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

*Corresponding author for this work

    Research output: Contribution to journalArticleAcademicpeer-review

    23 Citations (Scopus)

    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).

    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.

    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.

    Original languageEnglish
    Pages (from-to)110-115.e3
    Number of pages9
    JournalThe Journal of Pediatrics
    Volume169
    DOIs
    Publication statusPublished - Feb-2016

    Keywords

    • REFLUX DISEASE QUESTIONNAIRE
    • PNEUMATIC DILATATION
    • IDIOPATHIC ACHALASIA
    • ENDOSCOPIC MYOTOMY
    • CHILDREN
    • OUTCOMES
    • DILATION
    • ESOPHAGOMYOTOMY
    • VALIDATION
    • CHILDHOOD

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