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.

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: A considerable step forward in low rectal cancer resection has been done in the last decades. Maintaining total mesorectal excision as the gold standard treatment, new techniques such as Trans-Anal Mini-Invasive Surgery (TAMIS) and Trans-Anal Total Mesorectal Excision (TATME), which have been added to improve skills in laparoscopic and robotic surgery, currently represent the advancement of this procedure. Despite improvements in surgical techniques, complications after low anterior resection for rectal cancer still remain a challenge. Drainage and colostomy are the main treatments used to overcome the problem caused by anastomosis failure, and most patients will never been restored. Different techniques of redo surgery could be proposed to deal complex cases, although remaining high risk procedures.
METHODS: We present two clinical cases with a late complication of the colorectal anastomosis: one with a late leakage of low colorectal anastomoses, treated with Hartman procedure, that developed a pelvic chronic sinus; the another one with complete anastomotic disruption after massive suture bleeding; both treated with delayed pull-through anastomosis, according to Turnbull-Cutait technique. We also made a review of relative literature, in order to back our therapeutic iters.
CONCLUSIONS: Both the procedures were carried out satisfactorily, with restoration of intestinal continuity and good anastomotic result. It allows the resolutions of the chronic sepsis caused by the pelvic sinus and maintenance of intestinal continuity with a good Wexner incontinence score. Literary review demonstrated that this procedure still remains undervalued and not widely exploited.
CONCLUSIONS: Delayed pull-through coloanal anastomosis could be considered as a valid option, in order to preserve intestinal continuity in septic or complicated low colorectal anastomosis.

Theoretical advantages of Turnbull-Cutait pull-through delayed coloanal anastomosis (DCAA) are a reduced risk of anastomotic leak and therefore avoidance of stoma. Gradually abandoned in favor of immediate coloanal anastomosis (ICAA) with diverting stoma, DCAA has regained popularity in recent years in reconstructive surgery for low RC, especially when combined with minimally invasive surgery (MIS). The aim of this study was to perform the first meta-analysis, exploring the safety and outcomes of DCAA compared to ICAA with protective stoma.
A systematic search of MEDLINE, EMBASE, and CENTRAL and Google Scholar databases was performed for studies published from January 2000 until December 2020. The systematic review and meta-analysis were performed according to the Cochrane Handbook for Systematic Review on Interventions recommendations and Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines.
Out of 2626 studies screened, 9 were included in the systematic review and 4 studies in the meta-analysis. Outcomes included were postoperative complications, pelvic sepsis and risk of definitive stoma. Considering postoperative complications classified as Clavien-Dindo III, no significant difference existed in the rate of postoperative morbidity between DCAA and ICAA (13% versus 21%; OR 1.17; 95% CI 0.38-3.62; p = 0.78; I2 = 20%). Patients in the DCAA group experienced a lower rate of postoperative pelvic sepsis compared with patients undergoing ICAA with diverting stoma (7% versus 14%; OR 0.37; 95% CI 0.16-0.85; p = 0.02; I2 = 0%). The risk of definitive stoma was comparable between the two groups (2% versus 2% OR 0.77; 95% CI 0.15-3.85; p = 0.75; I2 = 0%).
According to the limited current evidence, DCAA is associated with a significant decrease in pelvic sepsis. Further prospective trials focusing on oncologic and functional outcomes are needed.

The majority of children who undergo operative management for Hirschsprung disease have favorable results. A subset of patients, however, have long-term dysfunctional stooling, characterized by either frequent soiling or obstructive symptoms. The evaluation and management of a child with poor function after pull-through for Hirschsprung disease should be conducted by an experienced multidisciplinary team. A systematic workup is focused on detecting pathologic and anatomic causes of pull-through dysfunction. This includes an exam under anesthesia, pathologic confirmation including a repeat biopsy, and a contrast enema, with additional studies depending on the suspected etiology. Obstructive symptoms may be due to technique-specific types of mechanical obstruction, histopathologic obstruction, or dysmotility-each of which may benefit from reoperative surgery. The causes of soiling symptoms include loss of the dentate line and damage to the anal sphincter, which generally do not benefit from revision of the pull-through, and pseudo-incontinence, which may reveal underlying obstruction. A thorough understanding of the types of complications associated with various pull-through techniques aids in the evaluation of a child with postoperative dysfunction. Treatment is specifically tailored to the patient, guided by the etiology of the patient\'s symptoms, with options ranging from bowel management to redo pull-through procedure. This review details the workup and management of patients with complications after pull-through, with a focus on the perioperative management and technical considerations for those who require reoperation.

OBJECTIVE: Anorectal malformation (ARM) is a rare congenital disorder of the anus and rectum. In the last 30 years virtually all patients born with ARM have survived and surgeons from adult care may be called to deal with new and long-term sequelae, including tumors of the pulled-through anorectum. Two new cases of colorectal carcinoma in young adults born with ARM and a review of the literature is reported to emphasize the importance of a multidisciplinary follow-up.
METHODS: A man and a woman, with previous history of ARM, presented at 34 years of age with symptoms of intestinal occlusion and a large pelvic mass. Both patients had no familial history of colorectal carcinoma.
RESULTS: The patients underwent biopsies (mucinous rectal adenocarcinoma) and stadiation (T4N0M0). In one case the microsatellite instability showed a stable profile. Despite maximal treatments, including surgery, chemo- and radio-therapy, they both died a few years after diagnosis for progression of disease.
CONCLUSIONS: Case studies are too limited to suggest guidelines for prevention and treatment of such complications, but the life-long follow-up is mandatory in the framework of a well-established network between pediatric and adult surgeons. The risk of tumor development in these patients should not be neglected and colleagues from adult care should be aware of the possibility this occurs in their practice.

BACKGROUND: The transanal endorectal pull-through (TERPT) procedure, the latest advancement in the surgical treatment of Hirschsprung\'s disease, has replaced most other surgical techniques in the last decade.
METHODS: Between October 2002 and March 2014, a total of 22 patients diagnosed with Hirschsprung\'s disease underwent a one-stage TERPT operation.
RESULTS: Resected segments included the rectosigmoid (seven patients), the descending colon (10 patients), and the transverse colon (five patients). The minimum length of the resected segments was 15 cm and the maximum length was 65 cm. The mean length was 39.18 ± 12.05 cm. Following surgery, the start of oral ingestion was 1-8 days (mean 3 ± 1.69 days) and the hospital stay after the operation lasted 4-11 days (mean 7.04 ± 2.05 days). The mean follow-up period was 48 ± 6 months (range of 24-166 months). Out of 22 patients, three patients had an anal stricture, which responded to anal dilatations; three patients had an enterocolitis episode that required hospitalization; two patients experienced constipation; and two patients had incontinence/soiling.
CONCLUSIONS: Our data suggest that the TERPT operation can be safely performed in terms of long-term complications.

OBJECTIVE: To compare outcomes following totally transanal endorectal pull-through (TTERPT) versus pull-through with any form of laparoscopic assistance (LAPT) for infants with uncomplicated Hirschsprung\'s disease.
METHODS: Systematic review and meta-analysis.
METHODS: Five hospitals with a paediatric surgical service.
METHODS: 405 infants with uncomplicated Hirschsprung\'s disease.
METHODS: TTERPT versus LAPT.
METHODS:
METHODS: mortality, postoperative enterocolitis, faecal incontinence, constipation, unplanned laparotomy or stoma formation, and injury to abdominal viscera.
RESULTS: Haemorrhage requiring transfusion of blood products, abscess formation, intestinal obstruction, intestinal ischaemia, enteric fistula formation, urinary incontinence or retention, impotency and duration of procedure.
RESULTS: Five eligible studies comprising 405 patients were identified from 2107 studies. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. Operative duration was 50.29 min shorter with TTERPT (95% CI 39.83 to 60.74, p<0.00001). There were no significant differences identified between TTERPT and LAPT for incidence of postoperative enterocolitis (OR=0.78, 95% CI 0.44 to 1.38, p=0.39), faecal incontinence (OR=0.44, 95% CI 0.09 to 2.20, p=0.32) or constipation (OR=0.84, 95% CI 0.32 to 2.17, p=0.71).
CONCLUSIONS: This meta-analysis did not find any evidence to suggest a higher rate of enterocolitis, incontinence or constipation following TTERPT compared with LAPT. Further long-term comparative studies and multicentre data pooling are needed to determine whether a purely transanal approach offers any advantages over a laparoscopically assisted approach to rectosigmoid Hirschsprung\'s disease.
BACKGROUND: PROSPERO registry- CRD42013005698.