Antegrade Continence Enema Alone for the Management of Functional Constipation and Segmental Colonic Dysmotility (ACE-FC): A Pediatric Colorectal and Pelvic Learning Consortium Study.

PURPOSE: The purpose of the study was to determine if antegrade continence enema (ACE) alone is an effective treatment for patients with severe functional constipation and segmental colonic dysmotility. METHODS: A retrospective study of patients with functional constipation and segmental colonic dysmotility who underwent ACE as their initial means of management. Data was collected from six participating sites in the Pediatric Colorectal and Pelvic Learning Consortium. Patients who had a colonic resection at the same time as an ACE or previously were excluded from analysis. Only patients who were 21 years old or younger and had at least 1-year follow-up after ACE were included. All patients had segmental colonic dysmotility documented by colonic manometry. Patient characteristics including preoperative colonic and anorectal manometry were summarized, and associations with colonic resection following ACE were evaluated using Fisher's exact test and Wilcoxon rank-sum test. p-Values of less than 0.05 were considered significant. Statistical analyses and summaries were performed using SAS version 9.4 (SAS Institute Inc., Cary, North Carolina, United States). RESULTS: A total of 104 patients from 6 institutions were included in the study with an even gender distribution (males n = 50, 48.1%) and a median age of 9.6 years (interquartile range 7.4, 12.8). At 1-year follow-up, 96 patients (92%) were successfully managed with ACE alone and 8 patients (7%) underwent subsequent colonic resection for persistent symptoms. Behavioral disorder, type of bowel management, and the need for botulinum toxin administered to the anal sphincters was not associated with the need for subsequent colonic resection. On anorectal manometry, lack of pelvic floor dyssynergia was significantly associated with the need for subsequent colonic resection; 3/8, 37.5% without pelvic dyssynergia versus 1/8, 12.5% (p = 0.023) with pelvic dyssynergia underwent subsequent colonic resection. CONCLUSION: In patients with severe functional constipation and documented segmental colonic dysmotility, ACE alone is an effective treatment modality at 1-year follow-up. Patients without pelvic floor dyssynergia on anorectal manometry are more likely to receive colonic resection after ACE. The vast majority of such patients can avoid a colonic resection.

Most children experience resolution of idiopathic pediatric rectal prolapse with bowel management alone.

BACKGROUND: Recent studies in children with idiopathic rectal prolapse report up to 48% require surgical intervention to manage refractory disease. We sought to examine outcomes of our non-surgical approach to managing rectal prolapse using a bowel management program. METHODS: A retrospective review was performed for all children with the diagnosis of rectal prolapse between 2011 and 2020. Children with a rectal polyp or hemorrhoid were excluded. RESULTS: 47 children with rectal prolapse were identified (median age at diagnosis of 4 years (IQR 3,7.75); age ≤ 4 years n = 30; age > 4 years n = 17). Associated diagnoses included constipation (n = 45, 96%) and psychiatric diagnoses (n = 7, 14%). Children underwent a bowel management program including stimulant laxatives in 44 (94%) and osmotic laxatives in 2 (4%). Median follow-up time was 181 days (IQR 77, 238). Median time to resolution of rectal prolapse was 9 months (IQR 4, 13) with a maximum time to resolution of 31 months. We compared children ≤ 4 years old (Group A) to those > 4 years old (Group B). Psychiatric diagnoses were less common in Group A (3.5 vs. 38.9%, p = 0.003). Median time to spontaneous resolution was 6.5 months (IQR 3.5, 9.5) in Group A versus 13.5 (IQR 4, 16) months in Group B, p = 0.13. No differences in surgical intervention were identified. Three (6.4%) patients required surgery for prolapse. CONCLUSIONS: A bowel management program is an effective treatment for most children with rectal prolapse. This data suggests that surgical intervention is unnecessary in most children. LEVEL OF EVIDENCE: III.

Decreased ER visits and readmissions after implementation of a standardized perioperative toolkit for children with IBD.

BACKGROUND: Pediatric inflammatory bowel disease (IBD) carries significant morbidity and requires extensive medical and often surgical intervention. The aim of this study was to evaluate the impact of a dedicated Multidisciplinary clinic on the outcomes of children with IBD. METHODS: A retrospective review of a prospective database, established to track quality and outcomes of children undergoing an abdominal operation for IBD, was performed. Children who were managed before (09/2017-03/2019) and after (04/2019-06/2020) establishment of the multidisciplinary clinic were examined. The clinic instituted several care process protocols including early recovery (ERAS) and garnered additional resources for patients (wound ostomy, nutrition, social work, etc.) Primary outcomes were unanticipated return to the operating room, length of stay, ER visits within 30 days of surgery and hospital readmissions within 30 days of surgery. RESULTS: We identified 41 children who underwent a total of 80 major abdominal operations; 46.3% of procedures occurred before and 53.7% occurred after instituting our clinic. There were no notable changes in disease distribution (e.g., ulcerative colitis vs. Crohn's), disease severity, medication exposure, or case urgency (elective vs. emergent). ER visits within 30 days of surgery decreased (4 (9.3%) vs. 10 (27%), p = 0.04) as did readmissions within 30 days of surgery (1 (2.3%) vs. 9 (24.3%), p = 0.005). CONCLUSIONS: Implementation of a dedicated multidisciplinary clinic for IBD and its attendant focus on protocols and appropriate use of adjunctive resources was associated with decreased emergency department visits and hospital readmissions in the post-operative setting. LEVEL OF EVIDENCE: III.

Use of expedited post-operative protocol for children undergoing appendicostomy reduces length of hospitalization.

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.

Use of an enterocolitis triage and treatment protocol in children with Hirschsprung disease reduces hospital admissions.

BACKGROUND: While a consensus for the definition of Hirschsprung associated enterocolitis (HAEC) is lacking, the mainstay of treatment includes rectal irrigations with or without antibiotics. This treatment is often effective when initiated as an outpatient. Our institution implemented a triage algorithm in an effort to standardize care thus providing more timely treatment and preventing unnecessary hospital admissions. We sought to review our short-term experience. METHODS: A retrospective review was performed of all Hirschsprung (HD) patients <6 years old over two distinct time periods from May 2016-2017 (pre-protocol, group A) and June 2017-2018 (post-protocol, group B). Patients with a colostomy were excluded. Primary end point was hospital admission. Presenting symptoms were categorized as moderate or severe, with patient triage based on number and quality of symptoms. RESULTS: Eighty-seven total patients were included. Rectosigmoid transition zone was most common (75%) and 20% of patients had trisomy 21. HAEC occurred in 22% of patients in the preprotocol group (group A, n = 78, 27 episodes) and 20% of patients in the post-protocol group (group B, n = 87, 32 episodes). In group A, 78% of episodes required an unplanned visit and 74% resulted in admission. In group B, 81% of episodes required an unplanned visit and 50% resulted in admission (33% reduction in hospital admission, p = 0.06). Irrigations only, without antibiotics, were used in 30% of episodes in group A versus 41% in group B. Of patients who initially contacted the office by phone (group A = 7 episodes, group B = 6 episodes), outpatient management was successful in 43% versus 100% respectively (p = 0.07). No patient experienced increased morbidity in group B. DISCUSSION: Implementation of a HAEC treatment algorithm shows promise in improving the management and resource utilization of this complex patient population. It is anticipated that continued education of caregivers and the treatment team will result in a greater effect. A multi-institutional implementation of this algorithm is needed to characterize risk factors associated with failure of outpatient management. LEVEL OF EVIDENCE: III, Treatment Study.

Intermediate and long-term outcomes of a bowel management program for children with severe constipation or fecal incontinence.

PURPOSE: We sought to examine the long-term clinical success rates of a bowel management program (BMP) for children with severe constipation or fecal incontinence. METHODS: A single center review was conducted of children (≤18 years) enrolled in a BMP and followed in a colorectal specialty clinic (2011-2017). All patients who completed an initial week of the BMP were included. Patients enrolled in a BMP after 2018 were excluded. Success was defined as no accidents and <2 stool smears per week. RESULTS: A total of 285 patients were reviewed. BMP was initiated at a median age of 7 years (9 months-17 years). Primary diagnoses included functional constipation (112), anorectal malformation (ARM) (104), Hirschsprung Disease (HD) (41), rectal prolapse (14), spina bifida (6), fecal incontinence (3) and other (5; 4 sacral coccygeal teratomas and a GSW to the buttocks). Initial bowel regimen included large volume enema in 54% and high dose stimulant laxative in 46%. The initial Bowel Management Week (BMW) was successful in 233 (87% of adherent patients) patients with 17 (6%) non-adherent. One hundred twenty-two patients had follow-up at 12 months (72% success amongst adherent patients, 7% of patient non-adherent) and 98 patients had follow-up at 24 months (78% success amongst adherent patients, 10% of patients non-adherent). 21/154 (14%) patients started on enemas were later successfully transitioned to laxatives and 13/132 (10%) patients started on laxatives subsequently required enemas in order to stay clean. Clinic phone contact occurred outside of scheduled visits for adjustment to the BMP in 44% of patients. 33% of patients had surgery to aid bowel management (antegrade colonic enema (ACE) = 81, resection + ACE = 13, diverting stoma = 4). Median follow up was 2.5 years (5 weeks-7 years). CONCLUSION: Children who follow a structured BMP with readily available personnel to provide outpatient assistance can experience successful treatment of severe constipation or fecal incontinence long-term. A multi-institutional collaboration is necessary to identify factors which predict failure of a BMP and non-adherence. TYPE OF STUDY: Single-center retrospective chart review. LEVEL OF EVIDENCE: 3.

The effect of multidisciplinary colorectal center development on short-term hospital readmissions for patients with anorectal malformations or Hirschsprung disease.

INTRODUCTION: Hospital readmissions have become a quality metric for both hospital systems and individual surgeons. The medical literature is replete with studies describing readmission rates and factors contributing to readmissions following surgical procedures. Relatively little, however, has been done to define potential solutions to these problems. Over the past decade there has been a movement toward the development of multidisciplinary colorectal centers at high volume children's hospitals. We hypothesized that the development of a colorectal center at our children's hospital decreased readmissions in our colorectal surgery population. MATERIALS AND METHODS: A retrospective review was performed including all patients with the diagnosis of anorectal malformation (ARM) or Hirschsprung disease (HD) at our institution between the years of 2005-2017. Patient level outcomes were compared between the cohort treated prior to (2005-2010) and the cohort treated after the development of the colorectal center (2012-2017). RESULTS: A total of 354 patients were identified. One hundred seventy-eight patients (113 ARM, 65 HD) were treated prior to and 176 patients (110 ARM, 66 HD) were treated after the development of the colorectal center. Forty-five (25.3%) patients underwent neonatal repair prior to development of the center compared to 15 (8.5%) after. 111 (62.4%) patients underwent colostomy prior to the colorectal center comparted to 95 (54%) after. The rate of readmission within 120 days of discharge in the early group was 63% compared to 52% in those managed in the multidisciplinary colorectal center (p = 0.04). Conversely, the rate of emergency room visits increased from 8.4% to 27.3% (p = 0.01). The decrease in readmission rates was more pronounced in the ARM group, while the HD cohort had similar readmission rates before and after the establishment of the center. Multivariate logistic regression revealed an odds ratio of 0.59 (95% CI 0.37-0.92) for readmission following the development of the multidisciplinary colorectal center. DISCUSSION: The development of a multidisciplinary colorectal center at our institution was associated with decreased hospital readmissions, but an increase in emergency department resource utilization. These findings suggest improved efficiency in patient care with the implementation of a multispecialty, patient centered approach while also identifying areas of focus for future quality improvement initiatives. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Single-stage repair of rectoperineal and rectovestibular fistulae can be safely delayed beyond the neonatal period.

PURPOSE: We sought to examine the short-term outcomes following single-stage repair of rectoperineal and rectovestibular fistulae in infants and identify risk factors for wound complication. METHODS: Patients with a rectoperineal or rectovestibular fistula treated with a single-stage repair beyond the neonatal period (>30days of age) at a pediatric colorectal center (2011-2016) were reviewed. RESULTS: 36 patients with a rectoperineal and 7 patients with a rectovestibular fistula were repaired using the Posterior Sagittal Anorectoplasty (PSARP) approach. Median follow-up was 31months. The median age and weight at the time of repair were 166days and 6.5kg. Four patients (11%) suffered a wound complication (3 rectoperineal, 1 rectovestibular). Two required a diverting colostomy to allow wound healing. Two patients suffered skin separation managed with local wound care. All 4 patients experienced satisfactory wound healing without anoplasty stricture. Two different patients developed a stricture of the neo-anus. Age and weight at time of repair, gender, and presence of a genitourinary anomaly were not associated with wound complications. CONCLUSION: Delayed single-stage repair of rectoperineal and rectovestibular fistulae can be performed safely in infants beyond the newborn period. With attentive treatment, satisfactory healing can be anticipated if a wound complication is encountered. LEVEL OF EVIDENCE: Retrospective Comparative Study, Level III.

A structured bowel management program for patients with severe functional constipation can help decrease emergency department visits, hospital admissions, and healthcare costs.

BACKGROUND: Published health-care costs related to constipation in children in the USA are estimated at $3.9 billion/year. We sought to assess the effect of a bowel management program (BMP) on health-care utilization and costs. METHODS: At two collaborating centers, BMP involves an outpatient week during which a treatment plan is implemented and objective assessment of stool burden is performed with daily radiography. We reviewed all patients with severe functional constipation who participated in the program from March 2011 to June 2015 in center 1 and from April 2014 to April 2016 in center 2. ED visits, hospital admissions, and constipation-related morbidities (abdominal pain, fecal impaction, urinary retention, urinary tract infections) 12 months before and 12 months after completion of the BMP were recorded. RESULTS: One hundred eighty-four patients were included (center 1 = 96, center 2 = 88). Sixty-three (34.2%) patients had at least one unplanned visit to the ED before treatment. ED visits decreased to 23 (12.5%) or by 64% (p < 0.0005). Unplanned hospital admissions decreased from 65 to 28, i.e., a 56.9% reduction (p < 0.0005). CONCLUSION: In children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care. LEVEL OF EVIDENCE: 3.

Reducing hospital admissions of healthy children with functional constipation: a quality initiative.

Functional constipation (FC) is a common medical problem in children, with minimal risk of long-term complications. We determined that a large number of children were being admitted to our children's hospital for FC in which there was no neurological or anatomical cause. Our hospital experienced a patient complication in which a patient died after inpatient treatment of FC. Subsequently, we developed a standardised approach to determine when paediatric patients needed hospitalisation for FC, as well as to develop a regimented outpatient therapeutic approach for such children to prevent hospitalisation. Our quality improvement initiative resulted in a large decrease in the number of children with FC admitted into the hospital as well as a decrease in the number of children needing faecal disimpaction in the operating room. Our quality improvement process can be used to decrease hospitalisations, decrease healthcare costs and improve patient care for paediatric FC.

Characteristics of the Contrast Enema Do Not Predict an Effective Bowel Management Regimen for Patients with Constipation or Fecal Incontinence.

BACKGROUND: A bowel management program using large volume enemas may be required for children with anorectal malformations (ARM), Hirschsprung's disease (HD), severe medically refractive idiopathic constipation (IC), and other conditions. A pretreatment contrast enema is often obtained. We sought to determine if the contrast enema findings could predict a final enema regimen. METHODS: A retrospective review was performed at a tertiary care children's hospital from 2011 to 2014 to identify patients treated with enemas in our bowel management program. Patient characteristics, contrast enema findings (including volume to completely fill the colon), and final enema regimen were collected. RESULTS: Eighty-three patients were identified (37 ARM, 7 HD, 34 IC, and 5 other). Age ranged from 10 months to 24 years, and weight ranged from 6.21 kg to 95.6 kg at the time bowel management was initiated. Linear regression showed contrast enema volume was of limited value in predicting effective therapeutic saline enema volume (R(2 )= 0.21). The addition of diagnosis, colon dilation, and contrast retention on plain x-ray the day after the contrast enema moderately improved the predictive ability of the contrast enema (R(2 )= 0.35). Median final effective enema volume was 22 mL/kg (range: 5 - 48 mL/kg). CONCLUSIONS: We were unable to demonstrate a correlation with contrast enema findings and the effective enema volume. However, no patient required a daily enema volume greater than 48 mL/kg to stay clean.

The morbidity of a divided stoma compared to a loop colostomy in patients with anorectal malformation.

PURPOSE: Loop colostomies may contaminate the genitourinary (GU) tract in patients with anorectal malformations (ARM) owing to incomplete diversion of stool. Stoma complications are also thought to be higher with a loop versus divided colostomy. We sought to compare the morbidity, including urinary tract infections (UTI), in these two types of colostomies in children with ARM. METHODS: A review was performed at a children's hospital (1989-2014). Children with ARM who had a colostomy performed were identified. Demographic data and outcome variables were collected. Analyses included Student's t-test, Fischer's exact and logistic regression as appropriate. RESULTS: 171 patients were identified (loop=78; divided=93). Thirty percent of patients with a divided colostomy and 24% with a loop experienced a stoma complication (p=0.5). A subgroup analysis of children with a rectourinary fistula (54 divided, 26 loop) was performed to assess for effect of colostomy type on UTI. After controlling for other UTI risk factors (major GU anomalies, vesicostomy, and prophylactic antibiotics), loop ostomies were not associated with risk of UTI (OR 0.83, 95% CI 0.27-2.63). No patient with a loop colostomy developed megarectum. CONCLUSIONS: Children with ARM who undergo a loop colostomy are not at a detectable increased risk of experiencing a UTI compared to a divided stoma. The rate of stoma complication is high regardless of the type of stoma created.

Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21.

BACKGROUND/PURPOSE: The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD). METHODS: A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000-2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011-2015) were performed. RESULTS: 26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD+T21 (p=0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD+T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD+T21 (p=0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD+T21). One child with HD+T21 was toilet trained by age 4years versus 12 with HD (p=0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD+T21. CONCLUSION: Enterocolitis rates in children with HD+T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent postoperative follow-up. LEVEL OF EVIDENCE: Retrospective Study - Level II.

Effectiveness of an organized bowel management program in the management of severe chronic constipation in children.

BACKGROUND: Chronic constipation is a common problem in children. The cause of constipation is often idiopathic, when no anatomic or physiologic etiology can be identified. In severe cases, low dose laxatives, stool softeners and small volume enemas are ineffective. The purpose of this study was to assess the effectiveness of a structured bowel management program in these children. METHODS: We retrospectively reviewed children with chronic constipation without a history of anorectal malformation, Hirschsprung's disease or other anatomical lesions seen in our pediatric colorectal center. Our bowel management program consists of an intensive week where treatment is assessed and tailored based on clinical response and daily radiographs. Once a successful treatment plan is established, children are followed longitudinally. The number of patients requiring hospital admission during the year prior to and year after initiation of bowel management was compared using Fisher's exact test. RESULTS: Forty-four children with refractory constipation have been followed in our colorectal center for greater than a year. Fifty percent had at least one hospitalization the year prior to treatment for obstructive symptoms. Children were treated with either high-dose laxatives starting at 2mg/kg of senna or enemas starting at 20ml/kg of normal saline. Treatment regimens were adjusted based on response to therapy. The admission rate one-year after enrollment was 9% including both adherent and nonadherent patients. This represents an 82% reduction in hospital admissions (p<0.001). CONCLUSIONS: Implementation of a structured bowel management program similar to that used for children with anorectal malformations, is effective and reduces hospital admissions in children with severe chronic constipation.

Fecal continence following complex anorectal trauma in children.

BACKGROUND: Complex injuries involving the anus and rectum are uncommon in children. We sought to examine long-term fecal continence following repair of these injuries. METHODS: We conducted a retrospective review using our trauma registry from 2003 to 2012 of children with traumatic injuries to the anus or rectum at a level I pediatric trauma center. Patients with an injury requiring surgical repair that involved the anal sphincters and/or rectum were selected for a detailed review. RESULTS: Twenty-one patients (21/13,149 activations, 0.2%) who had an injury to the anus (n=9), rectum (n=8), or destructive injury to both the anus and rectum (n=4) were identified. Eleven (52%) patients were male, and the median age at time of injury was 9 (range 1-14) years. Penetrating trauma accounted for 48% of injuries. Three (14%) patients had accompanying injury to the urinary tract, and 6 (60%) females had vaginal injuries. All patients with an injury involving the rectum and destructive anal injuries were managed with fecal diversion. No patient with an isolated anal injury underwent fecal diversion. Four (19%) patients developed wound infections. The majority (90%) of patients were continent at last follow-up. One patient who sustained a gunshot injury to the pelvis with sacral nerve involvement is incontinent, but remains artificially clean on an intense bowel management program with enemas, and one patient with a destructive crush injury still has a colostomy. CONCLUSIONS: With anatomic reconstruction of the anal sphincter mechanism, most patients with traumatic anorectal injuries will experience long-term fecal continence. Follow-up is needed as occasionally these patients, specifically those with nerve or crush injury, may require a formal bowel management program.

Complete VACTERL evaluation is needed in newborns with rectoperineal fistula.

PURPOSE: Given that a rectoperineal fistula is developmentally the most mature lesion in the spectrum of anorectal malformations, it is not clear whether it merits a complete VACTERL evaluation. We sought to determine if the same evaluation is required to rule out associated anomalies in newborns with rectoperineal fistula as those with more complex anorectal malformations. METHODS: We performed a retrospective review of the pediatric colorectal center database at our tertiary care children's hospital from 2000 to 2012. Patients with anorectal malformations were categorized as rectoperineal fistula or "other" using the Krickenbeck classification. Records were reviewed to identify associated anomalies. RESULTS: 308 patients (156 males) were treated at our institution during the time period (rectoperineal fistula=102). Thirty-five (34%) patients with a perineal fistula had at least one associated anomaly. The most common anomalies were cardiac lesions (29% excluding PFO and PDA), genitourinary (20.6%), and malformations of the spine (15.7%). The overall occurrence of anomalies was lower than the "other" group. CONCLUSION: Our review demonstrates that newborns with a rectoperineal fistula frequently have associated anomalies and should undergo an evaluation similar to more complex lesions. These findings illustrate the importance of a structured approach to the evaluation of even the most straightforward lesions.