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.

BACKGROUND: Diagnostic delay in Hirschsprung disease is uncommon. Different definitions have been proposed but that of a diagnosis achieved after 12 months of age seems to be the most reliable and resorted to. Some authors reported a worse outcome in case of delay. Our study aims at providing the most relevant features of a series of patients who received a delayed diagnosis of Hirschsprung disease.
METHODS: All consecutive patients admitted to our Center with a delayed diagnosis of Hirschsprung diseases between January 2017 and July 2023 have been retrospectively enrolled. Demographic data, phenotype, genotype, surgical complications, and outcome were assessed and compared to those of literature. A number of variables were also compared to those of a series of patients admitted during the same study period without a delayed diagnosis.
RESULTS: A total of 45 patients were included (16.4% out of a series of 346 patients with data regarding age at diagnosis). Male to female ratio was 3.1:1. Median age at diagnosis was 41 months with a wide variation (range between 17 months and 58 years). All patients but 2 suffered from classic rectosigmoid aganglionosis. Normal meconium passage (58%) was reported in a significantly higher number of patients compared to what observed in a series without diagnostic delay (p = 0.0140). All other variables (associated anomalies, preoperative enterocolitis, complications, and functional outcome) proved not to have statistically significant differences compared to a series of patients without a diagnostic delay.
CONCLUSIONS: The results of our study underline that a significant percentage of patients are basically missed in the neonatal period mostly due to mild symptoms. Overall outcome does not differ from that of patients without diagnostic delay. Nonetheless, we underline the importance of a throughout investigation of all patients with meconium delay/failure and that of adopting a low threshold for performing rectal suction biopsies in constipated children to avoid misdiagnosis to serve the best for our patients.

OBJECTIVE: Compromised swallowing, speaking, and local complications are the major disadvantages of established approaches to the posterior tongue and oropharynx. The mandibular split involves an esthetically unpleasant bipartition of the lower lip and is prone to bony non-union or sequestration. The conventional pull-through technique on the other hand lacks the secure reattachment of the lingually released soft tissues.
METHODS: The feasibility of a new modified pull-through approach was tested on three anatomical specimens. CAD/CAM cutting guides were used to design a retentive bone flap to properly refixate the genioglossus and geniohyoid muscles after the procedure. The radiographic assessment and treatment planning was performed on 12 cadavers. The entire procedure was tested surgically via dissection in three of those cases. This procedure was then applied in a clinical case.
RESULTS: Precise repositioning and dynamic compression of bony segments was possible reproducibly and without injury to adjacent structures. In all dissected cases, a median lingual foramen was found and in two cases vessels entering it could be dissected Radiologic anatomical landmarks were sufficient in all 12 cases to perform the clinical planning procedure. Clinically, the osteotomized segment demonstrated good blood supply and plateless repositioning was verified postoperatively via cone beam scan.
CONCLUSIONS: The method presented is safe and easy to perform. Individual cutting guides improve the safety and accuracy of the procedure, potentially eliminating the need for osteosynthesis. We provide the anatomical and radiologic basis for clinical evaluation of this pedicled bone flap procedure and present the clinical application of this modified pull-through approach.

OBJECTIVE: Inflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing.
METHODS: A single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull-through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30-days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6-months of ERPT.
RESULTS: Forty-nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT.
CONCLUSIONS: Our study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull-through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.