Colonic Hyperactivity Results in Frequent Fecal Soiling in a Subset of Children After Surgery for Hirschsprung Disease

Division of Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio 45229, USA.
Journal of pediatric gastroenterology and nutrition (Impact Factor: 2.63). 04/2011; 52(4):433-6. DOI: 10.1097/MPG.0b013e3181efe551
Source: PubMed


Fecal soiling is a challenging problem in some children after pull-through surgery for Hirschsprung disease (HSCR). The prevailing perception is that soiling results from overflow incontinence; however, its treatment with laxatives yields mixed results. Colonic manometry studies are reported to be normal in most patients in this population. The interpretation of these findings does not support the physiology of fecal overflow incontinence in these children. The aim of the present study was to define the physiology underlying daily, frequent fecal soiling in children after surgery for HSCR using manometric techniques.
Four pediatric motility centers in the United States participated in the study; medical records and manometric tracings (anorectal and colonic) of children (n = 59; 6.5 years; 48 boys) who had pull-through surgery for HSCR and presented with daily, frequent fecal soiling were examined. Children referred for evaluation of constipation who had normal colonic manometry served as controls (n = 25; 6.7 years; 12 boys). The patients with HSCR were divided into 2 groups (Hirschsprung disease groups 1 and 2 [HD1, HD2]) based on the absence or presence of high-amplitude propagated contractions (HAPCs). A control group that included children with chronic constipation was also studied. We compared the mean HAPC frequency between the HD2 and control groups.
HD1 included 21 patients who had no HAPCs in fasting or postprandial periods. HD2 included 38 patients who had an average of 0.07 HPACs/min while fasting and 0.13/min in the postprandial state. In this subset the number of HAPCs in the fasting state (P = 0.04) and the postprandial state (P < 0.001) was greater when compared with controls. Additionally, there was a significant increase in HAPCs/min from the fasting to the postprandial state (P = 0.01). In the HD2 group 40% had colonic hyperactivity.
Daily, frequent fecal soiling after pull-through surgery for HSCR may be due to colonic hyperactivity in some children. It is imperative that this unique subset be identified because the management strategy would include avoidance of laxatives, contrary to standard current practice.

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    • "Yet long term clinical follow-up of HSCR patients has found that surgical intervention does not completely ''cure'' intestinal symptoms in these individuals who continue to suffer from intestinal disorders, such as enterocolitis and chronic constipation, Contents lists available at ScienceDirect journal homepage: despite successful surgical removal of the aganglionic gut segment (Amiel et al., 2008; Austin, 2012; Chumpitazi and Nurko, 2011; Kaul et al., 2011). The occurrence of such chronic bowel dysfunction across a variety of surgical procedures (Chumpitazi and Nurko, 2011) and evidence from developmental studies of HSCR mouse models (Paratore et al., 2001, 2002; Walters et al., 2010) hint that defects in ENS development in the proximal, ganglionated portions of the intestine may be causative. "
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    ABSTRACT: Normal enteric nervous system (ENS) development relies on numerous factors, including appropriate migration, proliferation, differentiation, and maturation of neural crest (NC) derivatives. Incomplete rostral to caudal migration of enteric neural crest-derived progenitors (ENPs) down the gut is at least partially responsible for the absence of enteric ganglia that is a hallmark feature of Hirschsprung disease (HSCR). The thought that ganglia proximal to aganglionosis are normal has guided surgical procedures for HSCR patients. However, chronic gastrointestinal dysfunction suffered by a subset of patients after surgery as well as studies in HSCR mouse models suggest that aberrant NC segregation and differentiation may be occurring in ganglionated regions of the intestine. Studies in mouse models that possess enteric ganglia throughout the length of the intestine (non-HSCR) have also found that certain genetic alterations affect neural crest lineage balance and interestingly many of these mutants also have functional gastrointestinal (GI) defects. It is possible that many GI disorders can be explained in part by imbalances in NC-derived lineages. Here we review studies evaluating ENS defects in HSCR and non-HSCR mouse models, concluding with clinical implications while highlighting areas requiring further study.
    Developmental Biology 01/2013; 382(1). DOI:10.1016/j.ydbio.2013.01.024 · 3.55 Impact Factor
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    ABSTRACT: The main objective of this study is to evaluate the efficacy of pelvic floor muscle exercise for the treatment of fecal incontinence after Soave procedure for Hirschsprung disease (HD). A case series study was performed in 24 incontinent children after Soave pull-through surgery for HD. Out of the 24 patients, 16 patients (training group) received pelvic floor muscle training while the other 8 patients (control group) did not receive further treatment. For children who received pelvic floor muscle training, biofeedback treatment was given for 2 weeks in hospital and they were then instructed to carry out pelvic floor muscle exercise at home. At the baseline and after 1 year of training, anorectal manometry was performed to measure resting anal canal pressure, squeeze pressure, and rectal sensation. Efficacy of pelvic floor muscle exercise for the treatment of postoperative fecal incontinence was evaluated by the difference between baseline and 1-year follow-up values. At the baseline, the characteristics of the incontinent children were also compared with 18 children who were performed Soave operation for HD and had normal anal function. Lower resting anal canal pressure distinguished the incontinent children from the continent ones. Resting pressure of the incontinent children was significantly improved by pelvic floor muscle exercise: the baseline and 1-year follow-up values of the treatment group were 18.6 ± 6.2 and 35.4 ± 8.7 mm Hg, respectively. Squeeze pressure and clinical outcomes were also improved after the pelvic floor muscle training: only 3 out of the 16 patients had occasional soiling after the training. No significant changes in clinical outcome and manometry measurements were observed in the control group. The damage of internal anal sphincter might be one of the causes of fecal incontinence after Soave procedure. The damage of internal anal sphincter could be caused by lower level of dissection, vigorous anal dilation, and excessive anal canal traction during operation. Pelvic floor muscle training is one procedure of choice to treat this complaint.
    European Journal of Pediatric Surgery 05/2012; 22(4):300-4. DOI:10.1055/s-0032-1313351 · 0.99 Impact Factor
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    ABSTRACT: Background Antegrade continence enemas (ACE) have been used in the treatment of defecation disorders in children; little is known on their effect on colon motility and the utility of the colon manometry (CM) predicting long-term ACE outcomes. Methods Retrospective review of children with constipation undergoing CM before and after ACE to evaluate CM changes and their utility on predicting ACE outcome. Key Results A total of 40 patients (mean age 8.8 SD 3 years and 53% female patients) were included; 39 of 40 responded to the ACE. Of these 39, 14 (36%) were dependent and 25 (64%) had decreased it (11 of those or 28% discontinued it). On repeat CM we found a significant increase in the fasting (P < 0.01) and postprandial (P = 0.03) motility index, number of bisacodyl-induced high amplitude propagating contractions (HAPCs) (P = 0.03), and total HAPCs (P = 0.02). Gastrocolonic response to a meal, propagation and normalization of HAPCs improved in 28%, 58%, and 33%, respectively, with CM normalizing in 33% of patients. The baseline CM did not predict ACE outcome. The presence of normal HAPCs on the repeat CM was associated with ACE decrease. Progression and normalization of HAPCs (P = 0.01 and 0.02, respectively) and CM normalization (P = 0.01) on repeat CM were individually associated with ACE decrease. No CM change was associated with ACE discontinuation. Multivariate analysis showed that older age and HAPC normalization on CM predict ACE decrease and older age is the only predictor for ACE discontinuation. Conclusions & Inferences Colon motility improves after ACE and the changes on the repeat CM may assist in predicting ACE outcome.
    Neurogastroenterology and Motility 10/2012; 25(2). DOI:10.1111/nmo.12018 · 3.59 Impact Factor
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