• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권2호
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• pp.202-207
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• 2003

장신경 이형성증은 만성 가성 폐색 증후군의 드문 원인질환으로 임상 증상이 선천성 거대결장증과 유사하여 진단이 늦어지는 경우가 많기 때문에 임상적인 의심이 필요하다. 저자들은 쌍생아에서 신생아기에 태변이 배출되지 않고 복부 팽만을 보여 선천성 거대결장증이 의심되었으나 임상경과와 조직검사로 진단된 장신경 이형성증을 보고하는 바이다.

• Advances in pediatric surgery
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• 제8권1호
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• pp.54-61
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• 2002

Hirschsprung's disease (HD) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of meconium within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium ileus, meconium plug syndrome, distal ileal atresia and low imperforate anus often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, intestinal neuronal dysplasia (IND), hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.447-452
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• 2019

Purpose: Few studies have reported non-acute long-term morbidity rates in children with intestinal malrotation. The aim of this study was to investigate the rate of constipation in children undergoing Ladd's procedure for isolated intestinal malrotation. Methods: This retrospective study included children aged <15 years who underwent Ladd's procedure for intestinal malrotation between 2001 and 2016. Demographics, presence of volvulus perioperatively, need for bowel resection, short term (<30 days) and long-term complications, including mortality were recorded. Constipation was defined as treatment with laxatives at 1-year follow-up. Results: Of the 43 children included in the study, 49% were boys. The median age at surgery was 28 days (0-5, 293 days). Volvulus occurred in 26 children (60.5%), and bowel resection was required in 4 children (9.3%). Short-term complications categorized as grades II-V according to the Clavien-Dindo classification occurred in 13 children (30.2%). Of these, 5 children (11.6%) required re-operation. Constipation was observed in 9 children (23.7%) at the 1-year follow-up. No difference was observed in the rate of perioperative volvulus between children with and without constipation (44% vs. 65%, p=0.45). Excluding re-operations performed within 30 days after surgery, 3 children (6%) underwent surgery for intestinal obstruction during the study period. Conclusion: Many children undergoing Ladd's procedure require bowel management even at long-term follow-up, probably secondary to constipation. It is important to thoroughly evaluate bowel function at the time of follow-up to verify or exclude constipation, and if treatment of constipation is unsuccessful, these children require evaluation for dysmotility disorders and/or intestinal neuronal dysplasia.