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

@entity1 @entity1318 @entity354
OBJECTIVE: To assess incidence and clinical course of Dutch @entity1 with @entity1318 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 @entity1 <18  years of age or to 36-Item Short Form Health Survey for @entity1 >= 18  years of age. RESULTS: Between 1990 and 2013, 87 @entity1 (mean age 11.4      3.4  years, 60% male) diagnosed with @entity1318 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%) @entity1 and Heller myotomy (HM) in 18 (21%) @entity1 . 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%) @entity1 were prospectively contacted. Median Eckardt score was 3 (IQR 2-5), with 32 @entity1 (44.5%) having positive scores suggesting active disease. @entity354 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: @entity1318 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.
