Update Management Dysphagia in Children
As is true of diagnostic procedures, the development and application of therapeutic interventions for children with disorders of oropharyngoesophageal dysmotility has often lagged behind that for adults. Many of the treatments have not undergone rigorous controlled evaluation. This section discusses therapies for these disorders: general aspects, “conservative” lifestyle measures, pharmacologic agents, and procedural or surgical techniques.
Because of the complexity of these disorders, and because many of them impact the child at the intersection of gastrointestinal, respiratory, and neuromuscular systems, interdisciplinary teams of personnel often must participate in designing optimal treatments. For children, optimal teams consist of personnel with in-depth pediatric experience, and may include pediatric gastroenterologists, otolaryngologists, pulmonologists, speech-language therapists, rehabilitation therapists, nutritionists, surgeons, occupational and physical therapists, orthodontists, and so on. The setting for such therapy may be the acute pediatric hospital, rehabilitation hospitals, outpatient clinics, the home, or the school.
It is important to diagnose and begin therapy early for dysphagia in children; delay in management may interrupt normal development of swallowing during critical stages, producing feeding aversion and delaying rehabilitation. A Japanese group found that in 51 tube-fed infants and young children, a higher rate of progression to tube extraction occurred in those with older age of onset of dysphagia, shorter duration of dysphagia prior to treatment, and younger age at starting treatment.
- Conservative Lifestyle Measures Conservative measures to assist in management of oropharyngoesophageal dysmotility include optimizing body and head position (both during feedings and during sleep) to minimize the risk of aspiration; thickening and other adaptations of feeds and feeding equipment; performing specific maneuvers during swallowing; using the most favorable swallow intervals; “exercise” conditioning of swallowing musculature; and avoidance of fatigue. Further, optimizing the nutritional value of the meals consumed and management of associated disorders, including secondary feeding aversion, are crucial.
- Body and Head Position The position of the body and head during feeding or during sleep can influence the likelihood that swallowed material (or refluxed material) will be misdirected into the airway. Simply elevating the head above the stomach allows gravity to compensate for some deficits of motility, but optimal alignment and stabilization of trunk, head, and jaw (using intraoral appliances in some cases) may be useful for particular disorders. Children with cerebral palsy or primary myopathies, for example, may require specialized external supports for these functions. Specific uses for particular head positions are indicated in the paragraph on swallow maneuvers, below.
- Thickening and Other Adaptations of Feedings Thickening of feedings to alter the bolus flow and maneuverability in the oropharynx can improve airway protection. There is a need for rheologic standards, as clinicians’ subjective impressions may not be completely accurate or reproducible. Various descriptive textures (e.g., pureed, minced, and soft) have proved useful for different children, although the person doing the feeding is also an important variable. Because thickening of feedings also decreases regurgitant reflux, thickening of feeds has added potential benefit in those children, particularly neurologically impaired children, with dysphagia as well as gastroesophageal reflux disease. Other manipulations of foods are adding thermal or tactile stimulation to speed triggering of the pharyngeal swallow in order to prevent aspiration that would otherwise intervene during the pharyngeal delay. For this purpose, cold food, pressure from a spoon on the tongue, or a bolus that requires chewing are several useful techniques. Feeding equipment, including special nipples, spoons, etc., has been designed to be adapted to particular disorders and can be prescribed by speech therapists or feeding specialists.
- Swallowing Maneuvers The choice of specific head positions, maneuvers, and swallowing techniques used for individuals with oropharyngeal dysphagia depends on the disorder to be treated. The head is tilted back to treat inefficient oral transit due to poor propulsion of the bolus by the tongue, but this head position must be used carefully, as it may impair airway protection even in healthy individuals. The chin is tilted down, in contrast, to treat delayed triggering of pharyngeal swallow, reduced retraction of the tongue base leading to residue in the valleculae, or reduced laryngeal closure leading to aspiration during the swallow. Lateral head tilt toward the stronger side is used to treat unilateral oral and pharyngeal weakness leading to residue in the mouth and pharynx on that side. Head rotation toward the weak side is used to treat unilateral laryngeal dysfunction leading to aspiration during the swallow, or unilateral pharyngeal paresis leading to residue on one side of the pharynx. Head rotation is also used to pull the cricoid cartilage forward to reduce the upper esophageal sphincter tone in order to treat residue remaining in the pyriform sinuses. The empiric effect of these head and neck positions were evaluated in adults: 24 healthy controls, nine patients with unilateral lower cranial lesions, and 42 patients without laterality in their oropharyngeal muscle functioning. A randomized controlled trial of the efficacy of chin tilt down versus thickening of feedings for adults with Parkinson’s disease has been undertaken. Four swallowing maneuvers to exert voluntary control over aspects of the swallow have been described: supraglottic swallow, in which inspiration before swallowing allows voluntary expiration after a swallow to clear the vocal folds of contaminating liquids; supersupraglottic swallow, with more effortful breath holding; effortful swallow, to increase the posterior tongue base movement in cases in which it is reduced; and the Mendelsohn maneuver, in which the laryngeal elevation and upper esophageal sphincter opening are increased. The degree to which these positions and maneuvers are useful in children depends in part on their developmental stage and speech and communication skills. However, it is notable that extremely severe swallowing disability, manifest by completely absent pharyngeal swallow, has not precluded complete dependence on oral intake in a patient as young as 18 years of age, who used a sequence of devised maneuvers to completely compensate for an absent swallowing mechanism.
- Exercises Exercises specific to the musculature involved in swallowing, including tongue resistance and range of motion, and laryngeal adduction, may be useful. Intraoral appliances have been used to facilitate the learning of optimal oral movements. However, over the long term they may not be more beneficial than maturation alone. Methods of applying electrical stimulation to the patient with pharyngeal dysphagia have been developed.
- Fatigue Avoidance It is crucial to avoid fatigue during eating in children with dysphagia. During videofluoroscopic swallowing examinations, children with deep penetration early in the study were likely to advance to aspiration during an extended study. Furthermore, consistency in presentation of food seems important in oral-motor skill acquisition by children with severe neurologic impairment.
- Nutritional Management Maintaining nutrition comprises a crucial aspect of the treatment of any child with dysmotility of the upper gastrointestinal tract. If the above conservative methods, supplemented by any appropriate pharmacotherapy or surgical therapy, are insufficient to promote optimal nutrition, nasogastric feedings may be used for the very short term. If oral nutrition is not then feasible, percutaneous gastrostomies or jejunostomies may be required while other therapeutic interventions proceed. Children with oropharyngoesophageal dysmotility rarely require parenteral nutrition, unless other disabilities preclude the use of the intestinal tract.
- Management of Associated Disorders Management of associated disorders is beyond the scope of this chapter, but the fact that nearly 50% of children with dysphagia have multiple etiologic contributing factors must be kept in mind.14 Gastroesophageal reflux is a particular complicating factor to consider, because these children may be more likely to aspirate any refluxed material.
- Pharmacologic Agents Most pharmacologic agents to treat these disorders are, like many pharmacotherapies in use today, not adequately evaluated or approved by the Food and Drug Administration for use for these indications, in children. Nonetheless, clinicians use these drugs because of the absence of adequate alternatives. Some of the newer drugs in early use in children are reviewed elsewhere.
- Motility-Modifying Agents Agents to modify motility are the most obvious pharmacotherapies for motility disorders of the upper gastrointestinal tract. Contractility-reducing agents treat disorders that produce elevated sphincter pressures or high peristaltic amplitudes. These conditions include achalasia, diffuse esophageal spasm, nutcracker esophagus, and cricopharyngeal achalasia. The agents include calcium channel blockers (e.g., nifedipine, diltiazem), nitric oxide effect promoters (e.g., sildenafil) and nitrates, as well as botulinum toxin injected directly into the hypertonic lower or upper esophageal sphincter. Effects of each of them have been imperfect and inconsistent in clinical trials. Botulinum toxin injections used to treat achalasia in two pediatric studies (3 and 23 patients, respectively) produced symptomatic resolution, but symptoms recurred within 7 months in at least half of the children. Contractility-augmenting agents, on the other hand, treat the lower esophageal sphincter incompetence of gastroesophageal reflux disease. Agents used for this purpose include metoclopramide and several others not available in the United States, such as domperidone and cisapride. Inconsistent and imperfect results of clinical trials affect these drugs as well. Agents to inhibit the lower esophageal sphincter relaxations responsible for gastroesophageal reflux disease include the -aminobutyric acid (GABA)B agonist baclofen.
- Gastric Acid–Suppressing Agents Gastroesophageal reflux disease, itself the most common upper gastrointestinal motility disorder, may also play a secondary role in other upper motility disorders, such as postmyotomy achalasia patients, or patients who aspirate during reflux as well as during primary swallowing. In addition, gastroesophageal reflux disease may mimic the symptomatic presentations of many pediatric esophageal motility disorders. Therefore, empiric therapy for the symptoms or directed therapy for diagnosed gastroesophageal reflux disease becomes a logical early step in many algorithms for management of these disorders. The most consistently beneficial pharmacotherapy for gastroesophageal reflux disease is powerful acid suppression, which interrupts the acid damage of the esophagus and thus resulting vicious cycles of esophageal dysfunction. Proton pump inhibitors are thus currently the primary drugs in this category, with histamine-2 receptor antagonists, antacids, and topical agents such as sucralfate also playing a role.
- Glucagon for Food Impaction Because of its ability to decrease lower esophageal sphincter pressure, glucagon is sometimes used before more invasive means to treat solid food impacted in the esophagus of the patient with esophageal dysmotility. However, glucagon also may decrease lower esophageal body contraction pressures, and thus impair esophageal propulsion of the impacted bolus.
- Psychotropic Agents Antidepressants and benzodiazepines have found some use in ameliorating the symptoms of upper gastrointestinal dysmotility disorders, but generally without affecting the motility itself. Pediatric studies of this use of these agents are even rarer than adult studies.
Procedural or Surgical Treatments
Procedures to treat oropharyngoesophageal dysmotility include those directed at management of oral cavity disorders, provision of nutrition, improving the pharyngoesophageal motility disorder itself, or prevention of aspiration.
- Orthodontia Orthodontic procedures, which are beyond the scope of this chapter, may benefit children with abnormalities of biting and chewing; such procedures may improve their swallowing, as well as their speech and facial morphology.
- Gastrostomy and Enterostomy Tube Feedings Children unable to consume adequate nutrition orally for prolonged periods because of dysphagia may require placement of a gastrostomy or enterostomy tube, either surgically or percutaneously. In a prospective 1-year study at a single institution, 20 children (mean age 6.5 years) with severe mental impairment and oropharyngeal dysphagia underwent endoscopic gastrostomy. Sixteen had cerebral palsy, and two each had myopathy and traumatic brain injury. During half a year of follow-up, weight gain occurred in all. However, complications consisted of three (15%) perforations, four stomal infections, seven instances of granulation tissue, three reflux pneumonias, and one pneumoperitoneum. Another pediatric report cites a 3.5% rate of gastroen teric fistulae (12 of 343 percutaneous gastrostomies during a 5-year period) presenting up to 25 months postgastrostomy, often (8/12) with acute intestinal obstruction and universally involving the posterior gastric wall. Late complications of percutaneous (or surgically placed) gastrostomy or jejunostomy tubes include various malfunctions that prompt emergency department visits; a report of such visits by demented patients with malfunctioning feeding tubes cited 138 visits by 33 patients during 20 months, and a total estimated national cost extrapolated at almost $11 million annually. Tube-fed children are more likely to be able to resume oral feeding if they have a shorter period of dysphagia before tube placement, the onset of feeding impairment after 8 months of age, younger age at initiation of treatment for eating (specifically before 36 months of age), and less severe dysphagia.
- Dilations Dilation of the hypertensive sphincter in achalasia or in cricopharyngeal achalasia is widely used, and generally considered first-line therapy. It is less clear whether it should be used for nonobstructive dysphagia or in patients without endoscopically evident disease. Children, as well as adults, may be able to advance to unsedated dilation during a program of repeated dilations of strictures; the applicability of this to children with dysphagia is unknown. Esophageal dilation is not without risk; perforation and brain abscess are reported complications.
- Myotomies (UES, LES) Heller myotomy for achalasia of the lower esophageal sphincter (LES) is the most commonly used myotomy, but myotomy is performed on the upper esophageal sphincter (UES) for cricopharyngeal achalasia. Myotomy of the esophageal length and LES has been performed for intractable diffuse esophageal spasm.
- Esophageal Replacement Dysmotility or its sequelae may affect the native esophagus so severely that esophagectomy and esophageal replacement (e.g., by gastric tube or colon interposition) is warranted. However, a majority of 81 adults undergoing this procedure reported chronic disability in the form of heartburn, continuing dysphagia, and the necessity for smaller meal size at 10 months to 18.9 years (median 9.8 years) after the procedure.
- Surgical Approaches to Aspiration Owing to Dysmotility When nonsurgical approaches to aspiration, including discontinuation of all oral intake and aggressive pulmonary toilet, are inadequate, surgical approaches may be required. Tracheostomy, even using a cuffed tracheostomy tube, cannot ensure the prevention of aspiration. Minor procedures, such as injection of a unilateral paralyzed vocal cord with Teflon (or Gelfoam if functional recovery is expected within 6 weeks), or implantation of a noninjectable material like Silastic (laryngeal framework surgery), to bring it to the midline for apposition by the opposite, functional cord, can be helpful. More aggressive procedures include laryngectomy; laryngeal closure procedures using, for example, an epiglottic flap; and tracheoesophageal diversion with laryngotracheal separation. A pediatric series of thyroplasty for the treatment of vocal fold paralysis in eight children (2 to 17 years of age), aiming to manage both aspiration and dysphonia, indicated that the lower location of the pediatric larynx must be taken into account in designing such innovative surgical procedures, highlighting the importance of specific pediatric expertise