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Cover page of the Journal of Health Sciences

 Table of Contents  
Year : 2019  |  Volume : 12  |  Issue : 3  |  Page : 196-201

Pediatric airway diseases

1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India
2 Department of Pediatrics, IMS and SUM Hospital, Siksha “O” Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India

Date of Web Publication15-Oct-2019

Correspondence Address:
Dr. Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha "O" Anusandhan (Deemed to be University), Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kleuhsj.kleuhsj_12_19

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The diseases of the pediatric airway is often challenging to the clinician. It needs a dedicated team of clinicians for evaluation and treatment. Obstructing lesions of the pediatric airway due to congenital or acquired causes often create fatal situations. Majority of the pediatric airway lesions are due to prolonged intubation or tracheostomy and prematurity. Management of the pediatric airway lesions often complicated with additional congenital malformations or severe comorbidities. It is often important for the pediatrician to understand the different pediatric airway lesions and its issues relevant to the airway management of the pediatric patients. This review article will give an overview of commonly encountered airway problems in children and its management.

Keywords: Airway diseases, laryngotracheal stenosis, pediatric airway

How to cite this article:
Swain SK, Choudhury J. Pediatric airway diseases. Indian J Health Sci Biomed Res 2019;12:196-201

How to cite this URL:
Swain SK, Choudhury J. Pediatric airway diseases. Indian J Health Sci Biomed Res [serial online] 2019 [cited 2022 Aug 13];12:196-201. Available from: https://www.ijournalhs.org/text.asp?2019/12/3/196/269193

  Introduction Top

The pediatric airway has distinct anatomical features in comparison to the adult airway. The airway changes in shape, size, and position throughout its development from the neonatal period to adult. The exact knowledge for the functional anatomy of the pediatric airway helps to understand the pathological conditions of the airway. The dimensions of the pediatric airway are much smaller than the adult one. The cartilaginous parts of the pediatric airway are often malleable and cannot give the same stability as an adult. The inner diameter of the subglottis of a newly born child usually measure around 3–4 mm.[1] Any lesion in the subglottis of the newborn can lead to life-threatening near occlusion of the subglottic airway. In early ages of life or newborn, the lack of stability/rigidity of trachea often prone to tracheomalacia. Due to the poor stability of trachea in children often require prolonged postoperative stenting for supporting laryngotracheal reconstruction. Vascular rings also give rise to pressure on the airway, leading to the dynamic collapse, which often needs surgical correction.[2] In the children, the trachea is very flexible, which helps for longer segment resection without undergoing much tension on the anastomosis site.[3] The thyrohyoid membrane in pediatric cases is shorter than adult ages, and the thyroid notch projected behind the hyoid bone. The high position of the pediatric larynx and presence of epiglottis behind the uvula facilitate breathing and swallowing simultaneously.[4]

  Diseases of the Pediatric Airway Top


Most common laryngeal disease in infant is the laryngomalacia. In laryngomalacia, there is the collapse of the supraglottic structures during inspiration. It is the most common cause of stridor in infants. Stridor due to laryngomalacia is often mild and is exacerbated during feeding, crying, and lying in a supine position. In approximately 50% of the cases, symptoms worsen during the first 6 months of life, and symptoms resolve by 1 year of age.[5] In the case of severe laryngomalacia (approximately 10%), surgery is needed where the symptoms are cyanosis, apnea, severe retraction, and failure to thrive.[6] In extremely severe cases, cor pulmonale may be seen. The diagnosis of the laryngomalacia is confirmed by fiberoptic nasopharyngolaryngoscopy. The characteristic features of laryngomalacia are short aryepiglottic folds with prolapsed of the cuneiform cartilages [Figure 1]. The shape of the epiglottis is described as omega-shaped. Due to the Bernoulli effect, the characteristic collapse of the supraglottic structures is found in inspiration. Laryngomalacia has often a favorable outcome. Approximately 10%–20% of infants present with severe laryngomalacia which need surgical treatment.[7] Surgery is advised when laryngomalacia is associated with one or more symptoms such as dyspnea, obstructive sleep apnea, suffocation during feeding, and poor weight gain. The medical/conservative treatment includes lifestyle, and dietary measures must be instituted like thickened milk, no bottle of water before lying down, raising the head of the bed or mattress, and the maintenance of posture after feeding. Antacids in infants are prescribed to prevent regurgitation.
Figure 1: Endoscopic picture of laryngomalacia

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Laryngeal webs

Laryngeal webs [Figure 2] occur due to failure of recanalization of the glottic airway during embryogenesis of life. Approximately 95% of the glottic webs are present anteriorly however the laryngeal webs are rarely seen, comprising only 5% of the congenital laryngeal lesions. Although there is no association between the congenital glottic web and gene anomaly, an association is seen between chromosome 22q11.2 deletion syndrome and glottic web (velocardiofacial syndrome).[8] In >50% of cases with anterior glottic web, there are chromosome 22q11.2 deletion syndrome, and hence, all patients of glottic web need genetic counseling. Neonates with glottic web often present with an abnormal cry or breathing difficulty at birth. If respiratory distress is more in the first few hours or days of life, the neonates need airway intervention. The laryngeal web is properly evaluated by flexible bronchoscopy, which gives an excellent view of the anterior commissure, whereas the rigid bronchoscopy is better for evaluating the degree of associated subglottic stenosis. Infant with severe web may need intubation or emergent tracheotomy in an emergency. In infant with a thin anterior web, it needs division of the web with a sickle knife while suspended on a small Lindholm laryngoscope. In case of the thick glottic web, the decision must be made as to whether surgery/repair should be done early or later in childhood. In cases of the mild or moderate web without airway, compromise is often preferable to perform surgery in a larger larynx. Delayed repair is often done by the age of 4 years, so to improve the quality of the voice in children. In children with a severe degree of the glottic web with airway compromise need repair early. An alternative, a tracheotomy may be done, and late repair may be planned. The surgical option of the glottic web includes open keel placement and open reconstruction of the anterior commissure. Endoscopic laser division of the glottic web or endoscopic keel placement is not advocated although these are options in older patients.
Figure 2: Endoscopic picture of laryngeal web

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Vocal cord paralysis

In children, vocal cord paralysis [Figure 3] is often due to neurologic cause or sometimes occurs after cardiothoracic procedures. In the majority of cases, the left recurrent laryngeal nerve paralysis occurs due to its close relationship with the aortic arch and left pulmonary artery. Usually, immediate treatment is not advised in this case, whereas watchful waiting is done with follow-up in 3-month interval. If vocal cord paralysis persists, thyroplasty can be done in children.
Figure 3: Laryngoscopic picture showing unilateral vocal cord palsy

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Laryngotracheal stenosis

Laryngotracheal stenosis (LTS) is a partial or complete cicatricial narrowing of the endolarynx or trachea.[9] Traumatic intubation is the most common cause of LTS, which accounts for about 90% of all pediatric acquired airway stenosis.[10] The intubation trauma is often due to the use of an endotracheal tube being too large for the pediatric airway. In pediatric cases, the smallest endotracheal tube providing adequate ventilation should be used. The mild resistance during intubation should lead to downsizing to smaller endotracheal tube size. Endotracheal tube accompanied with a stylet has to be used with caution as it can cause significant injury to laryngotracheal airway. Cuffed endotracheal tubes are usually used when intubation is needed for a short period. Cuffless endotracheal tubes are usually considered for longer period intubation as these are less traumatic. Endotracheal tubes are often better tolerated in pediatric cases than adults, particularly if nasotracheal tubes are used. The right time for performing tracheostomy in infants and children those requiring prolonged ventilation is still a matter of discussion. In children, there are two sites of larynx prone to pressure related injury: the posterior commissure of the larynx and subglottis. Injury to the posterior commissure of the larynx leads to scarification and fixation of the cricoarytenoid joints (posterior glottic stenosis). Injury at the subglottis causes circumferential strictures at the level of cricoid cartilage [Figure 4]. The severity of the LTS is classified by using the modified Meyer-Cotton grading system. This grading system of the LTS incorporates the severity of the stenosis and its involvement of the glottis as well as additional comorbidities. Different comorbidities are severe congenital cardiovascular anomalies, neurological abnormalities, or syndromes. The goal of treatment for LTS is always decannulation and re-establishment of the airway along with the preservation of laryngeal function such as airway protection, voice, and swallowing.[11] Medical treatment has a limited role in LTS, whereas the surgical options are two types as follows: endoscopic or open. Endoscopic surgery is helpful in Grade-I or II stenosis and open is often done in Grade-III or IV stenosis.[12]
Figure 4: Endoscopic picture showing subglottic stenosis

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Localized tracheomalacia

External compression of the airway by the vascular ring (cardiovascular anomalies) leads to localized tracheomalacia. The vascular ring may be complete or incomplete. In children, the most common cause of localized tracheomalacia is an aberrant brachiocephalic artery which compresses the right anterolateral aspect of the trachea (incomplete ring). The commonly accepted treatment of this anomaly is aortopexy.[13] Complete rings such as vascular slings or double aortic arch often need complicated cardiovascular reconstructions with airway repair.[14] The differential diagnosis of localized tracheomalacia/vascular rings is diffuse congenital tracheomalacia in premature or preterm infants, caused by immature cartilage. Most often, the airway skeleton stabilizes with the growth of the child and needs supportive measures like humidifier air, which is sufficient to overcome this situation.

Laryngeal/laryngotracheal clefts

Laryngeal and laryngotracheal clefts are uncommon congenital lesions with an insufficient division of the respiratory tract and the upper digestive tract.[15] They can be classified as simple inter arytenoids clefts (type I) to extensive laryngotracheal defects with a complete split of the trachea up to one main bronchus. The clinical presentations in pediatric patients depend on the extent of the cleft. The type-I laryngotracheal cleft may be asymptomatic, but high degree laryngotracheal clefts can cause stridor, coughing aspiration, chocking, and recurrent pneumonia. Laryngeal endoscopy is often used for the workup. The anatomic classification for posterior laryngeal/laryngotracheal cleft was proposed by Benjamin and Inglis [16] which divides posterior laryngeal clefts into the four subtypes. The four types [Figure 5] of laryngeal clefts are Type 1: supraglottic interarytenoid cleft present to, but not below, the level of the true vocal folds. This cleft could also be considered a deep interarytenoid notch. Type 2: partial cricoid cleft extends into, but not through the posterior cricoid cartilage. Type 3: total cricoid cleft with or without extension of the cleft into the cervical trachea. Type 4: cleft extending to the thoracic trachea. The treatment is decided on the basis of the type of cleft. Laryngeal cleft can be treated endoscopically, whereas laryngotracheal cleft often needs open surgical repair.
Figure 5: Different types of laryngotracheal clefts

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Subglottic hemangioma

The subglottis of the larynx is the most common site for hemangioma within the airway. In the case of subglottic hemangioma, >50% of the cases have cutaneous hemangioma, which gives suspicion for the subglottic hemangioma. In the case of cutaneous hemangioma, the lesions initially proliferate followed by involution, whereas hemangioma in subglottis increases in size and progressively deteriorates. The clinical symptoms are often biphasic stridor with retraction, particularly when the child is feeding or upset. The subglottic hemangioma is properly evaluated by awake transnasal flexible laryngoscopy. It will give visualization of the subglottis and also rule out other causes of neonatal stridor such as laryngomalacia and vocal cord palsy. A pediatric patient with progressive stridor and normal supraglottic and glottic examination need a laryngoscopy and bronchoscopy in the operating room under general anesthesia. Magnetic resonance imaging with T2-weighted gadolinium contrast is usually advised as imaging for subglottic hemangioma. Biopsy of this lesion is not advised for the risk of hemorrhage. The treatment modality of subglottic hemangioma is often combined. Symptomatic children with stridor are often managed by systemic steroids along with propranolol, a nonselective beta-blocker. Propranolol therapy is now replaced both endoscopic and open resection and avoiding tracheostomy in case of subglottic hemangioma.[17] Premature stoppage of this therapy may lead to hemangioma growth.[18] Hence, careful monitoring is essential during the weaning process of propranolol treatment.

Saccular cyst

The saccule of the larynx is a diverticulum containing several mucous glands at the anterior end of the ventricle [Figure 6]. It extends superiorly between the vestibular fold and the inner surface of the thyroid cartilage.[19] Congenital saccular cysts are often filled with mucus and extend posterosuperior into the vestibular fold (false vocal fold) and aryepiglottic fold (lateral saccular cyst). These congenital saccular cysts usually present with airway obstruction, muffled cry, and dysphagia in the first few days of life. As the infant has low muscle tone, during induction of general anesthesia, there will be complete airway obstruction. It is always better to perform preoperative awake flexible nasopharyngolaryngoscopy for evaluation of the airway as it will forewarn of the potential problems following anesthesia. During the radiological examination of the infant with sedation or anesthetic may lead to disastrous. It is better to evaluate and secure the airway in the operating room and consider for imaging after the airway is once secured. There are several treatment options of laryngeal saccular cysts such as needle aspiration, marsupialization, or endoscopic removal, but the results are not satisfactory. In transcervical approach, the superior border of the thyroid cartilage is identified, and the incision is made on the thyrohyoid membrane along with superior border of the thyroid cartilage where the cyst is easily approached and cyst removed in intact.
Figure 6: Endoscopic picture showing saccular cyst

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Posttracheostomy tracheal stenosis/tracheomalacia

In pediatric patients, the site of tracheostomy is important for preventing tracheal stenosis. In the case of LTS, the ideal site for tracheostomy is the first tracheal ring. It will help the surgeon to include tracheostomy stoma/tract in the resection during repair later on. In cases of a longer neck, the tracheostomy site can be at the lower part of the trachea. In nonLTS, high tracheostomy is always avoided.[1] The symptoms of tracheal stenosis develop progressively within weeks/months after successful tracheostomy in adult, whereas children cannot tolerate the decannulation when there is an underlying problem.

Recurrent respiratory papillomatosis

Recurrent respiratory papillomatosis is a rare clinical entity which presented with multiple exophytic squamous warts like lesions [Figure 7] seen in the larynx and also in the tracheobronchial tree.[20] Recurrent papillomatosis is the most common neoplastic lesion of the pediatric airway. It is caused by human papillomavirus 6, and 11 and the lesions are usually exophytic and highly recurrent, affecting the airway mucosa, mainly the larynx. The children often present with hoarseness of voice and breathing difficulty. The treatment of choice is surgery for getting a patent airway and improved voice quality. However, recurrence is common after surgery in pediatric patients. As repeated surgery are often needed, may lead to scarring and stenosis.[21] Adjuvant treatments include interferon-α2a, indole-c-carbinol, retinoic acid, photodynamic therapy, and antiviral agents.[22]
Figure 7: Laryngoscopic picture showing multiple laryngeal papillomatosis

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The tongue is an indispensable structure of the oral cavity helpful for speech production, swallowing, and breathing.[23] Macroglossia refers to a tongue which protrudes beyond the alveolar ridge.[24] Common causes for macroglossia are hemangioma, glandular hyperplasia, and lymphangioma. The large tongue reduces the space of the oral cavity in children and easily blocks the airway. The lower muscle tone of the tongue also leads to passive obstruction of the airway. During the supine position of infants, the tongue becomes flatten against the soft palate during inspiration, and it will remain in the same position for the passive expiration through the nasal cavity.[25] If the head will be extended at the atlanto-occipital joint, may lead to improved hypopharyngeal airway patency but does not necessarily alter the position of the tongue.[26] The treatment of choice is surgery, and the surgical technique is either midline or peripheral glossectomy.

Adenoid hypertrophy

Adenoid is a lymphoid tissue situated at the junction of the roof and posterior wall of the nasopharynx. Adenoid hypertrophy is a common clinical entity during pediatric ages. It occludes the choana and lead to upper airway obstruction and presents with mouth breathing, hyponasal speech, and snoring in children.[27] It may lead to serous otitis media, obstructive sleep apnea, and accompanying cor pulmonale and growth retardation. The treatment is determined on the degree of airway obstruction and associated morbidity. The treatment is often surgery called adenoidectomy. The intranasal steroid may be useful in some cases of low-grade adenoid hypertrophy.[28]

  Conclusion Top

Pediatric airway diseases are always challenging clinical entity in the medical field, which needs a dedicated multidisciplinary team pediatricians, ENT surgeons, anesthetists, thoracic surgeons, and phoniatricians. The most common clinical presentation is noisy breathing. The management of pediatric airway diseases is often stressful to clinicians. Emergency physicians are often familiar with the adult airway management but not to pediatric airway, so face difficulty during managing the pediatric airway lesions as these are less frequently seen. Proper diagnosis and experienced hands can achieve excellent results. Management of the pediatric airway lesions is varied from patient to patient. The management includes observation, investigations, medical, and surgical treatment.

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Conflicts of interest

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  References Top

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]

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