Arch Dis Child 1999;81:21–27 21 Chronic intestinal pseudo-obstruction: treatment Arch Dis Child: first published as 10.1136/adc.81.1.21 on 1 July 1999. Downloaded from and long term follow up of 44 patients S Heneyke, V V Smith, L Spitz, P J Milla Abstract disease had been clarified, from one centre over Aims—To document the long term course a long period of time, to determine the place of chronic idiopathic intestinal pseudo- and type of surgery used in their management, obstruction syndrome (CIIPS) in children and to identify prognostic factors. with defined enteric neuromuscular dis- The histopathological features and motility ease, and the place and type of surgery abnormalities have been reported previously used in their management; in addition, to and will not be considered in detail here. identify prognostic factors. Methods—Children with CIIPS were in- vestigated and treated prospectively. Methods Results—Twenty four children presented Between 1979 and 1997, 44 children with congenitally, eight during the 1st year of recurrent symptoms of bowel obstruction in life, and 10 later. Twenty two had myo- the absence of a mechanical occlusion, in pathy and 16 neuropathy (11 familial). whom Hirschsprung’s disease was excluded, Malrotation was present in 16 patients, 10 were investigated prospectively to define the had short small intestine, six had non- cause and extent of the bowel disorder. hypertrophic pyloric stenosis, and 16 had We noted the age at onset of illness, the pre- urinary tract involvement. Thirty two senting symptoms, drugs given, specific nutri- patients needed long term parenteral tional support used, and the types of surgery nutrition (TPN): for less than six months performed. We performed marker transit stud- in 19 and for more than six months in 13, ies, abdominal ultrasound, and contrast x ray 10 of whom are TPN dependent; 14 are studies in all children; upper gastrointestinal now enteral feeding. Prokinetic treatment contrast studies in 32 children, and contrast improved six of 22. Intestinal decompres- studies of the lower gastrointestinal tract in 22. sion stomas were used in 36, colostomy If there was involvement of the urinary tract, relieved symptoms in five of 11, and ileos- the extent of involvement, the management, tomy in 16 of 31. A poor outcome (death and the outcome were noted. http://adc.bmj.com/ (14) or TPN dependence (10)) was seen with malrotation (13 of 16), short small HISTOPATHOLOGY bowel (eight of nine), urinary tract in- Full thickness intestinal biopsies were available volvement (12 of 16), and myopathic from 44 patients, but for two patients the histology (15 of 22). material was obtained postmortem. These Conclusions—In CIIPS drugs are not comprised both small and large intestine from helpful but decompression stomas are. 22 patients, small bowel alone from 17, and Outcome was poor in 24 of 44 children (15 large bowel alone from five. We performed on September 28, 2021 by guest. Protected copyright. muscle disorder, 10 nerve disease). routine histology, enzyme histochemistry, and (Arch Dis Child 1999;81:21–27) immunocytochemistry by light microscopy and ultrastructural studies by electron microscopy Keywords: chronic intestinal pseudo-obstruction; as described previously (VV Smith, PhD thesis, decompression stomas; prognostic factors; total 10–12 parenteral nutrition University of London, 1993). MOTILITY STUDIES Chronic idiopathic intestinal pseudo- Surface electrogastrography Institute of Child obstruction syndrome (CIIPS) is an intestinal We recorded fasting gastric electrical control Health and Great motility disorder in which impaired intestinal Ormond Street activity (ECA) in 16 patients who initially pre- Hospital for Children motor activity causes recurrent symptoms of sented after 1989 and the results were analysed NHS Trust, London, intestinal obstruction in the absence of me- using our previously described method.6 In UK chanical occlusion. It is usually caused by dis- summary, four pairs of Ag–AgCl electrodes S Heneyke ease of the enteric neuromusculature and may matched for impedance were placed along the V V Smith involve either segments of, or the entire, L Spitz greater curvature of the stomach from the fun- P J Milla gastrointestinal tract. It is an uncommon disor- dus to the first part of the duodenum and the der with a high morbidity and mortality. Until potential diVerences across these pairs of elec- Correspondence to: the last 10 years few patients survived long trodes were measured for one hour after a four Dr P J Milla, enough for the natural history of CIIPS to be hour fast or during small intestinal manometry. Gastroenterology Unit, Institute of Child Health, apparent, and the place of surgery in its The frequencies present were determined by 30 Guilford Street, London management is still not well defined.1–8 The autoregressive modelling using a running spec- WC1N 1EH, UK. aims of our study were to document the course tral analysis method.6 After running spectral email: [email protected] of the disorder in a large number of patients, in analysis of gastric ECA the dominant fre- Accepted 22 December 1998 whom the underlying enteric neuromuscular quency was determined if present. 22 Heneyke, Smith, Spitz, Milla Table 1 Clinical details of 44 patients with chronic intestinal pseudo-obstruction Arch Dis Child: first published as 10.1136/adc.81.1.21 on 1 July 1999. Downloaded from Presentation Weight centile Patients Birth Present Age Symptoms Radiology Histology Surgery Outcome 1–6 3rd to – Birth Vomiting Dilated whole gut 5M 6 Ladd’s procedure 4 dead 50th Intestinal obstruction Pyloric obstruction 1N 6 ileostomy 2 TPN Short small intestine 6 pyloromyotomy Malrotation 7–13 3rd to – Antenatal Vomiting Dilated bowel M Ileostomy 4 dead 25th Failure to pass urine Megacystis 1 enteral Intestinal obstruction Megaureter 2 TPN 3 malrotation 14–24 3rd to < 3rd to Birthto7 Constipation Megacystis 9M Ileostomy 3 dead 50th 10th years Obstruction Megaureter 2? 6 enteral Abdominal distension 1 hydronephrosis 2 TPN Dilated bowel 25–30 10th to 3rd to Birthto2 Constipation Slow transit 6 N 3 colostomy 2 gastrostomy 75th 25th years Dilated bowel 4 normal feeds 1 stricture 31–42 3rd to < 3rd to Birthto3 Constipation 10 contracted colon 8N 2 colostomy 3 dead 50th 10th years Obstruction 3 malrotation 4? 5 ileostomy 7 enteral Abdominal distension 4 short small intestine 2 TPN 43 10th < 3rd 2 years Obstruction Dilated bowel M Ileostomy TPN 44 25th < 3rd 10 years Severe constipation Dilated bowel N Ileostomy TPN Abdominal distension Present, presentation; M, myopathy; N, neuropathy; ?, unknown; TPN, long term total parenteral nutrition. Small intestinal manometry abdominal distension and bilious vomiting and We assessed motor activity in the duodenum an abdominal x ray suggestive of distal bowel manometrically in 30 patients investigated obstruction. Seven neonates presented with the since 1981 after an overnight 15 hour fast as diagnosis of an obstructed urinary system on described previously.13 We recorded fasting antenatal ultrasound scanning and failure to motor activity for three interdigestive cycles, pass urine. Symptoms of intestinal obstruction where this was present, or for four hours, developed during the 1st month of life in six whichever was longer. The manometric pat- and by the 4th month of life in two other terns were analysed by means of visual planim- infants. Five neonates, presenting with bilious etry by two blinded independent observers.13 vomiting and a paucity of intestinal gas on Because the infants were vomiting and had a abdominal x ray, had a midgut malrotation on severe intolerance to feeds, postprandial activ- contrast study. One child presented with a gas- ity was not studied. troschisis and one with a ruptured exomphalos, both had persistent abdominal distension and http://adc.bmj.com/ STATISTICS bilious vomiting during the postoperative Fisher’s exact probability test was applied to period. After the 1st month of life, the most determine whether there were any significant common mode of presentation was chronic prognostic factors. constipation and abdominal distension with episodic vomiting. In three of the older Results children a febrile illness preceded the onset of The criteria for the diagnosis of CIIPS were gastrointestinal symptoms by a few days. fulfilled by 44 patients (25 boys, 19 girls). Barium meal and follow through revealed a on September 28, 2021 by guest. Protected copyright. Histopathology was available in all and motil- midgut malrotation in 16, a short small ity, contrast, and marker transit studies in intestine in nine, and a non-hypertrophic most. Thirty of the 44 patients had survived at pyloric stenosis in six. Six of these presented as the time of our study and had a mean age of 12 neonates and had a midgut malrotation, short years (range, 3 months to 26 years). Details of small intestine, and a non-hypertrophic plyoric all 44 patients are summarised in table 1. stenosis. In four patients, three from one family, circular muscle morphogenesis was ONSET OF SYMPTOMS AND CLINICAL abnormal, with two circular muscle coats. In PRESENTATION general, a barium enema was not helpful but Twenty four children developed obstructive suggested pseudo-Hirschsprung’s disease with symptoms during the 1st month of life: 18 were a contracted left sided segment in 11 patients, symptomatic at birth and seven were evident nine of whom had nerve disease. on ultrasound scanning antenatally. A further In those with an obstructed urinary system eight developed symptoms during the 1st year prenatally (n = 7), urinary tract infections were of life and 10 before the 3rd year of life. Two common. But in nine of 44, urinary tract children did not develop constipation or anomalies were found on routine investigation obstructive symptoms until the age of 6 and 10 without initial obstructive or infective symp- years, respectively. Seven had evidence of active toms. All of these had muscle disease.