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Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2011) 12, 149153

Egyptian Society of Ear, Nose, Throat and Allied Sciences

Egyptian Journal of Ear, Nose, Throat and Allied


Sciences
www.esentas.org

ORIGINAL ARTICLE

Laryngomalacia: Diagnosis and management


Hisham Abedl Fattah a, Alaa Hazem Gaafar
a

a,*

, Zeyad Mohamed Mandour

Otolaryngology Head and Neck Surgery Department, Alexandria Faculty of Medicine, Egypt

Received 19 October 2011; accepted 5 December 2011


Available online 4 January 2012

KEYWORDS
Laryngomalacia;
Laryngoscopy;
Reux;
Conservative treatment;
Surgery

Abstract Background: Laryngomalacia is the term most widely used to describe the inward collapse of supraglottic structures of the larynx during inspiration. It is considered to be the most
common cause of congenital stridor. Laryngomalacia usually presents by high pitched inspiratory
stridor which is often present at birth and is usually noticed by 2 weeks of age. Stridor in laryngomalacia is usually positionally dependent and is worse at times of increased work of breathing.
Diagnosis depends mainly on visualization of the larynx during respiration. Spontaneous resolution
of symptoms is the rule, it usually occurs by the age of 25 years. Surgical intervention is indicated
only in severe cases of laryngomalacia.
Aim: The aim of this study was to assess clinical presentation, management and prognosis of
infants and children suffering from laryngomalacia presented to our department in the period of
5 years.
Methods: The medical sheath records of newborns and infants suffering of laryngomalacia were
reviewed regarding demographic data, clinical presentation, diagnosis and management.
Results: Fifty eight infant and child were included in the study. They were 33 males (57%) and 25
females (43%). Their age at presentation ranged from 2 to 13 months with mean age of 6.3 months.
Diagnosis was done using laryngoscopy under general anesthesia with spontaneous breathing in 49
patients (85%) and by using exible nasopharyngolaryngoscopy under topical anesthesia in 9
patients (15%). Conservative treatment was given for all cases in the form of diet modication,

* Corresponding author. Tel.: +20 1227435699.


E-mail address: gaafar_a@hotmail.com (A.H. Gaafar).
2090-0740 2011 Egyptian Society of Ear, Nose, Throat and Allied
Sciences. Production and hosting by Elsevier B.V. All rights reserved.
Peer review under responsibility of Egyptian Society of Ear, Nose,
Throat and Allied Sciences.
doi:10.1016/j.ejenta.2011.12.001

Production and hosting by Elsevier

150

H.A. Fattah et al.


lansoprazole (7.5 mg once daily) and Domperidone (1 mg/kg/day). For mild cases, gradual
improvement occurred within 1 to 3 months. For severe cases, surgical intervention was planned.
Indications for surgical intervention were severe airway obstruction with attacks of cyanosis, feeding difculties and aspiration, weight loss and failure to thrive.
Conclusion: Laryngomalacia is the most common congenital anomaly of the larynx. It usually presents within 2 weeks after birth. Diagnosis depends on visualization of the larynx during respiration. Conservative treatment is the rule. Surgical treatment is only indicated in 10% of cases.
2011 Egyptian Society of Ear, Nose, Throat and Allied Sciences.
Production and hosting by Elsevier B.V. All rights reserved.

1. Introduction
Laryngomalacia is the term most widely used to describe the
inward collapse of supraglottic structures of the larynx during
inspiration.1 It is considered to be the most common cause of
neonatal and infantile stridor.2 Pathogenesis of laryngomalacia is still unknown. The rst proposed mechanism of pathogenesis was oppiness of the airway secondary to infantile
cartilage abnormalities, but histologic study did not support
this.3 Other investigators4,5 suggest that there is poor neuromuscular control with relative hypotonia of the supraglottic
dilator muscles. Many studies68 cited reux disease as being
at least a comorbid factor for explaining the symptoms of
laryngomalacia.
Whatever the mechanism, laryngomalacia usually presents
by high pitched inspiratory stridor which is often present at
birth and is usually noticed by 2 weeks of age. Stridor in laryngomalacia is usually positionally dependent and is worse at
times of increased work of breathing.1 Diagnosis depends
mainly on visualization of the larynx during respiration. This
could be achieved by direct laryngoscopy under general anesthesia (without muscle relaxant)9 or via exible nasopharyngolaryngoscope under topical or no anesthesia.10 Short
aryepiglottic folds, bulky redundant mucosa over the arytenoids cartilages and omega shape epiglottis are the most common endoscopic ndings in cases of laryngomalacia.11 There
are many classications for laryngomalacia, however, Holinger and Konior in 198912 proposed a practical classication
depending on the direction of the supraglottic collapse which
is either posterolateral collapse (Type A) where there is collapse of the arytenoids and the aryepiglottic folds, complete
collapse of the supraglottic structures of the larynx (Type B),
or anterior collapse (Type C) where only the epiglottis collapses during inspiration.
Spontaneous resolution of symptoms is the rule, it usually
occurs by the age of 25 years.13 Surgical intervention is indicated only in severe cases of laryngomalacia. Severe laryngomalacia is associated with eight primary symptoms: inspiratory
stridor, suprasternal retraction, substernal retraction, feeding
difculty, choking, post-feeding vomit, failure to thrive, and
cyanosis.12,14 Persistence of these symptoms may result in poor
dietary intake, oxygen desaturation, greater respiratory energy
expenditure, ventilator insufciency, and even cardiac failure.15
Historically, the surgical management of laryngomalacia has
been tracheotomy,16 or hyomandibulopexy.17 However, both
of these procedures are associated with high morbidity and
longer ICU duration. In the last 20 years, endoscopic approaches for the treatment of laryngomalacia, namely epiglottoplasty or supraglottoplasty, have become the norm.

These procedures try to eliminate inspiratory obstruction by


widening the supraglottis and have demonstrated to be efcacious in correcting all anatomic anomalies associated with
laryngomalacia.18
2. Aim of the work
The aim of this study was to assess clinical presentation, management and prognosis of infants and children suffering from
laryngomalacia.
3. Patients and methods
Infants and children suffering from laryngomalacia presented
to the Department of Otolaryngology Head and Neck Surgery,
Alexandria University at the period from January 2004 till
January 2009 were included in the study. The study protocol
was approved by the Medical Faculty Ethics Committee of
Alexandria University. The following parameters were
reviewed:
 Demographic data including age at presentation and sex.
 Clinical presentation as noisy breathing, degree of stridor,
feeding difculties, cyanotic attacks, and general condition
affection especially weight gain and chest condition.
 Endoscopic ndings including anatomical changes in the
larynx, associated anomalies in the airway, signs of laryngopharyngeal reux like posterior laryngeal and posterior
pharyngeal wall congestion, degree and type of collapse
according to Holinger and Konior classication.12
 Management either conservative or surgical, type of surgical intervention done and its prognosis.

4. Results
Fifty eight infant and child were included in the study. They
were 33 males (57%) and 25 females (43%). Their age at presentation ranged from 2 to 13 months with mean age of
6.3 months. Noisy breathing and stridor was noticed to occur
starting from 5 days up to 6 weeks after birth with mean of
12 days. Stridor was severe and associated with feeding difculties and failure to thrive in 11 patients (19%). In the rest
of patients (47 patients 81%) stridor was mild, cyclic and
not associated with signicant feeding difculties.
Diagnosis was done using laryngoscopy under general anesthesia with spontaneous breathing in 49 patients (85%) and by
using exible nasopharyngolaryngoscopy under topical anes-

Laryngomalacia: Diagnosis and management

151
Table 1

The postoperative results of surgical treatment.

Stridor
Feeding diculty
Failure to thrive

Figure 1 (a) Preoperative endoscopic view of a case of laryngomalacia showing omega shaped epiglottis (arrow), short aryepigottic folds (*) and redundant mucosa over the arytenoids, (b)
postoperative view of the same case showing bilateral laser
division of the aryepiglottic folds with partial trimming of the
edges of the epiglottis and vaporization of the mucosa over the
arytenoids.

thesia in 9 patients (15%). Endoscopic ndings reported were


short aryepiglottic folds, omega shaped epiglottis and bulky
mucosa over the arytenoids in all cases. (Fig. 1a) Signs of
Larygopharyngeal reux (LPR) were documented in 39 patients (67%). As regards the collapsed portion of the supraglottic structure, it was type A (postrolateral collapse) in 19
patients (33%), type B (complete collapse) in 22 patients
(38%) and type C (anterior collapse) in 17 patients (29%).
Associated airway anomalies were reported in 4 cases;
namely vocal cord paralysis in one case, small glottis web in
one case, tracheal stenosis in one case and tracheomalacia in
one case.
Conservative treatment was given for all cases in the form
of diet modication, lansoprazole (7.5 mg once daily) and
Domperidone (1 mg/kg/day). For mild cases, gradual improvement occurred within 1 to 3 months. For severe cases, surgical
intervention was planned. Indications for surgical intervention
were severe airway obstruction with the attacks of cyanosis,
feeding difculties and aspiration, weight loss and failure to
thrive.
Surgical treatment was performed to 11 (19%) cases. Laser
supraglottoplasty was done in 10 cases and tracheostomy for
one case. Laser used was CO2 laser coupled to operating
microscope. Parameters used were super-pulse mode with
power 57 W for a spot size of 0.5 mm. As regards cases of
supraglottoplasty, laser division of the aryepiglottic fold was

Figure 2 (a) A case of laryngomalacia after cutting of the


arypiglottic folds. Notice the curled redundant edges of the
epiglottis. (b) After laser trimming of the epiglottis (arrows).

Resolved

Improved

Persistent

8 (80%)
9 (90%)
8 (80%)

1 (10%)
1 (10%)
2 (20%)

1 (10%)
0 (10%)
0 (0%)

done for all cases. (Fig. 1b) It was coupled with trimming of
the epiglottis in 2 cases, (Fig. 2) vaporization of the mucosa
over the lingual surface of the epiglottis in 2 cases, vaporization of the mucosa over the arytenoids cartilage in one case,
and excision of the suprahyoid portion of the epiglottis in
one case. Postoperatively, patients were transferred intubated
to pediatric ICU for 2448 h after which they were extubated.
Postoperative treatment was given in the form of antibiotic,
corticosteroids for 5 days and proton pump inhibitors to control reux for 6 weeks.
Post operative outcome was classied as resolved, improved
and persisted regarding the main presenting symptoms and
signs namely stridor, feeding difculties and failure to thrive.
Table 1 shows postoperative results as regards different clinical
presentation. Surgical complications reported were intraoperative bleeding in one case, transient aspiration in 2 cases, supraglottic stenosis in one case and persistence of stridor in one
case.
5. Discussion
The rst description of congenital stridor was reported as early
as 1853 by two French authors, Rilliet and Barthez. They described a child who developed stridor at birth, which was exacerbated by agitation and resolved by the age of ten months.
Sutherland and Lack (1897) were the rst to report a comprehensive review of the condition in their paper entitled Congenital laryngeal obstruction. They presented a detailed analysis
of 18 patients and the rst description of supraglottic collapse
in laryngomalacia. It was not until 1942 that the term laryngomalacia was rst used by Jackson and Jackson (Greek: malakia means morbid softening of part of an organ).19
We report 58 cases presented by airway symptoms and
feeding difculties secondary to laryngomalacia. The onset of
stridor occurred from 5th day up to 6 weeks after birth. This
was in agreement with most of the published data about laryngomalacia.913 This delayed onset could be explained by the
fact that inspiratory ow rates may not be high enough to generate the sounds at birth, however, with growth, there is increased activity and increased air demand.
Premature neuromuscular control to the supraglottic muscles was proposed as an explanation of the pathogenesis of
laryngomalacia.4,5 This theory was supported by the fact that
symptoms and signs resolve in the majority of cases by time.
However, children with laryngomalacia are developmentally
normal and usually are not suffering from any neurological
insults.5
Several authors have reported that laryngomalacia is often
associated with reux, the incidence ranging between 23% and
80%.2022 Signs of reux were found in 39 cases (67%) in the
form of posterior laryngeal congestion and edema over the arytenoids mucosa. Pandora et al.23 in 2003 documented pathological gastroesophageal reux (GOR) in 66% in a series of infants

152
suffering from laryngomalacia using 24-h pH monitoring. They
reported improvement of GOR after performing aryepiglottoplasty.23 It is not clear whether the reux is the primary problem or
if reux occurs secondary to the changes in intrathoracic pressure, with the increased work of breathing that occurs with more
signicant laryngomalacia.7 Peggy in 20059 proposed classication of laryngomalacia into (1) primary laryngomalacia, in
which there is actual anatomic variation such as the shortening
of the aryepiglottic folds or a more pronounced omega-shaped
epiglottis; and (2) secondary laryngomalacia, in which reux
disease is the inciting factor.9 It was obvious that cases of severe
laryngomalacia could be categorized as primary laryngomalacia
(according to Peggy classication) and therefore it did not improve with conservative treatment and it responded only to surgical intervention. In this series this accounts for 19% of
included cases.
The diagnosis of laryngomalacia is made when viewing the
larynx during inspiration and expiration, and correlating the
noise heard with infolding of the supraglottic structures. This
could be achieved by awake exible beroptic endoscopy or
under anesthesia/sedation.9 In our series, awake endoscopy
was done in 9 patients (15%) and endoscopy under anesthesia
in 49 patients (85%). We found that the technique using exposure of the larynx with intubating laryngoscope and visualization of the laryngeal collapse using rigid endoscope during
spontaneous respiration under light general anesthesia is very
practical. It allows good visualization of the larynx, sufcient
time for the documentation of supraglottic folding, better control of the airway, and assessment of vocal cord mobility. In
addition, the endoscope could be pushed distally to the subglottis and trachea to exclude any associated airway anomaly. Sivan
et al.24 compared awaked endoscopy versus endoscopy under
general anesthesia in diagnosis of laryngomalacia. They found
that the awake technique missed three cases of laryngomalacia
and overdiagnosed one healthy control subject. On the other
hand, the anesthesia technique was superior in diagnosis of
laryngomalacia with a sensitivity and specicity of 100%. They
concluded that the diagnosis of laryngomalacia is more accurate
using anesthesia.24
Endoscopic ndings documented were omega shaped epiglottis, short aryepiglottic folds and redundant bulky mucosa
over the arytenoids cartilages. Signs of reux were present in
67% of included cases. Total supraglottic collapse (Type B
laryngomalacia) was the most common encountered type of
laryngomalacia (38% of included cases). Synchronous airway
lesions were documented in 4 cases (6.9%) namely vocal cord
paralysis in one case, small glottis web in one case, tracheal stenosis in one case and tracheomalacia in one case. Several studies have shown that laryngomalacia may be associated with
other structural and functional airway lesions.2527 The frequency of such associated airway anomalies has been reported
to range from 7.5 to 45% of laryngomalacia cases.25,26,28 Thus,
although they are rare, pan endoscopy of the upper and lower
airway is recommended in cases of laryngomalacia to exclude
possible associated airway lesions.26,29
Conservative treatment in the form of diet modication and
anti reux treatment was given for all patients. Improvement
of respiratory and feeding symptoms occurred in 47 (81%)
of cases within 13 months. A medical trial with a proton
pump inhibitor for mild to-moderate airway erythema and
edema in the child who is not growing well or having feeding
difculties may result in improvement or resolution of the stri-

H.A. Fattah et al.


dor.30,31 If the child is then able to feed and gain weight consistently, surgery may be avoided, particularly for secondary
laryngomalacia.31
Surgical treatment was indicated in 11 cases (19%). Supraglottolplasty was done in 10 cases and tracheostomy in one
case. Among the cases of laser supraglottoplasty, stridor
resolved in 8 cases (80%), improved in one case (10%) and
persisted in one case (10%). As regards feeding difculties
and failure to thrive, they were resolved in 9 cases (90%)
and 8 cases (80%) respectively. The success rate of supraglottoplasty in different series ranged from 80% to 100%.3234
Complications of supraglottoplasty are rare. They account
for 7.4% of operated cases in previous series.34 In our series,
reported complications were intraoperative bleeding in one
case, transient aspiration in 2 cases, supraglottic stenosis in
one case, and persistent stridor in one case. Although few in
number, the complications of supraglottoplasty have signicant morbidity. Therefore, it is incumbent on the surgeon to
prevent and avoid as many of the complications as possible.33
6. Conclusion
Laryngomalacia is the most common congenital anomaly of
the larynx. It usually presents within 2 weeks after birth. Diagnosis depends on visualization of the larynx during respiration.
It could be primary or secondary. Conservative treatment is
the rule especially in secondary laryngomalacia. Surgical treatment is only indicated in 10% of cases. Supraglottoplasty has a
high success rate and low incidence of complications.
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