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

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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

Table 1 The postoperative results of surgical treatment.
Resolved Stridor Feeding diculty Failure to thrive 8 (80%) 9 (90%) 8 (80%) Improved 1 (10%) 1 (10%) 2 (20%)

151

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

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

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

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).

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. References
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Laryngomalacia: Diagnosis and management

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