TITLE: Treatment of Laryngomalacia SOURCE: Grand Rounds Presentation, UTM , !e"t# of Otolaryngology !$TE: %une &', ())' RESI!

E*T P+,SICI$*: Garrett $# +au"tman, M! -$CULT, P+,SICI$*: Matt.e/ 0# Ryan, M! SERIES E!ITORS: -rancis # 1uinn, %r#, M! and Matt.e/ 0# Ryan, M!
"This material was prepared by resident physicians in partial fulfillment of educational requirements established for the Postgraduate Training Program of the UTMB Department of Otolaryngology/ ead and !ec" #urgery and was not intended for clinical use in its present form$ %t was prepared for the purpose of stimulating group discussion in a conference setting$ !o warranties& either e'press or implied& are made with respect to its accuracy& completeness& or timeliness$ The material does not necessarily reflect the current or past opinions of members of the UTMB faculty and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion$"

Stridor is a harsh, high-pitched musical sound that results from turbulent airflow through the upper airway. Evaluation of stridor is dependent upon the clinical situation, as the underlying etiology may range from mild illness to a severe and life-threatening situation. When considering a pediatric patient, it should be noted that eighty-five percent of children under the age of 2.5 years presenting with stridor have a congenital etiology. Congenital stridor often times is not present at birth, but will typically present before the age of four months. he remaining cases are accounted for by inflammation, trauma, or foreign bodies. !ediatric stridor has a variable age of onset. he typical patient usually presents with a sudden onset of symptoms. "s a basic guideline, ac#uired stridor is more li$ely than congenital stridor to re#uire airway intervention. When assessing stridor, the location of the turbulent airflow may be determined by the respiratory phase in which the sound is noted. here are three %ones of the airway that are responsible for these sounds- supraglottic, glottic and subglottic, and intrathoracic. Supraglottic obstruction results in a high-pitched, inspiratory stridor. &bstruction of the e'trathoracic trachea, including the glottis and subglottis, is characteri%ed by biphasic stridor with an intermediate pitch. &bstruction of the intrathoracic trachea (including first and second order bronchi) results in e'piratory stridor, or whee%ing. his last area of obstruction is associated with retraction of the sternum, costal cartilage, and suprasternal tissue. he differential diagnosis of noisy breathing in children can be grouped into the anatomic location of the lesion or defect. *nderlying causes can be classified as congenital, inflammatory, neoplastic, neuromuscular, and traumatic. he following is a list of diagnoses depending on the location of the lesion or defect+ ,ose and nasopharyn'o Congenital+ choanal atresia or stenosis, pyriform aperture stenosis, craniofacial anomalies o -nflammatory+ nasal polyps, rhinitis, retropharyngeal abscess, adenoid hypertrophy o ,eoplastic+ encephalocele, dermoid, glioma o raumatic+ foreign body &ropharyn'.hypopharyn'o Congenital+ glossoptosis.macroglossia, lingual thyroid, vallecular cyst,

craniofacial anomalies o -nflammatory+ tonsil hypertrophy, retropharyngeal abscess o ,eoplastic+ dermoid, hemangioma, lymphangioma o ,euromuscular+ hypotonia, neurologic disease o raumatic+ foreign body Supraglottic laryn'o Congenital+ laryngomalacia, laryngocele.saccular cyst o -nflammatory+ epiglottitis, angioneurotic edema o ,eoplastic+ hemangioma, lymphangioma, papilloma o raumatic+ foreign body /lottic laryn'o Congenital+ web.atresia, laryngeal cleft, stenosis o -nflammatory+ laryngitis, spasm, stenosis o ,eoplastic+ hemangioma, lymphangioma, papilloma, granuloma o ,euromuscular+ vocal cord paralysis o raumatic+ hematoma, fracture, foreign body, stenosis Subglottic laryn'o Congenital+ stenosis, cysts o -nflammatory+ croup, stenosis o ,eoplastic+ hemangioma, papilloma o raumatic+ chondritis, stenosis, fracture, foreign body racheobronchialo Congenital+ stenosis.web, tracheomalacia, vascular ring.sling.complete tracheal rings, foregut cysts, tracheoesophageal fistula o -nflammatory+ membranous tracheitis, bronchitis, asthma o ,eoplastic+ mediastinal tumors, thyroid, thymus, papilloma o raumatic+ stenosis, foreign body (tracheal or esophageal)

0aryngomalacia is the most common cause of stridor in infancy. 1urthermore, it is the most common congenital laryngeal anomaly. 2ales are affected twice as often as females. 0aryngomalacia arises from a continued immaturity of the laryn'. Symptoms of laryngomalacia are often not present at birth. -n fact, onset of symptoms typically occurs days to wee$s after birth (most commonly within the first two wee$s of life) and they will resolve at twelve to eighteen months of age without intervention. he diagnosis is suspected when inspiratory stridor is auscultated and is confirmed by fle'ible nasopharyngoscopy. ypical stridor associated with laryngomalacia is low in pitch with a fluttering #uality secondary to the circumferential rimming of the supraglottic airway and aryepiglottic folds. -t is often associated with general noisy respiration. he stridor is most prominent when the child is in the supine position or when the child is agitated. 2ore forceable inspiration tends to result in a louder stridor #uality due to greater prolapse and thus greater obstruction. 3adiographic studies can suggest the diagnosis of laryngomalacia, however the mainstay of diagnosis is fle'ible nasopharyngoscopy. 1or completeness, we will include radiographic findings. "n inspiratory film with nec$ e'tension can show medial and inferiorly displaced arytenoids and epiglottis. 1luoroscopy can demonstrate the collapse of the supraglottic structures with inspiration. Chest radiographs can aid in evaluation of potential lower respiratory tract anomalies such as innominate artery compression, tracheomalacia, and vascular rings.

1le'ible nasopharyngoscopy is best performed in an unanestheti%ed child in an upright position with a 4.5mm nasopharyngoscope. he scope should be passed through both nasal passages when e'amining the patient. Classic findings with fle'ible nasopharyngoscopy are a cyclical collapse of the supraglottic laryn' with inspiration. he aryepiglottic folds are short and often times there is an omega shaped epiglottis. Short aryepiglottic folds draw the cuneiform and corniculate cartilages forward over the laryngeal inlet resulting in prolapse during inspiration. hese findings will be reviewed when discussing the five types of laryngomalacia. -t should be noted that the vocal cords are mobile in laryngomalacia. "lthough laryngomalacia can often ma$e visuali%ation of the vocal cords difficult, they still must be e'amined. 2ultiple factors may contribute to the development of laryngomalacia. he main contributors are thought to be anatomic, neurologic, and inflammatory. he underlying anatomic pathologies are shortening of the aryepiglottic folds and anterior collapse of the cuneiform cartilages. -mmature neuromuscular control and movement has also been thought to impact laryngomalacia. "dditionally, there is an association of gastroesophageal reflu' and laryngomalacia. 3eflu' can induce posterior supraglottic edema and secondarily laryngomalacia. " recent article published in "pril 2665 by 7r. SC 2anning et al. evaluated the anatomical impact on laryngomalacia. he specific ob8ective of the article was to compare the aryepiglottic length in pediatric patients who have severe laryngomalacia and are undergoing aryepiglottoplasty with the aryepiglottic length of a sample of control patients without laryngomalacia. he study design was a prospective case-control. 2easurements were compared by creating a ratio of the aryepiglottic fold length (distance from the most anterior arytenoids cartilage to the closest posterior lateral edge of the epiglottic cartilage) to the glottic length (distance from the anterior border of the interarytenoid muscle to the anterior commissure). he mean ratio of patients with severe laryngomalacia was calculated to be 6.9:6, while the mean ratio of control patients was calculated to be 6.595. here are five types of laryngomalacia. ype - is an inward collapse of the aryepiglottic folds (mainly the cuneiform and corniculate cartilages). hese cartilages are drawn inward during inspiration and open passively during e'piration. ype -- is a long, tubular epiglottis which curls on itself and contributes to obstruction during inspiration. his often occurs in association with ype - laryngomalacia. ype --- is anterior, medial collapse of the cuneiform and corniculate cartilages to occlude the laryngeal inlet during inspiration. ype -; is posterior inspiratory displacement of the epiglottis against the posterior pharyngeal wall or inferior collapse to the vocal folds. ype ; is short aryepiglottic folds. he re#uirement of surgical intervention in patients with laryngomalacia is rare, as it is generally a self-limiting condition. Severe symptoms such as inability to feed orally, cor pulmonale, failure to thrive, and life threatening airway obstruction may necessitate surgical intervention. !rior to the 45:6s, tracheotomy was used in this setting. he rationale for this intervention was that a tracheotomy bypassed the area of obstruction until the supraglottic pathology spontaneously resolved. oday, this strategy is only employed in the severely affected infant. -nstead, the strategy has changed to address the area of obstruction directly with a supraglottoplasty. his can be performed by ma$ing use of microlaryngeal instruments, a carbon dio'ide laser, or a microdebrider to e'cise redundant mucosa and.or cuneiform cartilages as well as releasing the shortened aryepiglottic folds and trimming the lateral edges of the epiglottis. -t is important to note that there is more than one obstructing mechanism in laryngomalacia and

when performing supraglottoplasty, all potential mechanisms should be addressed. When removing tissue, the surgeon should be conservative, as supraglottic stenosis can result from e'cessive tissue removal. *nilateral supraglottoplasty should be considered and the second side should be operated on only if symptoms do not resolve. "lso, direct laryngoscopy and bronchoscopy must be performed prior to supraglottoplasty to rule out concomitant pathology contributing to the airway obstruction. he direct laryngoscopy should be performed when the patient is breathing spontaneously under general anesthesia. !ost-operatively, the patients are usually left intubated over night with e'tubation the following morning. "ntibiotics should be given at least five days post-operatively to prevent infection. "ntireflu' precautions including both positioning and medication are recommended to minimi%e the raw mucosal surfaces e'posure to gastric secretions. <elow is a review of the literature he original description of endoscopic removal of supraglottic tissue with a nasal snare for laryngomalacia was first described in 4522 by 7r. S -glauer. -n 45:=, 7r. 3W 0ane et al. described a single report of the removal of the corniculate cartilages and redundant arytenoids mucosa in a child with laryngomalacia. -n 45:5, 7r. "< Seid et al. described the use of the carbon dio'ide laser for treatment of laryngomalacia in a series of three patients. -n 45:>, /? @al%al et al. presented epiglottoplasty as a new procedure. ?is data included a case series of ten patients. ?e described a laryngoscopic approach in which redundant mucosa was e'cised from the lateral edges of the epiglottis, aryepiglottic folds, arytenoids, and corniculate cartilages. his was done with the patient in the supine position while visuali%ing the laryn' through a laryngoscope. he tissue to be e'cised was grasped with cup forceps and trimmed with <ellucci scissors. 7r. @al%al reported that Aall patients who underwent epiglottoplasty achieved complete relief from their symptoms with the e'ception of one patient in whom mucosa from the aryepiglottic folds and arytenoids area had not been ade#uately e'cised. 1urther e'cision resulted in complete relief.B ?is indications for operating on a patient with laryngomalacia were severe stridor not resolved with time that may be associated with failure to thrive, cor pulmonale, feeding difficulties, and apnea. "dditionally, inability to view the vocal cords with a fle'ible nasopharyngoscope during inspiration because of laryngeal inlet collapse is a clear indication for surgery. -n 4555, / 3oger, et al. published a retrospective study of 445 cases of resection of the aryepiglottic folds with or without a carbon dio'ide laser. he success rate was determined to be 5:C with an average follow-up period of thirty months. 1ailure of the procedure was assigned to two children who needed tracheotomies. Seven patients re#uired a revision surgery. -n this paper, 7r. 3oger established criteria for the definition of severe laryngomalacia+ 4. 2. 9. =. 5. D. >. :. 5. dyspnea at rest and.or severe dyspnea during effort feeding difficulties height and weight growth rate stagnation sleep apnea or obstructive hypoventilation uncontrollable gastroesophageal reflu' history of intubation for obstructive dyspnea effort hypo'ia (46C higher than the normal values for the same age group) effort hypercapnia (46C higher than the normal values for the same age group) abnormal polysomnography with an increased apnea.obstructive hypoventilation inde'

he presence of at least three of these criteria was deemed to be a formal indication for

endoscopic surgery. 7r. S2 Eelly et al. evaluated the effectiveness of unilateral endoscopic supraglottoplasty for treatment of severe laryngomalacia in 4555. he study design was a retrospective review of eighteen patients with severe laryngomalacia that had undergone unilateral carbon dio'ide laser removal of redundant supraglottic tissue. hree of the eighteen patients re#uired a contralateral supraglottoplasty. 3elief of obstructive symptoms was achieved in 5=C of the patients. he one patient without obstructive relief had tracheomalacia secondary to previous tracheotomy for severe laryngomalacia. -n 4555, 73 &lney et al. performed a retrospective chart review aimed at determining the airway outcome of infants with laryngomalacia who do not undergo routine direct laryngoscopy and bronchoscopy, the age at which laryngomalacia resolves, and the outcome of supraglottoplasty as a function of the type of laryngomalacia and the presence of concomitant disease. 7r. &lney found that direct laryngoscopy and bronchoscopy as part of the routine evaluation of laryngomalacia is not warranted and should only be performed when there is clinical and physical evidence of a concomitant airway lesion. he median time to resolution of isolated laryngomalacia was 9D wee$s, and by >2 wee$s, >5C of infants were free of stridor. his finding did not vary significantly in infants with severe neurological compromise or in infants with other congenital anomalies. Supraglottoplasty was determined to be necessary in appro'imately 45-26C of affected infants which is attributed to episodes of apnea and failure to thrive. " chart review was performed in 2664 by CW Senders et al. to evaluate different carbon dio'ide laser procedures on children with various types of laryngomalacia and determine the role of associated anomalies on the outcome. wenty-three children were included in a retrospective chart review that underwent carbon dio'ide laser vapori%ation of redundant supraglottic mucosa of the aryepiglottic fold, arytenoids, and the epiglottis. !atients without associated anomalies did well, with >:C immediately resolving their respiratory symptoms and 466C resolving their respiratory symptoms within a wee$. *nfavorable immediate results, as well as long term surgical failures all had associated anomalies including "rnold-Chiari, Cerebral !alsy, C?"3/E association, and 3ieger syndrome. Conclusions determined by 7r. Senders were that laser supraglottoplasty is a safe and effective treatment for all types of laryngomalacia, but children with associated neurological or anatomic anomalies will have a more complicated immediate and short term course, as well as a significant incidence of failure. " retrospective review was performed in 2664 by SC oynton et al. loo$ing at one hundred patients that had endoscopic aryepiglottoplasty performed for severe laryngomalacia. Surgery was used for treatment of patients that had o'ygen saturation below 52C and feeding difficulties causing failure to thrive. ,inety-four percent of the patients had improvement of their stridor after one month, with 55C completely without stridor. !atients with slower progression of improvement were found to have a serious neurological condition. Seventy-two percent of patients with preoperative feeding difficulties improved their feeding. 7r. oynton concluded that endoscopic aryepiglottoplasty is the operation of choice for severe laryngomalacia. ?owever, underlying neurological disease decreases the success rate of operative intervention. -n 2664, 7r. 7E 3eddy et al. performed a retrospective review to evaluate the efficacy of unilateral supraglottoplasty compared to bilateral supraglottoplasty. his study included 46D

patients- 55 patients undergoing bilateral supraglottoplasty and => patients undergoing unilateral supraglottoplasty as the initial surgery. Surgical success was defined as the resolution of clinically significant laryngomalacia and was reported to approach 5DC in this study. 1ifteen percent of the patients that had unilateral supraglottoplasty performed needed an additional contralateral procedure performed for resolution of symptoms. wo patients (9C) that underwent bilateral supraglottoplasty initially developed supraglottic stenosis. ,o patients undergoing unilateral supraglottoplasty developed supraglottic stenosis. 7r. 3eddy recommended that unilateral supraglottoplasty is a reasonable initial surgical management of pediatric patients with severe laryngomalacia. 7r. 7 0o$e et al. performed a retrospective review in 2664 that e'amined the outcome of 92 cases of severe laryngomalacia that underwent a simple division of the aryepiglottic folds. "ppro'imately D5C showed complete resolution of stridor and associated complications of laryngomalacia, while 22C showed partial resolution of stridor with no further surgical intervention re#uired. Si' percent re#uired one additional procedure which included more e'tensive e'cision of redundant mucosa. &ne patient re#uired a tracheotomy. -t was concluded that endoscopic e'cision of the aryepiglottic folds is highly effective and should be the first-line treatment for severe laryngomalacia. -n 2662, isolated posterior displacement of the epiglottis was addressed by 7r. F" Werner et al. by performing an epiglottope'y for the treatment of severe laryngomalacia. Si' patients underwent epiglottope'y- = of which were solely epiglottope'y and 2 of which were combined epiglottope'y with transaction of the aryepiglottic folds. he procedure was performed transorally with a carbon dio'ide laser. "ll si' children demonstrated significant airway improvement without any further stridor. -t was also noted that deglutition was not affected by this procedure. he failures and complications of supraglottoplasty were analy%ed in 2669 by 7r. 1 7enoyelle et al. he study design was a retrospective review that included 49D patients, 462 of which had isolated laryngomalacia and 9= of which had additional congenital anomalies (!ierre 3obin, psychomotor retardation, C?"3/E association, 7own syndrome, miscellaneous). &utcome measures included persistence of dyspnea, sleep apnea, and.or failure to thriveG need for additional treatmentG presence of granuloma, edema or webG and supraglottic stenosis. 1ailure or only partial improvement of symptoms was only seen in patients with additional congenital anomalies (:.:C). he need for revision surgery was =.=C of patients, minor complications (granuloma, edema or web) occurred in 9.>C of patients and supraglottic stenosis occurred in =.=C of patients. 7r. 7enoyelle concluded that supraglottoplasty failed only in patients that had laryngomalacia and additional congenital anomalies. he complication rate was found to be similar between isolated laryngomalacia and patients that had laryngomalacia with additional congenital anomalies. his year (2665), 7r. /? @al%al et al. presented a new approach to supraglottoplasty by ma$ing use of the microdebrider. ?e reported on a series of five patients that had been diagnosed with sever laryngomalacia and underwent a microdebrider-assisted supraglottoplasty. he techni#ue involved initially dividing the aryepiglottic fold with microlaryngeal scissors. Subse#uently, the folds are resected with the microdebrider with tissue removal e'tended anteriorly to the lateral edge of the epiglottis and posteriorly to the arytenoids cartilage. -f redundant supra-arytenoid mucosa was identified, it was removed with the microdebrider. "ll five patients had resolution of their stridor postoperatively. ,o complications including

supraglottic stenosis have been identified and no revision surgeries have been performed. When evaluating an infant that presents with stridor, laryngomalacia tops the differential diagnosis. -t is established that the ma8ority of symptoms of laryngomalacia resolve without intervention by the ages of twelve to eighteen months. he otolaryngologist must decide if the associated symptoms 8ustify operative intervention. 1urthermore, it must be decided if there are associated congenital anomalies that could impact the success of the surgery. -f surgical intervention is pursued, all potential mechanisms contributing to laryngomalacia should be addressed.

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