Purpose: To clarify the status of segmental dilatation of the intestine (SD) using a nationwide retrospective cohort survey. Methods: Supported by the Ministry of Health, Labour and Welfare in Japan, preliminary questionnaires requesting the numbers of SD cases from 2000 to 2009 were sent to 161 Japanese major institutes of pediatric surgery and gastroenterology. We present the results of our analysis of the data from secondary questionnaires. Results: Twenty-eight cases of SD were reported, including 19 males and 9 females. The average gestational age was 30.2 weeks and the average birth weight was 2.319 g. The period of onset was neonatal in 18 cases and infancy in 6 cases, including 7 cases diagnosed prenatally. The dilated segment was ileum in 14 cases and colon in 10 cases, and symptoms included abdominal distention, vomiting, and chronic constipation. Concurrent intestinal malformation such as malrotation, intestinal atresia, and anal atresia were present in 4 cases and 27 of 28 cases were successfully treated by surgical resection. Histologically, abnormal muscle layers were found in three cases and ectopic tissue in two cases. Conclusion: We determined the clinical features of SD based on data from 28 cases occurring over 10 years.
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