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A CASE OF UPPER AIRWAY OBSTRUCTION IN LARYNGOMALACIA

WITH CEREBRALPALSY

Therry Tulus Prasetyawan, Agrina Nurlisyari, Dian Paramita Wulandari


Otorhinolaryngology Head and Neck Surgery Department
Faculty of Medicine Gadjah Mada University / Dr. Sardjito Hospital, Yogyakarta,

ABSTRACT

Introduction: Laryngomalacia is a congenital anomaly of the larynx leading to inward collaps


of the supraglottic airway with inspiration. It was the most common congenital laryngeal
abnormality, accounting for over 60% of all cases of congenital stridor and neonates.
Approximately 10% of cases develop severe symptoms. Severe laryngomalacia could
interfere with normal respiration, feeding and even cause life-threatening episodes, and
therefore aggressive management becomes necessary. Laryngomalacia often present with
GERD (65-100%) and neurological disease (20-40%). Some congenital and syndromic
disorder could also exist together with laryngomalacia
Case presentation: We report a case of 20 months old girl who presented with inspiratory stridor
worsened by feeding, agitation, supine crying, snoring, and dyspneu. The patient also had history
of cerebral palsy, growth retardation, and recurrent pneumonia. Flexible fiberoptic laryngoscopy
demonstrated an omega shaped epiglottic with airway obstruction and aspiration.
Supraglottoplasty preocedure was done to the patient after tracheostomy using direct
laryngoscopy. The patient was evaluated 2 days later using fiber optic laryngoscopy, and
followed by swallowing therapy.

Conclusion: Only 10 to 20% of infants present severe laryngomalacia requiring surgical


management. In this patient, surgery was proposed because laryngomalacia cause growth
retardation and recurrent pneumonia which could effect patients quality of life..

Keywords: laryngomalacia, tracheostomy, supraglottoplasty, cerebral palsy

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Introduction

Laryngomalacia is a congenital anomaly of the larynx leading to inward collaps of the

supraglottic airway with inspiration. Chevalier Jackson is credited with coining the term

laryngomalacia in 1942, but the condition was first described by Barthez an Rilliet in 1853. It

is literally meaning soft larynx, this condition refers to a dynamic prolapse of the supraglottic

structure during inspiration with endoscopic finding such as shortened aryepiglottic folds

and redundant soft tissue, with or without supraglottic edema.1,2

It is the most common congenital laryngeal abnormality, accounting for over 60% of

all cases of congenital stridor. It is the most common etiology of stridor in the neonate. In

pediatric patients presenting with stridor, laryngomalacia is the cause in 45-75% of case.

Affected infants often present with inspiratory stridor within the first 10 days of life that

typically resolves spontaneously by 12-18 months of age. Laryngomalacia often present with

GERD (65-100%) and neurological disease (20-40%). Some congenital and syndromic disorder

could also exist together with laryngomalacia 2,3

Approximately 10% of cases develop severe symptoms. Severe laryngomalacia can

interfere with normal respiration, feeding and even cause life- threatening episodes, and

therefore aggressive management becomes necessary. The mainstay treatment of severe

refractory to medical therapy is surgical supraglottoplasty to unblock the supraglottic and

excise the redundant tissue collapsing into the laryngeal inlet during inspiration.1,4

Supraglottoplasty was originally described in 1922, and became procedure of choice to

treat laryngomalacia. Historically, the surgical management of laryngomalacia has been

tracheotomy, or hyomandibulopexy. 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


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the norm.5,6

Case Presentation:
A 20 months old girl presented to the of Otorhinolaryngology Head and Neck Surgery

Department at Dr. Sardjito Hospital in Yogyakarta for evaluation with complain of inspiratory

stridor worsened by feeding, agitation, supine, crying, and snoring with supine position since

birth. The patients also occasionally show symptoms of dyspnea, and choking during feeding

with both liquid and solid food, and slow weight gain. The patient had history of cerebral palsy,

recurrent pneumonia, and growth retardation. The parents denied pain, bleeding and history of

injury, chronic irritation, or exposure to radiation. The family also denied the history of the same

symptom in family.

Orofaring examination showed normal pharyngeal wall, normal tonsil palatina with size

T1-T1, no mass. Anterior rhinoscopy was normal, no mass, no bleeding, no hypertrophy concha,

and no discharge. Otoscopy of both ear revealed normal ear canal and intact tympanic

membrane. Neck examination didnt show any mass, but there was retraction.

Flexible fiberoptic laryngoscopy demonstrated an omega shaped epiglottis which obstruct

airway and aspiration. There is no mass or bleeding, and vocal fold movement looks symmetric.

Figure 1. (Left)Flexible fiberoptic laryngoscopy


(Right) Direct laryngoscopy view before surgery

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Clinical history, physical examination, and endoscopy refer to the diagnosis of

laryngomalacia type 2 with cerebral palsy, recurrent pneumonia, and growth retardation . The

management was supraglottplasty procedure via direct laryngoscopy with general anesthesia

using cold knife microlaryngosurgery method. The bleeding was handled using cotton pledge

soaked with 1/10000 adrenalin/ephinephrine The tracheostomy procedure was done first before

supraglottoplasty. During this procedure the aryepiglottic fold was cut and widened.

Figure 2. Direct laryngoscopy view during surgery

Two days postoperative patient was evaluated again using flexible fiberoptic

laryngoscopy. The post operative wound healing is good, and no active bleeding. Patient was

discharged home later. After 3 months post operatively, the patient has started to gain more

weight, and there are less episode of pneumonia.

Figure 3
. Post operative : Flexible fiberoptic laryngoscopy view

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Discussion

Not all neonates have the same laryngeal anatomy, but all neonates potentially present

supraglottic structures that can invaginate during inspiration. The development of

symptoms results from variable combinations of the infants specific laryngeal anatomy, poor

control of the tone of supraglottic structures (either pathological neurological mechanisms or

simple physiological variations related to changes in tone during sleep), mucosal oedema, and

increased airflow.7

Historically, it was thought that laryngomalacia represented an anatomic abnormality of

the laryngeal cartilage. This theory was supported by a prospective study showing a lower

aryepiglottic fold to glottic length ratio in patients with severe laryngomalacia compared with

unaffected children, but failed to explain why some children with similar laryngeal

examinations were asymptomatic. The theory of immature and abnormally collapsible cartilage

was further discredited by histologic examinations demonstrating normal fibro-elastic

cartilage tissue in children with symptomatic disease.8

Recently, attention has focused on a neuromuscular etiology, consisting of immaturity or

abnormal integration of the peripheral nerves, brainstem nuclei and pathways responsible for

swallowing and maintenance of airway patency. This is supported by physiologic studies in

infants with laryngomalacia demonstrating increased stimulus threshold requirements for

elicitation of normal motor responses correlating with disease severity. Additional corroboration

is provided by histologic studies showing significant size differences in the superior laryngeal

nerve branches of patients with severe laryngomalacia compared to age matched

controls. Subsequent neurological and central nervous system maturation would provide a

reasonable explanation for the spontaneous resolution generally seen in the disease.8

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A presumptive clinical diagnosis of laryngomalacia can be made based on the classic

symptoms of inspiratory stridor worsened by feeding, agitation, supine positioning or crying.

Pertinent history should include birth circumstances (including gestational age and

endotracheal intubation), congenital or genetic abnormalities, respiratory symptoms with

aggravating or temporizing factors, and feeding concerns including retarded growth, choking or

gagging, reflux symptoms, or recurrent pneumonia. Physical examination should include

height and weight, respiratory sounds including timing in the respiratory cycle, chest

movement to determine the presence of retractions or pectus excavatum, and auscultation of the

lung fields.8

A definitive diagnosis of laryngomalacia can be made accurately by flexible fiberoptic

laryngoscopy alone in the vast majority of cases (88%), regardless of the experience level of

the examiner. The procedure can generally be performed on awake children in the arms of

their caregiver without the need for sedation. The flexible fiberoptic laryngoscope is passed

along the nasal floor and positioned above the larynx during several cycles of spontaneous

respiration. Topical anesthetics should be avoided when possible as they may exacerbate

airway collapse and alter the examination.8

The three basic abnormalities that may be present are 1) elongated, tubular epiglottis

that collaps inward and posteriorly on inspiration, 2) foreshortened aryepiglottic folds

that collaps medially, and 3) redundant mucosa overlying the arytenoid cartilages that

collaps anteriorly.1

Hollinger and Konior in 1989 defined laryngomalacia by the direction of supraglottic

collaps: posterolateral collaps (type A) where there is collaps of the arytenoids and the

aryepiglottic folds, complete collaps of the supraglottic structure of the larynx (type B), or

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anterior collaps (type C) where only the epiglottis collapses during inspiration. Kay and

Goldsmith classified laryngomalacia by the following: 1) foreshortened or tight aryepiglottic

folds, 2) presence of soft tussue in the supraglottis, or 3) consequence of other

etiologies, such as neuromuscular disorder.5,9

Only 10 to 20% of infants present severe laryngomalacia requiring surgical

management. Surgery should therefore be proposed when laryngomalacia is

associated with one or more of the following symptoms: dyspnoea with permanent and

severe intercostal or xyphoid retraction; episodes of respiratory distress; obstructive

sleep apnoea; episodes of suffocation while feeding or feeding difficulties; poor

weight gain.5,10,11

Another literature divides potensial indications for surgery in laryngomalacia according

to problem in respiratory and feeding. Respiratory problems includes stridor with

respiratory distress, dyspnea with retractions, pectus excavates, pulmonary

hypertension, cor pulmonale, severe obstructive sleep apnea. Feeding problems inludes

episodic cyanosis with feeding, recurrent aspiration pneumonia, failure to thrive.8

In the smaller group of children with laryngomalacia who fail to respond to conservative

treatment or in those with more severe respiratory or feeding difficulties upon presentation,

surgical intervention is often recommended.8

Supraglottoplasty is the principal surgical treatment option for moderate to severe

laryngomalacia and involves surgically modifying the supraglottis in an effort to reduce its

collaps during respiration. Supraglottoplasty includes one or more of the following procedures:

division of the aryepiglottic folds, debulking of the arytenoid mucosa and cuneiform cartilages,

making linear incisions on the lingual surface of the epiglottis, or trimming of the lateral

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edges of the epiglottis. The overarching goal of supraglottoplasty is to reduce or stabilize

laryngeal tissue, thereby preventing inspiratory collapse and airway obstruction. The

procedure is typically performed under sedation with spontaneous breathing after suspension of

the larynx.13,8,12

This procedure was first done with cold steel and was described by Zalzal et al. The

use of the carbon dioxide (CO2) laser was later introduced. Both cold-steel and laser have

been shown to have similar outcomes. Although CO2 laser supraglottoplasty was originally

thought to be more precise with less intraoperative bleeding, more recent studies have shown

coldsteel supraglottoplasty to be a reliable technique with very little bleeding. Microdebrider

assisted procedures were first described in 2005. Since coblation technology was introduced at

the beginning of the 21st century there has been little published on its use in supraglottoplasty.

A potential benefit of the coblator is a reduction in the risk of airway fires, which have been

reported with the laser. A systematic review by Preciado and Zalzal reported that therewere not

enough stratified data available to assess outcomes in supraglottoplasty by specific method.14

A combination of partial epiglottectomy, division of Aryepiglottic folds and removal of

redundant mucosa is performed as indicated by the anatomical abnormalities seen on flexible

fiberoptic evaluation and intra-operative findings. The interarytenoid mucosa has been preserved

to prevent postoperative scarring. Surgery can be performed using microlaryngeal instruments,

micro-debrider or CO2 laser. Similar success rates have been reported for each, therefore

instrumentation and technique is generally determined by surgeon preference and training.8

Conflicts of interest
The authors declare that there is no conflict of interests regarding the publication of this paper

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References

1. Bedwell J, Zalzal G. Laryngomalacia. Seminars in Pediatric Surgery.


2016;25:119-22.
2. Edmondson NE, Bent III JP, Chan C, Laryngomalacia: the role of gender and
ethnicity. International Journal of Pediatric Otorhinolaryngology. 2011;75:1562-4.
3. April M, Dana M, Laryngomalacia: Disease Presentation, Spectrum, and Management,
International Journal of Pediatrics, vol. 2012:
4. Soong WJ, Shiao AS, Jeng MJ, Lee YS, Tsao PC, Yang CF, et al. Comparison
between rigid and flexible laser supraglottoplasty in the treatment of severe
laryngomalacia in infants. International Journal of Padiatric Otorhinolaryngology.
2011;75:824-9.
5. Fattah HA, Gaafar AH, Mandour ZM. Laryngomalacia: diagnosis and
management. Egyptian Journal of Ear, Nose, Throat and Allied Sciences.
2011;12:149-53.
6. Netto JFL, Drummond RL, Oppermann LP, Hermes FS, Krumenauer RCP.
arryngomalacia surgery: a series from a tertiary pediatric hospital. Braz J
Otorhinolaryngology. 2012;78(6):99-106.
7. Ayari S, Aubertin G, Girschig H, Abbeele TVD, Mondain M. Pathophysiology and
diagnostic approach to laryngomalacia in infants. European Annals of
Otorhinolaryngology, Head and Neck disease. 2012;129:257-63.
8. Thorne MC, Garetz SL. Laryngomalacia: review and summary of current clinical
practice in 2015. Paediatric Respiratory Reviews. 2016;17:3-8.
9. Sheng LK, Nien CB, Chien YC, Chun CY. CO2 laser supraglottoplasty for severe
laryngomalacia: a study of symptomatic improvement. 2007;71:889-95.
10. Escher A, Probst R, Gysin C. Management of laryngomalacia in children with
congenital syndrome: the role of supraglottoplasty. 2014.
11. Ayari A, Aubertin G, Girschig H, Abbeele TVD, Denoyelle F, Couloignier V, et al.
Management of laryngomalacia. European Annals of Otorhinolaryngology, Head and
Neck disease. 2013;130:15-21.
12. Oconnor TE, Bumbak P, Vijayasekaran S. Objective assessment of
supraglottoplasty outcomes using polysomnography. International Journal of Pediatric
Otorhinolaryngology. 2009;73:1211-6.
13. Zainal A, Goh BS, Mohamed AS. Supraglottoplasty for laryngomalacia: who will
benefit?. Asian Journal of Surgery. 2011;34(2):92-6.
14. Ramprasad VH, Ryan MA, Farjat AE, Eapen RJ, Raynor EM. Practice patterns in
supraglottoplasty and perioperative care. International Journal of Pediatric
Otorhinolaryngology. 2016;86:118-23

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