Table 1

Clinical details of 44 patients with chronic intestinal pseudo-obstruction

PatientsPresentationHistologySurgeryOutcome
Weight centileAgeSymptomsRadiology
BirthPresent
1–63rd to 50thBirthVomiting
Intestinal obstruction
Dilated whole gut Pyloric obstruction Short small intestine Malrotation5 M
1 N
6 Ladd’s procedure
6 ileostomy
6 pyloromyotomy
4 dead
2 TPN
7–133rd to 25thAntenatalVomiting
Failure to pass urine Intestinal obstruction
Dilated bowel Megacystis
Megaureter
3 malrotation
MIleostomy4 dead
1 enteral
2 TPN
14–243rd to 50th< 3rd to 10thBirth to 7 yearsConstipation Obstruction
Abdominal distension
Megacystis
Megaureter
1 hydronephrosis
Dilated bowel
9 M
2?
Ileostomy3 dead
6 enteral
2 TPN
25–3010th to 75th3rd to 25thBirth to 2 yearsConstipation
Dilated bowel
1 stricture
Slow transit6 N3 colostomy2 gastrostomy
4 normal feeds
31–423rd to 50th< 3rd to 10thBirth to 3 yearsConstipation Obstruction
Abdominal distension
10 contracted colon
3 malrotation
4 short small intestine
8 N
4?
2 colostomy
5 ileostomy
3 dead
7 enteral
2 TPN
4310th< 3rd2 yearsObstructionDilated bowelMIleostomyTPN
4425th< 3rd10 yearsSevere constipation Abdominal distensionDilated bowelNIleostomyTPN
  • Present, presentation; M, myopathy; N, neuropathy; ?, unknown; TPN, long term total parenteral nutrition.