BACKGROUND: Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD.
METHODS: The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD.
RESULTS: This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations.
CONCLUSIONS: A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group\'s pre- and postoperative management.
METHODS: V.

Aim: To review the indications for rectal mucosal/submucosal biopsy (RMSBx) used for diagnosing Hirschsprung\'s disease (HD) in pediatric patients. Methods: The medical records of all children between 1 and 15 years old assessed for chronic constipation between 2012 and 2022 were reviewed. Until the end of 2018, enema usage (E+) was a major indication for RMSBx. In 2019, laxative use for 3 months irrespective of enema use was added as an indication (L+). To determine the relevance of enema usage, L+ was subdivided by enema usage into (L+E+) and (L+E-) groups. The effect of changing the indications for RMSBx on the incidence of HD was investigated. Results: Of 562 eligible subjects, E+ = 410, L+ = 152; demographics are similar. RMSBx rate in E+ (E+RMSBx) was 36/410 (8.8%) and in L+ (L+RMSBx) was 42/152 (27.6%;) (P < .05). For L+RMSBx, 15/42 were L+E+ and 27/42 were L+E-. HD incidence in E+RMSBx was 8/36 (22.2%; E+HD) and in L+RMSBx was 13/42 (31.0%; L+HD) (p = ns). In L+RMSBx, HD incidence in L+E+ was 5/15 (33.3%; L+E+HD) and in L+E- was 8/27 (29.6%; L+E-HD) (P = ns). Differences in daily bowel motion frequency 6 months postoperatively were not statistically significant; E+HD (1.75/d) versus L+HD (2.03/d) and L+E+HD (1.60/day) versus L+E-HD (2.31/day). Unassisted voluntary defecation was confirmed 12 months postoperatively in 7/8 (87.5%) E+HD, 11/13 (84.6%) L+HD, 4/5 (80.0%) L+E+HD, and 7/8 (87.5%) L-E-HD; differences were not significant. Laxatives were still required in 2/8 (25.0%) E+HD, 3/13 (23.1%) L+HD, in 1/5 (20.0%) in L+E+HD, and 2/8 (25.0%) L+E-HD; differences were not significant. Conclusion: Incidence of HD was higher in L+HD, but not significantly different suggesting that indications for RMSBx have potential to influence incidence of HD and hint that the incidence of HD could actually be higher. Further assessment of additional indications is warranted to diagnose HD with greater accuracy.

BACKGROUND: Patients with Hirschsprung disease (HD) can experience soiling after their pull-through. We evaluated the use of antegrade continence enema (ACE) for this patient population and investigated the timing and factors associated with getting the patient clean.
METHODS: We performed a single-institution retrospective review (from January 2020 to January 2023) of patients with HD and prior pull-through who had persistent soiling, failed laxative treatment or rectal enemas, and were treated with antegrade enemas. The primary outcome was time to become \"clean of stool\" which was defined as at least one bowel movement per day, no more than one stool accident per week, and no significant stool burden radiographically. Kaplan-Meier survival analysis was performed, and univariate cox proportional hazard regression was used to assess factors associated with time to continence.
RESULTS: Thirty patients who met the criteria underwent ACE creation at a median age of 6.5 y (interquartile range 5.3-9.8) with a median follow-up time of 11.5 mo (interquartile range 5.6-16.5). Most patients became clean by 4 mo (13 of 20, 65%) with similar results at 1-y follow-up (16 of 21, 76%). The median time to becoming clean was 4.3 mo (95% confidence interval: 1.7-15.0. Patients with hypermotility were more likely to continue to soil at 1 y (80% versus 13%, P = 0.01). There were no additional factors significantly associated with time to cleanliness.
CONCLUSIONS: ACE is a useful modality for HD patients with soiling. Most became clean of stool in 4 mo. The presence of hypermotility was associated with a higher incidence of persistent soiling at 1 y.

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%).
CONCLUSIONS: 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.
METHODS: Original research, retrospective cohort.
METHODS: III.

Surgery and management of rectal cancer have made significant progress in recent decades. However, there is still no coloanal anastomosis technique that offers a good compromise between functionality and low morbidity. The aim of this study is to evaluate the safety and efficiency of the modified delayed coloanal anastomosis (mDCA). In this retrospective study, we analyzed the morbi-mortality as well as functional outcomes of 19 patients treated with mDCA, out of 73 colorectal cancer patients treated at our institution from September 2021 to June 2023. The inclusion criteria were cancer of the mid and low rectum (tumor less than 10 cm from the anal verge). Morbidity represented by complications of Clavien-Dindo grade III or higher was estimated at 5.2%. Only one patient experienced an asymptomatic anastomotic leak (AL) grade A. Ischemia of the colonic stump occurred in one patient, taken back to the OR on the 5th postoperative day. No stump retraction was noted. Anastomotic stenosis appeared in one patient (5.2%) during the 90-day postoperative period, and was treated by instrumental dilation. Perioperative mortality was nil. The mean St Marks incontinence score at 90 days was 13.2 points. At the 3-month follow-up, 15 patients (78.9%) had major low anterior resection syndrome (LARS), three (15.7%) had minor LARS, and one patient (5.2%) had no LARS. None of the patients had a diversion loop ileostomy. The mDCA, by decreasing the rate of AL, without the need for diversion ileostomy, might be an interesting alternative to the conventional immediate coloanal anastomosis (ICA), for restoring the GI tract after proctectomy for cancer.

OBJECTIVE: Acquired rectovaginal fistulae (RVF) are a complication of paediatric HIV infection. We report our experience with the surgical management of this condition.
METHODS: We retrospectively reviewed the records of paediatric patients with HIV-associated RVF managed at Chris Hani Baragwanath Academic Hospital (2011-2023). Information about HIV management, surgical history, and long-term outcomes was collected.
RESULTS: Ten patients with HIV-associated RVF were identified. Median age of presentation was 2 years (IQR: 1-3 years). Nine patients (9/10) underwent diverting colostomy, while one demised before the stoma was fashioned. Fistula repair was performed a median of 17 months (IQR: 7.5-55 months) after colostomy. An ischiorectal fat pad was interposed in 5/9 patients. Four (4/9) patients had fistula recurrence, 2/9 patients developed anal stenosis, and 3/9 perineal sepsis. Stoma reversal was performed a median of 16 months (IQR: 3-25 months) after repair. Seven patients (7/9) have good outcomes without soiling, while 2/9 have long-term stomas. Failure to maintain viral suppression after repair was significantly associated with fistula recurrence and complications (φ = 0.8, p < 0.05).
CONCLUSIONS: While HIV-associated RVFs remain a challenging condition, successful surgical treatment is possible. Viral suppression is a necessary condition for good outcomes.

Background  Dupuytren\'s disease decreases quality of life significantly and often requires surgical treatment, nevertheless there is no actual gold standard. The aim of this study was to introduce the use of minimally invasive pull-through technique. Methods  From 2016 to 2020, 52 patients suffering from Dupuytren\'s contracture were treated with the minimally invasive pull-through technique. We evaluated the improvement in range of motion, pain, disability, and quality of life in the long term. Total extension deficit, quick disabilities of the arm, shoulder, and hand (QuickDASH), and EuroQol five dimensions-five levels index were systematically scored before each surgical intervention and reevaluated after 24 months. Results  Fourteen patients (26.9%) had already received a previous intervention (percutaneous needle aponeurotomy or collagenase Clostridium histolyticum ). The mean preoperative total active extension deficit was 84.0 ± 23.3 degrees (55-130 degrees). Mean follow-up was 36 months. There were no cases of tendon rupture or neurovascular injury. Total active extension deficit at the final follow-up was 3.4 ± 2.3 degrees (0-12 degrees). The mean active range of motion of the MCP and PIP joints were, respectively, 90.5 ± 3.3 degrees (85-96 degrees) and 82.7 ± 2.5 degrees (80-87 degrees). At 24 months after cord excision, a mean 10.7 points improvement in the QuickDASH questionnaire was registered ( p  < 0.001). Pull-through technique was equally effective both on patients with a primary or a recurrent disease. Eight patients (15.4%) had a recurrence of disease in the metacarpophalangeal joint or proximal interphalangeal joint. Conclusion  The pull-through technique is a simple, accessible, and effective technique for the treatment of Dupuytren\'s contracture. The use of palmar mini-incisions combined with minimal dissection has a low risk of iatrogenic injury to the neurovascular bundles and tendons, and has a low risk of recurrence rate. This study reflects level of evidence IV.

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown.
METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years).
RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001).
CONCLUSIONS: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through.
METHODS: III.

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).
CONCLUSIONS: 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.
METHODS: III.
UNASSIGNED: IRB14-00232.

BACKGROUND: Pull-through procedures for Hirschsprung disease (HD) can be performed during the Neonatal Intensive Care Unit (NICU) stay or delayed until discharge following home irrigations. This study assesses the safety of a delayed pull-through as an alternative to neonatal reconstruction in infants with successful abdomen decompression with home irrigations based on Hirschsprung-associated enterocolitis (HAEC) development.
METHODS: A single-institution retrospective review of neonates with HD who underwent delayed or neonatal pull-through from July 2018-July 2022. Endpoints included post-pull-through HAEC incidence, recurrence at an 18-month follow-up, time to the first HAEC episode, NICU length of stay (LOS), and HAEC-related LOS.
RESULTS: Twenty-four neonates were included. Eighteen were discharged from the NICU with home irrigations. Of these, 3 (28%) developed enterocolitis preoperatively, 12 (67%) underwent a delayed pull-through. NICU LOS in the delayed cohort was 3 times shorter than in the neonatal (6 vs. 18 days, p < 0.01). The incidence of enterocolitis (82% vs. 80%), time to the first episode (43 vs. 57 days), and HAEC-related LOS (median of 3 days) were similar.
CONCLUSIONS: Delayed HD pull-through is a viable neonatal reconstruction alternative that reduces NICU stay without increasing the risk of postoperative HAEC development.
METHODS: Original Research Article.
METHODS: III.