Clinical Variables as Indicative Factors for Endoscopy in Adolescents with Esophageal Atresia.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) occurs frequently in patients operated for esophageal atresia (EA). Longstanding esophagitis may lead to dysphagia, strictures, columnar metaplasia, and dysplasia with an increased risk of adenocarcinoma. Are clinical factors and non-invasive assessments reliable indicators for follow-up with endoscopy? MATERIAL AND METHOD: A follow-up study with inclusion of EA adolescents in Norway born between 1996 and 2002 was conducted. Clinical assessment with pH monitoring, endoscopy with biopsies, along with interviews and questionnaires regarding gastroesophageal reflux disease (GERD) and dysphagia were performed. RESULTS: We examined 68 EA adolescents. 62% reported GERD by interview, 22% by questionnaire. 85% reported dysphagia by interview, 71% by questionnaire. 24-hour pH monitoring detected pathological reflux index (RI) (>7%) in 7/59 (12%). By endoscopy with biopsy 62 (92%) had histologic esophagitis, of whom 3 (4%) had severe esophagitis. Gastric metaplasia was diagnosed in twelve (18%) adolescents, intestinal metaplasia in only one (1.5%). None had dysplasia or carcinoma. Dysphagia and GERD were statistically correlated to esophagitis and metaplasia, but none of the questionnaires or interviews alone were good screening instruments with high combined sensitivity and specificity. A compound variable made by simply taking the mean of rescaled RI and dysphagia by interview showed to be the best predictor of metaplasia (85% sensitivity, 67% specificity). CONCLUSION: The questionnaires and interviews used in the present study were not good screening instruments alone. However, combining dysphagia score by interview and RI may be helpful in assessing which patients need endoscopy with biopsy at each individual follow-up examination. LEVEL OF EVIDENCE: Level II prognostic study.

New insights in the prevalence of scoliosis and musculoskeletal asymmetries in adolescents with esophageal atresia.

BACKGROUND: Increased risk of scoliosis and musculoskeletal abnormalities in adolescents with esophageal atresia (EA) is reported, but the impact of these abnormalities on physical fitness and motor skills are not known. METHODS: Scoliosis was assessed radiographically and shoulder and chest abnormalities by a standardized protocol. Physical fitness was evaluated with Grippit, Six-minute walk test, and International Physical Activity Questionnaire and motor skills by Motor Assessment Battery for Children. RESULTS: Sixty-seven EA adolescents median 16 (13-20) years participated. The prevalence of significant scoliosis (≥ 20º) was 12% (8/67) whereas 22% (15/67) had mild scoliosis (10-19º). Vertebral anomalies occurred in 18/67 (27%), eight of them (44%) had scoliosis. The majority of adolescents (15/23) with scoliosis did not have vertebral anomalies. Musculoskeletal abnormalities were detected in 22-78%. Balance problems occurred three times more frequently than expected (44% vs. 15%, p = 0.004). Submaximal exercise capacity was significantly reduced compared to reference values (p < 0.001). Scoliosis ≥ 20º was related to reduced physical activity (p = 0.008), and musculoskeletal abnormalities to reduced physical activity and impaired motor skills (p = 0.042 and p < 0.038, respectively). CONCLUSIONS: Significant scoliosis was diagnosed in 12% of the EA adolescents and related to reduced physical activity. Musculoskeletal abnormalities identified in more than half of the patients, were related to reduced physical activity and impaired motor skills, and exercise capacity was significantly below reference group. EA patients with and without vertebral anomalies need health-promoting guidance to prevent impaired motor skills and consequences of reduced physical activity. LEVEL OF EVIDENCE: Prognostic Study, Level II.

Impaired motor performance in adolescents with esophageal atresia.

AIMS: The study prospectively assessed motor development from infancy to adolescence in patients with esophageal atresia (EA). METHODS: At one year of age motor performance was evaluated with the Psychomotor Developmental Index (PDI) of the Bayley Scales of Infant Development, Second Edition (BSID-II), and as adolescents reevaluated with Motor Assessment Battery for Children, Second Edition (MABC-2). Associations to clinical factors were assessed. RESULTS: 23 EA patients were followed from infancy to adolescence. The median total PDI score in infancy was 102 (56-118) and the corresponding mean z-score was -0.006 (SD 0.995) and not significantly different from the reference values (p = 0.48). The median total MABC-2 score in adolescence was 75 (32-93) and the corresponding mean z-score -0.43 (SD 0.998) which is significantly below normal (p = 0.03). Children with impaired motor function in adolescence underwent significantly more rethoracotomies than those with normal motor performance (p = 0.037); whereas the two groups did not differ with respect to other clinical characteristics. CONCLUSION: From infancy to adolescence the motor performance in the group of EA patients deteriorated from within normal range to significantly impaired compared to reference values. Interdisciplinary follow-up programs from infancy to adolescence with close monitoring for motor function is necessary to detect motor impairments.

Nutritional Status in Adolescents with Esophageal Atresia.

OBJECTIVES: To examine factors that may affect nutritional status in adolescents with esophageal atresia. STUDY DESIGN: Anthropometric measurements, blood samples, pH measuring, mapping of dysphagia with a modified Easting Assessment Test questionnaire, 4-day dietary record, and a semistructured interview about eating habits and nutrition counseling were performed in a cross-sectional cohort of adolescents with esophageal atresia. RESULTS: Out of 102 eligible patients, 68 (67%) participated. The median height-for-age Z score was -0.6 (-4.6 to 1.8). Ten (15%) were classified as stunted (height-for-age Z score <-2). Fourteen (21%) were overweight. More than two-thirds reported symptoms of dysphagia (Easting Assessment Test score ≥3) and avoided specific foods. Forty-eight (71%) completed the 4-day dietary record, which showed daily intake of energy below age-appropriate recommendations. One-third reported an energy intake below their estimated basal metabolic rate. Only 24% had received counselling from a dietitian. CONCLUSIONS: Adolescents with a history of esophageal atresia have growth below reference values and energy intake below recommendations. Energy intake and its relation to stunting needs to be further studied in patients with esophageal atresia.

Surgical repair of long-gap esophageal atresia: A retrospective study comparing the management of long-gap esophageal atresia in the Nordic countries.

BACKGROUND: Several surgical procedures have been described in the reconstruction of long-gap esophageal atresia (LGEA). We reviewed the surgical methods used in children with LGEA in the Nordic countries over a 15-year period and the postoperative complications within the first postoperative year. METHODS: Retrospective multicenter medical record review of all children born with Gross type A or B esophageal atresia between 01/01/2000 and 12/31/2014 reconstructed within their first year of life. RESULTS: We included 71 children; 56 had Gross type A and 15 type B LGEA. Delayed primary anastomosis (DPA) was performed in 52.1% and an esophageal replacement procedure in 47.9%. Gastric pull-up (GPU) was the most frequent procedure (25.4%). The frequency of chromosomal abnormalities, congenital heart defects and other anomalies was significantly higher in patients who had a replacement procedure. The frequency of gastroesophageal reflux (GER) was significantly higher after DPA compared to esophageal replacement (p = 0.013). At 1-year follow-up the mean body weight was higher after DPA than after organ interposition (p = 0.043). CONCLUSION: DPA and esophageal replacement procedures were equally applied. Postoperative complications and follow-up were similar except for the development of GER and the body weight at 1-year follow-up. Long-term results should be investigated. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level III.