• Advances in pediatric surgery
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• v.4 no.2
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• pp.166-171
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• 1998

Segmental dilatation of the small intestine is a rare form of the congenital intestinal anomaly. Many other combined anomalies have been reported in, but the association with colonic duplication has not been reported in the literature. We report a case of segmental dilatation of the distal ileum associated with colonic duplication. The main clinical and pathogenic aspects are discussed, and the literatures are reviewed.

• Advances in pediatric surgery
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• v.19 no.2
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• pp.156-161
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• 2013

Congenital segmental dilatation of the colon is a very rare entity of unknown etiology, characterized by a localized dilatation of a bowel segment of the colon of variable length and an abrupt transition between the normal and dilated intestine. It can affect any part of the colon, with the rectosigmoid colon being the most commonly affected site. The clinical and radiological features may resemble that of Hirschsprung disease, but differ in that the normal ganglion cells are found in the dilated and normal segment of the colon. We performed laparoscopic-assisted transanal endorectal pull-through for segmental dilatation of rectosigmoid colon in an 8-year-old boy with chronic constipation since the age of 5 months.

• Advances in pediatric surgery
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• v.1 no.1
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• pp.68-72
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• 1995

Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal t o the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.