Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2011) 12, 149–153

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,*, Zeyad Mohamed Mandour a

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

Received 19 October 2011; accepted 5 December 2011Available online 4 January 2012

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KEYWORDS

Laryngomalacia;

Laryngoscopy;

Reflux;

Conservative treatment;

Surgery

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Abstract Background: Laryngomalacia is the term most widely used to describe the ‘‘inward col-

lapse 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 laryngo-

malacia 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 2–5 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 flexible nasopharyngolaryngoscopy under topical anesthesia in 9

patients (15%). Conservative treatment was given for all cases in the form of diet modification,

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(A.H. Gaafar).

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, feed-

ing difficulties and aspiration, weight loss and failure to thrive.

Conclusion: Laryngomalacia is the most common congenital anomaly of the larynx. It usually pre-

sents within 2 weeks after birth. Diagnosis depends on visualization of the larynx during respira-

tion. Conservative treatment is the rule. Surgical treatment is only indicated in 10% of cases.

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1. Introduction

Laryngomalacia is the term most widely used to describe the‘‘inward collapse of supraglottic structures of the larynx duringinspiration’’.1 It is considered to be the most common cause of

neonatal and infantile stridor.2 Pathogenesis of laryngomala-cia is still unknown. The first proposed mechanism of patho-genesis was floppiness of the airway secondary to infantile

cartilage abnormalities, but histologic study did not supportthis.3 Other investigators4,5 suggest that there is poor neuro-muscular control with relative hypotonia of the supraglotticdilator muscles. Many studies6–8 cited reflux disease as being

at least a comorbid factor for explaining the symptoms oflaryngomalacia.

Whatever the mechanism, laryngomalacia usually presents

by high pitched inspiratory stridor which is often present atbirth and is usually noticed by 2 weeks of age. Stridor in laryn-gomalacia is usually positionally dependent and is worse at

times of increased work of breathing.1 Diagnosis dependsmainly on visualization of the larynx during respiration. Thiscould be achieved by direct laryngoscopy under general anes-

thesia (without muscle relaxant)9 or via flexible nasopharyngo-laryngoscope under topical or no anesthesia.10 Shortaryepiglottic folds, bulky redundant mucosa over the aryte-noids cartilages and omega shape epiglottis are the most com-

mon endoscopic findings in cases of laryngomalacia.11 Thereare many classifications for laryngomalacia, however, Holin-ger and Konior in 198912 proposed a practical classification

depending on the direction of the supraglottic collapse whichis either posterolateral collapse (Type A) where there is col-lapse 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 col-lapses during inspiration.

Spontaneous resolution of symptoms is the rule, it usuallyoccurs by the age of 2–5 years.13 Surgical intervention is indi-cated only in severe cases of laryngomalacia. Severe laryngoma-lacia is associated with eight primary symptoms: inspiratory

stridor, suprasternal retraction, substernal retraction, feedingdifficulty, choking, post-feeding vomit, failure to thrive, andcyanosis.12,14 Persistence of these symptoms may result in poor

dietary intake, oxygen desaturation, greater respiratory energyexpenditure, ventilator insufficiency, and even cardiac failure.15

Historically, the surgical management of laryngomalacia has

been tracheotomy,16 or hyomandibulopexy.17 However, bothof these procedures are associated with high morbidity andlonger ICU duration. In the last 20 years, endoscopic ap-proaches for the treatment of laryngomalacia, namely epiglot-

toplasty or supraglottoplasty, have become the norm.

These procedures try to eliminate inspiratory obstruction by

widening the supraglottis and have demonstrated to be effica-cious in correcting all anatomic anomalies associated withlaryngomalacia.18

2. Aim of the work

The aim of this study was to assess clinical presentation, man-

agement and prognosis of infants and children suffering fromlaryngomalacia.

3. Patients and methods

Infants and children suffering from laryngomalacia presentedto 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 protocolwas approved by the Medical Faculty Ethics Committee ofAlexandria University. The following parameters were

reviewed:

� Demographic data including age at presentation and sex.

� Clinical presentation as noisy breathing, degree of stridor,feeding difficulties, cyanotic attacks, and general conditionaffection especially weight gain and chest condition.

� Endoscopic findings including anatomical changes in thelarynx, associated anomalies in the airway, signs of laryngo-pharyngeal reflux like posterior laryngeal and posteriorpharyngeal wall congestion, degree and type of collapse

according to Holinger and Konior classification.12

� Management either conservative or surgical, type of surgi-cal intervention done and its prognosis.

4. Results

Fifty eight infant and child were included in the study. Theywere 33 males (57%) and 25 females (43%). Their age at pre-sentation ranged from 2 to 13 months with mean age of

6.3 months. Noisy breathing and stridor was noticed to occurstarting from 5 days up to 6 weeks after birth with mean of12 days. Stridor was severe and associated with feeding diffi-

culties and failure to thrive in 11 patients (19%). In the restof patients (47 patients – 81%) stridor was mild, cyclic andnot associated with significant feeding difficulties.

Diagnosis was done using laryngoscopy under general anes-

thesia with spontaneous breathing in 49 patients (85%) and byusing flexible nasopharyngolaryngoscopy under topical anes-

Figure 1 (a) Preoperative endoscopic view of a case of laryngo-

malacia showing omega shaped epiglottis (arrow), short aryepi-

gottic 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.

Table 1 The postoperative results of surgical treatment.

Resolved Improved Persistent

Stridor 8 (80%) 1 (10%) 1 (10%)

Feeding difficulty 9 (90%) 1 (10%) 0 (10%)

Failure to thrive 8 (80%) 2 (20%) 0 (0%)

Laryngomalacia: Diagnosis and management 151

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

short aryepiglottic folds, omega shaped epiglottis and bulkymucosa over the arytenoids in all cases. (Fig. 1a) Signs ofLarygopharyngeal reflux (LPR) were documented in 39 pa-

tients (67%). As regards the collapsed portion of the supra-glottic structure, it was type A (postrolateral collapse) in 19patients (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 inone case, tracheal stenosis in one case and tracheomalacia in

one case.Conservative treatment was given for all cases in the form

of diet modification, lansoprazole (7.5 mg once daily) and

Domperidone (1 mg/kg/day). For mild cases, gradual improve-ment occurred within 1 to 3 months. For severe cases, surgicalintervention was planned. Indications for surgical intervention

were severe airway obstruction with the attacks of cyanosis,feeding difficulties and aspiration, weight loss and failure tothrive.

Surgical treatment was performed to 11 (19%) cases. Laser

supraglottoplasty was done in 10 cases and tracheostomy forone case. Laser used was CO2 laser coupled to operatingmicroscope. Parameters used were super-pulse mode with

power 5–7 W for a spot size of 0.5 mm. As regards cases ofsupraglottoplasty, 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).

done for all cases. (Fig. 1b) It was coupled with trimming ofthe epiglottis in 2 cases, (Fig. 2) vaporization of the mucosaover the lingual surface of the epiglottis in 2 cases, vaporiza-

tion of the mucosa over the arytenoids cartilage in one case,and excision of the suprahyoid portion of the epiglottis inone case. Postoperatively, patients were transferred intubated

to pediatric ICU for 24–48 h after which they were extubated.Postoperative treatment was given in the form of antibiotic,corticosteroids for 5 days and proton pump inhibitors to con-

trol reflux for 6 weeks.Post operative outcome was classified as resolved, improved

and persisted regarding the main presenting symptoms and

signs namely stridor, feeding difficulties and failure to thrive.Table 1 shows postoperative results as regards different clinicalpresentation. Surgical complications reported were intraopera-tive bleeding in one case, transient aspiration in 2 cases, supra-

glottic stenosis in one case and persistence of stridor in onecase.

5. Discussion

The first description of congenital stridor was reported as earlyas 1853 by two French authors, Rilliet and Barthez. They de-

scribed a child who developed stridor at birth, which was exac-erbated by agitation and resolved by the age of ten months.Sutherland and Lack (1897) were the first to report a compre-

hensive review of the condition in their paper entitled ‘Congen-ital laryngeal obstruction’. They presented a detailed analysisof 18 patients and the first description of supraglottic collapse

in laryngomalacia. It was not until 1942 that the term ‘laryngo-malacia’ was first used by Jackson and Jackson (Greek: mala-kia means morbid softening of part of an organ).19

We report 58 cases presented by airway symptoms and

feeding difficulties secondary to laryngomalacia. The onset ofstridor occurred from 5th day up to 6 weeks after birth. Thiswas in agreement with most of the published data about laryn-

gomalacia.9–13 This delayed onset could be explained by thefact that inspiratory flow rates may not be high enough to gen-erate the sounds at birth, however, with growth, there is in-

creased activity and increased air demand.Premature neuromuscular control to the supraglottic mus-

cles was proposed as an explanation of the pathogenesis oflaryngomalacia.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 developmentallynormal and usually are not suffering from any neurological

insults.5

Several authors have reported that laryngomalacia is oftenassociated with reflux, the incidence ranging between 23% and

80%.20–22 Signs of reflux were found in 39 cases (67%) in theform of posterior laryngeal congestion and edema over the ary-tenoids mucosa. Pandora et al.23 in 2003 documented patholog-

ical gastroesophageal reflux (GOR) in 66% in a series of infants

152 H.A. Fattah et al.

suffering from laryngomalacia using 24-h pHmonitoring. Theyreported improvement ofGORafter performing aryepiglottopl-asty.23 It is not clear whether the reflux is the primary problemor

if reflux occurs secondary to the changes in intrathoracic pres-sure, with the increasedwork of breathing that occurs withmoresignificant laryngomalacia.7 Peggy in 20059 proposed classifica-

tion of laryngomalacia into (1) primary laryngomalacia, inwhich there is actual anatomic variation such as the shorteningof the aryepiglottic folds or a more pronounced omega-shaped

epiglottis; and (2) secondary laryngomalacia, in which refluxdisease is the inciting factor.9 It was obvious that cases of severelaryngomalacia could be categorized as primary laryngomalacia(according to Peggy classification) and therefore it did not im-

prove with conservative treatment and it responded only to sur-gical intervention. In this series this accounts for 19% ofincluded cases.

The diagnosis of laryngomalacia is made when viewing thelarynx during inspiration and expiration, and correlating thenoise heard with infolding of the supraglottic structures. This

could be achieved by awake flexible fiberoptic endoscopy orunder anesthesia/sedation.9 In our series, awake endoscopywas done in 9 patients (15%) and endoscopy under anesthesia

in 49 patients (85%). We found that the technique using expo-sure of the larynx with intubating laryngoscope and visualiza-tion of the laryngeal collapse using rigid endoscope duringspontaneous respiration under light general anesthesia is very

practical. It allows good visualization of the larynx, sufficienttime for the documentation of supraglottic folding, better con-trol of the airway, and assessment of vocal cord mobility. In

addition, the endoscope could be pushed distally to the subglot-tis and trachea to exclude any associated airway anomaly. Sivanet al.24 compared awaked endoscopy versus endoscopy under

general anesthesia in diagnosis of laryngomalacia. They foundthat the awake technique missed three cases of laryngomalaciaand overdiagnosed one healthy control subject. On the other

hand, the anesthesia technique was superior in diagnosis oflaryngomalacia with a sensitivity and specificity of 100%. Theyconcluded that the diagnosis of laryngomalacia is more accurateusing anesthesia.24

Endoscopic findings documented were omega shaped epi-glottis, short aryepiglottic folds and redundant bulky mucosaover the arytenoids cartilages. Signs of reflux were present in

67% of included cases. Total supraglottic collapse (Type Blaryngomalacia) was the most common encountered type oflaryngomalacia (38% of included cases). Synchronous airway

lesions were documented in 4 cases (6.9%) namely vocal cordparalysis in one case, small glottis web in one case, tracheal ste-nosis in one case and tracheomalacia in one case. Several stud-ies have shown that laryngomalacia may be associated with

other structural and functional airway lesions.25–27 The fre-quency of such associated airway anomalies has been reportedto range from 7.5 to 45% of laryngomalacia cases.25,26,28 Thus,

although they are rare, pan endoscopy of the upper and lowerairway is recommended in cases of laryngomalacia to excludepossible associated airway lesions.26,29

Conservative treatment in the form of diet modification andanti reflux treatment was given for all patients. Improvementof respiratory and feeding symptoms occurred in 47 (81%)

of cases within 1–3 months. A medical trial with a protonpump inhibitor for mild to-moderate airway erythema andedema in the child who is not growing well or having feedingdifficulties may result in improvement or resolution of the stri-

dor.30,31 If the child is then able to feed and gain weight con-sistently, surgery may be avoided, particularly for secondarylaryngomalacia.31

Surgical treatment was indicated in 11 cases (19%). Supra-glottolplasty was done in 10 cases and tracheostomy in onecase. Among the cases of laser supraglottoplasty, stridor

resolved in 8 cases (80%), improved in one case (10%) andpersisted in one case (10%). As regards feeding difficultiesand failure to thrive, they were resolved in 9 cases (90%)

and 8 cases (80%) respectively. The success rate of supraglot-toplasty in different series ranged from 80% to 100%.32–34

Complications of supraglottoplasty are rare. They accountfor 7.4% of operated cases in previous series.34 In our series,

reported complications were intraoperative bleeding in onecase, transient aspiration in 2 cases, supraglottic stenosis inone case, and persistent stridor in one case. Although few in

number, the complications of supraglottoplasty have signifi-cant morbidity. Therefore, it is incumbent on the surgeon toprevent 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. Diag-nosis depends on visualization of the larynx during respiration.It could be primary or secondary. Conservative treatment is

the rule especially in secondary laryngomalacia. Surgical treat-ment is only indicated in 10% of cases. Supraglottoplasty has ahigh success rate and low incidence of complications.

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