)
Other comorbid conditions, 7 (6%) 1 (25%)
total
Neurologic 2 0
Cardiac 3 0
Genetic or syndromic 3 0
Craniofacial abnormalities 2 0
Other 0 1
Presenting symptoms
Stridor 115 (100%) 5 (100%)
Dyspnea or accessory 21 (18.3%) 4 (80%) 0.007
muscle use
Feeding difficulties 14 (12.2%) 5 (100%) <0.005
Cyanotic spells 14 (12.2%) 3 (60%) 0.02
Apnea 12 (10.4%) 3 (60%) 0.014
Oxygen desaturation 5 (4.3%) 4 (80%) <0.005
Apparent life-threatening 5 (4.3%) 1 (20%)
event
Failure to thrive 1 (1%) 2 (40%) <0.005
Pectus excavatum 1 (1%) 0
Pulmonary hypertension 0 0
Cor pulmonale 0 0
Recurrent pulmonary 0 0
infections
*All p values were calculated with Fishers exact test.
GERD gastroesophageal reflux disease; LPR laryngopharyn-
geal reflux.
Results
One hundred twenty patients were identified who
met the study criteria. One hundred fifteen patients
were managed without surgery for stridor and oth-
er symptoms associated with laryngomalacia (Table
1). The remaining 5 patients in the study underwent
supraglottoplasty to address their laryngomalacia.
Table 2 lists the presenting symptoms and associ-
ated conditions found in the patients with laryn-
gomalacia. Syndromic conditions were found in 3
patients who had associated cardiac abnormalities
(DiGeorge, Downs, and Williams syndromes). In the
patients managed without surgery, additional mea-
sures beyond simple watchful waiting included the
use of higher-calorie formulas in 5 patients and use
of temporary nasogastric tube feedings in 3 patients
after aspiration was noted on modified barium swal-
low study. Additionally, 17 patients were provided
59 Wright & Goudy, Congenital Laryngomalacia 59
home apnea and/or pulse oximetry monitors when
prone positioning strategies were recommended to
ensure that apneic or hypoxic events were not occur-
ring. A review of family reports of stridor resolution
found that the average age at stridor resolution was
7.6 months, although in some cases it did resolve as
early as 4 months, and by 9 months, stridor had re-
solved in 93% of cases.
Five patients (4.2%) underwent supraglottoplas
ty. The average age at surgical intervention was 53
days. The surgical indications included feeding dif-
ficulties leading to failure to thrive (FTT) in 1 pa-
tient and the combination of life-threatening apnea,
cyanosis, and oxygen desaturation in 4 patients. Pa-
tients who were admitted to the hospital on telem-
etry had documentation of their cyanotic and apne-
ic events as indications for surgery. There were no
adverse surgical events or postoperative complica-
tions. Two of the 5 patients who underwent supra-
glottoplasty were noted to have secondary airway
lesions. Both had associated tracheomalacia, and 1
had mild subglottic stenosis. Another patient who
underwent supraglottoplasty experienced initial im-
provement in his stridor and then subsequent wors-
ening. Further workup and imaging revealed a ret-
ropharyngeal lymphatic malformation that was then
resected.
Discussion
Laryngomalacia is the most common cause of
neonatal stridor in infants and has been managed a
number of ways over time.1-5 The presenting symp-
toms of these patients include dyspnea with ac-
cessory muscle use, feeding difficulties, cyanotic
spells, apnea, oxygen desaturation, and FTT.1-5 In
our study, all of these symptoms were statistically
significant factors in patients who required supra-
glottoplasty (Table 2). This finding is not surprising,
as these symptoms are indications for surgical inter-
vention. However, our study examined patients with
laryngomalacia who were managed without surgery,
as nonoperative management is infrequently dis-
cussed or quantified in the literature.
Other reports suggest that 11.6% to 31% of pa-
tients with laryngomalacia will require surgical in-
tervention.2,7,9 In contrast, only 4.2% of patients in
our study required surgical intervention, and the re-
maining 95.8% were managed without surgery. At
the time of diagnosis, families were counseled that
FTT, ALTE, or documented cyanotic events were in-
dications for surgery. Patients managed without sur-
gery, with positioning or nasogastric tube feedings,
were given the option of surgery as well. Our surgi-
cal indications included feeding difficulties leading
to FTT and/or the combination of life-threatening
apnea, cyanosis, and oxygen desaturation; these cri-
teria are clearly more stringent than those of other
studies. We had 1 patient who did have FTT, pec-
tus excavatum, a history of an ALTE, and feeding
difficulties but whose parents refused surgery; the
inclusion of this patient would have increased our
surgical rate to 5%. We also had a patient who had
laryngomalacia that required surgery and a retro-
pharyngeal lymphatic malformation that, if exclud-
ed, would bring our rate of surgical intervention to
3.3% of patients. Several of the patients seen in our
clinic reported cyanotic spells or had a history of a
possible ALTE, but did not have any documented
observation of these episodes. Home pulse oximetry
and positioning were used in these cases to deter-
mine whether these were indeed true cyanotic spells,
ie, an indication for surgery. No patients on home
monitors experienced apneic or cyanotic events, and
thus, none required surgery. However, patients who
were admitted emergently for a history of an ALTE
were put on telemetry, and the apneic and/or cya
notic events were documented before surgery.
The most commonly reported age at the time of
supraglottoplasty is approximately 3 months2; the
mean age of 53 days at the time of surgery in our
patients is noticeably younger. This difference may
reflect the fact that patients with severe laryngoma
lacia are unable to tolerate airway obstruction in the
neonatal and early infancy period, and that it precip-
itates FTT and ALTEs, leading to an emergent pre-
sentation at the hospital and the need for emergent
surgery. Initial evaluation in the hospital setting on
an emergent basis proved to be predictive of patients
who would require surgical intervention, and these
patients tended to be younger (p = 0.13) and have
more associated symptom findings. When patients
were admitted to the hospital for a parent-reported
ALTE, telemetry was used to determine whether pa-
tients were experiencing true cyanotic events, which
then determined the need for surgical intervention.
Patients referred to our clinic with stridor are always
seen within 48 hours, so presentations to the emer-
gency department usually occurred in the context of
a significant ALTE. This finding suggests that se-
vere laryngomalacia that requires surgery may rep-
resent a neonatal or early infant disease, and it could
be postulated that maturation of the patient is cor-
related with a decreased likelihood of surgical in-
tervention.
In previous studies that report a higher percentage
of surgical intervention, otolaryngologists may have
offered surgery to more patients with feeding dif-
ficulties and poor weight gain, rather than offering
60 Wright & Goudy, Congenital Laryngomalacia 60
conservative management strategies.2,3,6,7,10 In fact,
one study acknowledged that the benefit of surgery
on feeding problems may have been one factor that
led to their higher percentage of patients having sur-
gery.2 Very little is stated in the literature about man-
agement strategies for patients who do not undergo
surgical intervention other than to optimize antire-
flux treatment. In our study, patients with feeding
difficulties and poor weight gain were instructed to
follow up with their pediatrician on a weekly basis
to ensure adequate weight gain between otolaryn-
gology follow-up visits. Five patients were placed
on higher-calorie formulas. Use of these nonoper-
ative measures allowed patients to safely feed and
gain weight while their laryngomalacia gradually
improved during the first months of life, which ap-
pear to be a critical milestone. It is not our intent
to suggest that patients with severe laryngomalacia
should be managed without surgery; however, we
feel that it is important to present data on nonoper-
ative management of patients with laryngomalacia
who have not experienced FTT or documented cy-
anosis or ALTEs. In this series, we did not perform
polysomnography, which would have better quanti-
fied these respiratory events; however, data from pa-
tients on monitors were reviewed for apneic or cy-
anotic events.
The literature is rich in information regarding sur-
gical techniques and outcomes for patients who un-
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that only 4.2% of our patients required surgery sug-
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adequate nutrition, close follow-up, positioning, and
noninvasive monitoring can be considered if symp-
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