Airway
Choanal atresia
Laryngomalacia
Glossoptosis
Vocal fold paralysis
Laryngotracheoesophageal cleft
Stenosis: glottic, subglottic, tracheal, transglottis
Tracheobronchomalacia
Tracheoesophageal fistula
Tracheostomy dependence
Pulmonary
Chronic lung disease of prematurity
Diffuse lung disease
Asthma
Bronchiectasis: aspiration, ciliary dyskinesia, immunodeficiency, post-obstructive
Chronic respiratory failure
Gastroenterology
Gastroesophageal reflux
Eosinophilic esophagitis
Esophageal structure
Failure to thrive
Feeding and swallowing
Swallow incoordination
Oral aversion
Behavioral feeding problems
Sleep
Obstructive sleep apnea
Central sleep apnea
Hypoventilation
Genetic
Trisomy 21
CHARGE association
Pierre Robin sequence
22q11 deletion
VATER/VACTERL
Craniofacial syndromes
Opitz syndrome
Cri du chat
Neurologic
Static encephalopathy
Chiari malformation
Hypotonia
There are a significant number of specialists that would be required to cover all of the possible conditions in children with aerodigestive disorders. Through consensus development by Boesch et al. [7], however, the list of essential core members whose input is required for all patients attending an aerodigestive program can be distilled to the following: care coordinator, nursing, speech language pathologist, pulmonologist, gastroenterologist, and otolaryngologist. An aerodigestive program should include these key players at a minimum.
Consensus was also achieved regarding the essential and defining functions and features of an aerodigestive team evaluation [7]. For maximal efficiency and efficacy, the care cycle for an aerodigestive patient would involve the following work flow: consultation request and care coordination, pre-visit intake, team meeting, prescheduling appointments and procedures, shared clinic visit, combined endoscopy with a single anesthetic encounter, wrap-up visit with the family, summary document, and provision of follow-up care if needed.
The typical aerodigestive program will see patients with a mix of medical and surgical needs. The interdisciplinary approach is important to effectively manage and plan the order of events leading to maximization of medical and surgical interventions and outcomes. Piccione et al. [8] also emphasized that there are several consistent structural elements of an aerodigestive program, namely, a (1) interdisciplinary medical and surgical team, (2) care coordination, (3) team meeting, and (4) combined endoscopy.
Common aerodigestive diagnostic tests
Diagnostic modality | Strengths | Weaknesses |
---|---|---|
Chest radiograph | Identification of lower respiratory tract disease | Low sensitivity for bronchiectasis |
Low radiation | Limited ability to differentiate causes of lung disease | |
Chest CT | Distribution and severity of lung findings of various types | Increased radiation |
Differentiation between airway and parenchymal disease | May require sedation for good imaging | |
Upper GI series | Evaluation of anatomy: peristalsis, stricture, hernia, gastric outlet obstruction, malrotation | Does not evaluate reflux |
Radionucleotide reflux scan | Physiologic conditions | Limited sensitivity |
May document aspiration from reflux | ||
Radionuclide salivagram | Assess for aspiration of saliva | Poor sensitivity |
Radionuclide parotid scan | Assess function of major salivary glands | |
FEES (fiber-optic endoscopic evaluation of swallowing) | Evaluate functional anatomy of swallowing | Blind to moment of pharyngeal swallowing and esophageal phase |
Evaluate airway protective reflexes | Not widely available | |
Portable | ||
No radiation | ||
VFSS (videofluoroscopic swallowing study) | Evaluates all phases of swallowing | Radiation exposure |
Evaluates for aspiration | Limited anatomic evaluation | |
Microlaryngoscopy and rigid bronchoscopy | Superior optical resolution | Difficult access to peripheral airways |
Evaluation of the posterior larynx | Limited assessment of airway dynamics | |
Access for instrumentation | Requires anesthesia | |
DISE (drug-induced sleep endoscopy) | Assessment of anatomic site of obstruction during sleep | Only an approximation of sleep state |
May miss REM specific obstruction | ||
Flexible bronchoscopy with lavage | Evaluation of static and dynamic airway lesions, nasal-bronchial | Limited evaluation of posterior larynx |
Access to difficult and peripheral airways | Limited optical resolution | |
Evaluation of airway inflammation and infections | Access for instrumentation | |
Requires anesthesia | ||
Esophagogastroduodenoscopy (EGD) | Evaluation of esophageal mucosal disease: acid and eosinophilic | Requires anesthesia |
Evaluation of esophageal, gastric, and duodenal anatomy | ||
Obtain intestinal secretions | ||
Evaluation of celiac disease | ||
Esophageal impedance | Identification and characterization of acid and nonacid reflux | Lack of normative data |
May identify dysmotility | Unclear relationship between impedance indices and extra-esophageal disease | |
Motility studies | Gold standard for dysmotility | Not widely available |
Polysomnography (PSG) | Characterization of sleep disordered breathing and sleep architecture | Expensive and cumbersome |
Titration of respiratory support | Availability issues |
Each of the core specialists will bring their perspective and process for evaluating the chief complaints and symptoms presented by the patient [8]. Although the group encounter, with all present for the clinic interview and the operative endoscopies, has been found to be the most efficient and efficacious, each provider brings unique and individual expertise. Each of the four core disciplines has overlap but also bring a unique role in the evaluation of these complex patients. The role of each core discipline will be outlined in this chapter.
Role of the Speech Language Pathologist
The multidisciplinary voice, swallow, and aerodigestive team can provide comprehensive, patient-centered and evidence-based care for children and adolescents with a variety of disorders impacting voice, swallow, and upper airway. Multidisciplinary team management of aerodigestive disorders in children has been found to be more cost-effective and has better outcomes than stand-alone care [6]. In voice disorders, the model of speech language pathologist and otolaryngologist working together in evaluation and treatment has been well established since the 1980s and became more common in pediatric voice around a decade later. The strength of these teams lies in both the diverse knowledge and skills of the team members and their ability to work collaboratively to evaluate and treat the patient. The speech language pathologist specializing in these areas provides a focused set of knowledge and skills for these patients. We can provide evaluation of structure, function, and behavior of upper airway as they relate to voice, swallow, and breathing. In many cases we can also provide behavioral therapy to change voice, breathing, and swallow function, provide education, and provide compensatory strategies when needed. We have specialized knowledge of laryngeal structure and function; the mechanics of voice, breathing, and swallowing; and neurologic controls of voice, swallow, and breathing. We provide valuable contributions with our in-depth understanding of behavior change. On any medical team, but especially with complex children, we do not operate in a vacuum and collaborate with surgical and medical personnel in both evaluation and treatment. According to the American Speech-Language-Hearing Association’s scope of practice statement, “SLPs share responsibility with other professionals for creating a collaborative culture. Collaboration requires joint communication and shared decision making among all members of the team, including the individual and family, to accomplish improved service delivery and functional outcomes for the individuals served” [9].
Evaluation of Swallow
Dysphagia is relatively common in children. A rate of 0.9% was found in children aged 3–17 [10], and incidence is higher in certain medically complex populations including those with cerebral palsy and craniofacial syndromes [11–13]. There has been a marked increase in diagnoses of dysphagia in the pediatric hospitalized population, from 0.08% in 1997 to 0.41% in 2012 [14]. While exact reasons for this are not clear, it is often attributed both to increased survival rates of extremely preterm infants and improved diagnosis of swallowing disorders. Often the SLP is the first contact a child with dysphagia has with the multidisciplinary team. Children may be referred directly to us for a swallow evaluation or for treatment of feeding or swallowing disorders, or we may care for the child in the NICU from birth. We have the benefit of being able to spend the time to get a comprehensive history and provide ongoing assessment in therapy sessions. The SLP has several methods of evaluating swallowing, including the clinical swallowing evaluation, flexible endoscopic evaluation of swallowing, and video fluoroscopic swallowing study, as well as less frequently used measures including manometry. These may be used in combination depending on the needs of the patient. According to ASHA, the role of the SLP in evaluation includes participating in determining the appropriateness of instrumental evaluation and follow-up, diagnosing pediatric oral and pharyngeal swallowing disorders, making appropriate referrals to other disciplines, and recommending a safe swallowing and feeding plan [9].
We require the expertise of others when evaluating and planning treatment beyond swallow recommendations for structural and functional deficits impacting swallowing, including (but not limited to) neurologic impairments, cerebral palsy (CP), sensory deficits, tracheoesophageal fistula, laryngeal cleft, acid reflux, esophageal dysmotility, laryngeal mobility impairment, and neurologic disorders.
Treatment of Dysphagia
SLPs on the multidisciplinary team as well as our colleagues working in more general outpatient settings, birth to three, and schools provide feeding and swallowing therapy to habilitate or rehabilitate swallowing and progress feeding skills. Feeding is defined as any aspect of eating or drinking and includes preparing food or liquid for intake, sucking or chewing, and swallowing [15]. Swallowing specifically refers to the complex processes involved in transporting solids, liquids, or saliva from the mouth to the digestive tract while maintaining airway protection [15]. Speech language pathologists are involved in evaluation and treatment of both.
A detailed description of all forms of feeding and swallowing therapy is beyond the scope of this chapter. Approaches to swallowing treatment may include positioning changes, changes in viscosity of bolus, changes in flow rate of bolus, maneuvers, sensory stimulation techniques, oral motor treatments, pacing, and cue-based feeding [15].
Evaluation of Voice
Incidence estimates of pediatric dysphonia are varied, ranging from 1.4% [10] to 26% [16]. Dysphonia rates in children are likely increasing for some of the same reasons dysphagia rates are increasing, and children are presenting with more complex etiologies of voice disorders, beyond benign lesions. As survival rates of children born extremely preterm, or with complex tracheal or laryngeal anomalies, increase, rates of hoarseness and the complexity of children seen in the voice clinic will increase. For example, 38% of a sample of children born extremely preterm were found to have moderate-severe dysphonia at school age, with only 6% having normal voice [17]. Speech language pathologists often work in collaboration with an ENT in diagnosis and evaluation of voice disorders. According to the ASHA scope of practice, SLPs can perform a comprehensive voice evaluation which includes clinical and instrumental evaluation, assess normal or abnormal vocal function, describe voice quality and function, diagnose a voice disorder, refer to appropriate professionals to provide diagnosis of the underlying cause of the voice disorder (e.g., nodules as a cause of dysphonia), and make referrals to other professionals for other medical, surgical, or behavioral evaluation [9]. We can perform perceptual, acoustic, and aerodynamic evaluation of vocal function. We can also visualize the larynx using rigid or flexible endoscopy with stroboscopy, as well as high-speed digital video imaging of the larynx, and provide skilled interpretation of structure and function based on this. We do not diagnose lesions but can identify and describe the parameters of laryngeal function based on these evaluations and contribute to planning treatment, whether it be behavioral, surgical, or a combination of the two. The voice evaluation is also an important time to assess for stimulability for change based on therapeutic probes.
Treatment of Dysphonia
SLPs in a voice clinic and in other settings plan and deliver skilled treatment to optimize vocal function given the current anatomy, provide pre- and postoperative therapy, and provide therapy to change ingrained vocal functional behaviors. A detailed discussion of the types of voice therapy provided is beyond the scope of this chapter but can be found in other sections of this book and in these and other resources [18–23].
Evaluation and Treatment of Breathing Disorders
Speech language pathologists are also experts in evaluation and management of laryngeal breathing disorders such as paradoxical vocal fold motion disorder, exercise-induced laryngomalacia, and chronic cough [9]. We can behaviorally and endoscopically evaluate laryngeal, pharyngeal, and respiratory function during breathing and provide interventions related to laryngeal sensitivity and control as well as optimizing respiratory coordination [24–28].
Conclusion
The benefits of working as a part of a multidisciplinary team cannot be overstated, for both clinician and patient. We are able to evaluate based on our areas of expertise and then discuss with other team members based on the findings of their specialized evaluations, providing optimal treatment for patients.
Role of the Otolaryngologist
As a specialist of disorders of the upper aerodigestive tract, the otolaryngologist shares the pathway to both the lungs and the gastrointestinal tract. This unique perspective positions them to be able to relate to both the pulmonologist and gastroenterologist. Working in conjunction with the speech language pathologist, the otolaryngologist can help assess the anatomy and physiologic function of the upper aerodigestive tract. Medical treatments of aerodigestive disorders in children are likely made in conjunction with the gastroenterology and pulmonology regarding reflux, inflammation, or infection. Dynamic surgical interventions of the airway may be suggested after functional assessment in collaboration with the speech pathologist. The typical aerodigestive problems evaluated by the otolaryngologist can be seen in Table 1.1. The role of the otolaryngologist centers primarily on evaluation of airway surgical issues and aspiration [8].
The otolaryngologist should elicit history specific to obstructive sleep apnea, voice and swallowing disorders, recurrent infection, previous surgical history, or instrumentation of the airway. An assessment of possible congenital or genetic disorders is also essential. Growth and weight gain curves are helpful to assess potential feeding or breathing problems.
The otolaryngologist can offer expertise in office and operative endoscopy to evaluate function and anatomy. Identifying sites of abnormal anatomy, obstruction, or function of the upper aerodigestive tract is the prime modality offered. Expertise in nasopharyngoscopy in the awake patient facilitates anatomical and functional evaluation for airway obstruction, voice disorders, and swallowing dysfunction. Expertise with flexible endoscopy with the patient in a state mimicking sleep is also essential for identifying sites of obstruction causing obstructive sleep apnea. Drug-induced sleep endoscopy (DISE) protocols continue to be developed to bring the patient as close to a state of true sleep as possible [29]. Typical dense general anesthesia for airway endoscopy changes muscular tone and can change the site of obstruction that occurs during this type of sleep and can misdirect the clinician during the evaluation. Accurately identifying the true site of obstruction during normal sleep is required to allow for successful surgical management of obstructive sleep apnea.