title: Pediatric Achalasia in the Netherlands: Incidence, Clinical Course, and Quality of Life.
abstract: 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).
  
  
  METHODS
  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.
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Marije
authors.initials: M
authors.lastname: Smits
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Marinde
authors.initials: M
authors.lastname: van Lennep
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Remy
authors.initials: R
authors.lastname: Vrijlandt
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Marc
authors.initials: M
authors.lastname: Benninga
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Jac
authors.initials: J
authors.lastname: Oors
authors.affiliation: Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, the Netherlands.
authors.forename: Roderick
authors.initials: R
authors.lastname: Houwen
authors.affiliation: Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, the Netherlands.
authors.forename: Freddy
authors.initials: F
authors.lastname: Kokke
authors.affiliation: Department of Pediatric Surgery, University Medical Center, Utrecht, the Netherlands.
authors.forename: David
authors.initials: D
authors.lastname: van der Zee
authors.affiliation: Department of Pediatric Gastroenterology, Sophia Children's Hospital, Erasmus MC, Rotterdam, the Netherlands.
authors.forename: Johanne
authors.initials: J
authors.lastname: Escher
authors.affiliation: Department of Pediatric Gastroenterology, University Medical Center, Maastricht, the Netherlands.
authors.forename: Anita
authors.initials: A
authors.lastname: van den Neucker
authors.affiliation: Department of Pediatric Gastroenterology, Vrije Universiteit MC, Amsterdam, the Netherlands.
authors.forename: Tim
authors.initials: T
authors.lastname: de Meij
authors.affiliation: Department of Pediatric Gastroenterology, Beatrix Children's Hospital, University Medical Center-Groningen, Groningen, the Netherlands.
authors.forename: Frank
authors.initials: F
authors.lastname: Bodewes
authors.affiliation: Department of Pediatric Gastroenterology, Willem-Alexander Children's Hospital, University Medical Center, Leiden, the Netherlands.
authors.forename: Joachim
authors.initials: J
authors.lastname: Schweizer
authors.affiliation: Department of Pediatric Gastroenterology, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands.
authors.forename: Gerard
authors.initials: G
authors.lastname: Damen
authors.affiliation: Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Olivier
authors.initials: O
authors.lastname: Busch
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Michiel
authors.initials: M
authors.lastname: van Wijk
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D004931:Esophageal Achalasia / Q000175:diagnosis* / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D011788:Quality of Life; D012189:Retrospective Studies