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INTRODUCTION: Duodenal atresia (DA) is the most common atresia of the small bowel. This study aims to assess the prevalence, mortality, and associated anomalies related to DA in Finland from 2004 to 2017. MATERIAL AND METHODS: A nationwide study based on registers maintained by the Finnish Institute for Health and Welfare and Statistics Finland containing data on all live births and stillbirths and terminations of pregnancy. The cases were identified based on the ICD-9 and 10 (International Classification of Diseases revisions 9 and 10) codes. Associated anomalies were classified based on the EUROCAT criteria; minor anomalies were excluded. RESULTS: There were 249 DA cases including 222 (89.2%) live births, 16 (6.4%) stillbirths, and 11 (4.4%) terminations. There was no significant change in the prevalence rates between 2004 and 2017. Live birth prevalence was 2.75/10,000 and total prevalence was 3.08/10,000 births. A total of 100 (40.2%) cases were isolated, 67 (26.9%) had other multiple congenital anomalies, and 83 (33.3%) were syndromic. There were no terminations in isolated DA. Most associated anomalies were cardiac (36.1%), followed by other gastrointestinal tract anomalies (23.7%) and limb deformities/defects (7.2%). Trisomy 21 was observed in 63 cases (25.3%). Neonatal mortality was 3.6% (n = 8) and at 1 year 95.0% were alive. Both neonatal and infant mortalities were associated with cardiac anomalies (p < 0.001 and p = 0.001, respectively). All neonatal deaths had associated cardiac defect(s). CONCLUSIONS: The prevalence of DA in Finland remains stable and among the highest reported. DA is often associated with cardiac anomalies, which portend a high risk for mortality. Despite the burden of associated anomalies, overall survival is high.
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Neuroblastoma is a malignant tumour affecting 10.5/1 million children annually. It arises from sympathetic nervous system precursor cells and is most frequently found in the adrenal gland and abdominal paravertebral ganglia. Diarrhoea as a presenting symptom of neuroblastoma is uncommon and usually linked to vasoactive intestinal peptide (VIP) tumour secretion. Even more rarely, postoperative diarrhoea may follow neuroblastoma tumour resection. Published studies generally associate postoperative diarrhoea with subadventitial tumour resection. These findings are however based on a handful of reports. This systematic review therefore aims to analyse the true incidence of postoperative diarrhoea and its morbid correlation with the extent/type of surgical resection. Pubmed/Embase databases were searched according to PRISMA guidelines. Final analysis consisted of 16 studies: N = 779 patients. Postoperative diarrhoea was significantly more common in all patients who underwent subadventitial resection compared to non subadventitial resection, p < 0.001 (OR 25.9, 95% CI 9.3-72.4). 5-year survival rates were equivalent in both groups. Preoperative diarrhoea was rarely reported in studies and always strongly linked to elevated VIP secretion. In the majority of neuroblastoma patients, preoperative diarrhoea ameliorated after gross tumour resection with elevated VIP normalized. The operative technique of subadventitial neuroblastoma resection portends significant risk(s) of post operative diarrhoea not seen in those patients undergoing other classical methods of tumour resection with 5-year survival rates strikingly similar. These findings affirm that subadventitial tumour resection should be avoided when undertaking surgery for neuroblastoma to minimize the risk(s) of persistent postoperative diarrhoea.