Hernia of the umbilical cord associated with segmental dilatation of the ileum (SDI) is a rare entity with only 17 cases reported in literature. We hypothesize that the prominent mesenteric vessels or folds that splay out towards the ends of the SDI signify the presence of an antecedent duplication cyst which eventually got resorbed to form the dilated segment.

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.

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

Segmental intestinal dilatation is rare, which causes symptom of bowel obstruction and requires resection. The resection is not only diagnostic but also curative procedure. Recently, author experienced 2 cases of segmental dilatation of the ileum due to focal agenesis of the intestinal muscularis in 7 day & 4 day-old female neonates. The postoperative recovery was excellant in the first case after resection of dilated ileum(l5cm in length) and end to end anastimosis, and discharged at 20th day. But in the second case, the passage disturbance was not relieved after resection of dilated ileum(30cm in length), and author re-resected 80cm more of dilated proximal ileum at 2 weeks after the first operation. This baby discharged after diarrhea control with Loperin on I month after the second operation.

Final histologic examination showed I) normal population of ganglion cells in both narrowed & dilated ileum in both cases, 2) focal abscence of muscularis propria in both cases, 3) relative hypertrophy of inner circular muscle layer and thinned, multiple fragmented outer longitudinal muscle layer in case 2.

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 to 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.