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Purpose: To investigate whether Leukotriene B4 receptor 2 (BLT-2), an upstream regulator of tight junction protein (TJP) Claudin-4, and TJPs could be etiologic factors in Hirschsprung-associated enterocolitis (HAEC) after pull-through (PT) for Hirschsprung disease (HD)., Methods: Normoganglionic colon (HD-N) and aganglionic rectum (HD-A) specimens from rectal/rectosigmoid (R/RS) or descending/transverse (D/T) HD were assessed using quantitative polymerase chain reaction (qPCR) for Occludin, TJP-1, TJP-2, Junctional adhesion molecule (JAM)-1, JAM-2, Claudin-1, Claudin-3, Claudin-4, and BLT-2 and immunoblotting for Claudin-4 using fresh specimens obtained intraoperatively (2021-2024; n = 17; R/RS = 15 and D/T = 2). Claudin-4 immunohistochemistry was also evaluated quantitatively using preserved (n = 29; R/RS = 20 and D/T = 9; 2009-2021) and fresh HD specimens for comparison with anorectal malformation patients having colostomy closure as controls (n = 42) and between HD-A versus HD-N, R/RS versus D/T, and HAEC (+) versus HAEC (-). Technically inadequate or transitional zone PT were excluded., Results: Subjects were 123 PT cases. Mean ages at PT/colostomy closure (years) were R/RS: 2.7 ± 2.9, D/T: 1.6 ± 2.2, and controls: 1.4 ± 0.7. Postoperative HAEC occurred 18 times in 14 PT cases (grade I = 5, grade II = 13). Post-PT HAEC was significantly more frequent in D/T (50.0% versus 6.4%; p < 0.001); Claudin-4 was significantly lower in HD-N from post-PT HAEC cases, especially D/T (p < 0.05) on immunohistochemistry. Claudin-4 was significantly lower in HD-N/HD-A compared with controls on immunoblotting (p < 0.05) and immunohistochemistry (p < 0.001). qPCR showed TJP-1, TJP-2, JAM-1, JAM-2, Claudin-4, and BLT-2 were significantly lower in HD-N/HD-A compared with controls., Conclusions: Lower Claudin-4 and BLT2 in post-PT HAEC HD-N (especially D/T) suggests generalized epithelial barrier derangement with possible etiologic implications for HAEC., Level of Evidence: Ⅱ., Competing Interests: Conflicts of interest The authors declare they have no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Colonic atresia (CA) is associated with Hirschsprung disease (HD) in up to 10% of cases. Therefore, some surgeons elect to complete proximal diversion at the initial operation. We sought to better define the incidence of concurrent HD and evaluate practice patterns regarding diversion for CA., Methods: The Pediatric Health Information System (PHIS) database was used to identify patients with CA from 2013 to 2022. Patients with small bowel atresia, anorectal malformation, gastroschisis, or first operation after 14 days of age were excluded. Index and subsequent operations were defined. Complications, time to enterostomy closure, and unplanned operations were evaluated., Results: HD was diagnosed in 8 (9.5%) patients and 7 of these were initially diverted. Diverted and anastomosed patients were demographically similar. In the 58 (69%) patients initially diverted, 19 (33%) had an ileostomy. Continuity was restored with an ileo-colic anastomosis in 63% of diverted and 27% of primarily anastomosed patients. Of those initially managed with a colostomy, 53% ultimately had a colo-colonic anastomosis. Patients with primary anastomoses had fewer operations and received more days of parenteral nutrition. Other outcomes did not vary., Conclusion: In a large population of infants with CA, 9.5% had concurrent HD. Almost 70% of CA patients underwent initial diversion and only one with HD had a primary anastomosis. Patients managed with a primary anastomosis were substantially more likely to retain the proximal colonic segment, but had a similar incidence of complications. When intraoperative colonic biopsies are obtained, primary anastomosis is a safe and effective strategy for CA., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Aim of the Study: We aimed to evaluate disease characteristics, associated malformations and surgical outcomes of congenital intestinal atresia., Methods: We identified all patients with jejunoileal (JIA) or colonic atresia (CA) treated at the Helsinki University Children's hospital during 1947-2019 and collected clinical data retrospectively from archived and electronic medical records., Results: Of the 180 included patients, 156 had JIA and 24 CA. Overall survival improved markedly from 34% during 1947-1977 to 93% during 1978-2019. Rate of primary anastomosis (81% in JIA, 21% in CA) remained unchanged while early surgical complications decreased (32% vs 18%, P = 0.04) and prematurity rate increased (21% vs 45%, P = 0.002) among JIA patients over time. Around half of patients had associated, mostly gastrointestinal malformations which occurred comparably in JIA and CA. During 1978-2019, 20 (21%) JIA patients, but none of CA patients, developed short bowel syndrome. Presence of type 3b atresia (n = 16) or gastroschisis (n = 14), but not the extent of intestinal resection or surgical complications, was associated with development of short bowel syndrome without decreasing survival. Shorter primary bowel resection without tapering surgery in JIA predisposed to repeated resections due to obstructive symptoms, and prolonged duration of parenteral nutrition., Conclusion: Survival of patients with JIA and CA has improved remarkably over time and is currently high despite frequent prematurity and associated malformations. In patients with JIA, apple peel atresia and gastroschisis predisposed to short bowel syndrome without jeopardizing high survival rates., Level of Evidence: IV., Competing Interests: Conflicts of interest Authors state no conflicts of interest., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Conflicts of interest The authors have no conflicts of interest to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population., Methods: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher's exact tests., Results: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005)., Conclusion: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE., Level of Evidence: III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Management of refractory constipation in children has not been standardized. We propose a protocolized approach which includes a contrast enema, anorectal manometry and exclusion of Hirschsprung disease (HD). For those without HD or with normal sphincters, an assessment of the colonic motility may be needed. The subgroups of dysmotility include (1) slow motility with contractions throughout, (2) segmental dysmotility (usually the sigmoid), or (3) a diffusely inert colon. We offered a Malone appendicostomy in all groups with the hope that this would avoid colonic resection in most cases., Methods: Patients with medically refractory constipation were reviewed at a single institution (2020 to 2021). For patients without HD or an anal sphincter problem, assessment of colonic motility using colonic manometry was performed followed by a Malone appendicostomy for antegrade flushes., Results: Of 196 patients evaluated for constipation refractory to medical management, 22 were felt to have a colonic motility cause. These patients underwent colonic manometry and Malone appendicostomy. 13 patients (59%) had a slow colon but with HAPCs throughout, 5 (23%) had segmental dysmotility, and 4 (18%) had a diffuse colonic dysmotility. 19 (86%) responded well to antegrade flushes with 17 reporting no soiling and 2 having occasional accidents. 3 patients (14%) failed flushes and underwent a colon resection within 6-month following Malone procedure., Conclusion: We propose a protocol for medically refractory constipation which provides a collaborative framework to standardize evaluation and management of these patients with antegrade flushes, which aids in avoidance of colonic resection in most cases., Level of Evidence: Level IV., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Abstract: We present an unusual case of total colonic aganglionosis with well-documented skip lesions and discuss our staged approach for diagnosis and surgical management. To date, there have been few reported cases of total colonic aganglionosis with skip areas. This type of presentation challenges the accepted theory regarding the etiology of colonic aganglionosis. Although skip lesions in Hirschsprung disease are extremely rare, their existence must be appreciated especially when a patient''s clinical and pathologic findings do not support classic Hirschsprung disease. If not considered, additional areas of aganglionosis can be missed at initial presentation, leading to a delay in definitive treatment. This case illustrates how careful mapping of bowel via multiple biopsies can identify and thereby preserve intervening segments of bowel with normal ganglions cells to yield the maximal amount of bowel possible. [Copyright &y& Elsevier]

Abstract: Purpose: This article is aimed at describing a series of 70 patients who underwent a redo pull-through in the period between January 1991 and January 2007 and to compare them with a series of consecutive patients with Hirschsprung disease who underwent a single pull-through. Methods: The notes were reviewed, and a comprehensive interview based on a disease-specific questionnaire for continence and quality of life was submitted to all eligible patients. We used a number of consecutive patients who underwent a single pull-through for Hirschsprung disease as control group. Results: Seventy patients were included. Residual aganglionosis in pulled-through bowel or retained aganglionic rectum represented the indications to reoperation in most cases. Intestinal obstruction represented the leading symptom in more than 60% of patients. We adopted either the endorectal pull-through, the Duhamel, or the Swenson procedure. Thirty-six patients were assessed for long-term outcome. One third experienced complications. Long-term continence was satisfactory in 70%. Enterocolitis, soiling, and perineal excoriations were complained by 30%. Excellent to good perspectives were described by 94%, and excellent to good cosmetic results by 22%. The only significant difference with control group (109 patients) involved cosmetic appearance, whereas soiling, although more frequent, could not be considered significantly increased. Conclusions: Patients who experience failure of a pull-through must go through multiple procedures to achieve definitive results. Although overall outcome does not significantly differ from that of a single effective pull-through, great efforts should be made to minimize complications and avoid the need for a redo, which requires experienced surgeons and highly committed families for a longer course of the disease. [Copyright &y& Elsevier]

Abstract: Background: Hirschsprung disease (HD) results from aganglionosis of the colon, is linked to acute and chronic enterocolitis (known as Hirschsprung-associated enterocolitis) despite successful corrective surgery, and can lead to bacteremia and even death. The genetic and molecular mechanisms underlying these disorders are largely unknown. Methods: We developed a microsurgical corrective pull-through procedure in mice, and applied that to Ednrb−/− mice, which manifest aganglionic megacolon that is very similar to HD. Wild-type littermates (Ednrb+/+) also underwent identical surgery. At prespecified time points postoperatively, mice were sacrificed, and histopathologic analyses of intestinal inflammation were performed. Mice of both genotypes were sacrificed after the postoperative recovery period to determine if corrective surgery itself caused inflammation. Stooling patterns were assessed as well to determine if intestinal function normalized after surgery. Results: There was no difference in histopathological enterocolitis scores after recovery from surgery. Stooling patterns in Ednrb−/− and Ednrb+/+ mice were similar postoperatively, suggesting normalization of intestinal function. However, with time, approximately 40% of Ednrb−/− mice developed clinical illness consistent with enterocolitis. No control (Ednrb+/+) mice developed clinical enterocolitis. Histopathological enterocolitis scores in the 40% of Ednrb−/− mice that developed clinical enterocolitis postoperatively were significantly worse than those of healthy postoperative Ednrb−/− mice. In contrast, none of the Ednrb+/+ control mice exhibited postoperative long-term inflammation. Conclusions: Microsurgical pull-through operation in Ednrb−/− mice produces a mouse model that closely resembles key features of Hirschsprung-associated enterocolitis, enabling controlled study of genetic and molecular mechanisms in Ednrb−/− mice and other genotypes that produce similar phenotypes. [Copyright &y& Elsevier]

Abstract: Caudal duplication syndrome is a very rare congenital deformity. A 13-year-old boy was born with duplicated colon-rectum and anus, diphallus, hydronephrosis of left kidney with megaureter, double bladders and urethras, and vertebral abnormalities. Multiple-stage correction was performed to remove the duplicated colon and the mucosa of the duplicated rectum. A new colon was reconstructed. The left kidney and megaureter were excised. The septum in the bladders was removed to convert the double bladders into a single bladder. The double phalluses were fused into a single penis. After these staged procedures, the boy is now living a normal life. [Copyright &y& Elsevier]

Abstract: Background: Many options exist in the surgical management of Hirschsprung''s disease (HD). To gain insight into contemporary management, we queried pediatric surgeons listed in the American Pediatric Surgical Association Directory on their management for the typical baby with HD. Methods: Surveys were sent electronically to the surgeons concerning a typical newborn diagnosed with HD. Questions included the preferred approach, number of stages, anastomotic technique, length of muscular rectal cuff, point of initiation of the anorectal dissection, and length of colonic resection. Surgeons performing laparoscopy were asked about how the colonic biopsy was performed. Other questions included the type of leveling colostomy, level of residents, and criteria for performing a primary transanal pull-through. The maximum margin of error was calculated using a 95% confidence interval based on the response percentages for discrete variables. Results: Surveys were sent to 719 surgeons with 270 responses. A minimally invasive approach is currently used by 80%, of which 42.3% favor laparoscopy and 37.7% prefer transanal dissection only. Only 5.4% of respondents prefer the Duhamel technique. A 1-stage approach is used by 85.6%. An average muscular cuff length of 2.4 cm (range, 0.5-6 cm) is reported. A divided muscular cuff is reported by 55%. On average, the anal anastomosis is 0.73 cm (range, 0-4.5 cm) above the top of the anal columns and 3.0 cm (0-12.5 cm) above the biopsy site on the ganglionic colon. Of the respondents using laparoscopy, 80.2% report using an intracorporeal colonic biopsy technique. Participation in a training program, either fellows and/or residents, is reported by 84.8% of respondents. The most common reason given for not performing a primary transanal pull-through is long segment disease (45.6%). Margin of error was no greater than 6% for any of the responses. Conclusions: A minimally invasive approach with a 1-stage operation has become the most common strategy for the surgical management of the typical baby with HD. Opinions vary about the amount of colonic resection, length of the rectal cuff, and site of initiation of the anorectal dissection, and these represent potential points for future studies. [Copyright &y& Elsevier]

Abstract: Background: Management guidelines with regard to congenital pouch colon (CPC) are not clearly defined with regard to the type of pouch present. The aim of this study was to outline the management strategy and surgical approach to CPC using the Saxena-Mathur classification based on anatomical morphology of the pouch. Methods: During a 12-year period (1995-2007), 426 patients were surgically managed for anorectal malformations at the RNT Medical College, Udaipur. Congenital pouch colon was documented in 80 patients and categorized into 5 types according the anatomical morphology. The management strategy depended upon the location of the pouch and its condition at the time of the surgery. Results: In type 1 and type 2 CPC, a 1-stage (pouch excision and pull-through) or 3-stage procedure (ileostomy, pouch-coloplasty with pull-through, and ileostomy closure) was performed depending on the condition of the pouch (ischemic or healthy). In type 3 and type 4 CPC, a 3-stage procedure (pouch excision with colostomy, pull-through, and colostomy closure) was performed in all patients. In type 5 CPC, a 3-stage procedure (distal pouch excision with proximal pouch-coloplasty with ileostomy, pull-through, and colostomy closure) was successful. Conclusion: Management of CPC patients according the Saxena-Mathur classification provides a well-defined algorithm in the surgical approach according to the anatomical morphology of the pouch. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The purpose of this report was to review the results of the antegrade continence enema (ACE) procedure and to compare the outcomes of right and left colon ACEs in children. Methods: Thirty patients who underwent an ACE between 1998 and 2005 were analyzed. Data were obtained based on the following parameters: postoperative soiling, catheter insertion time, colonic washout time, quality of life, and abdominal pain during and after the ACE. Twenty-nine patients were followed for an average of 3.8 years (range, 4 months-7.3 years). Results: Right colon ACEs were performed in 23 patients, and left colon ACEs were performed in 7 patients. The common complications of the ACE included abdominal pain during and after the ACE (51.7%) and stoma strictures (41.4%). The overall ACE success rate was 24/29 (82.8%; right colon ACE, 18/29; left colon ACE, 6/29). Twenty-three patients (95.8%) believed their quality of life was improved. There were no significant differences in complications or outcomes between the right and left ACEs. Conclusions: An ACE is an effective treatment for children with fecal incontinence. A left colon ACE has similar efficacy as a right colon ACE in managing fecal incontinence in children. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The study aimed to develop a mouse model of post-pullthrough Hirschsprung''s disease that will allow investigation of mechanisms that cause postoperative complications. Methods: We developed a novel microsurgical pullthrough operation on Balb/C mice and evaluated its effect on growth rate and stooling pattern. Histologic assessment of the pullthrough colon was performed. The pullthrough operation was then performed on Ednrb−/− mice that have aganglionic megacolon and Ednrb+/+ littermate controls, and the outcomes compared. Results: The Balb/C pullthrough group had 97% survival at 1 week and 70% survival at 2 weeks. Body weight of the pullthrough animals declined 15% in the first week after surgery and subsequently normalized. The stooling pattern showed consistently softer stools in the pullthrough group, but no difference in frequency compared to controls. Histopathologic analyses 4 weeks postoperatively showed well-healed coloanal anastomoses. Two-week survival after pullthrough surgery in Ednrb−/− and Ednrb+/+ mice was 50.0%, and 69.2%, respectively (P = NS). Increased mortality in the Ednrb−/− mice was related to the technical challenge of performing microsurgery on smaller-sized mice with poor baseline health status. Conclusions: Our microsurgical pullthrough operation in mice is feasible and allows systematic investigations into potential mechanisms mediating post-pullthrough complications and poor long-term results in mouse models of Hirschsprung''s disease. [Copyright &y& Elsevier]

Abstract: Purpose: A variety of procedures have been described for the treatment of total colonic aganglionosis (TCA); however, there is no consensus as to a superior operative method. The objective of this review is to evaluate the operative methods used to treat TCA and their effectiveness with respect to morbidity, mortality, rates of enterocolitis, and functional outcomes. Methods: We searched Medline (1950-2007) and the Cochrane Library for studies reporting the operative treatment of TCA. Search terms used were “aganglionosis,” “Hirschsprung''s disease,” “total colonic,” and “long-segment.” Studies evaluated were limited to those that solely discussed the operative outcomes for the treatment of TCA. A meta-analysis was then performed for morbidity outcomes in those studies describing a single operative procedure. Results: The search yielded 271 articles related to TCA. One hundred twenty-two articles discussed operative treatment of Hirschsprung''s disease, and only 26 of these articles discussed operative management of TCA. Nineteen articles were isolated for statistical analysis on the basis of results for perioperative outcomes for a single procedure. These procedures included Martin (4), colonic patch modifications (4), Duhamel (2), endorectal pull-through (1), Kimura (2), Boley (2), Rehbein (2), Soave (1), and J-pouch ileoanal anastomosis (1). Overall mortality was 1.9%. Meta-analysis revealed morbidity rates ranging from 10% to 64% depending on procedure category without significant outliers. Higher morbidity rates were found for the Martin-type procedure using the descending colon but with noted higher within-group variance due to small sample sizes. Enterocolitis was noted in 7 studies with a mean of 22%. Conclusions: There is no superior operative method for the treatment of TCA with respect to perioperative morbidity, mortality, enterocolitis, and functional outcomes. The operative technique performed should be selected on center and surgeon familiarity and expertise. [Copyright &y& Elsevier]

Abstract: Long gap esophageal atresia continues to be a therapeutic challenge for the pediatric surgeon. Although numerous methods have been described to achieve esophageal continuity in infants with esophageal atresia, esophageal replacement is often required if these methods fail. A common method of esophageal replacement in children is the use of a colon graft. Complications include cervical anastomotic leak, stricture, redundant intrathoracic colon with stasis, and cologastric reflux. We present an 11-year-old male with swallowing difficulties because of redundancy of the colon after undergoing colon interposition for long gap atresia. The patient underwent a successful transhiatal mobilization of the intrathoracic colon and stapled tapering coloplasty. The patient currently remains symptom-free. [Copyright &y& Elsevier]

Abstract: The author describes an extremely rare case of coexistence with imperforate anus, malrotation, and double zonal aganglionosis. A colostomy was performed on a 2-day-old male infant at the proximal sigmoid colon, because the finding of an invertgram revealed an intermediate type of imperforate anus. At the age of 1 month, a distal colostogram showed the low type without fistula. An upper gastrointestinal series revealed malrotation. At 4 months of age, Ladd''s procedure and an anoplasty were done. The function of the colostomy was not good. At laparotomy, a narrowing terminal ileum was removed for being a suspected intestinal obstruction. Histologic findings of the removed ileum revealed aganglionosis. A rectal suction biopsy showed the positive finding of acetyl cholinesterase staining. Therefore, the patient was diagnosed with extensive aganglionosis. At 9 months of age, a 1-stage ileoendorectal pull-through with a right colon onlay patch was performed. Histologically, a skipped ganglionic bowel segment at the right colon, a double zonal aganglionosis, was found among the extensive aganglionosis. Although the ganglionic right colon was used for the colon patch, which was placed for antiperistaltic movement, postoperatively the bowel function was excellent. The author herein describes an extremely rare case of coexistence of imperforate anus, intestinal malrotation, and double zonal Hirschsprung''s disease. A case with this association, to the author''s knowledge, has not been reported previously in the literature. [Copyright &y& Elsevier]

Abstract: Purpose: The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure. Methods: A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006. Results: We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group. Conclusion: Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The objective of this study is to assess the feasibility of primary posterior sagittal anorectoplasty in vestibular fistula without a covering colostomy. Methods: Girls presenting from July 1997 to July 2005 with vestibular fistula were included prospectively in the study, in a nonrandomized manner, after excluding those with megarectosigmoid and pouch colon. All underwent primary posterior sagittal anorectoplasty after total gut irrigation with normal saline. They were kept nil per oral until the fifth postoperative day. No patient was started on anal dilatation. Patients were assessed for immediate and delayed complications as well as voluntary bowel movements and continence. Results: A total of 72 patients with an age range of 1.5 months to 8 years (median, 9 months) were studied after excluding 7 with pouch colon and 3 with megarectosigmoid. Of the 72, 3 had undergone previous surgery. Follow-up ranged from 7 months to 8 years. No wound dehiscence or recurrence of fistula was noted. There were 5 mild wound infections. At 1 month postoperative, all patients had 1 to 3 stools per day with no episodes of soiling. None required anal dilatations, laxatives, or enemas. Conclusions: Primary posterior sagittal anorectoplasty in vestibular fistula can be performed without a covering colostomy provided fecal contamination of the wound can be kept to the minimum in the first postoperative week. We achieve this by thorough total gut irrigation preoperatively and keeping the child nil per oral for the first 5 postoperative days. Continence rates are excellent and postoperative constipation is unlikely if megarectosigmoid and pouch colon are ruled out before surgery. Anal dilatation is not required after surgery. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Primary pull-through for Hirschsprung disease has been reported to give comparable surgical outcomes to staged operations with less morbidity. The aim of this study was to review the early experiences in our institution with the primary pull-through option. Methods: Medical records of 50 consecutive patients with Hirschsprung disease who underwent a modified Duhamel pull-through operation from 2001 to 2004 were retrospectively reviewed. For analysis, cases were divided into 2 groups: primary pull-through (group I, 30 patients), and patients with a previously established colostomy who underwent a staged operation (group II, 20 patients). Comparisons were made regarding operating time, operative morbidity, and medium-term functional outcomes. Results: There was no difference between the groups with respect to median age and weight at time of surgery, sex ratio, presenting symptoms, and extent of aganglionosis. Seventy-four percent of the procedures were performed on infants (group I, 21 [70%] of 30 patients; group II, 16 [80%] of 20 patients). Acute postoperative complications were experienced by 4 (13.3%) of 30 patients in group I and 4 (20%) of 20 patients in group II (P <.05). Postoperative enterocolitis occurred in 11 (36.7%) of 30 patients in group I and in 7 (41.2%) of 17 patients in group II. Conclusions: Our experience indicates that the primary pull-through using a modified Duhamel technique gave neither difference in functional outcomes nor medium-term complications compared to a staged procedure. Considering a sparing of colostomy and a potential economic benefit, the procedure should be considered in selected patients. [Copyright &y& Elsevier]

Abstract: Purpose: Colostomy for patients with anorectal malformations decompresses an obstructed colon, avoids fecal contamination of the urinary tract, and protects a future perineal operation. The procedure is associated with several significant complications. Materials and Methods: The medical records of 1700 cases of anorectal malformations were retrospectively reviewed. A total of 230 patients underwent reconstruction without a colostomy. Of the remaining 1470 patients, 1420 had their colostomy performed at another institution (group A) and 50 did at our institution (group B) using a specific technique with separated stomas in the descending colon. Results: There were 616 complications identified in 464 patients of group A and in 4 patients in group B, an incidence of 33% vs 8% (P < .01). Complications in group A were classified into several groups. The first group was mislocation (282 cases), including 116 with stomas too close to each other, 97 with stomas located too distally in the rectosigmoid (which interfered with the pull-through), 30 with inverted stomas, 21 with stomas too far apart from each other, and 18 with right upper sigmoidostomies. The second largest group was prolapse (119 cases), which occurred mainly in mobile portions of the colon. The third group was composed of general surgical complications after colostomy closure (82 cases), such as intestinal obstruction (47 cases), wound infection (13 cases), incisional hernia (11 cases), anastomotic dehiscence (7 cases), sepsis (3 cases), and bleeding (1 case). Two of the septic patients died. Another group included 62 patients who received a Hartmann''s procedure, which we considered to be contraindicated in anorectal malformations. A total of 42 patients suffered from stenosis of the stoma; 29, from retraction. Conclusions: Most colostomy complications are preventable using separated stomas in the descending colon. Mislocated stomas lead to problems with appliance application, interference with the pull-through, megasigmoid, distal fecal impaction, and urinary tract infections. Loop colostomies lead to urinary tract infections, distal fecal impaction, and prolapse. Prolapse is a potentially dangerous complication that mostly occurs when the stoma is placed in a mobile portion of the colon. Recognizing this makes the complication preventable by trying to create colostomies in fixed portions of the colon or by fixing the bowel to the abdominal wall when necessary. The trend to avoid colostomies is justified; however, colostomy is the best way to prevent complications in anorectal surgery and, when indicated, should be done with a meticulous technique following strict rules to avoid complications. [Copyright &y& Elsevier]

Abstract: Purpose: The aim of this study was to evaluate postoperative anal function of high-type imperforate anus after laparoscopically assisted anorectoplasty (LAARP). Methods: Between 2000 and 2002, LAARP was performed in 13 patients with high-type imperforate anus. Clinical data of the LAARP group were compared with the posterior sagittal anorectoplasty (PSARP) group (n = 7) operated before 2000. All patients were treated with initial colostomy in the newborn period. After anorectoplasty, daily glycerin enemas were given for postoperative bowel management. Anorectal function of these patients was evaluated using the Kelly score and manometry at the age of 3 to 5 years (LAARP) and 5 to 6 years (PSARP). Results: Age at evaluation in the LAARP group was younger than that in the PSARP group (51 ± 10 vs 73 ± 12 months, P < .01). Kelly score was 3.8 ± 1.3 vs 3.4 ± 0.8, respectively. Resting pressure of internal sphincter was 31 ± 11 vs 33 ± 10 cm H2O, respectively. Relaxation reflex of the internal anal sphincter was observed in 62% (8/13) and 29% (2/7), respectively. Conclusions: Midterm follow-up study revealed that satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectoplasty may be a good alternative in this patient population. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP. [Copyright &y& Elsevier]

Abstract: Purpose: Gastrointestinal perforation is a catastrophic condition in neonates, especially in premature neonates. Although perforation is commonly observed in the small intestine during the neonatal period, perforation of the colon is a rare condition. This study analyzed the clinical findings and results of perforation of the colon in neonates. Methods: Between 1989 and 2004, 8 neonates were treated for spontaneous perforation of the colon at our institute. These patients were retrospectively reviewed. Results: Gestational ages ranged from 36 to 41 weeks. Seven patients weighed above 2500 g, whereas one patient weighed 1800 g at birth. Perforations developed within 7 days after birth in 6 patients and before birth in two. Associated bowel diseases included rectosigmoid type of Hirschsprung''s disease in two patients, immature ganglia in one, imperforate anus in one, colonic atresia in one, and necrotizing enterocolitis in one. An obvious cause was not identified in the remaining two. Six patients without definite anatomic obstructions, such as imperforate anus or colonic atresia, required evaluations for suspected Hirschsprung''s disease. All 8 patients underwent colostomy and recovered from peritonitis. Seven survived, but one died of sudden infant death syndrome. Conclusions: In this study, perforation of the colon during the neonatal period mostly occurred in term or near-term neonates and carried a good prognosis. During management, it was important to identify Hirschsprung''s and its allied disorders as a cause of perforation. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Colonic atresia occurs in 1:20,000 live births, offering a neonatal surgical challenge. Prenatal expression of fibroblast growth factor 10 (Fgf10), acting through fibroblast growth factor receptor 2b (Fgfr2b), is critical to the normal development of the colon. Invalidation of the Fgf10 pathway results in colonic atresia, inherited in an autosomal recessive pattern. Classically, disturbance of the mesenteric vasculature has been thought to cause many forms of intestinal atresia. The purpose of this study was to evaluate the role of vascular occlusion in the pathogenesis of colonic atresia. Methods: Wild type (Wt), Fgf10 −/−, and Fgfr2b −/− mutant mouse embryos were harvested from timed pregnant mothers. Immediately following harvest, filtered India ink was infused via intracardiac microinjection. The gastrointestinal tract was dissected, and photomicrographs of the mesenteric arterial anatomy were taken at key developmental time points. Results: Photomicrographs after India ink microinjections demonstrate normal, patent mesenteric cascades to the atretic colon at the time points corresponding to the failure of colonic development in the Fgf10 −/− and Fgfr2b −/− mutants. The mesenteric arterial anatomy of the colon demonstrates no difference between the Wt and mutant colonic atresia. Conclusions: The absence of embryonic expression of Fgf10 or its receptor Fgfr2b results in colonic atresia in mice. India ink microinjection is a direct measure of mesenteric arterial patency. Colonic atresia in the Fgf10 −/− and Fgfr2b −/− mutants occurs despite normal mesenteric vascular development. Thus the atresia is not the result of a mesenteric vascular occlusion. The patent colonic mesentery of the Fgf10 −/− and Fgfr2b −/− mutants challenges an accepted pathogenesis of intestinal atresia. Although colonic atresia can occur as a result of vascular occlusion, new evidence exists to suggest that a genetic mechanism may play a role in the pathogenesis of this disease. [Copyright &y& Elsevier]

Background/purpose: Distraction enterogenesis with intraluminal spring technology has been successfully used to lengthen segments of murine small intestine. We hypothesized that biocompatible springs could also be used to lengthen murine large intestine., Methods: Age and weight matched C57BL/6 mice underwent surgical insertion of nitinol spring-loaded capsules into the cecum. Segment lengths were measured at initial spring placement and at euthanasia after 7 and 14 days. Histologic adaptations were evaluated at scarification., Results: Cecal segments loaded with compressed springs lengthened an average of 150%, which was significantly longer than control segments loaded with either empty capsules or uncompressed springs. Muscularis layers tended to be thicker in the compressed spring groups compared to control groups., Conclusions: Insertion of a compressed nitinol spring into the cecum results in significant colonic lengthening in a mouse model. The ability to increase cecum length serves as proof of concept that distraction enterogenesis technology may be feasibly applied to large intestinal models. The use of distraction enterogenesis technology shows promise for application to clinical models in the treatment of pediatric intestinal disease., Competing Interests: Declaration of Competing Interest Dr. James C.Y. Dunn is the founder of Eclipse Regenesis, Inc. Remaining authors have no financial relationships relevant to this article to disclose., (Copyright © 2021. Published by Elsevier Inc.)

Background: Employing an institutional initiative to minimize variance in pediatric surgical care, we implemented a set of perioperative bundled interventions for all colorectal procedures to reduce surgical site infections (SSIs)., Methods: Implementation of a standard colon bundle at two children's hospitals began in December 2014. Subjects who underwent a colorectal procedure during the study period were analyzed. Demographics, outcomes, and complications were compared with Wilcoxon Rank-Sum, Chi-square and Fisher exact tests, as appropriate. Multivariable logistic regression was performed to assess the influence of time period (independent of protocol implementation) on the rate of subsequent infection., Results: One hundred and forty-five patients were identified (preprotocol=68, postprotocol= 77). Gender, diagnosis, procedure performed and wound classification were similar between groups. Superficial SSIs (21% vs. 8%, p=0.031) and readmission (16% vs. 4%, p=0.021) were significantly decreased following implementation of a colon bundle. Median hospital days, cost, reoperation, intraabdominal abscess, and anastomotic leak were unchanged before and after protocol implementation (all p > 0.05). Multivariable logistic regression found time period to be independent of SSIs (OR: 0.810, 95% CI: 0.576-1.140)., Conclusion: Implementation of a standard pediatric perioperative colon bundle can reduce superficial SSIs. Larger prospective studies are needed to evaluate the impact of colon bundles in reducing complications, hospital stay and cost., Level of Evidence: III - Retrospective cohort study., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: The outcome in HD has not been always satisfactory even after a technically sound operation., Purpose: To define the characteristic histopathological features of the pulled-through colon in patients with HD, and it is impact on clinical outcome., Patients and Methods: The study included patients with HD who underwent surgical repair between 2010 through 2016. The proximal margin of resected bowel segments (which corresponds to the pulled through colon) was subjected to detailed histopathological examination by two experienced pathologists. Based on the frequency of postoperative attacks of HAEC (fever, vomiting, abdominal distention, fluid offensive stools), cases included in the study were divided into two groups: Group A, those with less frequent attacks of HAEC; and Group B, those with recurrent attacks of HAEC (more than 3)., Results: The study included 35 patients (25 in group A; and 10 in group B). Their age ranged from 0.2 to 144 months (median 6 months). Comparing the histopathological findings in the two clinical groups, we have found that Group B (recurrent attacks of HAEC) had significantly more frequent focal disarray of nerve bundles and thicker nerve bundle diameter. Also, histopathological features of acute inflammation were more prevalent in examined specimens from group B., Conclusion: Several histopathological features of the examined bowel specimens in HD, other than presence or absence of ganglion cells, are indicative of postoperative functional outcome. These include the thickness and maturity of nerve bundles, in addition to the presence of histopathological features of acute inflammation., Level of Evidence: This is a case control study (level III evidence)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background and Objective: Several operating procedures have been reported for treating Hirschsprung Disease (HD), but the incidence of postoperative complications remains at a high level affecting both the patients' prognosis and postoperative life quality. Heart-shaped anastomosis as a novel surgery method for children with HD has been improved by our medical center. This new surgery method is characterized by a heart-shaped colorectal anastomosis after splitting the posterior rectum wall to 0.5 cm above the dentate line. The aim of this review was to research the outcomes and quality of life of laparoscope-assisted heart-shaped anastomosis (LHSA) for children with HD by comparing it with a more generally applied surgery method, the laparoscope-assisted Soave procedure (LSP)., Methods: A retrospective review was conducted for 198 patients who underwent operations from January 2005 to December 2014 in our institution, who were divided into 97 cases of the LHSA group and 101 cases of LSP group according to the treatment methods; all the outcomes and individual quality of life data were assessed and compared., Result: All the enrolled 198 children diagnosed with HD, LHSA and LSP had been successfully completed in all cases. For complications, the incidence of constipation and soiling in the LHSA group was lower than the LSP group (P = 0.030, P = 0.042 respectively). On aspects of quality of life after operation, the individual quality of life of children with fecal incontinence was scored; and the higher the scores, the better the quality of life. Patients in the LHSA group had higher scores in terms of soiling, unhappy or anxious and peer rejection than the LSP group (P = 0.003, P = 0.009, P = 0.021, respectively). Other surgical characteristics and outcomes did not significantly differ between the two groups., Conclusion: LHSA is a feasible and safe minimally invasive surgery method with good long-term follow-up outcomes for HD patients. Compared with LSP, LHSA has advantages of low incidence of constipation and soiling, and provides better quality of life. Therefore, LHSA may provide a better choice for HD patients., Levels of Evidence: The type of study was Clinical Research Paper and the level of evidence was level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To determine if there is a role for routine pouchogram before ileostomy reversal after IPAA in pediatric patients., Methods: The medical records of pediatric patients who underwent pouchogram between 2007 and 2017 prior to ileostomy reversal after IPAA at two affiliated hospitals were reviewed for concordance between exam under anesthesia (EUA) and pouchogram findings, management of abnormal pouchogram findings, and short and long-term outcomes after ileostomy reversal. Clinical notes were used to find patient-reported symptoms at the time of pouchogram., Results: Sixty patients (57% female) underwent pouchogram before planned ileostomy reversal. The median time from IPAA formation to pouchogram was 60.5 days (IQR: 46-77) and median follow-up was 4 years (IQR: 1-6). Fifty-seven patients (95%) were asymptomatic prior to reversal. Of the 40 asymptomatic patients with a normal EUA, pouchogram detected one stricture (3%), but reversal proceeded as planned. In the 16 patients with strictures on EUA, pouchogram only detected six (40%). One of 50 (2%) asymptomatic patients with normal pouchogram had anastomotic dehiscence found on EUA. Despite normal pouchogram and EUA, four asymptomatic patients required subsequent diversion for pouch-related complications between 13 and 60 months after ileostomy reversal. Three patients had pelvic pain prior to pouchogram; associated symptoms included perineal pain (n = 1) hematochezia (n = 1), and tenesmus (n = 1). EUA and pouchogram were concordant in two patients (n = 1 anastomotic complication, n = 1 pouch septum) and ileostomy reversal was delayed. In the remaining symptomatic patient, pouchogram detected an anastomotic leak where EUA detected only a stricture, and this prompted a delay in reversal. Long term, none of these patients required diversion or excision of their pouch., Conclusion: Routine pouchogram in asymptomatic pediatric patients does not change management and can be omitted, thereby sparing patients discomfort and unnecessary radiation exposure. Pouchogram may have diagnostic value in symptomatic patients., Level of Evidence: III., Type of Study: Study of Diagnostic Test., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Since we have started colonic replacement of the esophagus in children, several successive modifications have led to evolution of the surgical technique aiming to decrease complications and achieve the best functional outcome. Currently, our surgical group has reached a single standardized technique that has been applied to all cases included in this report. A colonic flap (including the transverse colon) is prepared based on the left colic vessels. The proximal end of the colonic flap is transferred upwards passing at first behind the stomach and then anteriorly through the lesser omentum to traverse the thoracic cavity via a previously prepared retrosternal tunnel. The proximal end of the colonic flap is anastomosed to the proximal esophagus in the neck (end to end anastomosis). Most specifically, the distal end of the colonic flap is anastomosed to the posterior wall of the body of the stomach in order to prevent regurgitation of gastric contents into the colonic flap. The retro-sternal colon bypass is among the successful options to replace the esophagus in children. Adding a posterior cologastric anastomosis to the procedure greatly prevents gastric regurgitation that can be responsible for short- and long-term complications. LEVEL OF EVIDENCE: This is a case series with no comparison group (level IV evidence)., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: 'Intractable constipation' (IC) is constipation unresponsive to 3 months of optimal conventional treatment. Colonic manometry (CM) is recommended as a diagnostic modality for evaluation of these children. This study aimed to review outcomes of children with IC who were managed surgically at a single tertiary care center., Methods: Records of children with IC who were treated with ACE (antegrade colonic enema), colostomy, or ileostomy (2006-2018) were reviewed. "Success" was defined as adequate evacuation without need for further unplanned surgery. Data are median (range)., Results: Sixty-seven children underwent surgery, of whom 56 with preoperative CM were included. Age at surgery was 8.6 (3.3-15.1) years. Eight had normal manometry and underwent ACE with 75% success. Thirty-six had left-sided dysfunction and underwent ACE (18), colostomy (14) or ileostomy (4) as initial intervention with 61, 70, and 100% success. Twelve with pancolonic dysfunction underwent ACE (6) or ileostomy (6) with 60 and 100% success. Twenty underwent repeat manometry 2.2 years (10 months-7.6 years) after surgery. Of 18 with stoma, 8 had resolution or improvement and of these, 7 underwent stoma reversal with a simultaneous ACE. Two patients with ACE had improvement, 1 is still on ACE washouts, and 1 is off all treatment. Ten with persistent dysfunction remain diverted. At 3.2 years (4 months-9.9 years) follow-up, 18 remain on ACE washouts, 9 have colostomy, 19 ileostomy, and 10 are off treatment and doing well., Conclusion: We present a large series of patients who were surgically managed for IC. CM may guide therapy in these children., Type of Study: Retrospective comparative study LEVEL OF EVIDENCE: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: At the severe end of the idiopathic constipation spectrum exist patients with chronic idiopathic constipation associated with an enormous megarectosigmoid, among whom few require surgery. We performed transanal proximal rectosigmoidectomy, involving preservation of a 5 cm rectal reservoir, to ameliorate inconsistent and unpredictable outcomes and fecal incontinence occurring in some patients operated on with other techniques., Methods: We retrospectively observed patients with chronic idiopathic constipation with megarectosigmoid who underwent transanal proximal rectosigmoidectomy during July 2017-May 2018. We analyzed sex, age, operation indication, complications, functional outcome, and time of follow-up. We statistically compared the dosage of laxative before and after the procedure., Results: Thirteen patients were included in the study. Median age during surgery was 8 years. In 9 cases, the indication for surgery was chronic intake of a daily high dose of Senna with failed weaning trials, and 4 had persistent fecal impaction with laxative-intolerance and refusal of rectal enemas. No intra/postoperative complications occurred. Currently, all 13 patients have daily voluntary bowel movements and no fecal accidents. Laxative dosage was significantly reduced (p = 0.007). Follow-up ranged 6-16 months., Conclusion: Preliminary results suggest transanal proximal rectosigmoidectomy as an adequate alternative for patients requiring surgery for chronic idiopathic constipation with megarectosigmoid., Type of Study: Clinical research., Level of Evidence: Level III., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)