Psychological outcome and quality of life in children born with congenital diaphragmatic hernia

OBJECTIVE: To assess psychological and social functioning as well as health-related quality of life and its early determinants in children born with congenital diaphragmatic hernia (CDH). DESIGN: Cross-sectional follow-up study. SETTING: Outpatient clinic of a tertiary care hospital. PARTICIPANTS: Thirty-three CDH survivors aged 6-16 years. Main exposure: Patients who developed CDH associated respiratory distress within 24 hours after birth. MAIN OUTCOME MEASURE: Psychological and social functioning assessed with the Wechsler intelligence scale for children (WISC-R), Bourdon-Vos test, Beery Developmental Test of visual motor integration, Child Behaviour Checklist (CBCL) and Teacher Report Form (TRF). Health-related quality of life assessed with the Child Health Questionnaire (CHQ) and the Health Utilities Index (HUI). RESULTS: Normal mean total IQ (100.0 +/- 13.2) and normal visual-motor integration, but significantly lower results for sustained attention (Bourdon-Vos test (38.8 +/- 11.2 points)) were found. Learning difficulties were reported by 30% of the parents. Eight children had scores in the clinical range on the CBCL and/or TRF, indicating clinical significant behavioural problems. Except for the CHQ scale "General health", health status was not different from the reference population. No significant correlations between test results and severity of CDH were found, except an association of general health and physical functioning with length of hospital stay. CONCLUSION: CDH patients are at risk for relevant subtle cognitive and behavioural problems, probably not related to CDH severity. These may contribute to school failure and need for special educational services. Perception of general health is reduced when compared to the reference population indicating that CDH survivors and their parents believe their health is poor and likely to get wors

Pulmonary function impairment after trachea-esophageal fistula: a minor role for gastro-esophageal reflux disease

Background: Long-term impairment of pulmonary function in trachea-esophageal fistula (TEF) patients is, at least in part, commonly ascribed to gastro-esophageal reflux disease (GERD). The objective of this study was to examine the independent effects of the underlying condition and GERD on cardiopulmonary function. Methods: Cardiopulmonary function of TEF patients, who had (severe) GERD (s-GERD) requiring antireflux surgery (TEF_GERD, n = 11) and TEF patients who did not have s-GERD (group TEF-GERD, n = 20) were compared with control patients who had isolated s-GERD requiring antireflux surgery (group GERD, n = 13). All patients performed spirometry, lung volume measurements, measurement of diffusion capacity and maximal cardiopulmonary exercise testing (CPET). Results: Mean age of the participants was 13.8 +/- 2.7 (group TEF_GERD). 13.2 +/- 2.9 (group TEF-GERD), and 14.7 +/- 1.5 years (group GERD). FVC and TLC were significantly lower in patients with TEF (with and without s-GERD) when compared to patients with isolated s-GERD. Most pulmonary function parameters were similarly affected in both TEF groups, but FEV1 was lower in the TEF+GERD group than in the TEF-GERD group. Cardiopulmonary exercise parameters were similar in all groups. Conclusions: TEF patients had restrictive lung function impairment when compared to patients with isolated s-GERD. This difference may be due to several causes, including thoracotomy. FEV1 was lower in TEF+GERD when compared to TEF-GERD indicating that GERD may affect large airway function in TEF patients. Other differences between TEF patients with and without s-GERD were not significant, suggesting only a minor role for GERD. Pediatr Pulmonol. 2011; 46: 348-355. (C) 2010 Wiley-Liss, In