Dysphagia in children with esophageal atresia: current diagnostic options

© 2017 Georg Thieme Verlag KGDysphagia or swallowing disorder is very common (range, 15–52%) in patients with esophageal atresia. Children present with a wide range of symptoms. The most common diagnostic tools to evaluate esophageal dysphagia, such as upper barium study and manometry, aim to characterize anatomy and function of the esophageal body and the esophagogastric junction (EGJ). Using these technologies, a variety of pathological motor patterns have been identified in children with esophageal atresia. However, the most challenging part of diagnosing patients with esophageal dysphagia lies in the fact that these methods fail to link functional symptoms such as dysphagia with the esophageal motor disorders observed. A recent method, called pressure-flow analysis (PFA), uses simultaneously acquired impedance and manometry measurements, and applies an integrated analysis of these recordings to derive quantitative pressure-flow metrics. These pressure-flow metrics allow detection of the interplay between bolus flow, motor patterns, and symptomatology by combining data on bolus transit and bolus flow resistance. Based on a dichotomous categorization, flow resistance at the EGJ and ineffective esophageal bolus transit can be determined. This method has the potential to guide therapeutic decisions for esophageal dysmotility in pediatric patients with esophageal atresia

The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus

Characterizing the esophageal function of infants in the NICU.

Infants admitted in the neonatal unit commonly present with feeding problems such as decreased oral intake and feeding intolerance. These infants may suffer from complex diseases such as prematurity or congenital malformations. The origin of these feeding problems may be disease-specific or may be secondary to many factors such as severity of the illness, concomitant medication and surgical interventions. "Healthy" preterm infants may have an immature nervous system leading to immature oral intake, and failure to coordinate sucking, swallowing and breathing. They may also have an immature motor gastro-intestinal function. Infants with bronchopulmonary dysplasia or central nervous system lesions are particularly prone to feeding problems. Recently, developmental changes in pharyngoesophageal physiology in the preterm infant were described explaining poor feeding in infants under 34 weeks. The physiology of distal esophageal motility has been described in healthy, preterm infants using water perfused manometry, but data on preterm infants with associated pathology are very limited. Term infants with severe congenital malformations also often present with feeding problems. In some cases, underlying respiratory, neurological or cardiac pathology impedes the ability to take oral feeds. In children with esophageal atresia, motility disorders are primarily due to the intrinsically disturbed development of the esophagus. End-to-end anastomosis of the esophagus can disturb vagal innervations, vascular supply or cause traction on the lower esophagus. In infants with congenital diaphragmatic hernia, multiple factors may play a role in the disturbed gastro-esophageal function leading to poor feeding. Development and position of lungs, stomach and lower gastrointestinal tracts are abnormal in those patients. Moreover, repositioning of the abdominal organs into the abdominal cavity can result in disorders based on numeric manometric parameters. For 15 years, we have had a unique setting of highly specialized clinical units focusing on pharyngeal as well esophageal dysphagia within the University Hospitals of Leuven. The current PhD project is part of an ongoing international collaboration between the KU Leuven (Experimental ORL, N Rommel) and the University of Adelaide, Australia (School of Paediatrics and Reproductive Health, T Omari) focusing on paediatric dysphagia. For three years, the clinical research has been expanded to adult dysphagia and become part of the Translational Research Centre for Gastrointestinal Disorders (TARGID). With this project we are now expanding the research to neonates.status: publishe

The Potential Benefits of Applying Recent Advances in Esophageal Motility Testing in Patients with Esophageal Atresia

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.Infants and children with esophageal atresia commonly present with swallowing dysfunction or dysphagia. Dysphagia can lead to a range of significant consequences such as aspiration pneumonia, malnutrition, dehydration, and food impaction. To improve oral intake, the clinical diagnosis of dysphagia in patients with esophageal atresia should focus on both the pharynx and the esophagus. To characterize the complex interactions of bolus flow and motor function between mouth, pharynx, and esophagus, a detailed understanding of normal and abnormal deglutition is required through the use of adequate and objective assessment techniques. As clinical symptoms do not correlate well with conventional assessment methods of motor function such as radiology or manometry but do correlate with bolus flow, the current state-of-the-art diagnosis involves high-resolution manometry combined with impedance measurements to characterize the interplay between esophageal motor function and bolus clearance. Using a novel pressure flow analysis (PFA) method as an integrated analysis method of manometric and impedance measurements, differentiation of patients with impaired esophago-gastric junction relaxation from patients with bolus outflow disorders is clinically relevant. In this, pressure flow matrix categorizing the quantitative PFA measures may be used to make rational therapeutic decisions in patients with esophageal atresia. Through more advanced diagnostics, improved understanding of pathophysiology may improve our patient care by directly targeting the failed biomechanics of both the pharynx and the esophagus

The Fate of Fat: Pre-Exposure Fat Losses during Nasogastric Tube Feeding in Preterm Newborns

Deficient nutritional support and subsequent postnatal growth failure are major covariates of short- and long-term outcome in preterm neonates. Despite its relevance, extrauterine growth restriction (EUGR) is still prevalent, occurring in an important portion of extremely preterm infants. Lipids provide infants with most of their energy needs, but also cover specific supplies critical to growth, development and health. The use of human milk in preterm neonates results in practices, such as milk storage, pasteurization and administration by an infusion system. All of these pre-exposure manipulations significantly affect the final extent of lipid deposition in the intestinal track available for absorption, but the impact of tube feeding is the most significant. Strategies to shift earlier to oral feeding are available, while adaptations of the infusion systems (inversion, variable flow) have only more recently been shown to be effective in "in vitro", but not yet in "in vivo" settings. Pre-exposure-related issues for drugs and nutritional compounds show similarities. Therefore, we suggest that the available practices for "in vitro" drug evaluations should also be considered in feeding strategies to further reduce pre-exposure losses as a strategy to improve the nutritional status and outcome of preterm neonates.status: publishe

Improvised Hand Injury Treatment Using Traditional Veterinary Medicine in Ethiopia

In remote wilderness environments, local people with traditional knowledge of medicinal plants are potentially important first-line health care providers. We present a case of a 31-year-old man who fell off a horse while trekking through a remote mountain landscape in Ethiopia and sustained blunt force trauma to the hand. A local mountain hut keeper examined the patient’s hand and used heated leaves of the succulent plant Kalanchoe petitiana to treat a suspected metacarpal fracture. As first responder in a low-resource setting, the hut keeper relied on his traditional knowledge of ethnoveterinary medicine to improvise a treatment for a human injury in a remote mountain environment. Although in this case the outcome of the traditional intervention was positive, our analysis shows that the massage component of the intervention could have led to complications. Conversely, reports from the use of related Kalanchoe species suggest that heated Kalanchoe leaves could be useful in the compression component of traditional care for hand injuries. Validation of traditional remedies and their therapeutic potential are needed if they are to complement wilderness wound care safely and reliably. The documentation and validation of these remedies are urgently needed, as many medicinal plants and indigenous knowledge of how to use these valuable natural resources are being lost.status: publishe

Short-Term Use of Parenteral Nutrition With a Lipid Emulsion Containing a Mixture of Soybean Oil, Olive Oil, Medium-Chain Triglycerides, and Fish Oil: A Randomized Double-Blind Study in Preterm Infants

BACKGROUND: For premature neonates needing parenteral nutrition (PN), a balanced lipid supply is crucial. The authors hypothesized that a lipid emulsion containing medium-chain triglycerides (MCTs) and soybean, olive, and fish oils would be as safe and well tolerated as a soybean emulsion while beneficially influencing the fatty acid profile. METHODS: Double-blind, controlled study in 53 neonates (<34 weeks' gestation) randomized to receive at least 7 days of PN containing either an emulsion of MCTs and soybean, olive, and fish oils or a soybean oil emulsion. Target lipid dosage was 1.0 g fat/kg body weight [BW]/d on days 1-3, 2 g/kg BW/d on day 4, 3 g/kg BW/d on day 5, and 3.5 g/kg BW/d on days 6-14. RESULTS: Test emulsion vs control, mean ± SD: baseline triglyceride concentrations were 0.52 ± 0.16 vs 0.54 ± 0.19 mmol/L and increased similarly in both groups to 0.69 ± 0.38 vs 0.67 ± 0.36 on day 8 of treatment (P = .781 for change). A significantly higher decrease in total and direct bilirubin vs baseline was seen in the test group compared with the control group P < .05 between groups). In plasma and red blood cell phospholipids, eicosapentaenoic acid and docosahexaenoic acid were higher, and the n-6/n-3 fatty acid ratio was lower in the test group (P < .05 vs control). CONCLUSIONS: The lipid emulsion, based on a mixture of MCTs and soybean, olive, and fish oils, was safe and well tolerated by preterm infants while beneficially modulating the fatty acid profile.status: publishe

Subcutaneous palivizumab (synagis) administration in an infant with congenital type 2B von Willebrand disease

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