Postoperative Enterocolitis Research Articles

Outcomes following endorectal pull-through for Hirschsprung disease: a retrospective study

PurposeTo share our experience in the surgical management of patients with Hirschsprung disease (HD) using the trans-anal endorectal pull-through “Soave” technique.MethodsThe study included cases diagnosed as HD who were operated during the period 2008 through 2018. Cases of total colonic aganglionosis were excluded to be discussed in a separate report.ResultsThe study included 67 consecutive cases of HD. All included cases underwent trans-anal endorectal pull-through which was purely trans-anal in 31 (46%), while abdominal-assisted trans-anal endorectal pull-through was applied in the remaining (54%). Early postoperative complications included 2 cases of partial dehiscence at the colo-anal anastomosis; 1 of them deteriorated after re-exploration and unfortunately died. Twenty-six cases were available to assess functional outcomes after corrective surgery. Their age at follow-up ranged between 44 months and 17 years (mean = 78.8 months; median = 72 months). All cases achieved voluntary defecation. However, fecal soiling was a common finding after surgery for HD (69%). Constipation was another common postoperative finding (73%). Lastly, a history of attacks of postoperative enterocolitis was prevalent in 19 cases (73%), which ranged between 1 and 7 attacks usually in the first 2 years after the operation.ConclusionThe trans-anal endorectal pull-through provides an effective surgical treatment for Hirschsprung disease. The high prevalence of fecal soiling after surgery highlights the importance of long-term follow-up to provide the required support for these patients during adolescence and transition into adulthood.

Histological transitional zone pull-through in Hirschsprung disease. Postoperative functional results and current recommendations.

Surgeons create a neorectum to repair patients with Hirschsprung's disease (HD), which should be formed from a normoganglionic bowel. However, the neorectum is occasionally created with a transition zone (TZ) bowel. A neorectum created with a TZ has been postulated as a cause of postoperative enterocolitis or constipation. This study compares the incidence of enterocolitis and constipation in patients with TZ neorectum and normoganglionic bowel. We conducted a retrospective review of patients with rectosigmoid HD who underwent primary pull-through. Patients were divided into normoganglionic neorectum (NNR) and TZ neorectum. The diagnosis was based on the final histopathologic report of the proximal margin. The incidence of enterocolitis and constipation was compared between these two groups. A total of 98 HD patients were analyzed. Seventy-one patients fulfilled the inclusion criteria. 65 (92%) had a NNR, and six patients (8%) had a TZ neorectum. From these patients, 42 (59%) presented with enterocolitis or constipation. However, there was no significant difference between both groups. The present study showed no difference in the incidence of enterocolitis or postoperative constipation in HD patients with normoganglionic or TZ neorectum. These results suggest that TZ neorectum does not cause postoperative obstructive symptoms.

Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis.

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

Comparison of the Duhamel Procedure and Transanal Endorectal Pull-through Procedure in the Treatment of Children with Hirschsprung's Disease: A Systematic Review.

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. 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. 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.

Functional outcome of Hirschsprung's disease in children: A single center study at The Children's Hospital Lahore.

Hirschsprung's disease (HD) is a congenital bowel disorder resulting in functional dysmotility. Advancement in surgical techniques has improved outcomes, but recent studies have identified problems having significant impact on patient's quality of life. The aim of this study is to analyze postoperative functional outcome after definitive surgery for Hirschsprung's disease. A retrospective study was conducted at the Department of Pediatric Surgery, The Children's Hospital Lahore. The record of 64 patients was reviewed from the year 2009 to 2019. Parents were interviewed on phone calls and a predesigned questionnaire was filled. Degree of constipation, incontinence and voluntary bowel movements was analyzed according to Krickenbeck classification. Median age at initial presentation was 11days, with male to female ratio of 3.9:1. Most common presentation was delayed passage of meconium (38%). Diagnosis was made on biopsy in 70.3%, but contrast enema and biopsy in 29.7%. Aganglionosis was limited to short segment in 81.3% and long segment in 14.5%. Decompressing enterostomy was made in 63% at a mean age of 191days. Definitive surgery included Duhamel pull-through in 89.1%. Mean age at follow-up was 10.6 ± 4.19years and alive patients were 95.4%. Postoperative enterocolitis was reported by 27%, while 35.5% reported fecal incontinence and 16.1% complained of constipation.80% could voluntarilyhold bowel movement, among which 5% had a feeling of urge and 15% had the capacity to verbalize. In 25% of thepatients, fecal incontinence was a constant social problem and in 25% of these patients constipation was resistant to diet and laxatives. On long-term follow-up, fecal incontinence was more worrisome, affecting quality of life and requiring help of the bowel management program. Such patients need close follow-up after definitive procedure, so that they have normal stooling habits in their adulthood and lead a normal life.

Outcome of Transanal Endorectal Pull-Through in Patients with Hirschsprung's Disease.

Transanal endorectal pull-through (TERPT) has become one of the preferred treatments for Hirschsprung's disease (HD) in our setting. This report aims to evaluate the current outcome of TERPT in the setting. A retrospective review of 71 children who had TERPT for histologically-confirmed HD in 11 years (2006-2017) in Nigeria. There were 48 boys and 23 girls; aged 3 days-12 years at initial presentation (median = 10 months). Three (4.2%) patients had associated anomalies (duodenal atresia; anorectal malformation and sensorineural deafness with hypopigmented skin patches each). Age at TERPT was 2 months to 15 years (median = 3 years), with surgery waiting time of 1 month-14.9 years (median = 18 months). Sixty-six (93.0%) patients had rectosigmoid, four (5.6%) patients had long segment and one (1.4%) had total colonic disease. Five (7.0%) patients with large megacolon and one (1.4%) with the total colonic disease had assisted abdominal resection of the colon at TERPT. Seventeen (23.9%) patients had post-operative complications, including post-operative enterocolitis 8 (11.3%); anastomotic dehiscence 3 (4.2%); retained aganglionic segment 2 (2.8%); anastomotic stenosis 2 (2.8%), resulting in prolonged hospital stay (P = 0.0001; range = 1-30 days; median = 5 days). The mortality rate was 4.2% (3) from malignant hyperthermia in one patient and in 2 patients, the cause of mortality was unclear. Patients were followed up for 3-6 years (median = 3.5 years). Bowel movement stabilised to 2-4 times daily by 6 weeks after surgery. TERPT is a safe treatment for HD in this setting with good short-term outcomes. Longer follow-up is necessary to further evaluate the long-term bowel movement outcomes.!

Comparison of clinical features and prognosis between ultrashort-segment and short-segment hirschsprung disease.

To compare the differences in clinical features, postoperative complications, and long-term bowel function outcomes of ultrashort-segment Hirschsprung disease (USHD) and short-segment Hirschsprung disease (SHD). A retrospective study was conducted to compare patients with USHD or SHD who underwent transanal endorectal pull-through (TEPT) at Beijing Children's Hospital between January 2014 and June 2021. Clinical details were collected from medical records. A long-term bowel function questionnaire (age > 4 years old) was completed by the patients' parents. A total of 84 patients (USHD = 15, SHD = 69) were included. Age at diagnosis and radical surgery in the USHD group were significantly older than the SHD group (46 [38, 66] vs. 34 [6, 55] months, p = 0.002; 51 [39, 68] vs. 37 [10, 68] months, p = 0.001, respectively). Compared with the SHD group, patients with USHD are more likely to suffer anastomosis leakage and postoperative enterocolitis after TEPT ([3/15, 33.3%] vs. [1/69, 1.4%], p = 0.017; [5/15, 33.3%] vs. [6/69, 8.7%], p = 0.023). In addition, patients in the USHD group are inclined to suffer lower bowel function scores (12.0 [7.5, 18.3] vs. 17 [15, 19], p = 0.018).Patients in the USHD group were more likely to suffer poorer ability to hold back defecation (p = 0.023), soiling (p = 0.011), fecal accidents (p = 0.004), and social problems (p = 0.004). Compared with patients with SHD, patients with USHD are diagnosed and performed TEPT at an older age. and they are inclined to suffer postoperative enterocolitis, anastomosis leakage, and poorer long-term bowel function following TEPT.

Robotic surgery in Hirschsprung disease: a unicentric experience on 31 procedures.

Robotic surgery has been increasingly applied to Hirschsprung patients with encouraging results. We report the results of a 5year unicentric experience. All consecutive HSCR patients older than 12months who underwent a surgical procedure with robotic approach between September 2017 and August 2022 were prospectively included. We collected data regarding demographics, extent of aganglionosis, associated anomalies, indications to surgery, and a number of perioperative data such as surgical details, intraoperative and perioperative complications, length of surgery, length of hospital stay, and functional outcome. A total of 28 patients underwent 31 robotic procedures during the study period. Median age at surgery was 82months. Eleven primary Totally Robotic Soave Pull-Through, 12 redoes, 5 innervative mapping, 2 redundant rectal pouch excision, and 1 Miles' procedures have been performed. Median console time was 145min. No conversion to either laparoscopy nor to laparotomy was required. Median length of hospital stay was 6days. Two patients experienced complications requiring reiterative surgery. One patient experienced mild postoperative enterocolitis. Normal continence was achieved by 70% of patients after a median of 16months postoperatively (80% for primary pull-throughs, 55% for redoes). To conclude, robotic surgery for older HSCR patients proved to be feasible, safe, and effective. Patients with complex surgical requirements seem to benefit most from this promising approach. Provided the economic burden is addressed and solved, robotic surgery will represent an excellent alternative for the surgical treatment of HSCR patients.

Do Histologic Features of the Proximal Margin of Resected Specimens Predict Clinical Outcomes in Hirschsprung Disease?

Objective Patients with Hirschsprung disease (HSCR) can experience obstructive symptoms despite adequate resection. We sought to determine if submucosal nerve thickness or length of ganglionated bowel in the resected specimen correlated with functional outcomes. Methods A retrospective study of patients who underwent surgery between 2015-2019 was performed. The resected specimen was scanned to measure areas of the thickest submucosal nerves and the length of the ganglionated segment. Functional outcomes were collected via chart review. Results Thirty patients were included. The median age at pull-through was 4.5 months (interquartile range {IQR} 0.5 - 6.7 months); 70% were male, and 57% had a Swenson pull-through. The median size of the thickest nerves was 28 micrometers (IQR 24, 32). Three specimens had a nerve thickness of >40 micrometers. The median length of the resected ganglionated segment was 4.4 cm (IQR 2.2, 7.2). Out of the total, 53% of patients experienced post-operative enterocolitis; 13% required further surgery. At a median of 25.3 months (IQR 17.6, 42.2 months) from pull-through, 33% did not require any bowel regimen therapy. Utilizing logistic regression, neither submucosal nerve thickness nor length of the resected ganglionic segment correlated with outcomes. Conclusion While continued bowel management therapy was common, no correlation was found between histologic findings and functional outcomes.

Comparative cohort study of Duhamel and endorectal pull-through for Hirschsprung's disease.

BackgroundThere are limited data available to compare outcomes between surgical approaches for Hirschsprung’s disease. Duhamel and endorectal pull-through (ERPT) are two of the most common procedures performed worldwide.MethodsObjective outcomes were compared between contemporary cohorts (aged 4–32 years) after Duhamel or ERPT using case–control methodology. Data were collected using prospectively administered standardized questionnaires on bowel and bladder function and quality of life (Pediatric Quality of Life Inventory, Short form 36 and Gastrointestinal Quality of Life Index). Patients were compared in two age groups (18 years and younger and older than 18 years) and reference made to normative control data. Multivariable analysis explored factors associated with poor outcomes.ResultsCohorts were well matched by demographics, disease characteristics and incidence of postoperative complications (120 patients who underwent Duhamel versus 57 patients who had ERPT). Bowel function scores were similar between groups. Patients who underwent Duhamel demonstrated worse constipation and inferior faecal awareness scores (P < 0.01 for both age groups). Recurrent postoperative enterocolitis was significantly more common after ERPT (34 versus 6 per cent; odds ratio 15.56 (95 per cent c.i. 6.19 to 39.24; P < 0.0001)). On multivariable analysis, poor bowel outcome was the only factor significantly associated with poor urinary outcome (adjusted odds ratio 6.66 (95 per cent c.i. 1.74 to 25.50; P = 0.006)) and was significantly associated with markedly reduced quality of life (QoL) in all instruments used (P < 0.001 for all). There were no associations between QoL measures and pull-through technique.ConclusionOutcomes from Duhamel and ERPT are good in the majority of cases, with comparable bowel function scores. Constipation and impaired faecal awareness were more prevalent after Duhamel, with differences sustained in adulthood. Recurrent enterocolitis was significantly more prevalent after ERPT. Clustering of poor QoL and poor functional outcomes were observed in both cohorts, with seemingly little effect by choice of surgical procedure in terms of QoL.

The outcomes in children with Hirschsprung’s disease treated with transanal endorectal pull-through method

Introduction and aim. The evaluation of functional results, complications and problems of children with Hirschsprung’s disease treated with one-stage surgery (TEPT) or two-stage surgery (colostomy, TEPT) in Paediatric Surgery Clinic in Rzeszow. Material and methods. Medical documentation of 41 children treated due to Hirschsprung’s disease in years 2006-2018 in Rzeszow were retrospectively analysed. The results of the questionnaires conducted among the parents of operated children were surveyed. Results. The average time of the radical surgery was 189 minutes. The mean length of the resected intestine in the classic form was 19 centimeters, in long-segment 35 centimeters. In the post-operative period, 15 patients had a blood transfusion. The mean time of the children’s stay calculated from the date of surgery until the discharge equaled 13.4 days. Early post-operative complications: enterocolitis occurred in 6 patients (1 death in a septic shock mechanism), total dehiscence of anastomosis in 1 patient, abscess of perirectal space in 1 patient, anastomotic retraction in 1 patient and in 3 patients inaccurate intra-operative evaluation of the section (intra), buttock dermatitis appeared in all patients. Late post-operative complications (a control trial of 38 patients): Soiling was confirmed in 9 patients, periodic constipation in one. The abnormal consistency of stool was signaled in 3 children. Two children were repetitively hospitalized due to enterocolitis. The frequency of defecation almost in all patients was reduced after a three, four-month period since the operation from 10-15 per day to the age norm. In one child, where the retraction of the anastomosis was diagnosed, the soiling and heightened frequency of defecation throughout the day persists. All of the parents of the older children view the outcome of the surgery as positive and the life quality of their children does not differ from their peers. Conclusion. TEPT is a method which can be performed in newborns, infants, babies as well as in case of a long-segment aganglionosis. Barium enema is not reliable in evaluation of the length of the aganglionic section in the long-segment type of Hirschsprung’s disease. The treatment of choice in the early post-operative enterocolitis should be colostomy. Worse functional results were observed in children after two-stage treatment – especially, in cases where the colostomy was created due to the intestinal re-distention.

Clinical Efficacy of an Indwelling Transanal Tube for the Prevention of Anastomotic Leakage After Hirschsprung's Disease: A Single Center Experience with Chinese Patients.

Background: The aim of this study was to investigate the clinical effectiveness of an indwelling transanal tube for the prevention of anastomotic leakage (AL) after a radical operation for Hirschsprung's disease (HD). Methods: We retrospectively analyzed the clinical data from 158 patients who had undergone laparoscopic-assisted Soave procedures for HD at our hospital from May 2015 to May 2019. Patients were divided into two groups depending upon whether the anal drainage tube was retained or not retained: an indwelling group (group A, n = 86) and a no-indwelling group (group B, n = 72). Results: All 158 children had a successful operation by a laparoscopic technique. There was no significant difference in the duration of the operation, the length of the incision, the amount of bleeding, or the postoperative hospitalization time between the two groups. Compared with the no-indwelling group, maintaining the transanal tube had significant advantages for preventing incidences of AL (P < .05). The 4-year follow-up showed that the incidence of postoperative enterocolitis with the indwelling transanal tube was significantly lower than in the group without the drainage tube (P < .05). Conclusions: The laparoscopic-assisted Soave procedure with an indwelling transanal tube is a safe and feasible method for the treatment of HD in children. This method can not only drain intestinal contents but also reduce the occurrence of AL.

Prevalence of Hirschsprung-associated enterocolitis in patients with Hirschsprung disease

PurposeHirschsprung's associated enterocolitis (HAEC) is a complication of Hirschsprung's Disease (HD) with considerable morbidity and mortality. The variability in presentation leads to a wide variety of the reported prevalence pre-and postoperatively. This systematic review aimed to clarify the prevalence of HAEC in short—(S-HD), long (L-HD), TCA and the type of operation used.MethodsA systematic literature-based search for relevant cohorts was performed using Pubmed/Medline, Cochrane Library from its inception to May 2021. Studies reporting on pre-and postoperative enterocolitis, segment length, and surgical procedure (Soave, Swenson, Duhamel) were included. Pooled prevalence and subgroup analysis have been calculated for pre-and postoperative HAEC.Results4738 articles were identified from the literature search, among which 57 studies, including 9744 preoperative and 8568 postoperative patients, were included. The groups were sorted by length of the aganglionic segment for further analysis. The pooled prevalence for preoperative HAEC was 18.3% for all types, 15.2% for S-HD and 26.1% for TCA. The pooled prevalence for postoperative HAEC was in total 18.2% for all segment lengths and used techniques. Subgroup analysis showed no significant difference in the occurrence of postoperative enterocolitis between the three techniques.ConclusionThe prevalence of preoperative HAEC increases with segment length. However, pooled data suggest that the postoperative risk for developing HAEC, independently of the employed method and segment length, is comparable to the preoperative risk.

Duhamel’s procedure for Hirschsprung’s disease and the functional outcome in a tertiary care center

Introduction: Hirschsprung’s disease (HD) is a developmental disorder caused by the migration failure of neural crest cells during intestinal development, resulting in an aganglionic colon and causing a functional obstruction in children. Duhamel’s procedure (DP) which involves excision of the aganglionic segment with retrorectal pull through, and anastomosis of the ganglionated bowel above dentate line, is one of the classical surgical procedures for HD. Materials and Methods: This retrospective study was conducted in a tertiary care center in South India. The medical records and follow-up details of 17 patients, who underwent DP for HD between January 2014 and December 2018, were obtained and evaluated. Outcomes evaluated included incidence of post-operative constipation, incontinence/soiling, enterocolitis, anastomotic stricture, and leak. Results: There were 17 patients with HD who underwent DP during the study period, among which 76.47% of patients had classical HD and 23.52% were long-segment HD. A total of 58.82% of patients presented at birth, with overall 76.47% of male predominance. Fecal soiling (29.41%), perianal excoriation (17.64%), wound infection (11.76%), post-operative enterocolitis (11.76%), stricture formation (11.76%), constipation (11.76%), and hypertension (11.76%), and adhesive bowel obstruction (5.88%) were some complications observed in this study. Most of these complications were successfully managed. Conclusion: Staged DP is safe and can address the entire spectrum of HD. Complications are less and comparable to other techniques, and with close follow-up to address the post-operative complications, good quality of life can be achieved for the children with HD.

Lack of Mucosal Cholinergic Innervation Is Associated With Increased Risk of Enterocolitis in Hirschsprung’s Disease

Background & AimsHirschsprung’s disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric neuronal cells (ganglia) in the distal gut. The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates innervation of acetylcholine-secreting (cholinergic) nerve fibers. Cholinergic signals have been reported to control excessive inflammation, but the impact on HSCR-associated enterocolitis is unknown.MethodsWe enrolled 44 HSCR patients in a prospective multicenter study and grouped them according to their degree of colonic mucosal acetylcholinesterase-positive innervation into low-fiber and high-fiber patient groups. The fiber phenotype was correlated with the tissue cytokine profile as well as immune cell frequencies using Luminex analysis and fluorescence-activated cell sorting analysis of colonic tissue and immune cells. Using confocal immunofluorescence microscopy, macrophages were identified in close proximity to nerve fibers and characterized by RNA-seq analysis. Microbial dysbiosis was analyzed in colonic tissue using 16S-rDNA gene sequencing. Finally, the fiber phenotype was correlated with postoperative enterocolitis manifestation.ResultsThe presence of mucosal nerve fiber innervation correlated with reduced T-helper 17 cytokines and cell frequencies. In high-fiber tissue, macrophages co-localized with nerve fibers and expressed significantly less interleukin 23 than macrophages from low-fiber tissue. HSCR patients lacking mucosal nerve fibers showed microbial dysbiosis and had a higher incidence of postoperative enterocolitis.ConclusionsThe mucosal fiber phenotype might serve as a prognostic marker for enterocolitis development in HSCR patients and may offer an approach to personalized patient care and new therapeutic options.

Çocuklarda Hirschsprung Hastalığı Cerrahi Tedavisi Sonuçları: 12 Yıllık Bir Deneyim

Objective: In the treatment of Hirschsprung’s disease, many surgical techniques have been developed and modified including laparotomy, resection of the affected colon segment, and pull-through of the functional bowel segment to the anus. Conventional surgical methods have many early and late postoperative complications, albeit at a reduced rate today. In this study, it was aimed to compare the treatment results of children with Hirschsprung Disease who were operated with single or two-stage techniques in our department. Method: Hospital records of the patients operated for Hirschsprung disease were retrospectively evaluated. Demografic characteristics, findings of physical and rectal examinations, X-rays findings, rectal biopsy results, surgical treatment methods and outcomes were analyzed. Results: Fifty-two children including 41-male (78.8%) and 11-female (21.2%) patients with age ranging from 1-day to 5-year and mean age of 6.8-day in neonates and 6.3-month in others were enrolled in the study. All patients presented with acute or subacute intestinal obstruction. Levels were observed on supine abdominal radiographs in all; transition zone and retention of opaque material were observed on opaque enema or retention radiographs.In patients whose rectal biopsies revealed aganglionic megacolon two staged procedures were performed including initial colostomy followed by definitive procedure of Duhamel with stapler in 30, and Soave in 12, and Swenson in 1 patient. Nine patients were operated with one-stage endorectal pull-through technique. The complication rate in 43 patients with Hirschsprung disease managed by two staged operative procedure was (78.8%), and it was 11.1% in 9 cases that underwent transanal endorectal pullthrough procedure. It was learned that effective anal dilatation was not performed in 22-patients who had postoperative enterocolitis attacks. Conclusion: In addition to the low complication rate, the morbidity rates in the transanal endorectal pull-through method, which achieved successful results with only one procedure, were very low compared to multiple-stage operations.

Postoperative enterocolitis assessment using two different cut-off values in the HAEC score in Hirschsprung patients undergoing Duhamel and Soave pull-through

BackgroundHirschsprung-associated enterocolitis (HAEC) is the most severe and potentially lethal complication of Hirschsprung disease (HSCR) which might occur following definitive surgery. Our objectives were: 1) to compare the incidence of HAEC after Duhamel and Soave procedures using different cut-off values of the HAEC scoring method; and 2) to associate them with the risk factors, including sex, aganglionosis type, mothers’ age at childbirth, gestational age, and mothers’ educational level.MethodsMedical records of patients with HSCR who underwent Soave and Duhamel procedures in our institution, Indonesia (January 2012 – December 2016) were reviewed retrospectively. Two cut-off values of the HAEC scoring system (i.e., ≥10 and ≥ 4) were utilized.ResultsEighty-three patients with HSCR were recruited in this study (Soave: 37 males and 7 females vs. Duhamel: 28 males and 11 females; p = 0.18). The incidence of HAEC after surgery was 14/83 (16.9%) and 38/83 (45.8%) for cut-off values of ≥10 and ≥ 4, respectively (p = 0.00012), and tended to have an association with sex (p = 0.09). Although it was not statistically significant (p = 0.07), the frequency of HAEC after Soave procedure tended to be higher in patients with their mother’s age of ≤35 years at childbirth than those with their mother’s age of > 35 years (OR = 7.9; 95% CI = 0.9–72.1). Multivariate analysis indicated none of the risk factors were associated with the frequency of HAEC after definitive surgery.ConclusionsThe lower cut-off value of ≥4 might increase the possibility to diagnose HAEC, particularly the mild cases. The incidence of HAEC after definitive surgery was not associated with any risk factors in our cohort patients. Further multicenter studies with a larger sample size are necessary to confirm our findings.

Comparison of Hirschsprung Disease Characteristics between Those with a History of Postoperative Enterocolitis and Those without: Results from the Pediatric Colorectal and Pelvic Learning Consortium.

The current understanding of Hirschsprung-associated enterocolitis (HAEC) is based mainly on single-center, retrospective studies. The aims of this study are to determine risk factors for postoperative HAEC using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database. We performed a multicenter, retrospective, case-control study of children with Hirschsprung disease (HD) who had undergone a pull-through procedure and were evaluated at a PCPLC member site between February 2017 and March 2020. The cohort with a history of postoperative HAEC was compared with that without postoperative episodes of HAEC to determine relevant associations with postoperative HAEC. One-hundred forty of 299 (46.8%) patients enrolled had a history of postoperative HAEC. Patients with a rectosigmoid transition zone had a lower association with postoperative HAEC as compared with those with a more proximal transition zone (odds ratio [OR]: 0.46, 95% confidence interval [CI]: 0.26, 0.84, p < 0.01). Private insurance was protective against postoperative HAEC on univariate analysis (OR: 0.62, 95% CI: 0.38, 0.99, p = 0.047), but not on multivariate analysis (OR: 0.62, 95% CI: 0.37, 1.04, p = 0.07). Preoperative HAEC was not associated with the development of postoperative HAEC. Patients with a rectosigmoid transition zone have less postoperative HAEC compared with patients with a more proximal transition zone. Multi-institutional collection of clinical information in patients with HD may allow for the identification of additional risk factors for HAEC and afford the opportunity to improve care.

Prospective study reveals a microbiome signature that predicts the occurrence of post-operative enterocolitis in Hirschsprung disease (HSCR) patients

ABSTRACT Hirschsprung disease (HSCR) is a birth defect with an approximate incidence of 1/5,000 live births, and up to one-third of HSCR patients develop Hirschsprung-associated enterocolitis (HAEC), the leading cause of HSCR-related death. Very little is known about the pathogenesis, prevention, and early diagnosis of HAEC. Here, we used a prospective study to investigate the enteric microbiome composition at the time of surgery as a predictor for developing postoperative HAEC. We identified a microbiome signature containing 21 operational taxonomic units (OTUs) that can potentially predict postoperative HAEC with ~85% accuracy. Furthermore, we identified exclusive breastfeeding as a novel protective factor for total HAEC (i.e., preoperative and postoperative HAEC combined). In addition, we discovered that breastfeeding was associated with a lowered risk for HAEC potentially mediated by modulating the gut microbiome composition characterized by a lower abundance of Gram-negative bacteria and lower LPS concentrations. In conclusion, modulating the gut microbiome by encouraging breastfeeding might prevent HAEC progression in HSCR patients.

The outcome of single-stage management of Hirschsprung's disease at a tertiary care center

Background: Hirschsprung's disease (HD) was initially treated as a multistage procedure. Subsequently, it has been recognized that surgery can be done as a single-stage at an early age as this approach is safe, cost-effective, and avoids the morbidity associated with stomas. We study the effectiveness and safety of single-stage management of HD using Langer's or Georgeson's techniques, to analyze the outcome of surgery, and its comparison with other similar studies. Patients and Method: The study was retrospectively carried out in our institution from October 2016 to September 2019. They were operated in a single-stage either by Langer's procedure or by laparoscopy-assisted Georgeson's procedure. Inclusion criteria were clinically diagnosed cases of HD with an obvious transition zone. Patients without a funnel (on barium enema films) were excluded. Results were analyzed using IBM SPSS statistics 20.00 software. Result: The present series included 28 patients. The mean age of the patients was 16.24 months. Mean intra operative blood loss for Langer's series was 30 ml and Georgeson's series was 20 ml. The mean operative time for Georgeson's procedure was 150 min and for Langer's, it was 90 min. Postoperative enterocolitis 3 (10.7%), persistent constipation 2 (7.5%), and perianal excoriation 9 (32.14%) were some of the important complications noted. Conclusion: Single-stage surgery for HD is safe and avoids the need for multiple operations including stoma and its related complications.