Early post-operative fever after colorectal surgery in infants is common and rarely associated with infection.

PURPOSE: Early post-operative fever (< 48 h) is common in adults and children and seldom indicative of an infection. Guidance to limit excessive evaluation in adults is well-characterized but similar studies for the pediatric population is scarce. This study was performed to better clarify which infants should undergo investigation for post-operative fever after colorectal reconstructive surgical procedures. METHODS: We performed a retrospective chart review of all infants under one year of age who underwent elective reconstruction for anorectal malformations (ARM) and Hirschsprung Disease (HD) between June 2018 and April 2020 at a single institution. Patient and perioperative characteristics were analyzed to evaluate for possible factors associated with infection. RESULTS: Sixty-eight infants met study criteria - 38 (55.9%) had HD and 30 (44.1%) had ARM. Twenty-two infants (32.4%) had early post-operative fever. A definitive infectious cause was identified in only two infants. The presence of a colostomy pre-operatively and longer operative times were associated with increased risk of post-operative fever (62.5% vs. 22.7% and 175 min vs. 150 min respectively, p < 0.05). CONCLUSION: Early post-operative fever in infants after colorectal surgery is common and rarely associated with an infection. Further research is needed to determine which infants require further work-up and which can be safely observed.

Hirschsprung disease associated enterocolitis: a systematic review and meta-analysis.

INTRODUCTION: Hirschsprung's disease-associated enterocolitis (HAEC) remains a substantial morbidity and mortality risk in Hirschsprung's disease. HAEC is a challenge. Its p athophysiology is still a mystery, and no adequate treatment strategy exists. The aim of the study is to analyse the pre-and post-operative, mortality and complications: strictures, anastomotic leak, constipation and incontinence associated with HAEC. MATERIALS AND METHODS: Adjust to the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) 2020; this study met all the requirements and was up-todate. The search approach was online publications between 2013 and 2023 in Pubmed and SagePub. It was decided not to consider review pieces that had already been published and half done. The STATA 18th version was used for metaanalysis. RESULTS: Our search results included 370 PubMed and 149 SagePub articles. Since 2013, 134 PubMed and nine SagePub articles have been obtained, and seven studies have met the criteria. CONCLUSION: Disorders of intestinal motility in the aganglionic segment and accumulation of faeces disrupt the balanced microbiota population, which are factors of preoperative HAEC. Major congenital anomalies and low birth weight worsen pre-operative HAEC. Pre-operative HAEC can continue and affect the post-operative. Constipation and fecal incontinence are still the main challenges after HSCR surgery.

Can the diameter of nerve fibers be effectively utilized to enhance the accuracy of determining the length of the aganglionic segment compared to intraoperative biopsy in patients with Hirschsprung's disease?

OBJECTIVE: The aim of this study is to investigate the accuracy of utilizing neural fiber trunk diameter in accurately diagnosing the length of the aganglionic segment in patients definitively diagnosed with Hirschsprung's disease. RESULTS: In this study, 40 patients (19 males, 21 females; mean age 2.5 ± 2.2646 years) were assessed for Hirschsprung's disease. Constipation was the main symptom (75%), followed by abdominal issues. All underwent contrast enema and rectal suction biopsy for diagnosis, followed by surgery (predominantly Soave and Swensen techniques). Majority (85%) had rectosigmoid involvement. Neural fiber diameter was measured, with 52.5% ≤40 µm and 47.5% >40 µm. Statistical analysis showed 40% sensitivity(CI:95%) and 47% specificity(CI:95%) with a cutoff of 40.5 µm. Cohen's kappa index for aganglionic segment size was 0.7.

Assessing fecal load with ultrasound in children with colorectal pathology: ReKiSo study.

PURPOSE: To evaluate bowel management for children with colorectal pathology by measuring transverse rectal diameter (TRD) and assessing fecal load with transabdominal rectal ultrasound (TRU). METHODS: Prospective case-control study of children receiving bowel management (BM) between 04/2023 and 04/2024 was done. There was inclusion of patients with Hirschsprung disease (HD), anorectal malformation (ARM) and functional constipation (FC). Patients with other congenital or neurological conditions were excluded. Control group consisted of inpatients and outpatients without abdominal complaints. FC was diagnosed according to ROM-IV-criteria. For HD and ARM, we followed a list of symptoms. To assess fecal load, we visualized the TRD using the Klijn (Klijn et al. in J Urol 172:1986-1988, 2004) method. The bladder was moderately full. The fecal load was assessed retrograde from the rectum. Follow-up was at 1/3/6 months. Secondary data were collected from medical records. Sample size calculated a priori and follow-up group with new gathered data. RESULTS: p value for TRD in all groups significant with p < 0.05 and in grouped follow-up. CONCLUSION: Ultrasound is a useful tool for assessing fecal load and helps diagnose constipation and monitor BM. Irrespective of colorectal pathology, a cut-off of 3 cm seems to discriminate between children without constipation/overload symptoms and asymptomatic patients. We present a radiation-free method for monitoring bowel management.

A multi-center cross-sectional comparison of parent-reported quality of life and bowel function between anorectal malformation and Hirschsprung's disease patients with versus those without Down syndrome.

PURPOSE: Down syndrome (DS) is a common abnormality associated with anorectal malformation (ARM) and Hirschsprung's disease (HD). However, quality of life (QOL) in ARM and HD patients with DS is under-researched. This study compares parent-reported QOL and bowel function in ARM and HD patients with DS to those without. METHODS: Between December 2020 to February 2023, parents of ARM and HD patients with and without DS aged 3-17 years who had undergone surgery > 12 months prior at four tertiary referral centers were recruited. We used the Pediatric Quality of Life Inventory™ (PedsQL™) Generic Core Scales, General Well-Being (GWB) Scale and Family Impact (FI) Module questionnaires, and the Rintala bowel function score (BFS). RESULTS: There were 101 ARM, 9 (8.9%) of whom had DS; and 87 HD, of whom 6 (6.9%) had DS. Parent-reported Core scores in ARM and HD with DS were comparable to those without DS. However, ARM and HD with DS had worse scores in the FI Module and bowel function than those without DS. CONCLUSION: Although parent-reported QOL in ARM and HD with DS is similar to those without DS, family impact and BFS are worse. Our findings are limited by small sample size in proportion of DS patients.

Enough is enough: how many rectal suction biopsies do you need to diagnose Hirschsprung's disease?

PURPOSE: Rectal suction biopsy (RSB) is the gold standard for diagnosing Hirschsprung's disease (HD) in infants. Despite being a common procedure, no standard exists on the number of biopsy specimens and their respective level within the rectum. METHODS: We conducted a retrospective review of epidemiological and pathological data of patients who underwent RSB at our institution between January 2011 and May 2022. During RSB we obtain 4 specimens: at 1 cm, 3 cm and 5 cm above the dentate line, besides one specimen at the dentate line. We used a logistic regression model for statistical analysis and included control variables (e.g. underlying disease, weight at first biopsy, gestational age). RESULTS: A total of 92 patients underwent 115 biopsies, with an average of 3.77 specimens per session. Of the specimens taken at 1 cm above the dentate line 73.9% were conclusive, at 3 cm 75.9% and at 5 cm 79.2%. Specimens taken at the dentate line were squamous or transitional epithelia in 31.5% and therefore of no use for HD diagnostics. The specimen at 3 cm shows the highest discriminative power whether the biopsy session was diagnostic (p-value < 1%). CONCLUSIONS: We propose that a total of three specimens, namely one at 1 cm, one at 3 cm and one at 5 cm above the dentate line, is enough to diagnose or exclude HD.

Mowat-Wilson syndrome: Case report.

BACKGROUND: Mowat-Wilson syndrome (MWS) is a rare genetic condition resulting in multiple congenital anomalies, including facial dysmorphism, structural anomalies of the internal organs, functional disorders, and, although less commonly, ocular abnormalities. To present a child with MWS and eye abnormalities. METHODS: A 3-year-old boy was born at 37 weeks of pregnancy with dysmorphic features, neurodevelopmental disorders, genetically confirmed MWS, nystagmus, strabismus, and suspicion of congenital glaucoma. Ophthalmic examination was carried out under general anesthesia; eyeball ultrasound and electrophysiological examination (flash visual evoked potentials) were also performed. RESULTS: The examinations revealed nystagmus, a normal response of pupils to light in both eyes, and normal intraocular pressure, that is, 17 and 18 mm Hg in the right and left eye, respectively. Corneal thickness was 606 µm in the right eye and 588 µm in the left eye. Gonioscopy revealed displacement of Schwalbe line anterior to the limbus of the cornea (posterior embryotoxon). Fundus examination revealed a pink optic disk with a cup-to-disc ratio of 0.5, macular pigment regrouping, and normal blood vessels. Flash visual evoked potentials: P2 latency was normal. P2 amplitude from the left hemisphere was reduced to 50%, and P2 amplitude over the right hemisphere was normal. CONCLUSION: Children with genetically determined congenital anomalies need regular ophthalmic checkups to accurately assess the eye and determine the prospects of vision function development.

Early intervention in Hirschsprung's disease: effects on enterocolitis and surgical outcomes.

BACKGROUND: The timing of surgical intervention for Hirschsprung's disease (HSCR) has been a topic of continued discussion. The objective of this study was to evaluate the significance of age at surgery in the management of HSCR by conducting a comparative analysis of the correlation between surgical age and midterm outcomes. METHODS: We conducted a retrospective analysis of children with HSCR who underwent one-stage laparoscopic assisted pull-through surgery with modified Swenson technology at our hospital between 2015 and 2019. The study population was stratified into two groups based on surgical age: patients who underwent surgery within a period of less than 3 months and those who underwent surgery between 3 and 12 months. The basic conditions, complications at 3-7 years after surgery, anal function (Rintala scale) and quality of life (PedsQLTM4.0) were compared between the groups. RESULTS: A total of 235 children (196 males and 39 females) were included in the study. No statistically significant differences in postoperative bowel function (P = 0.968) or quality of life (P = 0.32) were found between the two groups. However, there was a significant reduction in the incidence of Hirschsprung-associated enterocolitis (HAEC) among individuals under the age of three months prior to undergoing surgical intervention (69.1%) compared to the incidence observed postsurgery (30.9%). This difference was statistically significant (P < 0.001). CONCLUSION: In the current study, the age at which surgery was performed did not exhibit a discernible inclination towards influencing mid-term anal function or quality of life. Early surgical intervention can effectively diminish the occurrence of HAEC, minimize the extent of bowel resection, and expedite the duration of the surgical procedure.

The use of postoperative calibrations in Hirschsprung disease: a practice to reconsider?

PURPOSE: Daily postoperative anal dilations after endorectal pull-through for Hirschsprung disease (HD) are still considered a common practice. We analyzed the potential risks of this procedure and its effectiveness compared to a new internal protocol. METHODS: All infants (< 6 months of age) who underwent transanal endorectal pull-through between January 2021 and January 2023 were prospectively enrolled in a new postoperative protocol group without daily anal dilations (Group A) and compared (1:2 fashion) to those previously treated by postoperative anal dilations (Group B). Patients were matched for age and affected colonic tract. Patients with associated syndromes, extended total intestinal aganglionosis, and presence of enterostomy were excluded. Outcomes considered were: anastomotic complications (stenosis, disruption/leakage), incidence of enterocolitis, and constipation. RESULTS: Eleven patients were included in group A and compared to 22 matched patients (group B). There were no significant differences in the occurrence of anastomotic complications between the two groups. We found a lower incidence of enterocolitis and constipation among group A (p = 0.03 and p = 0.02, respectively). CONCLUSION: A non-dilation strategy after endorectal pull-through could be a feasible alternative and does not significantly increase the risk of postoperative anastomotic complications. Moreover, some preliminary advantages such as lower enterocolitis rate and constipation should be further investigated.

Urological outcomes in adult females born with anorectal malformation or Hirschsprung disease.

INTRODUCTION: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population. METHODS: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022. Female patients between the ages of 18 and 80 years were included. Lower Urinary Tract Symptom Questionnaires were administered through REDCap and the responses were compared to a reference population using Chi-squared or Fisher's exact tests. RESULTS: Sixty-six born female patients answered the questionnaires, two of them identified as non-binary. The response rate was 76%. Median age was 31.6 years. The majority were born with cloaca (56.3%), followed by other type of ARMs (28.1%), complex malformation (9.4%), and HD (6.3%). A history of bladder reconstruction was present for 26.6%. Catheterization through a channel or native urethra was present in 18.8%. Two had ureterostomies and were excluded from the analysis. Seven had chronic kidney disease or end-stage renal disease, three with a history of kidney transplantation. Patients with cloaca had significantly higher rates of urinary incontinence, urinary tract infection, and social problems due to impaired urological functioning, when compared to an age-matched reference population (Table 3). CONCLUSION: This study emphasizes the need for a multi-disciplinary team that includes urology and nephrology following patients with ARM long term, especially within the subgroup of cloaca. LEVEL OF EVIDENCE: III.

Surgical maneuvers for long-segment Hirschsprung pull-through in unique patients.

PURPOSE: Patients with Hirschsprung disease affecting the splenic flexure or more proximal segments present a surgical challenge. Mobilizing the transverse colon to the pelvis during a pull-through may obstruct the distal ileum, or the length may be insufficient to reach the lower pelvis. This retrospective study aimed to describe two surgical techniques that facilitate mobilization of the transverse colon and their outcome. METHODS: We included patients operated on between April 2017 and April 2024 and analyzed sex, comorbidities, type of pull- through, age at pull-through, history of previous surgeries, cause of the proximal transverse colon pull-through, technique used (Deloyers or Turnbull), complications , postoperative outcome and follow-up. The first technique used was the maneuver described by Turnbull. This operation creates a mesenteric defect and mobilizes the colon into this mesenteric window at the distal ileum level. The second technique was described by Deloyers and involves a 180-degree rotation of the right colon by dissecting the right colon attachment and the hepatocolic ligament. The cecum and the ileocecal valve are placed in the right upper quadrant, and the distal colon is mobilized into the pelvis. RESULTS: We included 13 patients, 12 boys and 1 girl. Eight patients had previous surgeries in another hospital: five had an initial transverse colostomy, and three had an ileostomy. The remaining five had the initial operation in our hospital: two had an ileostomy, two had a colostomy, and one had a primary pull-through. The median age at pull-through was 16 months (4-59 months). We used the Turnbull technique in four patients whose aganglionosis was limited to the middle transverse colon. The Deloyers technique was used in the remaining patients, with ganglion cells in the proximal transverse colon. We left a protective ileostomy in five patients. The median follow-up was 4.5 years (3 months to 10 years). The stoma takedown is pending in one patient. CONCLUSION: The Turnbull and Deloyers techniques were helpful in patients with aganglionosis affecting the transverse colon.

Are the complications after laparo-assisted endo-rectal pull-through for Hirschsprung disease related to the change of the anal tone?

The main advantage of the laparo-assisted transanal endorectal pull-through technique (LA - TERPT) for Hirschsprung Disease (HD) is the respect to the rectal-anal anatomy. Postoperative complications have been observed recently. The present study aims to determine how often these postoperative complications occur in these patients. From January 2009 to December 2018, a retrospective analysis was conducted on 36 children (25 males) with HD who underwent LA-TERPT. Data were collected on the age of diagnosis and surgery, sex, the presence of other pathologies, and cases of enterocolitis. In all cases, anorectal manometry (ARM) was performed to evaluate the anal tone. The median age at diagnosis was 2 months and the mean age at surgery was 5 months. Nine related pathologies were identified: five cases of Down syndrome, one case of hypertrophic stenosis of the pylorus, atresia of the esophagus, polydactyly, and anorectal malformation. A patient with total colonic aganglionosis was identified through laparoscopic serummuscular biopsies. Enterocolitis was diagnosed in 7 cases before and 6 after surgery. At follow-up, the complications recorded were: 5 cases of constipation (treated with fecal softeners), one case of anal stenosis (patient with anorectal malformation), 16 cases of soiling (treated with enemas) and 1 child with fecal incontinence (treated with a transanal irrigation system). The ARM was performed in all 36 cases and showed normal anal tone, except for one case with anal hypotonia. LA-TERPT is an important surgical technique for HD. According to the literature, soiling is the most main complication after HD surgery, probably due to "pseudo-incontinence" with normal anal sphincter tone.

Evaluation of Trans-Anal Endorectal Pull-Through Outcomes in Hirschsprung's Disease in Different Age Groups: A Comprehensive Systematic Review.

BACKGROUND: The timing of trans-anal endorectal pull-through (TAEPT) for Hirschsprung's disease (HD) is controversial. Early endorectal pull-through avoids the occurrence of preoperative enterocolitis. However, delayed pull-through (≥31 days) enables postnatal maturation of the anal canal and sphincter complex. The aim of this study was to identify the best age to perform trans-anal pull-through according to the literature. METHODS: This is a comprehensive systematic review. All articles published from 2010 to 2022 were searched in the Web of Science, Ovid Medline, PubMed, CINAHIL, and Embase databases, using the keywords HD, delayed or early treatment, trans-anal pull-through surgery, age, sex or gender, complications and outcomes. Articles that met the inclusion criteria with good to fair quality according to the Newcastle-Ottawa quality assessment and low bias score in the Cochran collaboration tool were reviewed. RESULTS: Sixteen studies were eligible to be reviewed. The overall results of this study showed that due to more common short-term complications at neonatal period and lower contrast enema diagnostic accuracy in determining the transition zone, it seems to be reasonable decision to postpone surgery until the child is several months old. There was also no difference in terms of complications and outcomes of trans-anal pull-through surgery between females and males. CONCLUSION: It is not recommended to delay surgery too much for ages over 1 year. Ages between 3 and 12 months can be a good time for interventional treatment for HD.

Hirschsprung's disease. Management.

BACKGROUND: Hirschsprung's disease (HD) is a rare congenital disease that is characterised by the absence of ganglion cells in the myenteric plexus starting in the distal bowel. This results in distal functional obstruction and may lead to complications like enterocolitis. The treatment is surgical and requires the resection of the aganglionic segment, and the pull-through of normal intestine into the anal opening. However, even after successful surgery, patients may continue to have symptoms. AIM: Discuss current surgical techniques and management strategies for patients with postoperative symptoms after surgical correction of Hirschsprung's disease. METHODS: A review of the literature was done through PubMed, with a focus on clinical management and approach. RESULTS: We describe the clinical problems that can occur after surgical correction. These include obstructive symptoms, enterocolitis, or faecal incontinence. A systematic approach for the evaluation of these patients includes the exclusion of anatomic, inflammatory, behavioural or motility related factors. Depending on the severity of the symptoms, the evaluation includes examination under anaesthesia, the performance of contrast studies, endoscopic studies, measurement of anal sphincter function and colonic motility studies. The treatment is focused towards addressing the different pathophysiological mechanisms, and may include medical management, botulinum toxin to the anal sphincter or rarely redo-operation. CONCLUSIONS: Patients with Hirschsprung's disease need to have surgical correction, and their postoperative long-term management is complex given a variety of associated problems that can occur after surgery. A systematic evaluation is necessary to provide appropriate therapy.

Faecal incontinence: Retentive, non-retentive and when to suspect organic pathology.

BACKGROUND: Faecal incontinence is a highly prevalent and very distressing condition that occurs throughout the entire paediatric age. AIM: To summarise advances in the understanding of the epidemiology, pathophysiology, evaluation and treatment of children with faecal incontinence due to either disorders of gut-brain interaction or organic diseases. METHODS: Literature review on prevalence, impact, diagnosis and treatment options for children with faecal incontinence, interspersed with observations from the author's lifelong career focused on evaluation of children with motility disorders. RESULTS: Faecal incontinence in children is most commonly due to unrecognised or insufficiently treated functional constipation with overflow incontinence. Non-retentive faecal incontinence (NRFI) is probably more common than previously thought and is particularly challenging to treat. Organic diseases such as anorectal malformations (ARMs), Hirschsprung disease and spinal defects are often associated with faecal incontinence; in these conditions, faecal incontinence has a profound impact on quality of life. Recognition of the different pathophysiologic mechanisms causing the incontinence is essential for a successful treatment plan. A thorough physical examination and history is all that is needed in the diagnosis of the causes of faecal incontinence related to disorders of gut-brain interaction. Colonic transit studies or x-rays may help to differentiate retentive from NRFI. Manometry tests are helpful in determining the mechanisms underlying the incontinence in children operated on for ARMs or Hirschsprung diseases. Multiple behavioural, medical and surgical interventions are available to lessen the severity of faecal incontinence and its impact on the daily life of affected individuals. CONCLUSIONS: Recent advances offer hope for children with faecal incontinence.

Identification of a founder effect involving n.197C>T variant in RMRP gene associated to cartilage-hair hypoplasia syndrome in Brazilian patients.

Cartilage-hair hypoplasia syndrome (CHH) is an autosomal recessive disorder frequently linked to n.72A>G (previously known as n.70A>G and n.71A>G), the most common RMRP variant worldwide. More than 130 pathogenic variants in this gene have already been described associated with CHH, and founder alterations were reported in the Finnish and Japanese populations. Our previous study in Brazilian CHH patients showed a high prevalence of n.197C>T variant (former n.195C>T and n.196C>T) when compared to other populations. The aim of this study was to investigate a possible founder effect of the n.197C>T variant in the RMRP gene in a series of CHH Brazilian patients. We have selected four TAG SNPs within chromosome 9 and genotyped the probands and their parents (23 patients previously described and nine novel). A common haplotype to the n.197C>T variant carriers was identified. Patients were also characterized for 46 autosomal Ancestry Informative Markers (AIMs). European ancestry was the most prevalent (58%), followed by African (24%) and Native American (18%). Our results strengthen the hypothesis of a founder effect for the n.197C>T variant in Brazil and indicate that this variant in the RMRP gene originated from a single event on chromosome 9 with a possible European origin.

National Centralization of Hirschsprung's Disease in Sweden:A Comparison of Preoperative Management and Outcomes.

BACKGROUND: Surgical treatment of Hirschsprung's disease (HSCR) in Sweden was centralized to two tertiary pediatric surgery centers 1st of July 2018. Although complex surgical care in adults seems to benefit from centralization there is little evidence to support centralization of pediatric surgical care. The aim of this study was to assess centralization of HSCR in Sweden, with special consideration to preoperative management and outcomes in this group of patients. METHODS: This study retrospectively analyzed data of patients with HSCR that had undergone or were planned to undergo pull-through at our center, from 1st of July 2013 to 30th of June 2023. Patients managed from 1st of July 2013 to 30th of June 2018 were compared with patients managed from 1st of July 2018 to 30th of June 2023 regarding diagnostic procedures, preoperative treatment, complications and time to definitive surgery. RESULTS: Thirty-six patients were managed during the first five-year period compared to 57 during the second period. There was an increased number of patients referred from other Swedish regions to our center following the centralization. Time from diagnosis to pull-through increased from 33 to 55 days after centralization. There were no significant differences in pre-operative management or complications, general or related to stoma. CONCLUSIONS: Despite increasing patient volumes and longer time from diagnosis to pull through, centralization of care for HSCR does not seem to change the preoperative management and risk of complications. With access to support from the specialist center, transanal irrigations remain a safe mode of at home management until surgery, regardless of distance to index hospital. LEVEL OF EVIDENCE: Level III.

Duhamel and transanal endorectal pull-throughs for Hirschsprung disease: a Bayesian network meta-analysis.

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%). CONCLUSION: 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.

Children with Hirschsprung's disease have high morbidity in the first 5 years of life.

BACKGROUND: Hirschsprung's disease is a rare congenital anomaly of the colon with absence of the ganglionic nerve cells. The treatment of the anomaly is surgical. METHODS: This population-based data-linkage cohort study was part of the EUROlinkCAT project and investigated mortality and morbidity for the first 5 years of life for European children diagnosed with Hirschsprung's disease. Nine population-based registries in five countries from the European surveillance of congenital anomalies network (EUROCAT) participated. Data on children born 1995-2014 and diagnosed with Hirschsprung's disease were linked to hospital databases. All analyses were adjusted for region and length of follow-up, which differed by registry. RESULTS: The study included 680 children with Hirschsprung's disease. One-year survival was 97.7% (95% CI: 96.4-98.7). Overall, 85% (82-87) had a code for a specified intestinal surgery within the first year increasing to 92% (90-94) before age 5 years. The median age at the first intestinal surgery up to 5 years was 28 days (11-46) and the median number of intestinal surgical procedures was 3.5 (3.1-3.9). Thirty days mortality after neonatal surgery (within 28 days after birth) was 0.9% (0.2-2.5) for children with a code for intestinal surgery within the first 28 days after birth and there were no deaths for children with a code for stoma surgery in the neonatal period. CONCLUSION: Children with Hirschsprung's disease have a high morbidity in the first 5 years of life requiring more surgical procedures in addition to the initial surgery. Mortality after neonatal surgery is low.