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

Marije Smits, Marinde van Lennep, Remy Vrijlandt, Marc Benninga, Jac Oors, Roderick Houwen, Freddy Kokke, David van der Zee, Johanne Escher, Anita van den Neucker, Tim de Meij, Frank Bodewes, Joachim Schweizer, Gerard Damen, Olivier Busch, Michiel van Wijk

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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). 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. 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. 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
Original languageEnglish
Pages (from-to)110-5.e3
JournalJournal of pediatrics
Publication statusPublished - 1 Feb 2016


  • DSQoL Disease-specific QoL
  • EoE Eosinophilic esophagitis
  • GER Gastroesophageal reflux
  • HM Heller myotomy
  • HMF HM with fundoplication
  • HRM High resolution manometry
  • HRQoL Health-related QoL
  • PD Pneumodilation
  • POEM Peroral endoscopic myotomy
  • QoL Quality of life
  • RDQ Reflux disease questionnaire
  • SF-36 36-Item Short Form Health Survey

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