Search

We compared patient- and family-reported overall and stool-related quality of life (QoL) before and after an antegrade continence enema (ACE) procedure (cecostomy tube insertion) for refractory chronic constipation or fecal incontinence (CCFI). We hypothesized that patients with functional diagnoses experience similar improvements in QoL compared to those with organic diagnoses.This is a cross-sectional study of patients undergoing cecostomy tube insertion for CCFI at a tertiary pediatric hospital from 2012 to 2019. Patients and/or primary caregivers completed validated stooling and overall QoL surveys based on three time points: before surgery, three months after surgery, and at the time of survey / date of last follow-up. Repeated measures analyses compared scores over time between subjects and within the diagnostic groups.The response rate was 65% (22/34 patients, 12 organic and 10 functional diagnoses). Mean age was 8.3 years and 32% of the participants were female. Organic diagnoses were: spina bifida (6), anorectal malformation (5), and Hirschsprung Disease (1). There was substantial improvement in stool-related and overall QoL at three months post-ACE procedure (both p0.001) for all patients; both scores continued to improve significantly until the date of last follow-up (median 4.1 years, IQR 2.3-5.6, p0.001). There was no statistically significant difference in scores between patients with organic and functional diagnoses.Caregivers perceive a significant, sustainable improvement in stooling habits and QoL following ACE therapy. The improvement is comparable between patients with a functional diagnosis and those with an underlying organic reason for their CCFI.

Background An appendicostomy (ACE) is a surgical option for antegrade enemas in children with severe constipation and/or fecal incontinence who have failed medical management. In 2019, we initiated an expedited post-operative protocol and sought to examine our short-term outcomes compared with our historical cohort. Methods A retrospective review was performed of all children undergoing ACE between 2017 and 2020. Children were excluded if they underwent an associated procedure (e.g. colon resection). Patients were divided into two cohorts: historical cohort (2017–2018, Group A) and the expedited protocol (2019 to present, Group B). The primary outcome was length of stay. Results 30 patients met inclusion (Group A = 16, Group B = 14). The most common indications for ACE were constipation (50%) and constipation or fecal incontinence associated with anorectal malformation (43%). Group B experienced a decreased length of stay (1 vs 3 days, P = 0.001) without differences in 30-day surgical site infection (7.1% vs 18.8%, p = 0.61) or unplanned visit (15.4% vs 18.8%, p = 1.0). Group B had a higher prevalence of MiniACE® button placed through the appendix vs. Malone (42.8% vs 12.5%, p = 0.10). Conclusions Our expedited post-op protocol decreased length of stay without other significant adverse clinical sequelae. Level of evidence Retrospective Comparative Study, Level III.

Purpose A protocol to treat idiopathic constipation is presented. Methods A contrast enema is performed in every patient and, when indicated, patients are initially submitted to a "clean out" protocol. All patients are started on a Senna-based laxative. The initial dosage is empirically determined and adjusted daily, during a one week period, based on history and abdominal radiographs, until the amount of Senna that empties the colon is reached. The management is considered successful when patients empty their colon daily and stop soiling. If the laxatives dose provokes abdominal cramping, distension, and vomiting, without producing bowel movements, patients are considered nonmanageable. Results From 2005 to 2012, 215 patients were treated. 121 (56%) were males. The average age was 8.2years (range: 1–20). 160 patients (74%) presented encopresis. 67 patients (32%) needed a clean out. After one week, 181 patients (84%) achieved successful management, with an average Senna dose of 67mg (range: 5–175mg). In 34 patients (16%) the treatment was unsuccessful: 19 were nonmanageable, 3 noncompliant, and 12 continued soiling. At a later follow-up (median: 329days) the success rate for 174 patients was 81%. Conclusion We designed a successful protocol to manage idiopathic constipation. The key points are clean out before starting laxatives, individual adjustments of laxative, and radiological monitoring of colonic emptying. Treatment study Level IV.

Fecal incontinence is a socially debilitating problem for many children. We hypothesized that in selected patients with medically-refractory encopresis, placement of an appendicostomy or cecostomy tube for administration of antegrade continence enemas (ACE) would improve quality of life (QOL).We reviewed all patients with encopresis who underwent appendicostomy or cecostomy placement from 2003 to 2014 at our institution. We contacted subjects' parents by phone and administered 3 surveys: a survey reflecting current stooling habits, a disease-specific QOL survey, and the PedsQL™ QOL survey. QOL surveys were completed twice by parents, once reflecting pre-operative QOL, then again reflecting current QOL. Pre-procedure and post-procedure scores were compared by paired t-test.Ten patients underwent appendicostomy/cecostomy for encopresis. Eight completed phone surveys. All procedures were performed laparoscopically. All patients experienced fecal soiling pre-operatively, whereas 5/8 surveyed patients (63%) noted complete resolution of soiling post-procedure (p0.01). General and disease-specific QOL improved from pre-procedure to post-procedure in the following domains: social habits, physical activity, ability to spend the night elsewhere, feeling, and overall QOL (p0.05). PedsQL™ scores improved significantly in physical functioning, social functioning, and overall functioning (p0.05).Antegrade continence enemas significantly improve quality of life in patients with medically-refractory encopresis, likely related to resolution of soiling.4.

Objective The primary aim of this study was to determine if there is a change in the quality of life in pediatric patients with unremitting functional constipation and/or encopresis after undergoing a MACE procedure. Methods Patients, ages 5 to 18years with unremitting constipation and a normal evaluation, including anorectal manometry and colonic manometry, who opted to undergo a MACE procedure were contacted to participate in the study. Patients with congenital anorectal malformations as well as spinal cord disorders were excluded from the study. The patient's parent/guardian completed the PedsQL TM Generic Core Scales QOL survey prior to the operation, 6months, and 12months after the procedure. Results A total of 15 consecutive patients meeting protocol criteria were recruited within a period of 20months. The mean age at the MACE procedure was 9.8years (range 7.0–11.1). 5 patients were female. The mean QOL score pre-MACE was 64.1. At 6months post-MACE the mean overall QOL score was 90.2, and it was 92.0 at 12months. All 15 patients at the 6month follow up had significant improvement in their QOL (p=1.9×10 −7 ) and all subcategories of QOL were significantly improved as well. Conclusions A MACE procedure is of benefit to otherwise normal pediatric patients who have unremitting functional constipation with failure of medical treatment. Our patients had a significant improvement in all QOL categories and overall QOL.

Purpose: Erythromycin is successfully used as a gastroduodenal prokinetic agent. Given the limited available treatments for colonic dysmotility, further investigation into erythromycin's effect on colonic motility is warranted. We aimed to study the effect of erythromycin on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders. Methods: Patients referred for colonic manometry were eligible for enrollment. Fasting motility was recorded for 1 to 2 hours, then erythromycin lactobionate (EL), 3 mg/kg, was administered intravenously, and colonic motility was monitored for 1 to 2 hours after erythromycin. Manometry was then continued per routine. The motility index (MI) of pressure tracings at each pressure transducer was calculated for each patient for a period of 15 and 60 minutes before and after EL infusion. Change in MI was compared by Wilcoxon signed rank test. Results: Twenty patients were enrolled. The most common indication was constipation with encopresis. Seventy percent of patients had normal colonic manometry, and 30% of patients demonstrated a neuropathy. Average MI for the 60-minute period before and after EL infusion were 254 ± 74 mm Hg/h and 253 ± 94 mm Hg/h, respectively (P = .55). Average MI for the 15-minute period before and after EL infusion were 64 ± 23 mm Hg/15 min and 69 ± 32 mm Hg/15 min, respectively (P = .45). Conclusions: Administration of intravenous EL resulted in no changes in colonic MI in pediatric patients referred for colonic manometry. Further studies on potential colokinetic agents are warranted in this population of patients.

Objective This study evaluated the Trap-door button use (Cook Medical, Bloomington, IL) for antegrade enemas in children. Methods Since 2002, patients with fecal incontinence or encopresis and constipation underwent percutaneous cecostomy under laparoscopy using a button. Technical details are described. Age at surgery, operative time, hospital stay, diagnosis, indications for cecostomy, and duration of follow-up were recorded. A survey was proposed via a questionnaire that was sent to the patients. Patients wearing the button for less than 1 month were excluded from this evaluation. The survey concerned volume and frequency of enemas, difficulties encountered, benefits and disadvantages of this method, and assessment of the antegrade enemas in continence. Results Twenty-nine patients, 18 males and 11 females, aged 3 to 21 years (mean, 8.5 years) underwent laparoscopic Trap-door button placement. The indications for all the patients were intractable fecal incontinence in 24 cases and constipation with encopresis in 5 cases. Incontinence was because of myelomeningocele (n = 10), anorectal malformations (n = 11), caudal regression syndrome (n = 1), 22q11 syndrome (n= 1), and Hirschsprung disease with encephalopathy with convulsions (n = 1). Constipation with encopresis was because of sacrococcygeal teratoma (n = 1), cerebral palsy (n = 1), and acquired megarectum with psychiatric and social disorders (n = 3). A total of 26 cecostomy button placements and 3 sigmoidostomy button placements were successful with no intraoperative complication. The mean operative time was 25 minutes (10-40 minutes), and the hospital stay was 2.5 days (1-4 days). Twenty-two parents or patients answered the questionnaire. At the time of this survey, 2 patients had improved their fecal continence and had had the button removed. A mean of 4 weekly enemas was enough to improve fecal continence troubles (range, 1 daily to 1 for 2 weeks). The volume for enemas was 250 to 1000 mL (mean, 700 mL). The time required for the irrigation of the bowel by gravity took from 5 to 60 minutes (mean, 25 minutes) for 20 patients. Before surgery, 14 patients needed a diaper, day and night, and 6 needed sanitary protection. Soiling was a very significant inconvenience for all the patients. After surgery, only 5 patients needed a diaper (cerebral palsy, 22q11, cloacal malformation, myelomeningocele, bladder exstrophy) because of moderate results or urinary incontinence and continued soiling. Patients were asked to give an assessment (null = 0, bad=1, fair=2, good=3, very good=4). None of the patients felt there had been no changes or a bad result. There were 5 patients who felt they had an average result, 5 a good result, and 12 a very good result. The mean grade was 3.44 (17.2/20). A total of 3 patients had hypertrophic granulation tissue formation around the cecostomy button, and 12 had tiny leakage. Conclusion Percutaneous placement of a cecostomy button under laparoscopic control is an easy and major complication-free procedure. The use of the Trap-door device by the patients or with the help of the parents for antegrade enemas is effective and satisfactory. It improves the quality of life and is reversible.

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.

Background Hirschsprung's disease (HD) patients after pull-through (PT) may have recalcitrant constipation or recurrent enterocolitis (EC). Posterior myotomy/myectomy (POMM) are possible options for these problems. This study analyzed the outcome of POMM in HD patients post-PT. Methods Records of 348 HD patients were reviewed, and 32 were found to have undergone a POMM post-PT (1981 to 2002). Outcomes after this procedure were assessed. Statistics used linear and logistic regression. Results Of the 32 patients, 29 had complete records for analysis. Of those with pure constipation (12), 6 had aganglionosis post-PT. Of those with recurrent EC (17) only 1 had aganglionosis post-PT. POMM was performed at a mean of 3.1 years post-PT (11 myotomy, 18 myectomy). Average follow-up was 8.6 years (range, 0.7 to 21). Type of POMM had no correlation with overall functional outcome ( P = .44). Of those with chronic constipation, 60% had good results after POMM; the remainder required a redo-PT or colostomy. Interestingly, most patients with retained aganglionosis and chronic constipation did not respond after POMM (83%). Of those with recurrent EC, 75% became free of symptoms; none of the patients not responding have required redo-PT. Conclusions POMM to treat chronic constipation or recurrent EC in patients with HD post-PT is moderately successful. Because of the unsuccessful outcome with POMM in patients with a combination of constipation and aganglionosis, one should defer to a redo-PT in this group.

Background/Purpose: The authors' 15-year experience with children shows a high percentage of recurrence of functional constipation (FC) with conventional treatment. These data, confirmed in the international literature, led them to develop a new therapeutic approach. The aim of this study was to achieve intestinal control and avoid recurrence of FC in children through use of medical-psychological treatment. Methods: The authors studied 25 children (18 boys; mean age, 4.7 years; range, 2.10 to 7), 20% of whom had anal fissure, 30% retentive soiling, 52% pain on defecation, and 88% fecal retention owing to FC. Children and parents were questioned about eating and sleeping habits, school, toilet training, and daily routine. Treatment included increasing water and fiber intake, laxatives, and family therapy including making rules and working on autonomy and paternal role. Results: Mean onset of FC was 3.5 years, after "stressful events" in 88%. The questionnaire shows that 68% lacked parental autonomy and authority; 84% of children decided on their own about eating habits and sleeping; 68% had a "peripheral" father with a mother-child symbiotic relationship. After one month of therapy, 92% of children showed a modification of at least 2 behavioral patterns; after 3 months, 88% had regular bowel movements. During follow-up (range, 6 to 28 months), 48% had 2 or 3 recurrent episodes. After one year, 68% had reinforced the new behavioral patterns with resolution of the pathologic aspects. Conclusions: A multidisciplinary approach in the treatment of childhood functional constipation showed consistent therapeutic results by making rules and by equalizing family roles. J Pediatr Surg 38:819-823. © 2003 Elsevier Inc. All rights reserved.

Purpose: The authors devised computerized equipment for use in the biofeedback therapy in the management of fecal continence after surgery for anorectal malformations. Methods: The therapy was used for two to eight sessions in 14 children (11 who had high-type anomalies and three who had intermediate-type anomalies). The ages ranged 5 to 14 years. A control group of 17 children, aged 5 to 11 years, who had encopresis, was also treated with the same biofeedback therapy. Results: Clinical improvement was noted in 5 of the 14 (36%) children who had fecal incontinence, and in 15 of the 17 children (88%) who had encopresis. Both in patients who had fecal incontinence and in those who had encopresis, anal resting pressures were not affected by biofeedback therapy. Furthermore, the anal resting pressure in children who had fecal incontinence was significantly lower than that in children who had encopresis. However, anorectal manometry showed that the biofeedback therapy improved voluntary sphincter function and rectal sensation in both groups. Conclusion: Biofeedback therapy appears to be effective in most children who have encopresis whose sphincter function is intact, and in some children who have fecal incontinence after surgery for anorectal malformations.

The multifactorial nature of functional constipation in children suggests that a multidisciplinary management approach may be effective. The authors tested this hypothesis in a newly created pediatric Bowel Management Clinic (BMC). Detailed data were collected prospectively on all patients seen in the clinic over the first 16 months. Both quantitative and qualitative analyses were performed to describe the index population and to demonstrate the impact of the intervention. Satisfaction with care in the clinic was measured using the Measure of Processes of Care tool, then compared with a normative sample. One hundred fourteen patients, all previously treated unsuccessfully for constipation, were referred to a team comprised of a physician, nurse practitioner, nurse educator, dietitian, and psychosocial nurse specialist. The mean age was 5.4 years with equal gender distribution. Between the first and last visits recorded, several variables including stool consistency and frequency, soiling frequency, abdominal pain, rectal pain, and rectal bleeding all showed statistically significant (P.05) improvement. Qualitative data analysis showed the significant psychosocial impact of constipation on patients and their families. In the Measures of Processes of Care questionnaire, scores for the BMC were higher than normal on all scales except in provision of information. A multidisciplinary approach to functional constipation leads to both patient and parent satisfaction and significant short-term improvement. Further studies will examine the long-term impact of the clinic.

Aims Transcutaneous electrical stimulation (TES) was used to treat children with slow-transit constipation (STC) for 1 to 2 months in a randomized controlled trial during 2006 to 2008. We aimed to determine long-term outcomes, hypothesizing that TES produced sustained improvement. Methods Physiotherapists administered 1 to 2 months of TES to 39 children (20 minutes, 3 times a week). Fifteen continued to self-administer TES (30 minutes daily for more than 2 months). Mean long-term follow-up of 30 of 39 patients was conducted using questionnaire review 3.5 years (range 1.9-4.7 years) later. Outcomes were evaluated by confidence intervals or paired t test. Results Seventy-three percent of patients perceived improvement, lasting more than 2 years in 33% and less than 6 months in 25% to 33%. Defecation frequency improved in 30%. Stools got wetter in 62% after stimulation and then drier again. Soiling improved in 75% and abdominal pain in 59%. Laxative use stopped in 52%, and 43% with appendicostomies stopped washouts. Soiling/Holschneider continence score improved in 81% ( P = .0002). Timed sits switched to urge-initiated defecations in 80% patients. Eighty percent of relapsed patients elected to have home stimulation. Conclusion TES holds promise for STC children. Improvement occurred in two thirds of children, lasting more than 2 years in one third, whereas symptoms recurred after 6 months in one third of children.

Idiopathic constipation is a source of significant morbidity in children. A subset of patients is refractory to medical therapy and requires surgical intervention. We present a novel surgical technique for the management of these patients.We reviewed the records of 288 patients with severe idiopathic constipation and soiling. Patients who were refractory to medical management and had a megarectosigmoid underwent a transanal full-thickness rectosigmoid resection with a primary colo-anal anastomosis.Fifteen patients underwent a transanal rectosigmoid resection. The preoperative contrast enema demonstrated an enormously dilated rectosigmoid in 14. An average of 43 cm (range, 8-98 cm) of rectosigmoid was resected. Of 14 patients with more than 3 months of follow-up, the preoperative laxative dose was 68 mg of senna/d (range, 52-95 mg), which decreased to 8.6 mg postoperatively (P.001). Nine patients are clean without soiling, 1 is more prone to diarrhea, but is clean. Two patients soil occasionally, but are noncompliant, and 2 were lost to follow-up.Transanal rectosigmoid resection for medically intractable idiopathic constipation resulted in a dramatic reduction or elimination in laxatives use while preserving continence. It is a useful alternative to surgical options such as other colonic resections, antegrade enemas, and stomas.

Background: Internal anal sphincter hypertonicity with nonrelaxation can cause persistent constipation and obstructive symptoms in children after surgery for Hirschsprung’s disease. Intractable symptoms traditionally have been treated with anal myectomy, which may be ineffective or complicated by long-term incontinence. The authors evaluated prospectively the use of intrasphincteric botulinum toxin for these patients. Methods: Eighteen children were studied (age 1 to 13; median, 4 years). Botulinum toxin was injected (total dose 15 to 60 U) into 4 quadrants of the sphincter. Resting sphincter pressure was measured in 14 patients before and after injection. Ten have had 1 to 5 additional injections (total dose, 30 to 60 U per injection). Results: Four patients had no improvement in bowel function, 2 had improvement for less than 1 month, 7 had improvement for 1 to 6 months, and 5 had improvement more than 6 months. Nine of those with symptomatic improvement longer than 1 month had pressures measured, with a documented decrease in 8. Five with no significant clinical improvement had pressure measurements, with a decrease in 3. There were no adverse effects associated with botulinum toxin injection. Four children had new encopresis postinjection, which was mild and resolved in each case. Conclusions: Intrasphincteric botulinum toxin is a safe and less-invasive alternative to myectomy for symptomatic internal sphincter hypertonicity. Persistent symptoms, despite a fall in sphincter pressure, suggest a nonsphincteric etiology. Repeat injections often are necessary for recurrent symptoms.

Background : Antegrade continent enema (ACE) procedure has been accepted worldwide as the salvage procedure for intractable constipation and faecal incontinence after anorectal malformation surgery. Its application only has been reported from the developed countries. Methods : The authors performed four such operations on incontinent children in a poor socioeconomic group in South Africa. Results : Three patients had previous surgery for anorectal malformation, and one had intractable encopresis. The patients kept clean with water washouts only, starting 1 week after the operation. Conclusions : The ACE procedure can be used easily by patients in disadvantaged communities of Africa, and its use requires minimal but sympathetic supervision only. The authors recommedn that all pediatric surgeons dealing with these unfortunate children should perform this procedure after a trial period of medical treatment. This is also the first report of the ACE procedure performed for an encopretic child.

Although most children who have Hirschsprung's disease have an excellent result after pull-through surgery, some experience persistent constipation caused by "internal sphincter achalasia." Anal myectomy has been advocated for this problem, but it results in permanent injury to the sphincter and is not universally effective. Botulinum toxin has been safely used to selectively and reversibly weaken a variety of voluntary muscles and sphincters in both adults and children. Injection of botulinum toxin into the internal anal sphincter (IAS) should theoretically produce the same functional result as anal myectomy without permanent sphincter injury. Four children aged 4 to 8 years presented with persistent constipation after a pull-through procedure for Hirschsprung's disease. Two had associated encopresis, both of whom had previous myectomies. The authors performed four-quadrant intrasphincteric botulinum toxin injection (total dose, 15 U). Resting IAS pressure decreased in all children 4 to 8 weeks after injection. Patients have been followed up for 7 to 9 months. One child (with Down's syndrome) remained symptomatically unchanged. The other three families reported significant improvement in bowel function in their children. In two of these, there was a return of symptoms 6 months after injection; one child underwent reinjection with good results. Postinjection incontinence occurred in three children, but resolved after several weeks in the one who did not have encopresis before botulinum toxin injection. These preliminary results suggest that botulinum toxin may represent a less invasive alternative to anal myectomy for children who have severe constipation after surgery for Hirschsprung's disease. If myectomy is contemplated, botulinum toxin may also be useful as a means of predicting which children may benefit.

Sixty-three patients with biopsy-proven Hirschsprung's disease were diagnosed at LeBonheur Children's Medical Center, Memphis, TN between 1955 and 1980. Fifty-eight of these patients had pull-through procedures performed by three similarly trained pediatric surgeons. The follow-up was 100 percent, averaging 8 years from initial diagnosis. Demographics, surgical procedures performed, and complications are reviewed. Significant findings are (1) anastomotic strictures occurred most frequently when the level of aganglionosis was at the sigmoid colon; (2) postoperative encopresis was most likely when the endorectal pull-through procedures were performed before the age of 10 months; and (3) with selective use of colostomies or enterostomies performed prior to the pull-through procedure, the incidence of enterocolitis was low, with 0% mortality. On the basis of these findings, we recommend that, when the most proximal level of aganglionosis is the sigmoid colon, it is important to critically inspect the angulation of mesenteric blood vessels and viability of the splenic flexure pull-through colon segment to prevent ischemia and therefore anastomotic strictures. The endorectal pull-through procedure should be delayed until after 10 months of age. Infants with Hirschsprung's disease should have a colostomy or enterostomy prior to a pull-through procedure. Patients diagnosed at 10 months of age or more, who have not had earlier bouts of enterocolitis, are not low percentile weight, and are without signs of severe obstruction, are candidates for pull-through without a prior fecal diversion procedure.

The measurement of the strength of the anal sphincters has heretofore involved voluntary cooperation by the patient. In some children one cannot count on the maximum squeeze pressure being generated and in others the child is too young to understand what is required. We have devised an objective method of measuring the anal sphincter force (ASF) and have collected data derived from over 256 children over the course of 24 months. These include patients with Hirschsprung's disease, constipation, postoperative incontinence, spina bifida, and normal children both conscious and anesthetized. The results are presented and the value of the test and its applications are discussed.