TY - JOUR
T1 - Pediatric Achalasia in the Netherlands: Incidence, Clinical Course, and Quality of Life
AU - Smits, Marije
AU - van Lennep, Marinde
AU - Vrijlandt, Remy
AU - Benninga, Marc
AU - Oors, Jac
AU - Houwen, Roderick
AU - Kokke, Freddy
AU - van der Zee, David
AU - Escher, Johanne
AU - van den Neucker, Anita
AU - de Meij, Tim
AU - Bodewes, Frank
AU - Schweizer, Joachim
AU - Damen, Gerard
AU - Busch, Olivier
AU - van Wijk, Michiel
PY - 2016/2/1
Y1 - 2016/2/1
N2 - 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
AB - 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
KW - DSQoL Disease-specific QoL
KW - EoE Eosinophilic esophagitis
KW - GER Gastroesophageal reflux
KW - HM Heller myotomy
KW - HMF HM with fundoplication
KW - HRM High resolution manometry
KW - HRQoL Health-related QoL
KW - PD Pneumodilation
KW - POEM Peroral endoscopic myotomy
KW - QoL Quality of life
KW - RDQ Reflux disease questionnaire
KW - SF-36 36-Item Short Form Health Survey
U2 - https://doi.org/10.1016/j.jpeds.2015.10.057
DO - https://doi.org/10.1016/j.jpeds.2015.10.057
M3 - Article
C2 - 26616251
SN - 0022-3476
VL - 169
SP - 110-5.e3
JO - Journal of pediatrics
JF - Journal of pediatrics
ER -