Marije Smits1, Marinde van Lennep1, Remy Vrijlandt1, Marc Benninga1, Jac Oors1, Roderick Houwen2, Freddy Kokke2, David van der Zee3, Johanne Escher4, Anita van den Neucker5, Tim de Meij6, Frank Bodewes7, Joachim Schweizer8, Gerard Damen9, Olivier Busch10, Michiel van Wijk1. 1. Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands. 2. Department of Pediatric Gastroenterology, Wilhelmina Children's Hospital, University Medical Center, Utrecht, the Netherlands. 3. Department of Pediatric Surgery, University Medical Center, Utrecht, the Netherlands. 4. Department of Pediatric Gastroenterology, Sophia Children's Hospital, Erasmus MC, Rotterdam, the Netherlands. 5. Department of Pediatric Gastroenterology, University Medical Center, Maastricht, the Netherlands. 6. Department of Pediatric Gastroenterology, Vrije Universiteit MC, Amsterdam, the Netherlands. 7. Department of Pediatric Gastroenterology, Beatrix Children's Hospital, University Medical Center-Groningen, Groningen, the Netherlands. 8. Department of Pediatric Gastroenterology, Willem-Alexander Children's Hospital, University Medical Center, Leiden, the Netherlands. 9. Department of Pediatric Gastroenterology, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands. 10. Department of Surgery, Academic Medical Center, Amsterdam, the Netherlands.
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 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.
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.
Authors: Froukje B van Hoeij; Leah I Prins; André J P M Smout; Arjan J Bredenoord Journal: Neurogastroenterol Motil Date: 2019-01-30 Impact factor: 3.598
Authors: Marinde van Lennep; Marin L Leijdekkers; Jac M Oors; Marc A Benninga; Michiel P van Wijk; Maartje M J Singendonk Journal: J Pediatr Gastroenterol Nutr Date: 2021-02-01 Impact factor: 3.288