Disorder
Criteria
Achalasia and esophagogastric junction outflow obstruction
Type I achalasia (classic)
Elevated median IRP (>15 mmHg)
100% failed peristalsis
DCI <100 mmHg
Type II achalasia (with esophageal compression)
Elevated median IRP (>15 mmHg)
100% failed peristalsis
Panesophageal pressurization on ≥20% of swallows
Type III achalasia (spastic achalasia)
Elevated median IRP (>15 mmHg)
No normal peristalsis
Premature spastic contractions with DCI >450 mmHg∗s∗cm on ≥20% of swallows
Esophagogastric junction outflow obstruction
Elevated median IRP (>15 mmHg)
Peristalsis present
Major disorders of peristalsis
Absent contractility
Normal median IRP
100% failed peristalsis
Distal esophageal spasm
Normal median IRP
≥20% premature contractions with DCI >450 mmHg∗s∗cm
Hypercontractile (jackhammer) esophagus
DCI >8000 mmHg∗s∗cm on at least two swallows
Minor disorders of peristalsis
Ineffective esophageal motility (IEM)
≥50% ineffective (failed or weak) swallows
Fragmented peristalsis
≥50% fragmented contractions with DCI >450 mmHg∗s∗cm
Sample normal high-resolution manometry (HRM) spatiotemporal plot. Time is on the x-axis, sensor position is on the y-axis (with upper esophageal sphincter at top of image and lower esophageal sphincter at bottom), and pressure represented by color. (From Kessing et al. [13], with permission)
Sample high-resolution manometry (HRM) spatiotemporal plots showing typical appearance of different pathologies within the Chicago Classification. (From Rohof et al. [63], with permission)
Several HRM metrics are used in the Chicago Classification. These include integrated relaxation pressure (IRP), measured in mmHg, or the mean of the 4 s of maximal deglutitive relaxation in the 10 s window beginning at upper esophageal sphincter relaxation and distal contractile integral (DCI), measured in mmHg∗s∗cm, or the amplitude∗duration∗length of the distal esophageal contraction exceeding 20 mmHg from the transition zone to the proximal margin of the LES [10]. IRP serves to identify EGJ outflow obstruction, with elevated values in the presence of obstruction. DCI is a surrogate for distal esophageal contractile vigor, with hypercontractile disorders having elevated values and hypocontractile disorders having lower values.
There are several limitations relevant to application to children with dysphagia. Most importantly, there are no large normative datasets for esophageal HRM in children [15], and cutoffs for relevant manometric variables described in the Chicago Classification have not been thoroughly studied in the pediatric population [16]. Additionally, esophageal manometry, though low risk, is still an invasive test that requires cooperation by the patient. Effects of catheter size on data collection should also be investigated.
Presentation
Presenting symptoms of esophageal dysmotility in children include dysphagia, pyrosis, chest discomfort, regurgitation, nausea, vomiting, chronic cough, and a change in feeding habits. More severe symptoms include malnutrition, weight loss, and recurrent pneumonia [17]. After starting solid food, children may also present with esophageal food impaction, which is characterized by acute onset of dysphagia, pain, and vomiting [18].
Associated Conditions
Esophageal dysmotility can occur primarily or secondarily in association with other disorders. Disorders commonly featuring esophageal dysmotility include eosinophilic esophagitis (EoE), esophageal atresia (EA) with or without tracheoesophageal fistula, neurologic impairment, and gastroesophageal reflux disease (GERD). Less common disorders associated with esophageal dysmotility include scleroderma and megacystis-microcolon-intestinal hypoperistalsis syndrome.
Eosinophilic Esophagitis
Videofluoroscopy and esophagram showing poor progression of a food bolus. This patient had symptoms of an atypical nonproductive cough and globus. (a–c) These images showed movement of the food bolus into the proximal esophagus where there was stasis and slow transit. The food bolus stayed in this position for 20 s. Her multidisciplinary endoscopy esophageal biopsies confirmed eosinophilic esophagitis, and her symptoms responded to treatment
Esophageal Atresia
Esophageal atresia with or without tracheoesophageal fistula is the most common congenital esophageal anomaly, with incidence ranging from 1 in 2500–4500 live births [28]. Survival has improved over the last several decades due to improvements in intensive care, anesthesia, nutritional support, respiratory support, and surgical techniques, to the point where mortality is primarily associated with those patients who have additional life-threatening comorbid anomalies [28]. Esophageal dysmotility is an important problem and the most common long-term issue for patients with esophageal atresia [29, 30]. Potential mechanisms underlying dysmotility include developmental neuronal defects, surgical trauma during repair of the atresia, and esophagitis [31]. These changes contribute to abnormalities including aperistalsis, isolated distal contractions, and pan-esophageal pressurization [32]. The dysmotility predisposes to gastroesophageal reflux disease, with consequent exposure of the esophageal mucosa to acid and corresponding inflammatory changes [33]. Thus, long-term follow-up is warranted with treatment targeted at decreasing acid exposure and inflammation as well as monitoring for complications of chronic acid exposure such as Barrett’s esophagus [34].
Neurologic Impairment
Children with neurologic impairment often experience feeding problems, in part related to esophageal dysmotility with gastroesophageal reflux disease [35–37]. These children are also more likely to exhibit persistent issues following fundoplication compared to children without comorbid neurologic impairment, potentially secondary to ongoing prolonged LES relaxation or esophageal body spasticity [35, 38, 39].
Gastroesophageal Reflux Disease
There is considerable overlap in symptoms of gastroesophageal reflux disease (GERD) and esophageal dysmotility, with patients with each disorder commonly reporting dysphagia, chest discomfort, regurgitation, and pyrosis. Whether one disorder preceded the other temporally can be debated, but one can certainly perpetuate the other. Patients with esophageal dysmotility may have defective acid clearance, with the persistence of acid within the esophagus causing esophagitis which then further impairs esophageal motor function [31]. For those patients with symptoms of GERD who do not benefit from antacid therapy, additional evaluation with endoscopy and manometry is warranted to evaluate for alternative or comorbid diagnoses, including dysmotility and eosinophilic esophagitis [40].
Scleroderma and Systemic Sclerosis
Juvenile systemic scleroderma is a rare disorder, occurring in approximately three per one million children [41]. Esophageal dysmotility can occur in these children [42, 43]. Presenting symptoms may include dysphagia, regurgitation, and pyrosis [44], and patients may exhibit low-amplitude peristaltic contractions, tertiary contractions, and low LES resting pressure, with poor esophageal bolus clearance [45]. Use of steroids to treat the underlying disorder can help improve esophageal symptoms [45].
Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome (MMIHS)
MMIHS, or Berdon’s syndrome, is a rare smooth muscle myopathy resulting in an enlarged bladder, microcolon, and small intestine hypoperistalsis [46]. It was first described in 1976 [47], and less than 300 cases have been reported [48]. Both autosomal dominant inheritance and de novo mutations have been described [49]. A recent series of six patients with the disease identified normal LES resting tone and relaxation but absent esophageal peristalsis in all patients [46]. Though this is a rare disorder, esophageal dysmotility is reasonable to consider in any child presenting with dysphagia in the setting of an underlying myopathy.
Speech-Language Pathologist Approach
Esophageal motility problems are extremely challenging when working with pediatric dysphagia. While it can be tempting to remain focused on the oropharyngeal swallow, events below the upper esophageal sphincter are also an essential component of effective bolus passage. Children with esophageal dysmotility are at increased risk for aspiration, food refusal, and poor weight gain. The American Speech-Language-Hearing Association (ASHA) states that speech-language pathologists should have knowledge and skills regarding the interrelationships of the oral, pharyngeal, and esophageal stages of swallowing, and “If esophageal screening is completed, describe any suspected anatomic and/or physiologic abnormalities of the esophagus which might impact the pharyngeal swallow, deferring to radiology for diagnostic statements” [50]. Thus, while we do not diagnose esophageal disorders, we are responsible for knowledge of typical and atypical esophageal structure and function and making appropriate referrals and recommendations for further evaluation.
Additionally, as we are treating increasingly complex infants and children, we are more likely to encounter esophageal dysmotility as sequelae of tracheoesophageal fistula, prematurity, neurologic conditions, and inflammatory conditions. Familiarity with the presentation and treatment of esophageal dysmotility is increasingly important. Speech-language pathologists should be aware of symptoms associated with dysmotility, as well as signs on clinical and instrumental evaluation, and know when to recommend further evaluation.
Presentation
Symptoms of esophageal dysmotility in infants may include spitting up, vomiting, slow feeding, food refusal, fussiness, poor weight gain, and even failure to thrive. In toddlers and older children, it may manifest in vomiting, food refusal, slow eating, and regurgitation. Verbal children may report food sticking or pain or discomfort in their chest [17, 51]. Coughing after eating or when lying down can be a symptom as well. When these symptoms are reported, an esophageal issue should be considered. These patients may be referred for a videofluoroscopic swallow study. It should be noted that even when an esophagram is a part of the evaluation, the esophagram has poor sensitivity in identifying esophageal motility disorders, in comparison with manometry [52].
Videofluoroscopy
The videofluoroscopic swallow study is intended to evaluate the oropharyngeal swallow and the cervical esophagus, and as such is not the ideal test for evaluating esophageal dysmotility. Ideally, a barium esophagram will have been planned as part of the evaluation based on history and presentation, but in some cases, it is not, or the child is unwilling or unable to take adequate volumes to complete the esophagram. When esophageal dysfunction is suspected, an esophageal screening should be done.
Signs on videofluoroscopic swallow study can include the following: stasis at the UES or proximal esophagus, retrograde motion of the bolus, and even aspiration of contrast that did not pass through the esophagus. On esophageal screening or barium esophagram, tertiary esophageal contractions, limited or inconsistent passage of the bolus through the esophagus, retrograde movement, stacking of the food in the esophagus, a “nutcracker” appearance of the esophagus, or a “bird beak” appearance of the lower esophageal sphincter may be seen [53, 54].
Treatment
Positional changes: upright feeding; remain upright after meals
Texture changes: thinning or mechanically altering solids to allow for easier passage and decreased stasis
Behavioral changes: recommending a liquid wash for solids if safe; recommending smaller, more frequent meals
Nonnutritive suck: especially when oral feeding is not a viable option, promoting nonnutritive sucking, maintaining interest in oral stimulation, and pre-feeding skills
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