Swallowing Disorders Curriculum

  1. Aspiration

    1. Clinical signs/symptoms

      1. Overt aspiration:  coughing, choking, wet congested breath sounds during oral intake

      2. Evidence of foods/liquids via trach tube

      3. Congestion 30 mins following PO suggestive of GERD

    2. Silent aspiration

      1. 80% of pediatric patients aspirate silently without immediate responses

      2. Aspiration manifested as asthma which does not respond to medical treatment, prolonged URI/ multiple PNA

      3. Treatment of aspiration which yields improvement in pulmonary health suggests that underlying etiology was in fact material entering the airway with PO

    3. How to assess:

      1. Objective- VFSS is the gold standard in the US

      2. Vs Managing clinical symptoms

    4. Clinical Management of Aspiration

      1. Thickening

      2. Sidelying Positioning

      3. Changing flow rate of nipple

      4. Neutral head positioning

 

  1. Airway Disorders and implications for swallowing issues
    1. Subglottic stenosis with/without a tracheotomy
      1. Take Home Points
    2. Laryngomalacia
      1. Take Home Points
    3.  Prolonged intubation
      1. Take Home Points
    4. Vocal fold paresis
      1. Take Home Points
    5. Laryngeal Clefts
      1. Take Home Points
    6. Cerebral Palsy/ Significant Hypotonia
      1. Take Home Points
    7. Combined Orofacial Anomalies/ Airway Disorders
      1. Take Home Points
      2. Drooling

 

  1. Nutrition
    1. Increasing Caloric Intake to promote weight gain
      1. Replace SODA/ JUICE:  
        1. Juice/soda displaces hunger and reduces drive for intake of calorically dense foods
        2. Juice/soda while containing calories does not have fats or micro and macro nutrients
        3. Replace juice with water to stimulate hunger for solids with higher fats, 
        4. Warning:  Bottle Rot.  Placing a baby to sleep with a bottle in the crib overnight results in rapid tooth decay.  Data suggests…..
      2. Increasing caloric density of milk/formula: 
        1. increasing amount of powdered milk per same volume of liquids to increase calories,
        2. Add Incamparina to milk to add both thickness and additional calories
        3. Cautiously add additional powder to formula under supervision of a dietician
      3. Increasing hunger/satiation cycle : 
        1. Structured meals and snacks to promote hormone regulation of a hunger/satiation cycle.  Intake at mealtime and then allow for .25 hours with no calories prior to next meal
        2. reduce grazing (snacking) behaviors and increase hunger for solid foods/meals

 

Typical referrals that involve the speech language pathologist can include: coughing with swallowing and respiratory complications, delayed developmental feeding skills and poor weight gain, poor progression with age appropriate foods, upper airway anomalies that are affecting swallow safety, or oral/facial anomalies that compromise feeding efficiency. Common diagnoses that present with swallowing and/or feeding issues include: prematurity with persistent respiratory and reflux issues, neuromuscular deficits, airway anomalies (e.g., tracheo or laryngomalacia, subglottic stenosis) that may or may not require a tracheostomy; and syndromes or neuroanatomical anomalies (e.g., Down Syndrome; velocardiofacial, Cri Du Chat, Pierre Robin Sequence, Chiari Malformation, or various cerebellar malformations). When pneumonia occurs in these types of cases it is reported to often be related to an aspiration event.4

 

Airway Anomalies

Upper airway anomalies that compromise breathing or function of the anatomy for swallowing will be considered in this section. For example, laryngomalacia and/or tracheomalacia, subglottic stenosis, vocal cord paralysis, laryngeal or pharyngeal cysts or tumors (e.g., hemangioma), laryngeal web, or laryngeal cleft.13,14 When breathing is obstructed, such as with tracheomalacia and/or laryngomalacia, subglottic stenosis, or a laryngeal cyst/tumor or web, then swallow coordination may be compromised because of difficulty coordinating breathing with swallowing. In contrast, when laryngeal and/or pharyngeal movement is altered secondary to anatomical anomalies (e.g., laryngeal cleft, airway cysts or tumors, compression of artery anomalies) the integrity of the swallow sequence may be compromised. Subsequently, in both scenarios, the risk of aspiration can occur, especially with thin liquids.15 The other factor to keep in mind in these cases is potential nutrition compromise related to high calorie consumption from the airway obstruction as well as poor ability to manage oral and pharyngeal secretions.16

 

 

Laryngomalacia and Tracheomalacia

Definition. Laryngomalacia and/or tracheomalacia involves immature development of the laryngeal cartilages or trachea resulting in potential for partial collapse during respiration. Stridor or audible inspiration can be one of the key markers of this diagnosis.

 

Case Illustration:   Jack

Clinical Evaluation. Jack, age 1 month, was admitted to the acute care unit of the hospital with audible stridor at rest, choking with bottle feeding, and increased breathing effort during and after feeding. He was eager to eat and had vigorous sucking behavior on the pacifier. When placed on a monitor, his breathing rate was periodically tachypnic and there were periods of oxygen desaturation. The chest x-ray showed signs of congestion in the upper lobes. Weight gain was poor.

Instrumental Evaluation.  The videofluoroscopic swallow study (VFSS) showed no aspiration episodes.  However, during clinical evaluation of swallowing, the speech language pathologist reported concern with risk of microaspiration given periodic coughing while drinking from the bottle and documented pulmonary congestion. Nasopharyngoscopy exam by the ORL service indicated prolapsing of the arytenoids into the laryngeal vestibule and a tight omega shaped epiglottis.

Recommendations and Outcomes. The speech language pathologist implemented various compensatory strategies, including a slow flow bottle nipple, thickening the feeds, more upright positioning, and external pacing with periodic removal of the bottle to offer rest periods. GI service maximized the PPI dosage for reflux management. These changes only made mild improvements in the distress behavior during feeding and degree of stridor. Subsequently, the ORL physician conducted glottoplasty surgery. Feeding improved 7-10 days after surgery with markedly reduced stridor.

General Trends.  Generally VFSS studies show no aspiration for infants with mild to moderate laryngo and/or tracheomalacia despite the presence of stridor. The risk for aspiration, especially with thin liquids, increases as the severity of malacia in the airway approaches the moderate and severe range secondary to further compromise of breathing/swallowing coordination. For older infants, a FEES or fiberoptic exam of the swallow can be helpful to capture potential for microaspiration and poor secretion management.

Long Term Concerns:  Generally these children do well with time, as maturation resolves the issue. For those more medically complex cases, such as when there may be craniofacial or other genetic or developmental complications present, the tracheo/or larnygomalacia may persist into the preschool or primary school years. A tracheostomy tube may be placed in these cases and there maybe long term dysphagia issues that will require systematic management by the ORL and SLP, and possibly an Airway Team (when available). Of note, laryngomalacia and tracheomalacia can be associated with congenital heart disease, asthma and tracheo-esophageal fistula that can impact the course of management.19

 

 

Subglottic stenosis

Definition. Subglottic stenosis is graded by severity of narrowing of the subglottic region, either congenital or acquired from prolonged intubation. Mild narrowing (Type I-II) to moderate to severe narrowing (Type III-IV).  
 

Case Illustration:  Steven

Clinical Evaluation.  Steven is a four year old with grade III subglottic stenosis and bilateral vocal cord paralysis (see Figure 1). He had a tracheostomy at 7 months of age related to severe tracheomalacia and tracheal narrowing. As an infant, he had issues of oxygen desaturation and choking with bottle feeding. Once a tracheosotomy was completed, feeding issues gradually resolved and he was reported to show no signs of dysphagia. A vocal cord chordotomy was completed at 3 years of age with good outcomes. At four years of age, Steven did not tolerate decannulation and laryngotracheal reconstruction was planned along with decannulation. Steven presented with a hoarse vocal quality five days post-op. The SLP conducted a bedside swallow exam that showed choking with thin liquids, delayed coughing with nectar thick liquids and no difficulties with puree food consistencies. Further instrumental swallow assessment was subsequently recommended.

 

FIGURE 1:

figure1.png

 

Instrumental Evaluation:  A VFSS exam completed by the SLP on the same day of the bedside exam (day 5 post-op LTR) demonstrated aspiration with thin liquids during the swallow, and penetration with nectar liquids. The swallow was safe with puree consistencies.

Management and Outcomes. Honey thick liquids and soft moist solid foods were recommended for 3 days. During a bedside swallow exam on day 8, Steven tolerated thin liquids without evidence of dysphagia. He was discharged home from the hospital on a regular diet without restrictions.

General Trends. Children with mild subglottic stenosis generally present with a safe swallow unless there are additional complicating medical issues, such as laryngomalacia, or neurodevelopmental delay. Many of these children may have a background of prematurity and a history of respiratory compromise, including chronic lung disease. Children with more severe cases of subglottic stenosis (stage III-IV) may or may not require a tracheostomy. These children generally are feeding well, pending other medical diagnoses. There tends to be short term dysphagia after tracheal reconstruction surgery that resolves in 5-10 days post surgical repair. Children with baseline dysphagia prior to airway reconstruction, such as secretion pooling in the hypopharynx, premature spillage into the airway prior to swallowing, and reduced pharyngeal persistalsis with incomplete clearing of the pharynx after swallowing are at greater risk for persistent dysphagia after airway repair.16

 

 

Laryngeal Clefts

Definition. Laryngeal clefts are a congenital anomaly involving the interaytenoid muscles, and potentially cricoid cartilage and trachea. A laryngeal cleft can range from mild to severe on scale of 1-4. Type I cleft involves the interarytenoid muscles, Type II: partial cleft of the cricoid cartilage, Type III: a complete cleft of the cricoid, and Type IV: cleft of the cricoid and involves the upper trachea.20 The case illustration for this section will focus on mild laryngeal clefts. A photograph of Types I and II can be viewed in Figure 2.

 

FIGURE 2:

figure2.png

 

One proposed theory is a lateral subluxation of the cricoarytenoid joint. The subluxation results from unopposed actions of the posterior cricoarytenoid muscles as a result of interarytenoid muscle atrophy. Subsequently, there is potential for compromised laryngeal function during the swallow21 and risk for aspiration. Overall, thin liquids pose a greater risk of dysphagia in the presence of a laryngeal cleft because of the quicker transit time and speed of entry into the pharynx compared to a puree or solid food consistency.15, 22

 

Case Illustration:  Maria 

Clinical Evaluation.  Maria, four weeks of age, presented with choking with thin liquids, pulmonary congestion, stridor and one episode of aspiration pneumonia. She was born full term with noted low muscle tone, poor feeding. She also had moderate reflux and was taking Prilosec. There was a family history of an esophageal web and laryngeal cleft.

Instrumental Evaluation. The initial VFSS exam at 4 weeks of age showed aspiration along the posterior tracheal wall with thin liquids and a safe swallow with nectar thick liquids. Thickened oral feedings were recommended to a nectar thickness. ORL subsequently completed a bronchoscopy with a high index suspicion of a laryngeal cleft. A type I cleft was reported. One month later, increased congestion was reported. A repeat swallow study showed silent aspiration with thin and nectar thick liquids.  

Management and Outcomes .   Prior to surgical repair of the cleft, conservative approaches to dysphagia management included thickened liquids for four months. Due to persistent dysphagia and pulmonary issues, a surgical repair was conducted by the ORL. The otolaryngologist then closely monitored the upper airway and recovery from the laryngeal cleft repair as well as management of secretions and persistent mild stridor. The gastroenterologist maintained maximum dosage of reflux therapy. In addition, the pulmonologist was assessing respiratory function and tolerance of aspiration episodes. A repeat swallow study completed 6 weeks after the repair showed penetration and mild aspiration with thin liquids. At one year of age, the aspiration issues finally resolved. The developmental delay and mild low muscle likely contributed to the swallow dysfunction and prolonged resolution of the dysphagia with thin liquids.

Our institution has developed a protocol for managing swallow ability post repair of laryngeal clefts based on clinical experience of common difficulties and recovery patterns as summarized in Table 2. Thickened feeds are continued after the repair for a designated time period to allow for recovery from post surgical swelling and resolution of normal laryngeal motion. Initially, VFSS exams were conducted 1-2 weeks post-op. However, the frequency of aspiration on thin liquids on these early post-op swallow exams led us to defer the timing of the initial instrumental exam.

 

TABLE 2:

table2.png

 

General Trends.  The pattern of aspiration with unrepaired mild laryngeal clefts is typically either deep penetration (See Figure 3) or trace aspiration along the posterior tracheal wall. On FEES exam, a patient with penetration on VFSS, may present with mild aspiration episodes with a pattern of anterior-posterior (AP) aspiration (i.e., the material passing between the arytenoids into the airway). Therefore, the FEES may offer a more sensitive assessment of swallow function.22,23 However, pending on the age of the child, the FEES may or may not be tolerated. It is not uncommon for aspiration with thin liquids to persist 3 months or more after laryngeal cleft repair of Type I-II clefts, especially in the presence of other medical and/or developmental problems.  In contrast to the more common AP aspiration pattern that we have seen with laryngeal cleft cases.

 

FIGURE 3:

figure3.png

 

Long Term Outcomes.  Once aspiration issues resolve, children with isolated laryngeal clefts tend to do well with oral feeding. However, there are often multiple other complications including reflux, laryngomalacia, cardiac anomalies and sometimes developmental delay. Subsequently, there may be persistent feeding issues, including slow progression to more complex food textures, delayed feeding skills, and concerns with weight gain and nutrition. Multidisciplinary management is essential with these children including the community health care team from the pediatrician to the developmental therapists in Early Intervention.

 

 

Combined Airway and Oral/facial Anomalies

Oral/facial anomalies are congenital malformations of the face and mouth that may or may not be associated with a syndrome. Particular anomalies that may impact feeding and swallowing may include micrognathia, cleft lip and/or palate, hemi-facial microsomia, macro or microglossia, moderate to severe anklyoglossia, and abnormal mandibular joint.15,24-25

 

Case Illustration:  John:

Clinical Evaluation.  John was born with a U shaped cleft of the soft palate, micrognathia consistent with Pierre Robin Sequence. Additional complications included GERD, tracheomalacia, subglottic stenosis and poor management of oral secretions. Though the suck reflex was coordinated and he was eager to feed by mouth, the process was difficult for him. He experienced severe nasal regurgitation during feeding, reflux issues resulted in coughing and arching part way through bottle feeding efforts, and episodes of audible stridor would escalate as the feeding progressed. There were frequent episodes of oxygen desaturation with bottle feeding and visible physical distress.

Instrumental Evaluation.  VFSS findings showed no aspiration, but marked NP reflux with regurgitation from the nasal cavity. O2 desaturations occurred during the exam. In addition, there was one episode of transient airway penetration. A trial of thickened liquids was recommended. Nasopharyngoscopy and broncoscopy revealed significant tracheomalacia and subglottic stenosis.

Management/Outcomes.  John did not tolerate oral feeding despite the thickened feeds secondary to the degree of respiratory distress and nasal congestion. His airway issues, significant reflux, and nasal regurgitation made oral feeding unsafe. A g-tube and fundoplication were performed and oral feeding was discontinued for the first 6 months of life secondary to persistent distress. Tastes of formula were offered via pacifier, and later tastes of baby food. At 10 months of age, NP reflux resolved following palate repair. In addition, maturation resulted in improvement in tracheomalacia and GE reflux. Subsequently, he was able to reinitiate oral feeding with the support of structured feeding therapy.

 

 

Tracheostomy and Swallowing

The presence of a tracheostomy tube does not necessarily create dysphagia issues or risks. Two VFSS studies can be viewed. The first study illustrates a normal swallow despite the presence of a tracheostomy, while the second illustrates aspiration during the swallow related to cerebral palsy and vocal cord paralysis. The more important factor is the underlying etiology for the tracheostomy.26 Typical airway obstruction issues that may lead to a tracheostomy can include:  subglottic stenosis, laryngomalacia, and growths/hemangiomas which have been previously covered in this chapter (e.g., Airway Anomalies). Children with tracheostomy tube placement for management of secretions may experience aspiration of their saliva. There has been reported success in some pediatric cases using CPAP (constant positive airway pressure) via the tracheostomy to reduce the aspiration episodes.27

 

 The addition of a trach tube may provide some additional complications to the feeding/swallowing process. Reasons for this may include:  reduced upper airway sensation from lack of air directed through the larynx, pharynx and mouth and some impact on laryngeal excursion during swallowing.28 Complex cases with multiple issues underlying the need for a tracheostomy benefit from a team approach. The speech language pathologist and otolaryngologist collaborate closely in these cases.

 

A component of the initial bedside evaluation by the SLP, may include a “blue dye” test for assessment of swallowing function in tracheostomy patients. Caution in the literature regarding use of blue dye testing, equates the specificity and sensitivity to this test to that of flipping a coin. Although, useful in identifying gross aspiration, the clinician is reminded that a negative test result is not indicative of normal swallow function. Therefore, instrumental assessment, such as VFSS or FEES, is a more valid means of identifying dysphagia. Further limitations of the blue dye test include lack of insight for etiology of dysphagia or effectiveness of compensatory swallowing strategies.28   

 

Tracheostomy and Speaking Valves OR  Management and Outcomes.  In addition to feeding and swallowing problems, the use of speaking valves is a consideration with a tracheostomy. Evaluation for speaking valve candidacy is conducted by the SLP, respiratory therapist, and otolaryngologist. The child must have stable vital signs, good oral secretion management (i.e., not too thick or copious), intra-tracheal pressure ideally less than 5-10 cm H2O, and adequate pulmonary capacity.29 Contraindications for valve use may include, but are not limited to, 1. moderate to severe airway obstruction; 2. weak/ineffective protective cough; 3. vocal fold paralysis or paresis; and 4. insufficient cognition and/or alertness.30,31 The speaking valve not only assists with opportunities for verbal/vocal communication, but offers better pressure dynamics during swallow attempts and fosters increased sensory awareness to the upper airway to also aid in better swallow safety.32  

 

 

Digestive and Feeding Issues

Compromise of the gastrointestinal system may contribute significantly to the development of feeding and swallowing disorders. In our Airway Center, there are many patients who present with gagging, choking or retching upon solid food presentation, significantly restricted diets, or reliance on non-oral feedings. Gastrointestinal issues which affect oral feeding can include but are not limited to gastreoesophageal reflux (GER), esophilic esophagitis, delayed gastric emptying, esophageal dysmotility, or a tracheoesophageal fistula (TEF). Several patients present to our center with failure to thrive (FTT) as a result of reduced oral intake. Children status post fundoplication typically present with periodic gagging and retching, thereby developing oral aversions, feeding refusal and dependence on g-tube for caloric intake.34, 35

Premature infants frequently present with long term gastrointestinal issues given their immature digestive system. Congenital anomalies which initially restrict oral feedings in the premature infant population, include omphalocele, necrotizing enterocolitis (NEC) and gastroschisis. Gastrointestinal and oral feeding issues may also be associated with neurodevelopmental disorders such as autism.

 

 

Gastroesophageal Reflux

Definition. The relationship between GER, aspiration and respiratory compromise has gained more popularity in recent literature. GER, is defined as acid reflux in the esophagus, while laryngopharyngeal (LPR) reflux is characterized by refluxate that enters the pharynx, secondary to GER. For the purpose of this case illustration, the term GER will describe both GER and associated LPR. Studies have shown that longstanding GER/LPR may also result in edematous arytnoids.7,15 Figure 4 provides an example of airway edema secondary to GE reflux.

FIGURE 4:

figure4.png

 

Case Illustration: Jeffrey

Clinical Evaluation.  Jeffrey is a 10 month old baby with a diagnosis of GER and feeding refusal. Upon presentation to the Airway Center at 3 months of age, concerns included spitting up, coughing, arching and discomfort with feeds and mild inspiratory stridor. A Fiberoptic exam performed by the otorhinolaryngologist (ORL) revealed mild laryngomalacia  and marked erythema of the arytnoids. Given the signs and symptoms of GER, in addition to the physiological changes in the upper airway and breathing difficulties, the gastroenterologist initiated a trial of proton pump inhibitors (PPI). By 7 months of age Jeffrey presented with improved breathing, but ongoing spitting, coughing and discomfort with feeding. He was now refusing the bottle, and exhibiting gagging with introduction of textured baby foods. At this point further evaluation by the speech pathologist was conducted.

Instrumental Evaluation.  A VFSS was performed by the speech language pathologist. The study revealed consistent deep penetration into the laryngeal vestibule with thin liquids. Penetration resolved when the SLP presented a thickened liquid consistency. This pattern of deep penetration places the patient at an increased risk for microaspiration. Similar patients with GER who exhibit deep penetration on VFSS, when evaluated via FEES demonstrate microaspiration on a FEES exam. It is then not surprising that these patients present with breathing and/or upper airway issues in addition to reflux.

Follow-Up Clinical Evaluation Jeffrey was subsequently for a clinical evaluation to address the issues of gagging with solids. Gagging with solids is not a reflection of pharyngeal swallow dysfunction and did not need to be viewed under videofluoroscopy. Rather, gagging is a reflection of oral hypersensitivity and feeding aversion. GER/LPR increases the sensitivity of the gag response in the oral cavity. Jeffrey required a structured feeding therapy program to normalize his sensory responses to textures.

Recommendations and Outcomes.  In relation to Jeffrey’s pattern of deep penetration and likely microaspiration, the gastroenterologist decided to maximize the dose of PPIs. The ultimate goal was to decrease acidic LPR, improve pharyngeal sensation, and facilitate better swallow coordination. In the interim, thickened feedings were recommended to manage potential episodes of microaspiration. Respiratory symptoms gradually improved with this approach and feeding refusal slowly declined. Following the increase in PPI dosage, there is typically a several week delay prior to clinical improvement in feeding

General Trends.  GER and resulting LPR may result in reduced laryngopharyngeal sensation and subsequently pharyngeal dysphagia. The literature has described that LRP may result in “functional anesthesia of the larynx,” reducing sensory responses during the swallow, and affecting airway protection both from above (during swallowing), and from below (following a reflux or LPR event).36 Most commonly, these patients present with microaspiration, or deep penetration on VFSS. Dysphagia is managed in the short term, by increasing the viscosity of the liquid. Recent literature suggests that as reflux is managed “aggressively” in these patients with a history of GER/LPR, microaspiration and/or penetration may resolve.36

The second trend includes a feeding disorder component. When gagging occurs as food enters the oral cavity, this is an oral sensory based feeding issue and is not indicative of pharyngeal swallow dysfunction. Repeated gagging may lead to feeding refusal, extremely restricted diets, weight loss and failure to thrive.34

Finally, children with GER may have multiple GI complications which affect feeding. These may include constipation, delayed gastric emptying and motility disturbances.35,38,39 Hunger-satiation cycles are often a large motivator for oral feeding. Delayed gastric emptying and chronic constipation, negatively impact this hunger drive, and subsequently contribute to reduced oral intake.

 

 

Tracheoesophageal Fistula

Definition. A TEF is defined as an anatomic communication between the trachea and the esophagus. There may be a tracheoesophageal fistula to the proximal segment of the esophagus, or more commonly, in the distal, or lower esophageal region. An H-type fistula may be present between the cricoid cartilage and the midesophagus, and may be diagnosed later in infancy.40

 

Case Illustration: Trevor

Clinical Evaluation.  Trevor is a 4 year old boy with a history of a TEF repaired in infancy, esophageal atresia, and cardiac anomalies. He is status post g-tube placement and removal secondary to poor weight gain. Medical history is also remarkable for tracheomalacia, asthma, and reflux. In infancy, prior to diagnosis, Trevor presented with episodes of frequent choking and oxygen desaturation with oral feeding. The speech pathologist conducted a VFSS to evaluate swallow function and incidentally, during this study, a TEF was identified. Shortly after the TEF repair, he experienced intermittent periods of choking. A repeat VFSS displayed narrowing at the site of repair of the TEF, and delayed esophageal motility. There were also some episodes of retrograde flow with increased volume entering the esophagus. Trevor returns at age 4 with poor oral intake and a “choking” incident at school.    

Instrumental Evaluation. The VFSS completed at age 4 years revealed oral and esophageal dysphagia. Oral dysphagia was characterized by oral aversion and a highly sensitive gag reflex. Esophageal dysphagia was characterized by slowed motility through the esophagus, especially at the TEF repair site. Trevor required increased time to clear each bolus. This certainly puts him at risk for back-up at the level of the TEF repair, with larger volumes of food, and retrograde flow of liquids and solids, placing him at risk for aspiration from below.  

Recommendations and Outcomes. Trevor’s slowed motility limits the rate and volume of what he can eat, common to children with a history of TEF and resultant slowed esophageal motility. Oral feeding recommendations included reducing bite size, alternating intake of solids with a liquid ‘wash down.’ Trevor’s esophageal motility needs to be maximized, his reflux minimized, and weight gain closely followed. His upper respiratory and upper airway issues as a result of retrograde flow of stomach contents should be closely followed. The Airway team is a perfect avenue for Trevor to obtain the needed multidisciplinary follow-up.

General Trends: TEF and Dysphagia.  Aspiration from below is often a major contributor to respiratory complications. Dysphagia is almost always the result of disordered esophageal peristalsis with or without the presence of GER.42 Dysphagia with solids is the primary issue presented in this population.39 Following initial repair of the TEF, it is not uncommon for esophageal strictures to develop, which may require dilatation. As the child returns to oral feeding, the process of swallowing facilitates stretching at the site of repair. Improvement is often noted with time and practice with oral feeds.42 Careful monitoring of respiratory and feeding status is suggested given potential for recurrence of the TEF.41

Location of the TEF- either in the upper esophageal region, versus the more common fistula in the lower esophageal region will have an impact on the severity of the oral feeding issues. A more proximal TEF will predispose the patient to more severe oral feeding difficulties including esophageal and potential pharyngeal retrograde flow due to backup at the site of repair.   

Long Term Concerns.  Dysphagia issues can persist into early childhood characterized by difficulty with managing volumes of solid foods secondary to persistent narrowing at the site of repair. This may result in a filling of the esophagus above the site of repair and subsequent choking episodes. Compensatory strategies are needed to maintain safe swallowing and ease of feeding. Strategies may include encouraging smaller and more frequent meals, adequate mastication of foods, and alternating liquids and solids during meals.43

 

Neurological Impairment and Genetic Syndromes

Pediatric patients with neurological impairment, and identified genetic syndromes are at high risk for feeding and swallowing issues. For example, intractable seizure disorder and cerebral palsy in particular, predispose the patient to multiple airway, GI and swallowing issues. Additional diagnoses to consider include mitochondrial disorder, progressive neurogenic disorders, cerebellar tumors, brainstem malformation, and acquired brain injuries.

Identified genetic syndromes such as Down’s Syndrome, Velocardiofacial Syndrome (VCFS), Prader Willi, Corneila de Lange, and Pierre Robin sequence result in both neurological and/or structural deficits contributing to reduced or impaired oral feeding and swallow dysfunction. These patients may have a sustained a period of initial non-oral feeding. Transition to oral feeding, and careful advancement of oral feeding trials is essential to protect against laryngeal aspiration. 

 

 

The cases below will highlight some of the more frequent referrals to the Airway Center.

 

Velocardiofacial Syndrome (VCFS)

Definition.  VCFS, as its name describes, is associated with palatal anomalies, occult or overt submucous cleft palate, cardiac defects, and cognitive and developmental problems. Patients diagnosed with VCFS present with a 22q11 chromosome deletion.

 

 

Case Illustration: Nadia

Clinical Evaluation. Nadia is a 16 month old baby with a diagnosis of VCFS and history of chronic aspiration. Neonatal issues included cardiac issues now corrected, longstanding dysphagia, severe reflux, chronic respiratory involvement, and g-tube placement and fundoplication at 1 month of age. Previous videofluoroscopic swallow studies have revealed fluctuating swallow function with consistent documentation of aspiration with liquids. She relies on her g-tube for nutrition and hydration. Oral intake recommendations had fluctuated over the past year and a half from non oral feeding, to small volumes by mouth. Nadia’s care was subsequently transferred to our medical facility.  

Instrumental Assessment.  At the time of presentation to the Airway Center Nadia was exclusively g-tube fed. As a first diagnostic step, an endoscopy and flexible bronchoscopy were conducted with the pulmonologist and ORL, which revealed tracheobronchomalacia.    

The speech pathologist conducted a repeat VFSS. The exam revealed severe oral pharyngeal dysphagia. Nadia required multiple swallows per teaspoon to clear the pureed food from the pharynx. She had significant nasopharyngeal regurgitation of pureed consistencies. No direct aspiration was documented. However, the residue build up in the pharynx, and the multiple swallows to clear the food, placed her at a high risk for aspiration.

Recommendations and Outcomes.   Nadia is an excellent case to demonstrate the benefit of multidisciplinary problem solving. Should Nadia’s VFSS have been interpreted by any one specialty independently, likely a recommendation for NPO status would have been made. However, together the ORL, pulmonologist, gastroenterologist and SLP reviewed the VFSS study and the possible recommendations. Given a combination of factors including a) no overt documentation of aspiration, b) stable pulmonary and airway status and c) well managed reflux, the team decided to proceed with a joint plan. Nadia was cleared to reinitiate oral feeding trials. The SLP outlined a schedule for oral feeding in collaboration with the community feeding therapist. Given her history of prolonged non-oral feeding, swallow function and medical complications, she was presented with a program of oral sensory therapy with limited volumes. Her g-tube continued to be the primary source of nutrition and hydration. It was judged that her ability to achieve full oral feedings would be a long term process and would be dictated by her respiratory health and developmental progression.  

General Trends:  VCFS and dysphagia.  Given the velopharyngeal insufficiency typical of this genetic disorder, nasopharyngeal reflux is significant on a large percentage of VFSS studies. Concerning, is the presence of nasopharyngeal reflux in combination with neurological impairment, which compromises the safety of the swallow.2 Patients with VCFS may also present with impaired coordination and motility of the pharynx, cervical esophagus and the cricopharynx. Pharyngeal asymmetries or “bulging” of the superior pharyngeal wall during the swallow45, reduced thickness and differences in type 1 muscle fibers of the superior pharyngeal constrictor46, and/or a cricopharyngeal prominence may be common in this population and result in pharyngeal dysphagia.44 VIDEO 13 provides an example of cricopharyngeal dysfunction.

Dysphagia patterns in patients with VCFS also include poor coordination of the suck-swallow-breathe sequence secondary to palatal anomalies. Children with VCFS present with poor texture progression related to both cricopharyngeal incoordination and by difficulty with oral transport.44 

 

 

Intractable Seizure Disorder in the setting of Cerebral Palsy

Definition.  Cerebral Palsy refers to a cerebral lesion resulting in impairments in tone, posture and motor movements. Intractable seizures include seizures which are unresponsive to medications and require additional methods of controlling the seizure activity. In these cases, additional treatment strategies including a ketogenic diet or the placement of a vagal nerve stimulator (VNS) may be implemented dependent on age and severity.

 

Case Illustration:  Thomas

Clinical Evaluation.  Thomas is a 15 year old adolescent who presents with cerebral palsy and intractable seizure disorder. He had a vagal nerve stimulator (VNS) placed 5 years prior. Upon placement of the VNS, he experienced an escalation of dysphagia issues as well as intermittent hoarse vocal quality. Thomas continues to present with persistent seizures which are poorly controlled. At the time of meeting Thomas was a full oral feeder. He has had multiple upper respiratory infections and pneumonias consistent with aspiration. He has had multiple choking episodes at home requiring the Heimlich maneuver. On exam he presented with low oral muscular tone and coordination consistent with his CP.

Instrumental Assessment:  Initial VFSS.   VFSS showed gross, silent aspiration with all liquids. There was a significantly delayed cough which was ineffective to clear the aspirated material. In addition there was penetration with purees. Given reduced chewing, there was documentation of swallowing of table foods whole. This certainly places him at risk for choking on solid foods.

Recommendations and Outcomes.  Although the most conservative approach would include elimination of all liquids and purees, requiring placement of a g-tube, the team decided to continue to allow Thomas to consume purees in attempt to maintain hydration and continue oral feeding. All solids needed to be easy-to-chew and cut into small pieces. Given reports of several episodes of administration of the Heimlich maneuver, it was not safe for Thomas to continue to with hard to chew, or high-choke solids.

VNS Deactivation.  The neurologist deemed that the VNS was not providing substantial control of seizures. Therefore a collaborative decision was to temporarily deactivate the VNS and reassess swallowing. A repeat VFSS following deactivation demonstrated improved swallow function with pureed consistencies and thickened liquids, despite continued silent aspiration with thin liquids. Residue is present in the airway from a previous aspiration event with thin liquids.

Long Term Concerns.  Close collaboration with neurology is essential in managing dysphagia and seizure control. Consideration of non-oral feedings may be required if either the VNS cannot be deactivated from a seizure control perspective or they cannot meet daily caloric and hydration needs safely by mouth.  

It has been well documented that children with CP and neuromotor involvement exhibit oral motor dysfunction, poor bolus control and impaired pharyngeal swallow function.47,48 Monitoring of the safety of the swallow – specifically monitoring safety with solids given the trends of post swallow stasis and tendency to swallow foods whole, in addition to airway protection with liquids, will contribute to consideration of non-oral feedings and possible g-tube placement.

General Trends.  Research suggests that side effects of VNS may include vocal fold paresis with varying degrees of glottic closure resulting in hoarseness and swallow dysfunction. This may either be transient, attributed to affects from the surgery, and resolve within 4 months of the implantation, or more permanent. Aspiration related to VNS function is typically associated with the firing of the VNS during the “on” phase of the cycle. Close interaction between the ORL, neurologist and SLP is critical to manipulate the settings to maximize seizure control, and minimize vocal fold paresis and dysphagia.49 Research suggests that pediatric patients with concomitant motor and cognitive disabilities were more consistently affected.

 

 

Down Syndrome

General Trends.  Trends include subset of infants with Down Syndrome who present with aspiration with thin liquids.50 Hypotonia is a large contributing factor to dysphagia given decreased laryngeal elevation and reduced pharyngeal constriction during the swallow. Modifications to the viscosity of the liquid is often effective in establishing airway protection. As neuromuscular development matures, and hypotonia improves, issues with aspiration of thin liquids tends to resolve. Improved motor control and resolving GER are factors which both contribute to improved swallow function over time.51 

 

Finally, children with Down Syndrome also present with impaired chewing skills, and therefore require a diet consistent with their oral motor development, and not with their chronological age.51  

TAKE HOME POINTS

  • The presence or risk of aspiration does not always limit oral feeding, pending pulmonary function and clinical findings from a team approach.
  • VFSS and FEES exams can be complimentary in particular with children with upper airway issues.  
  • Short-term (or transient) aspiration is not uncommon post-op for upper airway surgical procedures, such as: tracheotomy, laryngo-tracheal reconstruction, laryngeal cleft repair.
  • Potential for aspiration with a tracheostomy is related more to the underlying diagnosis or medical complications than the presence of the tracheotomy tube.
  • Gagging on textured foods is frequently an oral-sensory based feeding issue and not a swallowing problem.
  • If reflux is not well controlled, oral intake may be restricted secondary to food refusal or oral aversion.
  • Children status post TEF repair are at risk for long-term feeding difficulties with solid foods.
  • Vagal nerve stimulators used for seizure control may impact swallow coordination.
  • Infants with Down Syndrome can be at risk for aspiration on thin liquids.