BACKGROUND: This study aimed to examine the characteristics of Hirschsprung\'s Disease (HD) in patients aged<18 who underwent surgical procedure at Dr. Zainoel Abidin (RSUDZA) General Hospital, Banda Aceh, Indonesia, between January 2010 and December 2020.
METHODS: This retrospective study collected and analyzed data from medical records of 18-yearold or younger children (n = 180) diagnosed with HD at RSUDZA. The surgical procedures included the Duhamel procedure, Soave procedure, the Soave Transanal Endorectal Pull-through (TEPT) procedure, and the Swenson TEPT procedure. Early outcomes of the surgery were then compared between males and females. The comparrative analysis was determined based on Chisquare analysis, where p< 0.05 was considered significant.
RESULTS: There were 111 (61.7%) male patients and 69 (38.3%) female patients, with a mean age of 15.2 months. The Soave TEPT is the most frequently performed procedure (91.7%). Emerging clinical manifestations include constipation (176; 97.8%) and soiling (171; 95%). Preoperative barium enema and postoperative pathological examination confirmed that almost all patients (99.4%) had an aganglionic segment confined to the rectosigmoid area. The average length of operation was 69.7 ± 65 minutes and average bleeding time was 5.4 ± 34 mL. The average discharge time was 3.3 ± 73.3 days. No significant difference was found in post-surgery complications between males and females (p<0.5). The immediate complications were not associated with surgical methods (p = 0.83).
CONCLUSIONS: Our descriptive study has suggested the Soave TEPT technique as appropriate to manage HD.

BACKGROUND: To comprehensively compare the effects of open Duhamel (OD), laparoscopic-assisted Duhamel (LD), transanal endorectal pull-through (TEPT), and laparoscopic-assisted endorectal pull-through (LEPT) in Hirschsprung disease.
METHODS: PubMed, Embase, Cochrane Library, Web of Science, CNKI, WanFang, and VIP were comprehensively searched up to August 4, 2022. The outcomes were operation-related indicators and complication-related indicators. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to evaluate the quality of evidence. Network plots, forest plots, league tables and rank probabilities were drawn for all outcomes. For measurement data, weighted mean differences (WMDs) and 95% credibility intervals (CrIs) were reported; for enumeration data, relative risks (RRs) and 95%CrIs were calculated.
RESULTS: Sixty-two studies of 4781 patients were included, with 2039 TEPT patients, 1669 LEPT patients, 951 OD patients and 122 LD patients. Intraoperative blood loss in the OD group was more than that in the LEPT group (pooled WMD = 44.00, 95%CrI: 27.33, 60.94). Patients lost more blood during TEPT versus LEPT (pooled WMD = 13.08, 95%CrI: 1.80, 24.30). In terms of intraoperative blood loss, LEPT was most likely to be the optimal procedure (79.76%). Patients undergoing OD had significantly longer gastrointestinal function recovery time, as compared with those undergoing LEPT (pooled WMD = 30.39, 95%CrI: 16.08, 44.94). The TEPT group had significantly longer gastrointestinal function recovery time than the LEPT group (pooled WMD = 11.49, 95%CrI: 0.96, 22.05). LEPT was most likely to be the best operation regarding gastrointestinal function recovery time (98.28%). Longer hospital stay was observed in patients with OD versus LEPT (pooled WMD = 5.24, 95%CrI: 2.98, 7.47). Hospital stay in the TEPT group was significantly longer than that in the LEPT group (pooled WMD = 1.99, 95%CrI: 0.37, 3.58). LEPT had the highest possibility to be the most effective operation with respect to hospital stay. The significantly reduced incidence of complications was found in the LEPT group versus the LD group (pooled RR = 0.24, 95%CrI: 0.12, 0.48). Compared with LEPT, OD was associated with a significantly increased incidence of complications (pooled RR = 5.10, 95%CrI: 3.48, 7.45). Patients undergoing TEPT had a significantly greater incidence of complications than those undergoing LEPT (pooled RR = 1.98, 95%CrI: 1.63, 2.42). For complications, LEPT is most likely to have the best effect (99.99%). Compared with the LEPT group, the OD group had a significantly increased incidence of anastomotic leakage (pooled RR = 5.35, 95%CrI: 1.45, 27.68). LEPT had the highest likelihood to be the best operation regarding anastomotic leakage (63.57%). The incidence of infection in the OD group was significantly higher than that in the LEPT group (pooled RR = 4.52, 95%CrI: 2.45, 8.84). The TEPT group had a significantly increased incidence of infection than the LEPT group (pooled RR = 1.87, 95%CrI: 1.13, 3.18). LEPT is most likely to be the best operation concerning infection (66.32%). Compared with LEPT, OD was associated with a significantly higher incidence of soiling (pooled RR = 1.91, 95%CrI: 1.16, 3.17). Patients with LEPT had the greatest likelihood not to develop soiling (86.16%). In contrast to LD, LEPT was significantly more effective in reducing the incidence of constipation (pooled RR = 0.39, 95%CrI: 0.15, 0.97). LEPT was most likely not to result in constipation (97.81%). LEPT was associated with a significantly lower incidence of Hirschprung-associated enterocolitis (HAEC) than LD (pooled RR = 0.34, 95%CrI: 0.13, 0.85). The OD group had a significantly higher incidence of HAEC than the LEPT group (pooled RR = 2.29, 95%CrI: 1.31, 4.0). The incidence of HAEC was significantly greater in the TEPT group versus the LEPT group (pooled RR = 1.74, 95%CrI: 1.24, 2.45). LEPT was most likely to be the optimal operation in terms of HAEC (98.76%).
CONCLUSIONS: LEPT may be a superior operation to OD, LD and TEPT in improving operation condition and complications, which might serve as a reference for Hirschsprung disease treatment.

Objective: To compare the Duhamel and transanal endorectal pull-through (TERPT) procedures in the treatment of children with Hirschsprung\'s disease.
METHODS: Studies comparing the Duhamel and TERPT procedures were included until 22 July 2023. R software (version 4.3.0) was used to perform the meta-analysis.
RESULTS: Ten studies with a sum of 496 patients were included. The length of postoperative hospital stay and incidence of postoperative constipation were longer and higher after the Duhamel procedure than the TERPT procedure (p < 0.0001 and p = 0.0041, respectively). The incidence of postoperative anastomotic stricture was higher after the TERPT procedure than the Duhamel procedure (p = 0.0015). No significant differences were found in the incidence of postoperative fecal continence, fecal incontinence/soiling, anastomotic leak, or ileus between these two procedures. The operation time seemed to be similar for both procedures, but it became longer for the Duhamel procedure than the TERPT procedure after sensitivity analysis. While the incidence of postoperative enterocolitis seemed to be higher after the TERPT procedure, it became similar for both procedures in the subgroup analysis.
CONCLUSIONS: The Duhamel procedure seems to be associated with a longer length of postoperative hospital stay, a higher incidence of postoperative constipation, and a lower incidence of postoperative anastomotic stricture than the TERPT procedure. However, the effect of these two procedures on the operation time and the incidence of postoperative enterocolitis remains unclear.

BACKGROUND: The aim of this study was to assess long-term outcomes of neonatal patients with Hirschsprung disease (HD) after single-stage transanal endorectal pull-through (TEPT) and to explore the predictive factors contributing to subnormal bowel function.
METHODS: Patients aged > 3 years operated for HD with TEPT during neonatal period between 2007 and 2019 answered the bowel function score (BFS) questionnaire. The patients were retrospectively divided into two groups according to whether they had normal bowel function. The clinical variables were compared between the subnormal and normal BFS groups. Univariate and multivariable logistic regression analysis were performed to identify the predictive factors contributing to subnormal bowel function.
RESULTS: A total of 160 children (71.7%) were included in this study, with mean follow-up time of 7.3 years (range 3.0-15.1 years). The level of aganglionosis were determined to be the short-segment (124/160, 77.5%), long-segment (33/160, 20.6%), and TCA (3/160, 1.9%). One hundred and thirty-four patients (83.8%) had a BFS ≥ 17, and 26 patients (16.2%) with subnormal bowel function (BFS < 17). Univariate and multivariate logistic regression analysis showed that level of aganglionosis with long-segment or TCA and postoperative hospital stay > 8.5 days were independent risk factors with OR of 3.213 (1.252, 8.246) and 3.813 (1.371, 10.606) for subnormal BFS, respectively.
CONCLUSIONS: Most HD patients who underwent one-stage TEPT in the neonatal period have favorable long-term results, and the level of aganglionosis with long-segment or TCA and long postoperative hospital stay may be closely related to subnormal bowel function.

OBJECTIVE: To evaluate the safety and efficacy of transanal endorectal pull-through (TEPT) and the long-term outcomes in newborns with Hirschsprung disease (HD).
METHODS: A total of 229 newborns with HD underwent one-stage TEPT between 2007 and 2020, and the diagnoses were confirmed by rectal biopsy. The perioperative clinical course for all patients was reviewed, and the postoperative short- and long-term outcomes were assessed.
RESULTS: A total of 229 neonates (187 male and 42 female) had a median age at TEPT of 17 days (range 6-28 days). Sixty-eight patients (29.7%) underwent TEPT combined with an abdominal approach or laparoscopy. Early postoperative complications (using the Clavien-Dindo grading system) were documented in 36 patients (15.7%), and late postoperative complications were noted in 9 patients (3.9%). The follow-up period in the remaining 165 children ranged from 1.2 to 14.0 years (median 5.0 years). A total of 106 of the patients older than four years old took part in an interview about bowel function, and 85 patients (80.2%) had bowel function scores (BFS) ≥ 18.
CONCLUSIONS: TEPT is effective and safe for HD in the neonatal period and presents with a low rate of complications and an acceptable outcome.

Hirschsprung\'s disease is a congenital anomaly in the distal part of the gastrointestinal tract, characterised by the absence of neuronal ganglion cells in the myenteric and submucosal plexus, causing the accumulation of faeces in the proximal portion. Dehiscence of coloanal anastomosis is one of the complications of Transanal Endorectal Pull-through (TEPT) surgery. This paper reports a case of Hirschsprung\'s disease in a six-year-old girl who experienced anastomotic dehiscence after undergoing TEPT. Due to post-TEPT anastomotic dehiscence, we performed a re-do pull-through with the modified Swenson-like method using a temporary stump. The stump is maintained for two weeks, then removed. On long-term evaluation, there was normal digestive function, no incontinence, and an excellent anal outcome. The modified Swenson-like pull-through with temporary stumps can be an alternative surgical technique for re-do pull-through after failed TEPT, with satisfactory results.
مرض هيرشسبرونغ هو شذوذ خلقي في الجزء البعيد من الجهاز الهضمي، ويتميز بغياب الخلايا العقدية العصبية في الضفيرة العضلية المعوية وتحت المخاطية، مما يتسبب في تراكم البراز في الجزء القريب. يعتبر تفكك مفاغرة القولون أحد مضاعفات جراحة السحب داخل المستقيم عبر الشرج (تيبت). تبلغ هذه الورقة عن حالة مرض هيرشسبرونغ في فتاة تبلغ من العمر ست سنوات عانت من إفراز تفاغر بعد خضوعها لجراحة تيبت. بسبب تفكك التفاغر بعد جراحة تيبت، أجرينا عملية سحب متكررة باستخدام طريقة شبيهة بسوينسون باستخدام جذع مؤقت. يتم الاحتفاظ بالجذع لمدة أسبوعين، ثم يتم إزالتها. في التقييم طويل الأمد، كانت هناك وظيفة هضمية طبيعية، ولا سلس بول، ونتائج شرجية ممتازة. يمكن أن تكون طريقة السحب المعدلة التي تشبه سوينسون مع جذوعها المؤقتة تقنية جراحية بديلة لإعادة السحب بعد فشل جراحة تيبت، مع نتائج مرضية.

BACKGROUND: Transanal endorectal pull-through (TERPT) is a common surgical procedure in Hirschsprung disease (HD). Aim of this study was to gain insight in the prevalence and severity of postoperative complications within 30-days after TERPT and to identify patient and perioperative characteristics, associated with the development of short-term postoperative complications.
METHODS: This study retrospectively analyzed data of children with HD and treated with TERPT in our center between 2005 and 2020. Complications emerging within 30-days after surgery were assessed using Clavien-Dindo (CD). Patient and perioperative characteristic as predictor of a complication were tested using (multivariable) logistic regression analysis.
RESULTS: Twenty-two of 106 (21%) included patients (17 transanal only; 77 laparoscopic-assisted; 12 laparotomy-assisted) developed 35 complications, including two patients (1.8%) that deceased. We suspect postoperative rectal irrigation leading to perforation as cause of death in both patients. Six patients (6%) had a minor (CD<3) and 16 patients (15%) a major (CD≥3) complication. Anastomotic leakage (n = 4, 11%), abdominal abscess (n = 3, 9%) and anastomotic stricture (n = 3,9%) occurred most frequently. Predictive factors for developing a complication were older age at time of surgery (OR 1.03 1.00-1.01, p = 0.041), laparotomy-assisted surgery (OR 12.65, CI 1.712-93.07, p = 0.013) and long-segment HD (OR 4.09 CI 1.09-15.39, p = 0.037).
CONCLUSIONS: We found a CD-graded short-term postoperative complication rate of 21% following TERPT, reporting anastomotic complications most frequently. In patients at risk a diverting stoma should be considered. We suspect postoperative rectal irrigation being the cause of two lethal perforations. Therefore, we recommend to place a rectal transanastomotic tube in all patients receiving TERPT.
METHODS: Level III.

OBJECTIVE: Few studies have focused on the operative age for Hirschsprung\'s disease (HD). We evaluated the optimal timing of surgery in HD patients based on their long-term bowel function.
METHODS: HD was diagnosed in 65 pediatric patients in our institute between 1992 and 2018. Twenty-five patients underwent the Soave-Denda procedure (SD) and 40 underwent transanal endorectal pull-through (TA). We divided these patients into two groups: those who underwent surgery at < 6 months of age (younger group) and those who underwent surgery at 6-12 months of age (older group). We assessed bowel function at 5, 7, and 9 years of age.
RESULTS: The bowel function of the patients who underwent the SD did not differ significantly between the groups. Similarly, the total bowel-function scores of the patients who underwent TA did not differ between the groups at any age. However, the soiling score at 7 years of age in the older group of patients who underwent TA was significantly lower than that in the younger group (p = 0.02).
CONCLUSIONS: Our data suggest that to achieve optimal bowel function, TA should be performed at < 6 months of age.

BACKGROUND: Transanal endorectal pull-through (TEPT) is considered the most preferable treatment method for Hirschsprung disease (HSCR) since it is less invasive and has fewer morbidities than transabdominal pull-through. Here, functional outcomes in short-segment HSCR patients after TEPT were assessed and associated with the prognostic factors.
METHODS: Krickenbeck classification was used to assess the functional outcomes in patients with HSCR after TEPT surgery at our institution from 2012 to 2020.
RESULTS: Fifty patients were involved in this study. Voluntary bowel movement (VBM) was achieved in 82% of subjects. Nine (18%) subjects had soiling grade 1, while two (4%) and two (4%) patients suffered constipation that was manageable with diet and laxative agents, respectively. Patients who underwent TEPT at ≥ 4 years old tended to have soiling more than patients who underwent TEPT at < 4 years old (OR = 16.47 [95% CI 0.9-301.61]; p = 0.06), whereas patients with post-operative complications had 10.5-fold higher risk for constipation than patients without post-operative complications (p = 0.037; 95% CI 1.15-95.92). Multivariate analysis showed male sex was significantly associated with VBM (OR = 9.25 [95% CI 1.34-63.77]; p = 0.024), while post-operative complications were strongly correlated with constipation (OR = 10 [95% CI 1.09-91.44]; p = 0.04).
CONCLUSIONS: The functional outcomes of HSCR patients after TEPT in our institution are considered relatively good. Moreover, the VBM, soiling, and constipation risk after TEPT might be affected by sex, age at TEPT performed, and post-operative complications, respectively, while the age at TEPT performed might not be associated with functional outcomes. Further multicenter studies with a larger sample size are necessary to clarify and confirm our findings.

UNASSIGNED: The growth of children with Hirschsprung disease (HSCR) can be affected by many factors, including the environment, nutrient intake, and surgery. Our study compared the long-term (i.e., at least 3 years of follow-up) growth outcomes in HSCR children after transabdominal Soave and Duhamel and transanal endorectal pull-through (TEPT) surgeries.
UNASSIGNED: A cross-sectional study was conducted in children <18 years of age diagnosed histopathologically with HSCR who underwent pull-through between January 1, 2012-December 31, 2015 in our institution. The postoperative anthropometric data were obtained prospectively through interviews during the outpatient clinic appointment or by telephone.
UNASSIGNED: We recruited 21 patients (Soave: 7 vs. Duhamel: 4 vs. TEPT: 10; p = 0.06). There were no significant differences between the three surgical methods in terms of preoperative and postoperative nutritional status categories (p = 0.52). Concerning the changes in nutritional status, after Soave surgery, it was improved, steady, and worsened in 28.6%, 57.1%, and 14.3% of the children, respectively. The nutritional status of the Duhamel group was worsened and steady in 25% and 75% of the children, respectively, while in the TEPT group, it was improved and steady in 40% and 60% of the children, respectively. However, these differences were not statistically significant (p = 0.42).
UNASSIGNED: While some HSCR children show an improvement in their nutritional status after Soave and TEPT procedures, the overall nutritional status is similar among different procedures. Further multicenter studies with a larger sample size are important to clarify our findings.