Figure - available from: Current Treatment Options in Pediatrics
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Esophagram of a patient with achalasia. a An air-fluid level in the proximal esophagus (arrow).b The classic bird’s beak appearance to the lower esophageal sphincter.
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Purpose of the review
Gastroesophageal reflux disease (GERD) is frequently implicated as a cause for respiratory disease. However, there is growing evidence that upper gastrointestinal dysmotility may play a significantly larger role in genesis of respiratory symptoms and development of underlying pulmonary pathology. This paper will discuss the di...
Similar publications
Achalasia cardia is a rare esophageal motility disorder. Although a primary neurological disorder, the treatment modalities of achalasia are primarily endoscopic or surgical. Pneumatic dilatation (PD) or laparoscopic Heller's myotomy (LHM) have been the mainstay of achalasia management for several decades. With the introduction of third space endos...
Citations
... Gastrointestinal dysmotility is often under-recognized in pediatric aerodigestive patients, and its clinical presentation may mimic symptoms of other gastrointestinal conditions such as GERD [21]. While there is no specific data on the prevalence of dysmotility in the aerodigestive population, there is evidence that dysmotility plays an important role in gastrointestinal dysfunction and may significantly impact respiratory function [22]. Dysmotility can affect any part of the gastrointestinal tract, with conditions ranging from achalasia to gastroparesis and chronic intestinal pseudoobstruction [23]. ...
... Manifestations of esophageal dysmotility include regurgitation, dysphagia, and vomiting. However, respiratory symptoms such as chronic cough and wheezing may be seen [22]. In conditions such as achalasia, respiratory compromise may be caused by tracheal compression due to a dilated esophagus [24]. ...
... Esophageal and gastric dysmotility are significant contributors to disease burden in aerodigestive patients and have been shown to influence respiratory function [22]. The effect of gastric dysmotility on respiratory symptoms and the biochemical mechanisms by which this occurs is poorly understood. ...
Purpose of Review
This review provides a comprehensive overview of the diagnosis and management of common gastrointestinal conditions found in pediatric patients with aerodigestive disorders. In particular, this review will address the impact of these gastrointestinal conditions on the airway and its contribution to respiratory disease.
Recent Findings
Multidisciplinary programs help to optimize management for medically complex aerodigestive pediatric patients with gastrointestinal disorders.
Summary
Aerodigestive patients are susceptible to multiple gastrointestinal conditions which may worsen pulmonary function. Timely diagnosis and treatment of these conditions have improved respiratory outcomes and decreased complications in this patient population.
... Improvements in feeding difficulties in this study were wide ranging and included increased oral intake and improved tolerance of tube feeds. GI dysmotility is described in patients with neurologic impairment and can be a contributor to aspiration-associated respiratory symptoms in patients with pulmonary disease, making these groups important potential targets for use of prucalopride [31][32][33]. While this study was not able to investigate the mechanism for benefit in patients with pulmonary and neurologic disorders, we suspect that these patients experienced particular benefit due to improvements in esophageal clearance and gastric emptying, which may also result in improvements in aspiration-related symptoms. ...
Background
Upper gastrointestinal (GI) symptoms are common in pediatrics, and few prokinetics for children exist. The goal of this study was to determine the efficacy of prucalopride for treatment of upper GI symptoms and feeding difficulties in children.Methods
This was a retrospective study of patients prescribed prucalopride for upper GI symptoms at a single tertiary care center from July 2019 to January 2021. Demographic data, the indication for prucalopride, comorbidities, and feeding data were recorded. The primary outcome was improvement in the primary upper GI symptom at first follow-up after prucalopride initiation. Univariable and multivariable analyses were used to assess for factors associated with improvement.ResultsThe final study population included 71 patients who received prucalopride for treatment of upper GI symptoms. The most common indications were nausea (44%), feeding difficulties (20%), and reflux (11%). Patients had a median age of 16.7 years (range 1.9–21.8 years), and they had received 4 ± 4.8 years of care in our GI clinic and trialed 3.0 ± 2.0 other GI medications by the time of the prucalopride prescription. At follow-up 3.6 ± 2.9 months after the prucalopride was prescribed, 46 patients (65%) had symptomatic improvement of the upper GI symptom. Improvement was more likely in patients with enteral tubes (p = 0.04), pulmonary comorbidities (p = 0.006), and neurologic comorbidities (p = 0.02). Amongst patients with feeding difficulties, 79% of patients showed improvements in oral or tube feeding.Conclusions
In this sample of children treated for refractory upper GI symptoms at a single tertiary care center, patients showed improvements in symptoms like nausea, reflux, and feeding difficulties after starting prucalopride.
... La chirurgie anti-reflux était associée à ces ré-hospitalisations, mais pas le RGO en lui-même(Lejeune et al., 2021). En effet, l'association du RGO à la morbidité respiratoire et l'efficacité d'un traitement anti-reflux empirique reste controversée(Ambartsumyan et al., 2019;de Benedictis & Bush, 2018;Rosen et al., 2018). ...
Les progrès des dernières décennies dans la prise en charge néonatale et chirurgicale des enfants présentant une atrésie de l’oesophage (AO) ont permis une amélioration significative de leur espérance de vie. Cependant une morbidité importante persiste - en particulier digestive - à court, moyen et long terme, même si la prévalence de ces complications et leurs facteurs de risque restent actuellement mal identifiés. L’objectif de ce travail était d’étudier chez les enfants nés avec une AO, en population, trois complications digestives et leurs déterminants à trois périodes différentes de l’évolution d’une AO.Le premier travail s’est intéressé à la prévalence du dumping syndrome (DS) à l’âge de 3 mois, à l’aide d’une étude prospective multicentrique (n=38). Les nouveau-nés bénéficiaient systématiquement d’une hyperglycémie provoquée par voie orale entre l’âge de 2,5 et 3,5 mois. La glycémie était évaluée de façon discontinue pendant 4 heures, ce qui permettait de repérer les hyperglycémies précoces et les hypoglycémies tardives. Trente pour cent des patients avaient un DS, sans association identifiée avec les signes cliniques observés. Aucun facteur associé au DS n’a pu être mis en évidence dans cette étude.Le deuxième travail s’est intéressé aux facteurs de risque de sténose de l’anastomose (SA) à 1 an, par une étude prospective longitudinale multicentrique en population (n=1082). Les données à la naissance et à un an étaient relevées grâce au registre national français de l’AO (RENATO). Vingt-trois pour cent des enfants présentaient une SA à l’âge de 1 an, et les seuls facteurs de risque de SA identifiés étaient chirurgicaux (anastomose sous tension et anastomose retardée).Le troisième travail a porté sur les facteurs de risque et l’histoire naturelle du reflux gastro-oesophagien (RGO) à l’âge de 6 ans, par une étude prospective longitudinale multicentrique d’une cohorte nichée dans le registre RENATO (n=286). Les données à 6 ans étaient relevées grâce au réseau des centres participant au registre, avec la même méthodologie que le registre. Un tiers des enfants présentaient un RGO à l’âge de 6 ans, et 20% d’entre eux en avaient été opérés. La présence d’un RGO à l’âge de 1 an, un antécédent de gastrostomie avant l’âge d’un an, le sexe masculin et la dénutrition étaient les 4 facteurs prédictifs de RGO à l’âge de 6 ans. Le RGO pouvait disparaître, persister ou apparaître de novo à l’âge de 6 ans.Ce travail a permis la description du DS, nouvelle complication fréquente observée à court terme dans l’AO, d’individualiser des groupes à risque de complication digestives à court et moyen terme (1 an pour les SA et 6 ans pour le RGO) et de décrire l’évolution naturelle à moyen terme du RGO. Ses perspectives sont d’étudier la physiopathologie du DS dans l’AO et de poursuivre le suivi de la cohorte nichée à 12-13 ans, afin de mieux décrire l’histoire naturelle et d’aider à repérer les populations à risque pour adapter le suivi et le traitement de ces patients.
... Oesophageal dysmotility resulting in stasis and direct aspiration can have a profound effect on development of respiratory symptoms and pulmonary sequelae in aerodigestive patients. 18 However, there is a paucity ...
Abnormal oesophageal motility may impair oesophageal bolus transport leading to symptoms of chest pain and regurgitation. Oesophageal pathophysiology may include neuromuscular and/or structural defects leading to weak, incoordinate or absent peristalsis and impaired oesophago‐gastric junction relaxation. Understanding these mechanisms is important to determine the appropriate course of therapy. Whilst, barium oesophagram is the mainstay for clinical investigation of oesophageal motility, high‐resolution manometry is now more widely available. This review describes the potential value of high‐resolution manometry in paediatric gastroenterology practise.
... Such factors include dysphagia and swallowing impairment (eg, inability to or delayed bolus formation, pooling of secretions in the pyriform sinuses etc), GER, and regurgitation of foodstuff that is stuck in the esophagus. [57][58][59][60] All of these factors can result in aspiration if the patient is also unable to protect the airways with laryngospasm and/or clear the trachea after laryngeal penetration using cough. ...
... However, the techniques are not readily available in every hospital and therefore they are not recommended at this point. Asymptomatic children with EA/TEF who have positive cultures in BAL should be evaluated for possible aspiration Recommendation 32: In infants with TEF, the CXR may show characteristic changes consistent with aspiration Recommendation 33: Chest CT should be considered in cases of known or highly suspected chronic aspiration Recommendation 34: Presence of lipid laden macrophages in BAL of children without acute or chronic lung disease is suggestive of aspiration Recommendation 35: Modified Barium Swallow should be performed in all patients prior to initiating oral feedings Abbreviations: BAL, bronchoalveolar lavage; CT, computed tomography; CXR, chest X-ray; EA, esophageal atresia; TEF, tracheoesophageal fistula.Patients with EA-TEF are predisposed to aspiration and the possibility should be investigated in cases of recurrent pneumonia.Conditions to be ruled out include undiagnosed occult communications (eg, laryngeal cleft and/or undiagnosed fistulae)18,[58][59][60][73][74][75]116 or functional abnormalities such as impaired swallowing, vocal cord dysfunction and esophageal dysmotility that predispose to aspiration.Laryngeal clefts in particular can be found in almost 20% of the cases of EA-TEF, and rigid airway endoscopy with probing of the posterior glottis is the gold standard for its diagnosis.117,118 Presence of contrast material in the tracheobronchial tree after a contrast study (eg, upper GI series) or foodstuff seen in the trachea of a patient with tracheostomy are definitive of the diagnosis butrather "accidental" findings. ...
... The association of GER with respiratory morbidity and the effectiveness of empiric anti-GER treatment remain highly controversial.57,60,168 This is in part, due to the fact that respiratory symptoms from the upper and/or from the lower airways can be caused by miniscule amounts of refluxate and as a result the usual diagnostic studies for GERD may likely be within the normal range.The RCWG did not address in detail the management of GER,GERD, and aspiration because they have been recently reviewed in detail in the INoEA Consensus Guidelines on GastrointestinalComplications of EA-TEF.19,168 ...
Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, C based on whether they were based on “strong”, “moderate” or “weak” agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Non‐invasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long‐term follow‐up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients’ age were also developed.
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... Sin embargo, en estos pacientes puede haber no solamente RGE, sino también trastornos de motilidad esofágica que impidan el paso descendente del contenido deglutido, entre ellos, acalasia esofágica o disfunción cricofaríngea, evaluable a través de manometría esofágica, en que se produce retención faringea y posterior paso a vía aérea del material deglutido. Otras alteraciones de la motilidad esofágica como la acalasia esofágica o la atresia esofágica operada, pueden dificultar la llegada del bolo alimentario al estómago, o promover la permanencia de los alimentos a nivel esofágico y faringeo (22). En algunos de estos casos, el tratamiento del reflujo a través del uso de IBP no disminuye el riesgo de neumonía aspirativa, dado que persiste el riesgo de aspiración del contenido oral, y se ha asociado -con evidencia aún no concluyente-con mayor frecuencia a infecciones respiratorias. ...
El reflujo gastroesofágico es una condición frecuente en la vida diaria de lactantes y niños mayores. Cuando produce síntomas, se denomina enfermedad por reflujo gastroesofágico. Se ha atribuido frecuentemente diferentes síntomas extraesofágicos al reflujo, sin embargo, nuevas técnicas diagnósticas disponibles, como la pHmetría con medición de impedanciometría, han permitido evaluar y eventualmente descartar tales asociaciones. En este artículo se revisa la relación entre el reflujo gastroesofágico y patología laríngea, tos crónica, asma y aspiración. En términos generales, no se recomienda el tratamiento empírico de un supuesto reflujo en pacientes asintomáticos, en quienes tampoco esté demostrada su presencia por técnicas como la pHmetría con impedanciometría, dado que la respuesta terapéutica es baja y similar a placebo, con potenciales efectos adversos.
Dysphagia is a common symptom in many neurologic disorders. Patients with oropharyngeal dysphagia present with difficulties when they start to swallow, often with coughing and choking; whereas patients with esophageal dysphagia describe the feeling that swallowed food stops in the chest. Chronic neurologic diseases such stroke, Parkinson's disease, or dementia often have dysphagia as a symptom, particularly oropharyngeal dysphagia, and the term “neurogenic dysphagia” is often used. A disruption of the sophisticated, integrated sensorimotor swallowing system is usually the main reason behind dysphagia. Dysphagia can be associated with aspiration leading to aspiration pneumonia, and chronic dysphagia can lead to weight loss and malnutrition. Patients with dysphagia, when accurately and promptly diagnosed through medical history, physical examination, and diagnostic tests, often can be treated and experience improved quality of life. The pathophysiological mechanisms behind dysphagia, its diagnosis, and potential treatments are discussed in this manuscript.
Esophageal motility disorders can present with common symptoms such as feeding difficulties in early childhood and dysphagia or chest pain in adolescents. If motility disorders are suspected, high-resolution esophageal manometry (HRM) is now considered the gold standard diagnostic test, both in adults and children (Omari and Krishnan, J Paediatr Child Health 56(11):1754-1759, 2020; Gyawali and Penagini, Dig Liver Dis 53:1373, 2021). Additionally, even in the absence of a strictly defined esophageal motility disorder, HRM can be helpful to elucidate the origin of symptoms. HRM records pressures throughout the esophagus with a large number closely spaced sensors between which pressures can reliably be interpolated. These data can be shown as a continuum of pressures using pressure topography (iso-contour) plots. For adults, protocols have evolved for the performance, analysis, and clinical interpretation of an HRM test (Yadlapati et al. Neurogastroenterol Motil 33(1):e14058, 2021). In children, existent protocols need further validation in the clinical setting, but HRM has proven to be very useful in a number of settings (Rosen et al. Neurogastroenterol Motil 30, 2018).
Objective:
To evaluate gastrointestinal (GI) risk factors for bronchiectasis in children. We hypothesized that upper GI tract dysmotility would be associated with increased risk of bronchiectasis.
Study design:
Subjects in this retrospective cohort study included those evaluated for persistent pulmonary symptoms in the Aerodigestive Center at Boston Children's Hospital who underwent chest computed tomography (CT) between 2002 and 2019. To determine gastrointestinal predictors of bronchiectasis, baseline characteristics, comorbidities, enteral tube status, medications received, gastroesophageal reflux burden, adequacy of swallow function, esophageal dysmotility, gastric dysmotility, and neutrophil count on bronchoalveolar lavage (BAL) were compared between patients with and without bronchiectasis. Proportions were compared with Fisher's exact test and binary logistic regression with stepwise selection was used for multivariate analysis. ROC analyses were utilized to compare BAL neutrophils and bronchiectasis.
Results:
Of 192 subjects, 24% were found to have evidence of bronchiectasis on chest CT at age 7.9±0.5 years. Enteral tubes (OR 5.77, 95% CI 2.25-14.83, p<0.001) and increased BAL neutrophil count (OR 5.79, 95% CI 1.87-17.94, p=0.002) were associated with increased risk while neurologic comorbidities were associated with decreased risk (OR 0.24, 95% CI 0.09-0.66, p=0.006). Gastroesophageal reflux was not found to be a significant risk factor. Neutrophil counts >10% had 72% sensitivity and 60% specificity for identifying bronchiectasis.
Conclusions:
Enteral tubes were associated with significantly increased risk of bronchiectasis but gastroesophageal reflux was not. Providers should consider obtaining chest CT to evaluate for bronchiectasis in children found to have unexplained elevated BAL neutrophil count. This article is protected by copyright. All rights reserved.
Pediatric achalasia is a rare disorder of the esophagus that results in functional obstruction at gastroesophageal junction and impedes antegrade movement of bolus into stomach. Children present with vomiting/regurgitation, dysphagia, weight loss, and nocturnal and respiratory symptoms. Diagnosis may be delayed as symptoms mimic common pediatric disorders. Diagnosis follows adult diagnostic pathways yet remains challenging given absence of normative values in children. Treatment goals are to reduce symptoms, improve esophageal emptying, and prevent megaesophagus. Laparoscopic myotomy, pneumatic dilation, and peroral endoscopic myotomy are definite treatment options in children. Follow-up is necessary to monitor/treat reflux and complications, disease recurrence/persistence, and megaesophagus.
