Outcomes of children with constipation and autism spectrum disorder treated with antegrade continence enemas.

BACKGROUND: Treatment of functional constipation (FC) in children with autism spectrum disorder (ASD) is challenging due to sensory and behavioral issues. We aimed to understand whether antegrade continence enemas (ACEs) are successful in the treatment of FC in children with ASD. METHODS: A single-institution retrospective review was performed in children diagnosed with ASD and FC who underwent appendicostomy or cecostomy placement from 2007 to 2019. Descriptive statistics regarding soiling and complications were calculated. RESULTS: There were 33 patients included, with a median age of 9.7 years at the time of ACE initiation. The average intelligence quotient was 63.6 (SD = 18.0, n = 12), the average behavioral adaptive score was 59.9 (SD = 11.1, n = 13), and the average total Child Behavioral Checklist score was 72.5 (SD = 7.1, n = 10). Soiling rates were significantly lower following ACE initiation (42.3% vs. 14.8%, p = 0.04). Behavioral issues only prevented 1 patient (3.0%) from proper ACE use. Eleven patients (36.6%) were able to transition to laxatives. There were significant improvements in patient-reported outcomes measures and quality of life. CONCLUSION: Placement of an appendicostomy or cecostomy for management of FC in children with severe ASD was successful in treating constipation and improving quality of life.

Rates of Hirschsprung-Associated Enterocolitis Decrease With Increasing Child Opportunity Index.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). There is a correlation between social determinants of health (SDOH) and outcomes in children with HD. The Child Opportunity Index (COI) is a publicly available dataset that stratifies patients by address into levels of opportunity. We aimed to understand if a relationship exists between COI and HAEC. METHODS: A single-institution, IRB-approved, retrospective cohort study was performed of children with HD. Census tract information was used to obtain COI scores, which were stratified into categories (very low, low, medium, high, very high). Subgroups with and without history of HAEC were compared. RESULTS: The cohort had 100 patients, of which 93 had a COI score. There were 27 patients (29.0%) with HAEC. There were no differences in demographics or clinical factors, including length of aganglionic colon, operative approach, and age at pull-through. As child opportunity score increased from very low to very high, there was a statistically significant decrease in the incidence of HAEC (p = 0.04). CONCLUSION: We demonstrate a significant association between increasing opportunity and decreasing incidence of HAEC. This suggests an opportunity for targeted intervention in populations with low opportunity. LEVEL OF EVIDENCE: III. IRB NUMBER: IRB14-00232.

Laxative Weaning Protocol for Patients With Functional Constipation: A Pilot Study.

BACKGROUND: Children with functional constipation require prolonged laxative administration for proper emptying. Whether these laxatives can be weaned after better functioning is achieved is unknown. We aim to describe a standardized protocol for stimulant laxative weaning and its early outcomes. METHODS: Patients were candidates for weaning if they had been on a stable laxative dose for six months, defined as one bowel movement per day with no soiling, impaction, or enemas. Laxative dose was decreased by 10-25% with re-evaluation every two weeks. If patients remained well without constipation, dose was weaned further by 10-25%. If there were worsening of symptoms, lower dose was maintained for 3-6 months until re-evaluation. RESULTS: There were a total of sixteen patients evaluated. Median age was 12.7 years [IQR: 11.7-15.3] with laxative duration of 8.0 years [IQR: 5.4-10.7]. All patients were on senna; some were on fiber. Median starting senna dose was 71.3 mg [IQR: 54.3-75.0] and median fiber dose was 5.5 g [IQR: 4.0-6.0]. As of most recent follow up, nine patients (56.3%) had weaned off laxatives in 3.7 months [IQR: 1.3-11.6]. For those still on laxatives, median reduction in dose was 41.4 mg [30.0-75.0], and over half weaned their dose by >50%. Almost all (90.9%) of those on high doses were able to wean. CONCLUSION: A standardized laxative weaning process can be successful in patients with functional constipation, especially on high doses. Further prospective studies will be necessary to confirm the success of this protocol. LEVEL OF EVIDENCE: III.

Social Determinants of Health Are Associated with Failed Bowel Management for Children with Anorectal Malformations.

INTRODUCTION: Children with anorectal malformations (ARMs) benefit from bowel management programs (BMPs) to manage constipation or fecal incontinence. We aimed to understand the role of social determinants of health (SDOH) in outcomes following BMPs in this population. MATERIALS AND METHODS: A single-institution, institutional review board (IRB) approved, retrospective review was performed in children with ARM who underwent BMP from 2014 to 2021. Clinical, surgical, and SDOH data were collected. Children were stratified as clean or not clean per the Rome IV criteria at the completion of BMP. Descriptive statistics were computed. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. RESULTS: In total, 239 patients who underwent BMP were identified; their median age was 6.62 years (interquartile range [IQR]: 4.78-9.83). Of these, 81 (34%) were not clean after completing BMP. Children with prior history of antegrade enema procedures had a higher rate of failure. Children who held public insurance, lived within driving distance, had unmarried parents, lived with extended family, and lacked formal support systems had a significant association with BMP failure (p < 0.05 for all). Type of ARM, age at repair, type of repair, age at BMP, and type of BMP regimen were not significantly associated with failure. CONCLUSIONS: There is a significant correlation of failure of BMPs with several SDOH elements in patients with ARM. Attention to SDOH may help identify high-risk patients in whom additional care may lead improved outcomes following BMP.

Laparoscopic-Assisted Colonic Derotation in Patients With Hirschsprung Disease.

BACKGROUND: Children with Hirschsprung disease (HSCR) proximal to the splenic flexure or those needing a redo pull-through (PT) are at risk for tension and ischemia of the PT which could result in leak, stricture, or loss of ganglionated bowel. Colonic derotation is a technique used to minimize tension and avoid duodenal obstruction. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. METHODS: All patients underwent initial diversion and colonic mapping. The derotation procedure involves mobilization of the remaining colon, counterclockwise rotation via the stoma closure site, placement of the pull through (the right colon) lying on the right of the pelvis, and ligation of the middle colic artery with preservation of the marginal branch running from the ileocolic artery. This maneuver prevents compression of the duodenum by the mesenteric vessels and allows for an isoperistaltic, tension-free anastomosis. Intraoperative indocyanine green fluorescence angiography (ICG-FA) was utilized in many of the cases to map the blood supply of the pull-through colon. We reviewed outcomes for all children with HSCR who underwent colonic derotation from 2014 to 2023. Descriptive statistics were performed. RESULTS: There were 37 children included. Most were male (67.5%) with the original transition zone proximal to the rectosigmoid (81.1%). The median age at PT was 9.3 months [6.1-39.7]. Median operative time was 6.6 h [4.9-7.4] and 19 cases (51.4%) used ICG-FA. Most children had no 30-day postoperative complications (67.6%); in those who did develop complications, readmissions for electrolyte imbalance was most common (50.0%). There were zero cases of anastomotic leak at PT anastomosis. At long-term follow up, median 4.4 years [2.3-7.0], three children (8.1%) developed an anastomotic stricture, all were amenable to anal dilation, and five experienced episodes of enterocolitis (14.7%). Most children had between 1 and 4 stools per day (58.8%). CONCLUSION: Colonic derotation is a useful strategy to ensure well-perfused colonic length, protect the marginal artery blood supply, avoid duodenal compression, and ensure a tension-free anastomosis with minimal complications. TYPE OF STUDY: Original research, retrospective cohort. LEVEL OF EVIDENCE: III.