• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.202-207
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• 2003

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

• Advances in pediatric surgery
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• v.8 no.1
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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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• v.22 no.5
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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.