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We wish to define colonic motor function in children with slow-transit constipation (STC) using manometry catheters introduced through appendiceal stomas, previously sited for controlling fecal retention by colonic irrigation.We undertook 24-hour pancolonic manometry of 6 children (5 boys; mean, 11.5 years; SD, 3.0) using a multilumen silastic catheter.were compared to nasocolonic motility studies obtained in healthy young adults.Antegrade propagating sequences (APSs) originated less frequently in the cecum compared to controls. There were fewer APS (mean +/- SEM: STC, 13 +/- 6 per 24 hours; controls, 52 +/- 6 per 24 hours; P.01) and high-amplitude propagating contractions (HAPCs: STC, 5 +/- 2 per 24 hours; controls, 9.9 +/- 1.4 per 24 hours; P.05). The amplitude of APS and HAPC was less in STC (APS, 39 +/- 9 mm Hg; controls, 54 +/- 3 per 24 hours; P.05) (HAPC: STC, 94 +/- 10 mm Hg; control, 117 +/- 3 mm Hg; P.01), whereas the amplitude of retrograde propagating sequences was greater in STC (43 +/- 6 mm Hg; control, 27 +/- 1 mm Hg; P.01). The distances propagated by HAPC were significantly less in STC (36 +/- 4.5 vs 47 +/- 2.3 cm, controls; P.05), and there was no evidence of a region-specific difference in propagation velocity of APS. Neither meal ingestion nor waking significantly increased colonic motor activity in patients with STC.Despite the small numbers available to be studied, we found that children with STC in whom an appendicostomy had been placed show significant abnormalities in pancolonic motor function.

Purpose Chronic idiopathic constipation (CIC) with soiling in children may result from slow colonic transit (SCT) or anorectal dysfunction and/or psychological problems known as functional fecal retention (FFR). Evidence is accumulating that SCT and FFR need different treatments, but they are poorly distinguished by solid marker studies. The authors used radionuclear transit scintigraphy to categorize children with CIC as having either FFR or SCT. Methods Children (N = 101) with CIC (and soiling) who were referred for further investigation after failure of standard treatments (diet, laxatives) received radiolabeled colloid orally, and scintillation images were collected at 0 to 2, 6, 24, 30 and 48 hours (total radiation dosage=2 standard x-rays). Radioactivity in 6 regions (precolonic, ascending, transverse, descending, rectosigmoid, and evacuated feces) was measured, and the median position (geometric center) of radioactivity at each time was determined. Results In children, meals normally reach the cecum at 6 hours and are evacuated in 30 to 58 hours. Fifty patients had retention of radioactivity in the proximal colon at 48 hours, indicating SCT. Analysis of the images and the geometric center showed that passage through the ascending colon and transverse colon was delayed in SCT. In 24 patients, radioactivity was passed by 30 hours, indicating normal transit or possible FFR. Twenty-two patients had retention in the rectum, indicating definite FFR. Five studies were borderline. Conclusions Radionuclear transit scintigraphy is useful for categorizing patients with CIC as having either FFR or SCT, allowing for different treatments. Radionuclear transit scintigraphy provides more detail and greater sensitivity than solid marker studies in diagnosing CIC. Radionuclear transit scintigraphy showed that half of our patients had SCT.

Tachykinins (TKs) colocalize with acetylcholine in excitatory motor neurones supplying human colonic circular muscle (CCM). Some children with slow-transit constipation (STC) have reduced TK-immunoreactivity in nerve terminals in CCM suggesting a deficit in neuromuscular transmission. This study aimed to test this possibility. Seromuscular biopsies of transverse colon were obtained laparoscopically from STC children (37, 17 with low density of TK-immunoreactivity). Specimens of transverse (17) and sigmoid colon (20) were obtained from adults undergoing colonic resection for cancer. CCM contractions were measured isotonically and responses to carbachol, neurokinin A (NKA) and electrical field stimulation (EFS) recorded. Carbachol and NKA-evoked contractions in adult and STC colon. Hyoscine (2 micromol L-1) significantly depressed responses to EFS in all preparations. Blockade of NK2 receptors (SR 48968, 2 micromol L-1) significantly depressed EFS-evoked contractions of adult transverse CCM, but had no effect on STC preparations. Thus, neuromuscular transmission in both adults and STC children is predominantly cholinergic and this component is unimpaired in the latter, indicating that reduced TK-immunoreactivity is not a marker for depressed cholinergic responses. Although pharmacologically responsive TK receptors are present in STC colon, we did not detect neuromuscular transmission mediated by release of TKs in these preparations.

Background/Purpose: Children with anorectal dysfunction can now be treated by antegrade continence enema (ACE), as described Malone et al. Those with idiopathic constipation, however, are not thought to be suitable for this treatment. Over 150 children attend the authors' department with proven slow transit constipation (mostly proven on nuclear transit/X-ray study), and the authors reviewed the outcome in the 40 of these who have had the ACE procedure. Families completed a questionnaire and attended interview with an independent assessor. Methods: Of the 40 patients, 32 patients were assessed. Follow-up ranged from 3 to 54 months (median, 18 months) and age ranged from 5 to 17 years (median age, 10 years). Three of 32 stomas were no longer in use. Frequency of soiling was reduced significantly in 20 patients, and a further 6 patients were clean ( P P Results: Stomal complications were frequent, (stenosis in 16 of 29, mucus leak in 20 of 29, fecal leak 3 of 29, catheter-related pain in 20 of 29). Slow evacuation (12 of 29) and pain with enema (17 of 20) also were common. Conclusion: Malone appendicostomy does improve the well being of patients with slow transit constipation, but the advantages are less dramatic than in children with normal motility. J Pediatr Surg 36:1227-1230. Copyright © 2001 by W.B. Saunders Company.

Slow-transit constipation (STC) is recognized in children but the etiology is unknown. Abnormalities in substance P (SP), vasoactive intestinal peptide (VIP) and nitric oxide (NO) have been implicated. The density of nerve fibers in circular muscle containing these transmitters was examined in colon from children with STC and compared to other pediatric and adult samples.Fluorescence immunohistochemistry using antibodies to NO synthase (NOS), VIP and SP was performed on colonic biopsies (transverse and sigmoid colon) from 33 adults with colorectal cancer, 11 children with normal colonic transit and anorectal retention (NAR) and 51 with chronic constipation and slow motility in the proximal colon (STC). The percentage area of nerve fibers in circular muscle containing each transmitter was quantified in confocal images.In colon circular muscle, the percentage area of nerve fibers containing NOSVIPSP (6 : 2 : 1). Pediatric groups had a higher density of nerve fibers than adults. In pediatric samples, there were no regional differences in NOS and VIP, while SP nerve fiber density was higher in sigmoid than proximal colon. STC children had lower SP and VIP nerve fiber density in the proximal colon than NAR children. Twenty-three percent of STC children had low SP nerve fiber density.There are age-related reductions in nerve fiber density in human colon circular muscle. NOS and VIP do not show regional variations, while SP nerve fiber density is higher in distal colon. 1/3 of pediatric STC patients have low SP or VIP nerve fiber density in proximal colon.

Gastrointestinal stromal tumours (GIST) are rare in children. A 7-year-old boy presented acutely with a severe upper gastrointestinal bleed and was found following angiography to have such a tumour in the duodenum, which was resected at laparotomy. The presence of CD 117 positive immunostaining was a confirmatory diagnostic marker. The prognosis and underlying molecular biology of the tumour is discussed. Understanding of the molecular pathogenesis has given rise to promising new therapies.

The authors describe their experience with a series of 11 patients in whom they have carried out Chait cecostomy catheter insertion into a laparoscopically formed appendicostomy. This technique has several advantages compared with percutaneous insertion and we now insert the device at the primary procedure. The authors have had one minor complication in their 11 patients, with mean follow-up of 8 months. This technique is recommended for the management of refractory constipation, for etiologies including spina bifida, anorectal malformation, distal intestinal obstruction syndrome, and slow-colonic transit.

Background Studies in animals suggest that enteric neurons decrease in density or number with increasing age. Neurons containing nitric oxide (NO), vasoactive intestinal peptide (VIP) and Substance P (SP) have been implicated. In human large intestine, NO-utilizing neurons decrease during childhood or early adulthood but it is not known if the innervation of the muscle changes. This study examined the density of nerve fibres containing these transmitters in sigmoid colon circular muscle from children and adults. Methods Fluorescence immunohistochemistry using antibodies to neuronal NO synthase (nNOS), VIP and SP was performed on sigmoid colon from 18 adults with colorectal cancer, two children with familial adenomatous polyposis, and normal colon from nine children with Hirschsprung’s disease. The percentage area of immunoreactive (IR) nerve fibres containing each transmitter in circular muscle was quantified in confocal images. Key Results In the adult sigmoid colon circular muscle, the percentage area of nerve fibres containing nNOS>VIP>SP (6 : 2 : 1). Paediatric groups had significantly higher percentage area of nerve fibres containing nNOS, VIP or SP-IR than adults, with the decrease in nerve fibre density occurring from birth to 30 years. Circular muscle thickness increased between 12 and 30 years. Total nerve fibre area remained constant, while the muscle increased in thickness. Conclusions & Inferences In human sigmoid colon circular muscle, there are reductions in nNOS-, VIP- and SP-IR nerve fibre density with growth from newborn to late adolescence but little further change with aging. The reduction in nerve density is due to an increase in circular muscle thickness rather than a loss of nerve fibres.