Flexible Bronchoscopy

Flexible bronchoscopy offers several advantages over rigid bronchoscopy. It facilitates identification of areas that can cause airway obstruction and that may be underappreciated or missed with a rigid bronchoscope. More specifically, flexible bronchoscopy provides better assessment of disorders such as glossoptosis, laryngomalacia, tracheomalacia, and bronchomalacia. Evaluation of the distal airway may provide additional information on vascular compression, bronchiectasis, and assessment of aspiration by BAL. (See Chapter 9 for a more detailed discussion.)

Microlaryngoscopy and Rigid Bronchoscopy

Prior to evaluation in the operating room, vocal cord mobility should be assessed in an office setting in the awake child with transnasal flexible laryngoscopy, because vocal cord movement is poorly assessed with the child under general anesthesia. This may be done as part of a fiberoptic endoscopic evaluation of swallowing (FEES) or as part of a voice evaluation. Microlaryngoscopy and rigid bronchoscopy are performed with the primary goal of identifying anatomic levels of airway obstruction from the larynx to the carina. The supraglottis is evaluated with attention given to the possibility of supraglottic obstruction, such as laryngomalacia and supraglottic stenosis. The vocal fold level is then evaluated for posterior glottic stenosis, anterior glottic web, and laryngeal cleft. It should be noted that the posterior glottis is an area that is suboptimally evaluated by flexible bronchoscopy. If vocal fold immobility is suspected or seen on the FEES evaluation, or on voice evaluation, the cricoarytenoid joints should be palpated to determine if there is any fixation of the joint.

Rigid bronchoscopy is performed using a combination of Hopkins rod telescopes and rigid bronchoscopes. The subglottis is then evaluated. If subglottic stenosis (SGS) is present, it is classified by the Myer-Cotton scale and sized using increasingly larger endotracheal tubes until the largest tube that has a leak at less than 20 cm of water pressure is determined ( Fig. 79.1 ). In addition, the length of stenosis and the proximity to the vocal folds is assessed and documented. If a tracheotomy is in place, attention is paid to the evaluation of the suprastomal area, considering the possibility of suprastomal collapse, granuloma, intratracheal skin tract, and a high tracheotomy. The trachea is evaluated to the level of the carina, looking for additional pathology, including tracheal stenosis, complete tracheal rings, tracheoesophageal fistula (TEF), TEF pouches, vascular compression, and tracheomalacia.

Myer-Cotton grading scale.

The entire airway should also be evaluated for the presence of inflammation because operating on an inflamed airway has a far lower success rate. Common signs of airway inflammation include cobblestoning of the mucosa of the laryngeal surface of the epiglottis, eversion of the laryngeal ventricles, edema of the subglottic mucosa, and loss of definition of tracheal rings due to tracheal mucosal edema.

Esophagogastroduodenoscopy

Evaluation of the upper gastrointestinal tract can provide information that is crucial in decision making with regard to future surgery. Inflammation in the laryngotracheal complex can be caused by conditions of the upper gastrointestinal tract, resulting in an “active” (i.e., inflamed) larynx. Poor wound healing and scarring are more likely to occur in this setting. The two gastrointestinal conditions associated with laryngeal inflammation are eosinophilic esophagitis (EoE) and gastroesophageal reflux disease (GERD); the diagnosis of EoE is made on esophageal biopsy. Laryngeal inflammation may resolve with appropriate treatment of the underlying condition, permitting surgical reconstruction with a lower risk of complication.

Swallowing and Voice Evaluations

Preoperative swallowing and voice evaluations can be crucial because both may alter an otherwise sound surgical plan.

If there is a suspicion of ongoing aspiration, if surgery will involve the glottis, or if surgery will repair a stenosis that may be preventing aspiration, then a swallowing evaluation should be pursued. The two most commonly used evaluations of swallowing are the videofluoroscopic swallow study (VSS) and FEES. These complimentary evaluations can assess ongoing aspiration with swallowing, as well as the likelihood of future aspiration. The advantages of FEES are that the laryngeal protective mechanisms can be visualized by the surgeon, vocal fold motion can be documented, and the potential risk of aspiration can be assessed prior to airway surgery.

Another evaluation that may be useful in children who have a tracheotomy is dye testing. Through the use of green food coloring, aspiration in general, as well as specific causes of aspiration, can be assessed as follows: dye can be placed on the tongue to evaluate aspiration of saliva or secretions; a particular consistency of food can be dyed to assess for consistency-specific aspiration; and gastrostomy tube feeds can be dyed to assess aspiration of refluxed feeds. Aspiration is suspected if stained secretions or feeds are noted from the tracheotomy during feeding or at any time after feeding. Dye testing provides no value in the evaluation of aspiration in children with a grade IV (complete) stenosis. Information obtained from a swallowing evaluation becomes crucial in planning the operative procedure and in counseling the family about the potential risk of aspiration.

Because many airway reconstructive procedures involve a compromise between voice quality and airway improvement, preoperative voice evaluation has become increasingly important. This evaluation provides key information regarding the impact of the initial surgery on voice, the potential impact of further surgery on voice, and the status of vocal cord mobility. In some cases, operative planning can be modified to offer a better balance between long-term voice and airway concerns. When vocal fold immobility is noted on the voice evaluation, the surgeon should search for the specific etiology of the immobility, as various conditions may appear similar on voice evaluation but may be treated differently (e.g., posterior glottic stenosis, vocal cord paralysis, and fixation of the cricoarytenoid joints). This investigation is best done during microlaryngoscopy and bronchoscopy. Counseling families about the impact of airway surgery on future voice quality and the options of voice therapy is also an important part of the overall process. A new indication for structural airway surgery is voice improvement rather than just airway improvement.

Bacterial Colonization

Because of the prevalence of colonization of methicillin-resistant Staphylococcus aureus (MRSA) and Pseudomonas aeruginosa in our patients with complex aerodigestive problems (approximately 30%), we recommend screening for these bacteria by culturing the nares and tracheal aspirate. Both MRSA and P. aeruginosa have a predilection for cartilage and can lead to operative failure. Patients who are found to be positive are treated with perioperative and postoperative antibiotic therapy ( Table 79.1 ). In our collective experience, this protocol decreases the risk of infection-related morbidity.

Eosinophilic Esophagitis

EoE is an uncommon disorder that, if left untreated, may have a significant effect on the aerodigestive tract. Many patients who have EoE also have esophageal, laryngotracheal, and sinonasal complaints; however, some patients are asymptomatic. The diagnosis is made by histologic examination of biopsies taken from the esophagus at the time of esophageal endoscopy. In patients who have active EoE, the laryngotracheal complex is often inflamed. Surgery in the presence of active EoE often elicits a brisk inflammatory response that can lead to graft failure and/or restenosis. If EoE is present, the authors recommend medical management followed by repeat endoscopy with biopsies. After biopsies demonstrate no active EoE, surgery may be performed. See Chapter 78 for a more detailed discussion on EoE and its treatment.

Gastroesophageal Reflux Disease

The evaluation for GERD can include esophagoscopy with biopsies, esophagram, impedance probe, and dual pH probe testing. Because of the potential impact of this condition on postoperative healing, the authors routinely administer prophylactic preoperative and postoperative therapy to patients undergoing airway reconstruction. Most patients are managed with a daily proton pump inhibitor and nighttime H2 blocker therapy. Patients continue the antireflux regimen for up to 1 year following successful reconstruction. Nonacidic reflux may also contribute to laryngeal inflammation and compromised healing. The authors believe that in some cases nonacidic reflux can cause damage in the reconstructed airway and potentially lead to operative failure. When medical treatment fails or nonacid reflux is suspected, a Nissen fundoplication or jejunal feeding should be considered before airway reconstruction.

Obstructive Sleep Apnea

Obstructive sleep apnea (OSA) can be difficult to diagnose and treat, and it can cause failure in an otherwise well-executed operative plan. In children who have multilevel airway obstruction, including a known fixed airway lesion above a tracheotomy, OSA may be difficult to identify, because the tracheotomy cannot be capped during a sleep study. In these cases, a two-stage airway reconstruction is often performed, leaving the tracheotomy in place, which allows for later assessment of OSA.

Pulmonary Disease

Unrecognized or untreated pulmonary disease can increase the risk of operative failure. This broad classification of pathology encompasses numerous diseases that affect the upper and lower respiratory systems, including unrecognized but significant aspiration, bronchopulmonary dysplasia, tracheomalacia, bronchomalacia, cystic fibrosis, and reactive airway disease. Failure to identify any of these disease processes, even when the more commonly found SGS is identified and treated, can result in significant airway obstruction and operative failure. Collaboration with a pediatric pulmonologist is important not only in the diagnosis but also in both the short- and long-term management of these patients. When significant pulmonary disease is identified, it is crucial that surgery be delayed until it is treated.

Patient Selection

Patient selection can significantly affect overall clinical outcome. Although the goal of creating an anatomically normal airway at the site of reconstruction may be achieved from a technical perspective, if a child remains dependent on a tracheotomy because of oxygen or ventilation requirements or suffers from chronic aspiration, then in a more global sense the operation has failed. A tracheotomy is not benign, and tracheotomy-related death or neurologic damage remains a risk for tracheotomy-dependent children. In a small number of cases the primary goal is not decannulation or avoidance of a tracheotomy. Alternatively, the goal may be to create a safer airway in the event of accidental decannulation, to improve or restore voice, or to decrease aspiration events.

Laryngomalacia

Laryngomalacia is the most common cause of stridor in newborns. Symptoms are usually observed at birth or within the first few days of life. Stridor is generally mild but typically worsens with feeding, crying, and lying in a supine position. In 50% of patients, stridor worsens during the first 6 months of life. A subset of children with severe laryngomalacia (5%) may present with a spectrum of symptoms, including apnea, cyanosis, severe retractions, and failure to thrive. Many patients also suffer from clinically significant reflux. In extremely severe cases, cor pulmonale is seen. Although laryngomalacia usually resolves spontaneously by 1 year of age, severe disease necessitates surgical intervention.

Diagnosis is confirmed by awake flexible transnasal fiberoptic laryngoscopy. Characteristic findings include short aryepiglottic folds, with prolapse of the cuneiform cartilages. In some patients a tightly curled (Ω shaped) epiglottis is observed. Because of the Bernoulli effect, characteristic collapse of the supraglottic structures is seen on inspiration. Inflammation indicative of reflux laryngitis may also be seen.

Determining whether to intervene surgically is based more on the severity of symptoms than on the endoscopic appearance of the larynx. Patients with laryngomalacia rarely present with acute airway compromise. In the 5% who require surgical intervention, this may be planned within 1–2 weeks of presentation. Preoperative management of GER is recommended.

Supraglottoplasty, also referred to as epiglottoplasty, is currently the operative procedure of choice. This procedure is quick and effective and can be adapted to the infant’s specific laryngeal pathology. Both aryepiglottic folds are divided, and one or both cuneiform cartilages may also be removed. Postoperative intubation is generally not required unless the child has some additional pathology; in such cases, overnight intubation may be necessary.

Following supraglottoplasty, we recommend that patients be observed overnight. In some children, obstruction persists postoperatively. Repeat fiberoptic laryngoscopy at the bedside is valuable in determining whether this can be attributed to laryngeal edema or persistent laryngomalacia that necessitates further surgery. Reflux management is helpful in minimizing laryngeal edema. Occasionally, although the postoperative appearance of the larynx is adequate, obstructive symptoms are ongoing. Such cases may have an underlying neurologic component, which becomes more evident with time. In neurologic variant laryngomalacia, supraglottoplasty often fails, thus requiring tracheotomy placement.

Vocal Cord Paralysis

Vocal cord paralysis is the second most common cause of stridor in newborns and may be either congenital or acquired. Congenital vocal cord paralysis generally manifests bilaterally. Although it is usually idiopathic, it is sometimes seen in children with central nervous system pathology (e.g., hydrocephalus and Chiari malformation of the brainstem). Most children with bilateral paralysis present with significant airway compromise, although with an excellent voice. They usually do not aspirate unless there is a significant Chiari II malformation. Acquired disease is generally, although not always, a unilateral condition arising from iatrogenic injury to the recurrent laryngeal nerve. Because of the length and course of the left recurrent nerve, this is far more likely to be damaged than the right recurrent laryngeal nerve. As such, acquired disease usually affects the left vocal cord. Risk factors for acquired paralysis include patent ductus arteriosus repair, the Norwood cardiac repair, and esophageal surgery, particularly TEF repair. In older children, thyroid surgery is an additional risk factor. Unlike children with bilateral vocal cord paralysis, most children with unilateral disease have an acceptable airway but a breathy voice. These children are at a slightly higher risk of aspiration.

The diagnosis of vocal cord paralysis is established with awake flexible transnasal fiberoptic laryngoscopy or stroboscopy. After paralysis has been confirmed, management depends on a number of factors. Children with acquired vocal cord paralysis (whether unilateral or bilateral) may experience spontaneous recovery several months after nerve injury; however, this occurs only if the nerve is stretched or crushed but is otherwise intact.

Children with unilateral paralysis can be initially managed with observation, medialization by temporary injection, or speech and voice therapy. Neonates with acquired unilateral vocal cord paralysis are managed based on their symptoms and comorbidities. Determining the appropriate option is based on a discussion with the patient’s family, taking into account the need for restoration of normal voice and improvement of aspiration. Regardless of which option is chosen, these children should be observed for at least 1 year prior to any permanent intervention. If paralysis persists after this period of time and there is a functional deficit, long-term interventions, such as ansa-cervicalis reinnervation, permanent medialization laryngoplasty, or long-term injection medialization, are considered. These options are discussed with the family and are often influenced by the age of the child and the presence of comorbidities. Medialization laryngoplasty is best performed after puberty.

For patients with bilateral paralysis associated with an underlying disease process, successful treatment of that disease may reverse the paralysis; however, up to 90% of these infants ultimately require tracheotomy placement. Given that up to 50% of children with congenital idiopathic bilateral vocal cord paralysis have spontaneous resolution by 1 year of age, surgical intervention to achieve decannulation is almost always delayed until patients are older than 1 year of age. A more recent innovation to prevent the need for tracheotomy placement in neonates with bilateral cord paralysis is the introduction of the endoscopic anterior-posterior cricoid split.

Several surgical options have been used for patients with bilateral paralysis, and no particular option offers a universally acceptable outcome. The aim of surgery is twofold: (1) to achieve an adequate decannulated airway while maintaining voice and (2) to prevent aspiration. Surgical options include laser cordotomy, partial or complete arytenoidectomy (endoscopic or open), vocal process lateralization (open or endoscopically guided), and posterior cricoid cartilage grafting. In a child with a tracheotomy, it is often prudent to maintain the tracheotomy to ensure an adequate airway prior to decannulation. In a nontracheotomized child, a single-stage surgical procedure can be carried out. Acquired bilateral vocal cord paralysis that does not resolve spontaneously is usually less responsive to treatment than idiopathic cord paralysis. In these cases, more than one operative intervention may be required to achieve decannulation. In patients who have undergone such interventions, postextubation stridor may respond to continuous positive airway pressure (CPAP) or high-flow nasal cannula. The postoperative risk of aspiration should be evaluated by a video swallow study before the child returns to a normal diet. During the initial postoperative weeks, some children have an increased risk of aspirating with certain textures, especially thin liquids.

Laryngeal Webs

Laryngeal webs result from a failure of recanalization of the glottic airway in the early weeks of embryogenesis. In severe cases, as recanalization commences posteriorly and progresses anteriorly, complete laryngeal atresia may occur. In less severe cases a thin anterior glottic web may be the only remnant of the recanalization process. Glottic webs are associated with 22q11.2 deletion syndrome in 60% of cases. The web is typically thickened anteriorly and thins out towards the posterior edge.

Although some anterior glottic webs are gossamer thin, most are thick and generally associated with a subglottic “sail” that compromises the subglottic lumen. Patients have varying degrees of glottic airway compromise, which usually manifests in an abnormal cry or respiratory distress. Thin webs may evade detection because neonatal intubation for airway distress may lyse the web.

Thick webs require open reconstruction with either reconstruction of the anterior commissure or placement of a laryngeal keel. These webs have a high association with SGS, and usually a stenosis will need to be addressed concurrently. The presence of thick membranous webs requires placement of a tracheotomy in approximately 40% of patients, although early single-stage reconstruction may be performed in selected cases.

Subglottic Stenosis

SGS can be either congenital or acquired. Congenital SGS in the neonate is defined as a lumen 4.0 mm in diameter or less at the level of the cricoid. SGS is thought to result from a failure of the laryngeal lumen to recanalize and is one of a continuum of embryologic failures that includes laryngeal atresia, stenosis, and webs. Congenital SGS is often associated with other congenital head and neck lesions and syndromes (e.g., a small larynx in a patient with Down syndrome). Acquired SGS is far more common and is typically a sequela of prolonged neonatal intubation, often with an inappropriately large endotracheal tube (one that does not permit an air leak at less than 20 cm of water pressure). Other cofactors for the development of acquired SGS include reflux and EoE.

Levels of SGS severity are graded according to the Myer-Cotton grading system (see Fig. 79.1 ). In its mildest form (no obstruction to 50% obstruction), congenital SGS appears as a normal cricoid with smaller than average diameter, usually elliptical in shape. Mild SGS may manifest in recurrent upper respiratory infections (often diagnosed as croup) in which minimal subglottic swelling precipitates airway obstruction. In a young child, the greatest obstruction is usually 2–3 mm below the true vocal cords. More severe cases may present with acute airway compromise at delivery. If endotracheal intubation is successful, the patient may require intervention before extubation. When intubation cannot be achieved, tracheotomy placement at the time of delivery may be life-saving. Important to note, infants typically have surprisingly few symptoms. Even those with grade III SGS (71%–99% obstruction) may not be symptomatic for weeks or months.

Children with mild acquired SGS may be asymptomatic or minimally symptomatic. Observation rather than intervention may thus be appropriate. This is often the case for children with grade I or II SGS. However, those with more severe SGS (grades III and IV) are often symptomatic, with either tracheotomy dependency or stridor and exercise intolerance.

Radiologic evaluation of an airway that is not intubated may provide the clinician clues about the site and length of the stenosis. Useful imaging modalities include inspiratory and expiratory lateral soft tissue neck films, fluoroscopy to demonstrate the dynamics of the trachea and larynx, and a chest x-ray. However, the single most important investigation is high-kilovoltage airway films. These films are taken not only to identify the classic “steepling” observed in patients with SGS but also to identify possible tracheal stenosis. The latter condition is generally caused by complete tracheal rings, which may predispose the patient to a life-threatening situation during rigid endoscopy or even during intubation.

Whether SGS is congenital or acquired, evaluation requires endoscopic assessment, which is considered the gold standard. Endoscopy is necessary for the diagnosis of laryngeal stenosis. Precise evaluation of the endolarynx should be carried out, including grading of the SGS. Stenosis caused by scarring, granulation tissue, submucosal thickening, or a congenitally abnormal cricoid can be differentiated from SGS with a normal cricoid, but endoscopic measurement with endotracheal tubes or bronchoscopes is required for an accurate evaluation.

In a patient with congenital SGS, the larynx will grow as the patient grows. As such, after initial management of SGS, the patient may not require further surgical intervention. However, if initial management requires intubation, the risk of developing an acquired SGS in addition to the underlying congenital SGS is considerable.

Unlike congenital SGS, acquired SGS is unlikely to resolve spontaneously and thus requires intervention. Reconstruction of the subglottic airway is a challenging procedure, and the patient’s general condition should be optimized before surgery. In children with mild symptoms and a minor degree of SGS, endoscopic intervention may be effective. Endoscopic options include radial incisions (cold steel or laser) through the stenosis, laryngeal dilatation, the application of topical or injected steroids and topical mitomycin. More severe forms of SGS are better managed with open airway reconstruction. Laryngotracheal reconstruction using costal cartilage grafts placed through the split lamina of the cricoid cartilage is reliable and has withstood the test of time. Costal cartilage grafts may be placed through the anterior lamina of the cricoid cartilage, the posterior lamina of the cricoid cartilage, or both. These procedures may be performed as a two-stage procedure, maintaining the tracheal tube and temporarily placing a suprastomal laryngeal stent above the tracheal tube. Alternatively, in selective cases a single-stage procedure may be performed, with removal of the tracheal tube on the day of surgery and with the child requiring intubation for 1–14 days. Higher decannulation rates have been achieved with cricotracheal resection than with laryngotracheal reconstruction in the management of severe SGS. However, cricotracheal resection is a technically demanding procedure that carries a significant risk of complications.

Vascular Compression

Although vascular compression of the airway is not uncommon, most affected children are either asymptomatic or only minimally symptomatic. Forms of vascular compression affecting the trachea include innominate artery compression (most common), double aortic arch, and pulmonary artery sling. Although symptomatic vascular compression of the trachea or bronchi is rare, it is associated with marked symptoms, including biphasic stridor, retractions, a brassy cough, and “dying spells.” Symptoms tend to become worse when the child is distressed. Vascular rings that result from a retroesophageal subclavian artery and a ligamentum arteriosum are less likely to be associated with airway compromise. Bronchial compression by either the pulmonary arteries or aorta may be significant, but in the absence of associated major cardiac anomalies, it is typically a unilateral problem.

The diagnosis of airway compression is best established with rigid or flexible bronchoscopy or both procedures. Thoracic imaging is then useful in assessing the intrathoracic vasculature. Imaging modalities generally include high-resolution computed tomography (CT) with contrast enhancement and three-dimensional reconstruction, magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and echocardiography. In some cases, angiography is required.

In a neonate with acute airway compromise, intubation may be required to maintain the airway prior to definitive treatment. In some cases, CPAP offers a degree of temporary improvement because segmental tracheomalacia may be present in the region of the vascular compression. Prolonged intubation should be avoided because of the risk of forming an arterial fistula from erosion of an endotracheal tube into the area of compression. Similarly, although tracheotomy will establish an unobstructed airway, there is also an increased risk of an arterial fistula into the airway.

The surgical management of symptomatic vascular compression varies, depending on individual pathology. Strategies for managing innominate artery compression include thymectomy and aortopexy; however, if little thymus is present, an alternative procedure is reimplantation of the innominate artery more proximately on the aortic arch. A double aortic arch requires ligation of the smaller of the two arches, which is usually the left one. A pulmonary artery sling is transected at its origin, dissected free, and reimplanted into the pulmonary trunk anterior to the trachea. There is a high incidence of complete tracheal rings in children with a pulmonary artery sling, and these should be repaired at the same time.

Although alleviating vascular compression improves the airway, it takes time for the airway to completely normalize. This is a consequence of long-standing vascular compression having adversely affected the normal cartilaginous development of the compressed segment of trachea, with resultant cartilaginous malacia or stenosis. Until the airway normalizes, children who are persistently symptomatic may require stabilization with a tracheotomy. Tracheal stabilization with the use of intratracheal stents is alluring, but the incidence of complications is high. Placement of a temporary tracheotomy is therefore a more desirable alternative.

Posterior Laryngeal Clefts

Posterior laryngeal clefts result from a failure of the laryngotracheal groove to fuse during embryogenesis. In a widely used anatomic classification system, these clefts are divided into four subtypes associated with varying levels of severity; type I cleft is the least and type IV cleft the most severe. Other associated anomalies are common and may be divided into those that affect the airway and those that do not. Associated airway anomalies include tracheomalacia (>80%) and TEF formation (20%). Nonairway associations include anogenital anomalies and GER. The most common syndrome in which posterior laryngeal clefting occurs is Opitz-Frias syndrome; hypertelorism and anogenital anomalies are other features of this syndrome.

Although aspiration is the hallmark clinical feature of this disorder, signs and symptoms may be nonspecific, making the diagnosis elusive. Symptoms may also include apnea, recurrent pneumonia, feeding difficulties, and airway obstruction.

VSS and FEES may suggest the risk of aspiration in children with clefts; however, definitive diagnosis requires rigid laryngoscopy and bronchoscopy, with the interarytenoid area being specifically probed to determine if a posterior laryngeal cleft is present.

Initial management decisions should consider whether the infant requires tracheotomy placement, gastrostomy tube placement, or Nissen fundoplication. Although none of these procedures is essential, each increases the likelihood of a successful cleft repair. Protection against aspiration is also crucial, and nasojejunal feeding may be a useful preventative measure. Surgical repair may be performed endoscopically for most type I and type II clefts, and some type III clefts; however, longer clefts that extend into the cervical or thoracic trachea require open repair. The transtracheal approach is advocated in that it provides unparalleled exposure of the cleft while protecting the recurrent laryngeal nerves. A two-layer closure is recommended, with the option of performing an interposition graft if warranted; a useful interposition graft is a free transfer of clavicular or tibial periosteum, or costal cartilage. Because all clefts are prone to anastomotic breakdown, repeat endoscopy and postoperative swallowing studies should be performed to evaluate for aspiration and to confirm a successful repair. Despite successful repair, patients may experience continued problems with dysphagia.

Recently there has been an increasing emphasis placed on type I clefts and associated deep interarytenoid notches. Endoscopic repair of minor clefts has become far more common in children who are diagnosed because of a high index of suspicion or evidence of recurrent or chronic aspiration. This suggests that in such patients, type I clefts may be more of a functional diagnosis than an anatomic diagnosis.

Tracheomalacia

Tracheomalacia is the most common congenital tracheal anomaly. Most children are either asymptomatic or minimally symptomatic, and most cases involve posterior malacia of the trachealis, with associated broad tracheal rings. Commonly associated abnormalities include laryngeal clefts, TEF, and bronchomalacia. Presenting symptoms include a brassy cough, wheezing, respiratory distress when agitated, and “dying spells.” Diagnosis is established with rigid or flexible bronchoscopy, while maintaining spontaneous respiration. The key diagnostic elements include: (1) ascertaining the severity of the malacia; (2) ascertaining the location of the malacia, particularly the possible presence of associated bronchomalacia; and (3) determining whether positive pressure support improves the malacia.

Although mild tracheomalacia is watched expectantly and anticipated to improve with time, more severe symptoms warrant intervention. The most common intervention is tracheotomy placement, with the tip of the tracheotomy tube bypassing the malacic segment. Positive pressure support delivered through the tracheotomy tube assists with the management of associated bronchomalacia. Although there is currently no definitive surgical approach to repair tracheomalacia, this is an area of active research.

Complete Tracheal Rings

Complete tracheal rings are a rare but life-threatening anomaly that presents with progressive worsening of respiratory function over the first few months of life, stridor, retractions, and marked exacerbation of symptoms during intercurrent upper respiratory tract infections. Children with distal tracheal stenosis usually have a characteristic biphasic wet-sounding breathing pattern that transiently clears with coughing; this pattern is referred to as “washing machine breathing.” The risk of respiratory failure increases with age.

An initial high-kilovolt airway film may indicate tracheal narrowing; however, the diagnosis is established with rigid bronchoscopy. This should be performed with utmost caution, using the smallest possible telescopes, as any airway edema in the region of the stenosis may turn a narrow airway into an extremely critical airway. If the stenosis is severe, the stenotic airway should not be instrumented, even with the smallest telescope. The initial bronchoscopic view is often sufficient to establish the diagnosis, thereby avoiding the risk of airway edema. Because 50% of children have a tracheal inner diameter of approximately 2 mm at the time of diagnosis, the standard interventions for managing a compromised airway are not applicable. More specifically, the smallest endotracheal tube has an outer diameter of 2.9 mm, and the smallest tracheotomy tube has an outer diameter of 3.9 mm; hence the stenotic segment cannot be intubated. This may leave extracorporeal membrane oxygenation (ECMO) as the only viable alternative for stabilizing the child. This situation is best avoided by performing bronchoscopy with the highest level of care. More than 80% of children with complete tracheal rings have other congenital anomalies, which are generally cardiovascular. As such, investigation should include a contrast-enhanced high-resolution CT scan of the chest and an echocardiogram. Specifically, a pulmonary artery sling should be excluded because this is a common association and, if present, should be repaired concurrent with the tracheal repair. Most children with complete tracheal rings require tracheal reconstruction. The recommended surgical technique is the slide tracheoplasty. This approach yields significantly better results than any other form of tracheal reconstruction and is applicable to all anatomic variants of complete tracheal rings.