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.

Primary segmental volvulus of the small intestine is not associated with malrotation, malfixation of the midgut, nor other primary small bowel lesions such as small bowel tumors. This entity is known to be more prevalent in adult and in certain global areas associated with particular diet habits. There have been very few reports in neonates, but not in this country so far. The author reports two cases of primary segmental volvulus. Case 1 was a septic 4-day-old girl with hematochezia due to jejunal volvulus with partial necrosis and panperitonitis. Resection of the segment and Bishop-Koop enterostomy were successful. Case 2 was a 3-day-old boy, who had ileal volvulus with ultra-short length of ileal atresia, probably due to intrauterine segmental volvulus. Limited resection of the atresia and spreading of the mesenteric base were enough to recovery. The rarity of the pathognomonic findings and limitation of the diagnostic workup due to rapid prqgression limit early diagnosis and good survival rate in this particular condition.

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.

A 2-day-old male {Premie, Large for gestational age(LGA), Intrauterine period (IUP) 33 weeks, birth weight 2,955 gram} was transferred with marked abdominal distention, bilious return via the orogastric tube, respiratory difficulty, and generalized edema (hydrops fetalis). He was born by cesarean section to a 36 year-old mother. Antenatal ultrasonogram at IUP 31 weeks demonstrated multiple dilated bowel loops suggestive of intestinal obstruction. There was no family history of cystic fibrosis. Simple abdominal films disclosed diffuse haziness and suspicious fine calcifications in the right lower quadrant. Barium enema demonstrated a microcolon. Sweat chloride test was not available in our institution. At laparotomy, there noted 1) a segmental volvulus of the small bowel with gangrenous change, associated with meconium peritonitis, 2) an atresia of the ileum at the base of the volvulus, and 3) the terminal ileum distal to the volvulus was narrow and impacted with rabbit pellets-like thick meconium. These findings appeared to be very similar to those of a complicated meconium ileus. In . summary, the ileal atresia and meconium peritonitis seemed to be caused by antenatal segmental volvulus of the small intestine in a patient with probable meconeum ileus.

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.