Pediatric Achalasia in the Netherlands: Incidence, Clinical Course, and Quality of Life.

OBJECTIVE: To assess incidence and clinical course of Dutch patients with achalasia diagnosed before 18 years of age as well as their current symptoms and quality of life (QoL). STUDY DESIGN: Retrospective medical chart review and a cross-sectional study assessing current clinical status using the Eckardt score and reflux disease questionnaire. General QoL was measured using Kidscreen-52 for patients <18 years of age or to 36-Item Short Form Health Survey for patients ≥18 years of age. RESULTS: Between 1990 and 2013, 87 children (mean age 11.4 ± 3.4 years, 60% male) diagnosed with achalasia in the Netherlands were included. Mean incidence was 0.1/100,000/y (range 0.03-0.21). Initial treatment was pneumodilation (PD) in 68 (79%) patients and Heller myotomy (HM) in 18 (21%) patients. Retreatment was required more often after initial PD compared with initial HM (88% vs 22%; P < .0001). More complications of initial treatment occurred after HM compared with PD (55.6% vs 1.5%; P < .0001). Three esophageal perforations were seen after HM (16.7%), 1 after PD (1.5%). Sixty-three of 87 (72%) patients were prospectively contacted. Median Eckardt score was 3 (IQR 2-5), with 32 patients (44.5%) having positive scores suggesting active disease. Reflux disease questionnaire scores were higher after initial HM vs PD (1.71 [0.96-2.90] vs 0.58 [0-1.56]; P = .005). The 36-Item Short Form Health Survey (n = 52) was lower compared with healthy population norms for 7/8 domains. Kidscreen-52 (n = 20) was similar to population norms. CONCLUSIONS: Pediatric achalasia is rare and relapse rates are high after initial treatment, especially after pneumodilation, but with more complications after HM. Symptoms often persist into adulthood, without any clinical follow-up. QoL in adulthood was decreased.

Pressure-flow characteristics of normal and disordered esophageal motor patterns.

OBJECTIVE: To perform pressure-flow analysis (PFA) in a cohort of pediatric patients who were referred for diagnostic manometric investigation. STUDY DESIGN: PFA was performed using purpose designed Matlab-based software. The pressure-flow index (PFI), a composite measure of bolus pressurization relative to flow and the impedance ratio, a measure of the extent of bolus clearance failure were calculated. RESULTS: Tracings of 76 pediatric patients (32 males; 9.1 ± 0.7 years) and 25 healthy adult controls (7 males; 36.1 ± 2.2 years) were analyzed. Patients mostly had normal motility (50%) or a category 4 disorder and usually weak peristalsis (31.5%) according to the Chicago Classification. PFA of healthy controls defined reference ranges for PFI ≤142 and impedance ratio ≤0.49. Pediatric patients with pressure-flow (PF) characteristics within these limits had normal motility (62%), most patients with PF characteristics outside these limits also had an abnormal Chicago Classification (61%). Patients with high PFI and disordered motor patterns all had esophagogastric junction outflow obstruction. CONCLUSIONS: Disordered PF characteristics are associated with disordered esophageal motor patterns. By defining the degree of over-pressurization and/or extent of clearance failure, PFA may be a useful adjunct to esophageal pressure topography-based classification.

Development of the reflux finding score for infants and its observer agreement.

OBJECTIVE: It is hypothesized that laryngeal edema is caused by laryngopharyngeal reflux (LPR) (ie, gastroesophageal reflux extending into the larynx and pharynx). The validated reflux finding score (RFS) assesses LPR disease in adults. We, therefore, aimed to develop an adapted RFS for infants (RFS-I) and assess its observer agreement. STUDY DESIGN: Visibility of laryngeal anatomic landmarks was assessed by determining observer agreement. The RFS-I was developed based on the RFS, the found observer agreement, and expert opinion. An educational tutorial was developed which was presented to 3 pediatric otorhinolaryngologists, 2 otorhinolaryngologists, and 2 gastroenterology fellows. They then scored videos of flexible laryngoscopy procedures of infants who were either diagnosed with or specifically without laryngeal edema. RESULTS: In total, 52 infants were included with a median age of 19.5 (0-70) weeks, with 12 and 40 infants, respectively, for the assessment of the laryngeal anatomic landmarks and the assessment of the RFS-I. Overall interobserver agreement of the RFS-I was moderate (intraclass correlation coefficient = 0.45). Intraobserver agreement ranged from moderate to excellent agreement (intraclass correlation coefficient = 0.50-0.87). CONCLUSION: A standardized scoring instrument was developed for the diagnosis of LPR disease using flexible laryngoscopy. Using this tool, only moderate interobserver agreement was reached with a highly variable intraobserver agreement. Because a valid scoring system for flexible laryngoscopy is lacking up until now, the RFS-I and flexible laryngoscopy should not be used solely to clinically assess LPR related findings of the larynx, nor to guide treatment.

Gastroesophageal reflux, esophageal function, gastric emptying, and the relationship to dysphagia before and after antireflux surgery in children.

OBJECTIVES: To assess gastroesophageal reflux (GER), esophageal motility, and gastric emptying in children before and after laparoscopic fundoplication and to identify functional measures associated with postoperative dysphagia. STUDY DESIGN: Combined impedance-manometry, 24-hour pH-impedance, and gastric-emptying breath tests were performed before and after laparoscopic anterior partial fundoplication. Impedance-manometry studies were analyzed with the use of conventional analysis methods and a novel automated impedance manometry (AIM) analysis. RESULTS: Children with therapy resistent GER disease (n = 25) were assessed before fundoplication, of whom 10 (median age 6.4 years; range, 1.1-17.1 years; 7 male; 4 with neurologic impairment) underwent fundoplication. GER episodes reduced from 97 (69-172) to 66 (18-87)/24 hours (P = .012). Peristaltic contractions were unaltered. Complete lower esophageal sphincter relaxations decreased after fundoplication (92% [76%-100%] vs 65% [29%-91%], P = .038). Four (40%) patients developed postoperative dysphagia, which was transient in 2. In those patients, preoperative gastric emptying was delayed compared with patients without postoperative dysphagia, 96 minutes (71-104 minutes) versus 48 minutes (26-68 minutes), P = .032, and AIM analysis derived dysphagia risk index was greater (56 [15-105] vs 2 [2-6] P = .016). Two patients underwent a repeat fundoplication. DISCUSSION: Fundoplication in children reduced GER without altering esophageal motility. Four patients who developed dysphagia demonstrated slower gastric emptying and greater dysplasia risk index preoperatively. AIM analysis may allow detection of subtle esophageal abnormalities potentially leading to postoperative dysphagia.

Interobserver and intraobserver variability in pH-impedance analysis between 10 experts and automated analysis.

OBJECTIVE: To determine interobserver and intraobserver variability in pH-impedance interpretation between experts and accuracy of automated analysis (AA). STUDY DESIGN: Ten pediatric 24-hour pH-impedance tracings were analyzed by 10 observers from 7 world groups and with AA. Detection of gastroesophageal reflux (GER) episodes was compared between observers and AA. Intraobserver agreement was assessed in 3 observers after 3 to 5 months. RESULTS: Overall, 1242 liquid and mixed GER events were detected, 490 (42%) were scored by the majority of observers, yielding moderate agreement (Cohen's kappa [κ] = 0.46). Intraclass co-efficient for numbers of GER per study was 0.84 (P < .001). AA has 94% sensitivity rate and 74% specificity rate compared with majority consensus (≥6 observers). Agreement for gas GER was poor (κ = 0.11). Intraobserver agreement was κ = 0.49, κ = 0.71, and κ = 0.85 in 3 observers. CONCLUSION: Interobserver agreement in combined pH-multichannel intraluminal impedance analysis in experts is moderate; only 42% of GER episodes were detected by the majority of observers. Detection of total GER numbers is more consistent. Considering these poor outcomes, AA seems favorable compared with manual analysis because of its reproducibility. However, the lower specificity rate suggests the need for refinement of AA before widespread use can be advocated.

Small volumes of feed can trigger transient lower esophageal sphincter relaxation and gastroesophageal reflux in the right lateral position in infants.

OBJECTIVE: To investigate the threshold amount of constantly infused feed needed to trigger lower esophageal sphincter relaxation (TLESR) in the right lateral position (RLP) and left lateral position (LLP). STUDY DESIGN: Eight healthy infants (3 male; gestational age: 32.9 +/- 2.4 weeks; corrected age: 36.1 +/- 1.3 weeks) were studied using an esophageal impedance-manometry catheter incorporating an intragastric infusion port. After tube placement, infants were randomly positioned in RLP or LLP. They were then tube-fed their normal feed (62.5 [40 to 75] mL) at an infusion rate of 160 mL/h. Recordings were made during the feed and 15 minutes thereafter. The study was repeated with the infant in the opposite position. RESULTS: More TLESRs were triggered in the RLP compared with LLP (4.0 [3.0 to 6.0] vs 2.5 [1.0 to 3.0], P = .027). First TLESR occurred at a significantly lower infused volume in RLP compared with LLP (10.6 +/- 9.4 vs 21.0 +/- 4.9 mL, P = .006). The percentage of feed infused at time of first TLESR was significantly lower in RLP compared with LLP (17.6% +/- 15.5% vs 35.4% +/- 8.02%, P = .005). CONCLUSIONS: In the RLP, TLESRs and gastroesophageal reflux are triggered at volumes unlikely to induce gastric distension.

Magnetic resonance imaging of the lumbosacral spine in children with chronic constipation or non-retentive fecal incontinence: a prospective study.

OBJECTIVE: To determine the prevalence of lumbosacral spine (LSS) abnormalities in children with defecation disorders, intractable constipation, or non-retentive fecal incontinence (NRFI) and evaluate whether LSS abnormalities on magnetic resonance imaging (MRI) are clinically detected by neurologic examination. STUDY DESIGN: MRI of the LSS and complete neurologic examination by a pediatric neurologist blinded to the MRI results were performed in patients with intractable defecation disorders. RESULTS: Patients with intractable constipation (n = 130; 76 males; median age, 11 years; range, 6-18 years), and patients with NRFI (n = 28; 18 males; median age, 10 years; range, 7-15 years) participated. One occult spina bifida (OSB) and 3 terminal filum lipomas were found in patients with a normal neurologic examination. One patient had a terminal filum lipoma and neurologic complaints. Gluteal cleft deviation was found in 3 of 4 patients with LSS abnormalities. Neurosurgical treatment was not required in any patient during the 12-week follow-up. CONCLUSIONS: MRI showed LSS abnormalities in 3% of patients with defecation disorders and normal neurologic examination, all of whom reported symptom relief at the 12-week follow-up without neurosurgical intervention. Thus, whether or not LSS abnormalities play a role in defecation disorders remains unclear.

Effect of body position changes on postprandial gastroesophageal reflux and gastric emptying in the healthy premature neonate.

OBJECTIVE: To identify a body-positioning regimen that promotes gastric emptying (GE) and reduces gastroesophageal reflux (GER) by changing body position 1 hour after feeding. STUDY DESIGN: Ten healthy preterm infants (7 male; mean postmenstrual age, 36 weeks [range, 33 to 38 weeks]) were monitored with combined esophageal impedance-manometry. Infants were positioned in the left lateral position (LLP) or right lateral position (RLP) and then gavage-fed. After 1 hour, the position was changed to the opposite side. Subsequently, all infants were restudied with the order of positioning reversed. RESULTS: There was more liquid GER in the RLP than in the LLP (median, 9.5 [range, 6.0 to 22.0] vs 2.0 [range, 0.0 to 5.0] episodes/hour; P = .002). In the RLP-first protocol, the number of liquid GER episodes per hour decreased significantly after position change (first postprandial hour [RLP], 5.5 [2.0 to 13.0] vs second postprandial hour [LLP], 0.0 [0.0 to 1.0]; P = .002). GE was faster in the RLP-first protocol than in the LLP-first protocol (37.0 +/- 21.1 vs 61.2 +/- 24.8 minutes; P = .006). CONCLUSIONS: A strategy of right lateral positioning for the first postprandial hour with a position change to the left thereafter promotes GE and reduces liquid GER in the late postprandial period and may prove to be a simple therapeutic approach for infants with GER disease.