Children with Hirschsprung disease exhibited alterations in host-microbial co-metabolism after pull-through operation.

PURPOSE: This study aims to compare the fecal metabolome in post pull-through HD with and without HAEC patients and healthy young children using nuclear magnetic resonance (NMR) spectroscopy. METHODS: Fresh fecal samples were collected from children under 5 years of age in both post-pull-through HD patients and healthy Thai children. A total of 20 fecal samples were then analyzed using NMR spectroscopy. RESULTS: Thirty-four metabolites identified among HD and healthy children younger than 5 years were compared. HD samples demonstrated a significant decrease in acetoin, phenylacetylglutamine, and N-acetylornithine (corrected p value = 0.01, 0.04, and 0.004, respectively). Succinate and xylose significantly decreased in HD with HAEC group compared to HD without HAEC group (corrected p value = 0.04 and 0.02, respectively). Moreover, glutamine and glutamate metabolism, and alanine, aspartate, and glutamate metabolism were the significant pathways involved, with pathway impact 0.42 and 0.50, respectively (corrected p value = 0.02 and 0.04, respectively). CONCLUSION: Differences in class, quantity, and metabolism of protein and other metabolites in young children with HD after pull-through operation were identified. Most of the associated metabolic pathways were correlated with the amino acids metabolism, which is required to maintain intestinal integrity and function.

The Effect of the Ganglionic Segment Inflammatory Response to Postoperative Enterocolitis in Hirschsprung Disease.

INTRODUCTION: We examined the relationship between proinflammatory cytokines that occur in the inflammatory reaction in the intestine in Hirschsprung disease (HD) and Hirschsprung-associated enterocolitis (HAEC). METHODS: Thirty cases (M:27, F:3) operated on due to HD. The cases were divided into three groups: group 1 with pre and post operative EC, group 2 with post-operative, and group 3 with pre-operative EC. The intestinal segments were evaluated by immunohistochemistry for interleukin 1 beta (IL-1ß), tumor necrosis factor-alpha (TNF-α), and interleukin 6 (IL-6). RESULTS: IL-1ß staining was significantly higher in the ganglionic zone of groups with enterocolitis compared to the control group (p = 0.012). TNF-α staining in the transitional zone of Group 3 and IL-1ß staining in the ganglionic zone of Group 1 was significantly higher than the control group (p = 0.030, p = 0.020). CONCLUSION: In our study, older age at diagnosis and more than 20% IL-1ß staining in the ganglionic segment were found to be risk factors for HAEC. It is noteworthy that the increase in IL-1ß can be associated with HAEC.

Comparation of robotic-assisted surgery and laparoscopic­assisted surgery in children with Hirschsprung's disease: a single-centered retrospective study.

BACKGROUND: There are few studies comparing robotic-assisted surgery (RAS) and laparoscopic-assisted surgery (LAS) in Hirschsprung's disease (HSCR). This study aimed to compare intraoperative and postoperative outcomes between RAS and LAS performed during the same period. METHODS: All consecutive 75 patients with pathologically diagnosed as HSCR who underwent Swenson pull-through surgery from April 2020 to Nov 2022, were included. Patients were divided into RAS group and LAS group and a retrospective analysis was performed based on clinical indexes and prognosis. RESULTS: A total of 75 patients were included, among which, 31 patients received RAS and 44 received LAS. The RAS and LAS groups had similar ages, sex, weight, postoperative hospital stays, and fasting times. Compared with LAS, blood loss (p = 0.002) and the incidence of Hirschsprung-associated enterocolitis (p = 0.046) were significantly lower in the RAS group. The first onset of Hirschsprung-associated enterocolitis in patients younger than 3 months occurred significantly earlier (p = 0.043). Two patients experienced anastomotic leakage in the LAS group and one patient experienced incisional hernia in the RAS group. The cost of RAS was significantly higher than that of LAS (p < 0.0001). CONCLUSIONS: RAS is a safe and effective alternative for HSCR children, and a delaying primary surgery until later in infancy (> 3 months) may improve outcomes.

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. METHODS: Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. RESULTS: We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). CONCLUSION: Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. LEVEL OF EVIDENCE: Level III.

Transition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes?

INTRODUCTION: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. METHODS: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. RESULTS: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). CONCLUSION: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.

Effect of Transumbilical Single-Port Laparoscopic-Assisted Duhamel Operation on Serum CRP and IL-6 Levels in Children with Hirschsprung's Disease.

Objective: To explore the clinical intervention effect of transumbilical single-port laparoscopic-assisted Duhamel operation on children with Hirschsprung's disease (HD) and to analyze the effect of treatment on children with serum C-reactive protein (CRP) and interleukin-6 (IL-6) effects. Methods: Retrospectively select 80 children with HD who underwent surgery in our hospital from May 2017 to May 2020 as the research subjects and they are classified as group A according to the difference of the children's surgical procedures (receiving transumbilical single-port laparoscopic-assisted Duhamel surgery, 40 cases) and group B (receiving conventional laparoscopic surgery, 40 cases), compare the perioperative period (operating time, intraoperative blood loss, surgical posthospitalization, and postoperative gastrointestinal function recovery time), early postoperative complications (perianal dermatitis, urinary retention, enterocolitis, and anastomotic leakage), and late postoperative complications (anastomotic stenosis, dirty stool, recurrence of constipation, and enterocolitis), compare the differences in the levels of CRP and IL-6 between the two groups of children before and after the operation, and conduct a 1-year follow-up of the two groups of children to compare the long-term defecation status. Results: The surgical time of children in group A, postoperative hospitalization time, and postoperative gastrointestinal function recovery time were significantly shorter than those of group B, and the differences between groups were statistically significant (P < 0.05). A group of patients: the total incidence of postearly complications was 5.00% lower than 22.50% (P < 0.05) in group B (P < 0.05), and the total incidence of previous complications after group A of patients was 10.00% lower than 27.50% of group B (P < 0.05). The two groups of serum CRP and IL-6 in two groups were not statistically significant (P > 0.05), and the serum CRP and IL-6 levels of children in group A after surgery were 3 days. It is obviously lower than those in group B, and the differences between groups have statistical significance (P < 0.05). At 1 month after surgery, the average bowel movement time in group A is significantly lower than those of group B (P < 0.05); during the 1-12 months, the difference between the defecation frequency group of the group A and group B did not have statistically significance (P > 0.05). Conclusion: Transumbilical single-port laparoscopic assistant Duhamel operation of HD has a good intervention effect, compared to traditional laparoscopic surgery, the operation time, postoperative hospitalization time, and postoperative gastrointestinal function recovery time, and also help to reduce postoperative near-long complications The incidence improves the stress reactions and long-term defecation functions in children.

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

BACKGROUND: There 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. METHODS: Objective 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. RESULTS: Cohorts 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. CONCLUSION: Outcomes 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.

Inpatient management of Hirschsprung's associated enterocolitis treatment: the benefits of standardized care.

INTRODUCTION: Patients with Hirschsprung's disease (HSCR) remain at risk of developing Hirschsprung-associated enterocolitis (HAEC) after surgical intervention. As inpatient management remains variable, our institution implemented an algorithm directed at standardizing treatment practices. This study aimed to compare the outcomes of patients pre- and post-algorithm. METHODS: A retrospective review of patients admitted for HAEC was performed; January 2017-June 2018 encompassed the pre-implementation period, and October 2018-October 2019 was the post-implementation period. Demographics and outcomes were compared between the two groups. RESULTS: Sixty-two episodes of HAEC occurred in 27 patients during the entire study period. Sixteen patients (59%) had more than one episode. The most common levels of the transition zone were the rectosigmoid (50%) and descending colon (27%). Following algorithm implementation, the median length of stay (2 vs. 7 days, p < 0.001), TPN duration (0 vs. 5.5 days, p < 0.001), and days to full enteral diet (6 days vs. 2 days, p < 0.001) decreased significantly. Readmission rates for recurrent enterocolitis were similar pre- and post-algorithm implementation. CONCLUSION: The use of a standardized algorithm significantly decreases the length of stay and duration of intravenous antibiotic administration without increasing readmission rates, while still providing appropriate treatment for HAEC. LEVEL OF EVIDENCE: III level. TYPE OF STUDY: Retrospective comparative study.

Primary laparoscopic endorectal pull-through procedure with or without a postoperative rectal tube for hirschsprung disease: a multicenter perspective study.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is a significant complication of HD both in the pre- and postoperative periods. This was a large multicenter series study to determine the effect of preserving a postoperative rectal tube on preventing HAEC after primary laparoscopic endorectal pull-through procedure. METHODS: Between 2014 and 2017, a total of 383 consecutive patients with rectosigmoid segment HD were randomly divided into group A (n = 190) and group B (n = 193). All of them underwent primary laparoscopic pull-through procedure, with the same postoperative treatment protocols except for group A with a rectal tube after surgery for 5 days, while group B did not have it. The mean time of follow-up was 2.0 ±â€¯0.53 years (0.5-3.6 years). Demographics, operative data, postoperative complications, and clinical outcomes were compared between these two groups. RESULTS: Outcomes within 1 month after surgery indicated a lower incidence of abdominal distention (4% vs 15.5%, P < 0.05) and postoperative HAEC (1.2% vs 6.8%, P < 0.05) in group A compared to group B. Beyond 1 month after surgery, the overall incidence of HAEC was not significantly different (12% vs 13.1%, P = 0.54). However, further analysis revealed that the patients who suffered HAEC twice or above twice in group A were significantly less than those in group B (3.6% vs 8.3%, p = 0.02). There were no significant differences in the defecation frequency and other complications. CONCLUSIONS: Primary laparoscopic endorectal pull-through procedure with a postoperative rectal tube can reduce the early-stage postoperative incidence of abdominal distension and HAEC and the risk of HAEC recurrence in the long term, and is beneficial to postoperative management. LEVEL OF EVIDENCE: Level 2.

Correlación entre el neumoperitoneo y los hallazgos quirúrgicos y morbimortalidad en recién nacidos con enterocolitis necrosante / Correlation between pneumoperitoneum and surgical findings and morbidity and mortality in newborns with necrotising enterocolitis

INTRODUCCIÓN: La intervención quirúrgica en las enterocolitis necrosantes (EN) es precisa cuando existe gangrena intestinal, hecho evidente cuando produce perforación y neumoperitoneo, siendo este la única indicación radiológica aceptada universalmente para la intervención quirúrgica. OBJETIVO: Analizar a los pacientes intervenidos de EN, saber por qué se les intervino, cómo evolucionan y si los pacientes perforados presentan neumoperitoneo. MÉTODO: Estudio retrospectivo de una cohorte de recién nacidos con EN intervenidos durante un periodo de 10 años (2006-2015). Se analizan los hallazgos radiológicos preoperatorios y se correlacionan con los quirúrgicos y con la morbimortalidad, dependiendo de la presencia de neumoperitoneo (N+) o no (N-). Se evaluó la concordancia interobservador con radiólogo pediátrico enmascarado a la clínica mediante el índice de acuerdo kappa. RESULTADOS: Se analizó a 53 pacientes. El 36% se intervino tras la visualización de neumoperitoneo; en el resto, la indicación fue deterioro clínico y metabólico, junto con hallazgos radiológicos asociados. En el 39% del grupo N- se objetivó perforación. No se encontraron diferencias significativas en ambos grupos con respecto a longitud intestinal resecada, días de intubación, día de inicio de nutrición enteral y mortalidad. La comparación entre duración de síntomas y estancia hospitalaria total en ambos grupos (N-/N+) fue significativa (7 vs. 2 días, p = 0,008; 127 vs. 79 días, p = 0,003 respectivamente), siendo más favorable en el grupo N+. Estas diferencias se mantuvieron al ajustar por peso. CONCLUSIONES: La indicación quirúrgica ha de basarse en un conjunto de datos clínicos y radiológicos, ya que el 39% de los pacientes sin neumoperitoneo presentaron perforación. En nuestro estudio la presencia de neumoperitoneo no se correlaciona con peor pronóstico

INTRODUCTION: Surgical intervention in necrotising enterocolitis (NEC) is correct when there is intestinal gangrene. This is evident when gangrene produces perforation and pneumoperitoneum, with this being the only universally accepted radiological indication for the surgical intervention of NEC. OBJECTIVE: To perform an analysis on patients with surgically managed NEC, including determining how the decision to intervene is reached, the outcomes, and if patients with perforation had a pneumoperitoneum. METHODS: Retrospective review of neonates with surgical NEC over a period of 10years (2006-2015). An analysis was made of pre-surgical x-ray findings, which were compared with surgical ones, in addition to the morbidity and mortality, depending on the presence (N+) or absence (N-) of pneumoperitoneum. An evaluation was also made of the interobserver concordance with a paediatric radiologist blinded to the clinical reason using the kappa agreement index. RESULTS: A total of 53 neonates were included in the study. Surgical treatment was indicated after observing pneumoperitoneum in 36%. In the remaining neonates, the surgical decision was made after noting a clinical and metabolic deterioration with classical x-ray findings. Intestinal perforation was observed in 39% of the N- neonates. There were no statistical differences between either group on analysing the excised intestinal length, days of intubation, starting of enteral nutrition, and the mortality rate. Comparisons in terms of duration of symptoms and total hospital stay were statistically significant (7 vs. 2 days, P = .008; 127 vs. 79 days, P = .003, respectively), with both being more favourable in the N+ group. These differences remained when the groups were adjusted by birthweight. CONCLUSIONS: Surgical indication has to be done on an ensemble of clinical and radiological evidence, as 39% of the neonates in the N- groups were perforated. In our study, the presence of a pneumoperitoneum did not correlate with a worse prognosis

Laparoscopic-assisted Duhamel procedure with ex-anal rectal transection for total colonic aganglionosis.

PURPOSE: Laparoscopic-assisted Duhamel procedure has a larger anastomosis and a reservoir which allows early recovery of defecation frequency, but concerns have been raised regarding the long operative time, high incidence of pouchitis and Hirschsprung associated enterocolitis (HAEC). The purpose of this study was to evaluate the postoperative complications and functional outcomes for patients with TCA undergoing modified laparoscopic-assisted Duhamel procedure (MLDP) with ex-abdominal partial colectomy and ex-anal rectal transection. METHODS: From 2011 to 2014, 16 patients with TCA who underwent MLDP were reviewed at our institution. Main modified techniques were to mobilize partial bowel through abdominal stoma opening, mobilize remaining colon, and dissect the retro-rectal space using laparoscopy, pull out and transect rectum ex-anally using a linear stapling device for creation of a short rectal pouch of 35~45mm. Seven patients who underwent classical laparoscopic Duhamel procedure (CLDP) with a long rectal pouch of 50-60mm between 2009 and 2011 were used as control group. Data were collected including demographics, laparoscopic technique, operative time, stool frequency, complications and continence outcomes. RESULTS: The operative time in MLDP group was significantly shorter than control group (3.0h vs. 4.7h, p=0.02). The incidence of postoperative HAEC in MLDP group was lower than control group (12.5% versus 42.9%; p=0.03) within the second postoperative year. Two patients (28.6%) experienced episodes of pouchitis in CLDP group and none was found in MLDP group. There was no significant difference in overall functional outcome between two groups, but the performance of MLDP group was better in terms of diapers required than CLDP group (1.80±0.45 vs. 1.00±0.64; p=0.02). All patients after 4years of age had a normal defecation frequency in both groups. CONCLUSIONS: MLDP is a safe, simple, and reliable technique for TCA. It has fewer postoperative complications due to the short rectal pouch. However, longer follow-up and a larger sample size are necessary to prove the efficacy in the treatment of TCA. LEVEL OF EVIDENCE: Level 3.

Pathologically assessed grade of Hirschsprung-associated enterocolitis in resected colon in children with Hirschsprung's disease predicts postoperative bowel function.

PURPOSE: The aim of this study was to investigate the relationship between the grade of enterocolitis on pathological assessment of resected colon and postoperative bowel function in children with Hirschsprung's disease (HD). METHODS: Children with HD who were seen at a large tertiary center from January 2012 to December 2013 were enrolled into this study. Resected colon was assessed using the histopathologic grade scoring system for Hirschsprung-associated enterocolitis (HAEC), and the relationship of these scores to postoperative bowel function was assessed. Time of recovery to normal defecation was the primary outcome measure. The t-test, analysis of variance, and Kaplan-Meier, univariate, and multiple regression analyses were performed. RESULTS: Eighty children with HD (median age at repair 7.9months; range 1.3months to 9years) were included in the study. Nineteen children dropped out of the study and were considered as providing censored data, giving a follow-up rate of 76.3%. A total of 21 children (34.4%) were admitted to hospital with at least one episode of enterocolitis. Multivariate Cox proportional hazards models showed that compared with patients with a normal proximal colon, those with an inflamed proximal segment had a 1.5-fold higher risk of a poor recovery. Logistic regression analyses suggested that postoperative HAEC admissions increased by 57% with each HAEC pathological grade of the transitional segment and by 50% with each grade of the overall segment. Compared with normal bowel in the transitional segment, the detection of grade ≥3 HAEC in the transitional area increased the incidence of postoperative HAEC by 4.75-fold. CONCLUSIONS: Children whose resected proximal colon showed inflammation on pathological assessment were at risk of poor recovery after surgery. A higher pathological HAEC score for the sum of the overall three segments suggested an increased risk for the subsequent development of enterocolitis. Among three segments, the severity of enterocolitis in the transitional segment was the most significant factor in predicting postoperative HAEC. TYPE OF STUDY: Clinical study. LEVEL OF EVIDENCE: Moderate.

Redo pull-through surgery in Hirschsprung disease: Short-term clinical outcome.

INTRODUCTION: Although surgery is effective in most patients with Hirschsprung disease (HD), some have persistent obstructive symptoms. Additional medical treatment is generally sufficient, but a small fraction of these patients needs secondary surgery. Series on redo surgery are scarce. Aim of this study is to evaluate complications and clinical outcome of patients in need of redo surgery for HD. MATERIALS AND METHODS: Sixteen patients underwent redo endorectal pull-through surgery in our center between 2007 and 2015. Medical records were reviewed and demographics, indication for redo surgery, surgical procedures, complications, and clinical outcome were scored. RESULTS: The median age at the time of redo was 4.6years (range: 2months-21years). Median follow-up after redo was 3years (range: 9months-7years). Before redo surgery, all patients (100%) had obstructive symptoms, one patient had recurrent enterocolitis, and four patients were fecally incontinent despite adequate attempts of bowel management. Surgical procedure consisted of a transanal endorectal pull-through (TERPT) in all patients, with additional laparotomy in 7 (44%) and protective stoma in 8 patients (50%). Complications within 30days after redo surgery were anastomotic dehiscence (3; 19%), wound abscess (2; 13%), rectovaginal fistula (1; 7%) or enterocutaneous fistula (1; 7%). During follow-up, nine patients needed additional surgery, mainly to close the stoma. At final follow-up there were no patients with stenosis, obstructive symptoms, remaining rectovaginal fistula, or small bowel obstruction. Only one patient experienced enterocolitis. Six patients (43%) reported soiling or fecal incontinence. CONCLUSION: TERPT for redo surgery for HD is effective in resolving sustained severe obstructive symptoms after primary surgery, but the outcome is complicated by a relatively high rate of soiling and fecal incontinence.

Altered tryptophan hydroxylase 2 expression in enteric serotonergic nerves in Hirschsprung's-associated enterocolitis.

AIM: To determine if expression of colonic tryptophan hydroxylase-2 (TPH2), a surrogate marker of neuronal 5-hydroxytryptamine, is altered in Hirschsprung's-associated enterocolitis. METHODS: Entire resected colonic specimens were collected at the time of pull-through operation in children with Hirschsprung's disease (HSCR, n = 12). Five of these patients had a history of pre-operative Hirschsprung's-associated enterocolitis (HAEC). Controls were collected at colostomy closure in children with anorectal malformation (n = 10). The distribution of expression of TPH2 was evaluated using immunofluorescence and confocal microscopy. Protein expression of TPH2 was quantified using western blot analysis in the deep smooth muscle layers. RESULTS: TPH2 was co-expressed in nitrergic and cholinergic ganglia in the myenteric and submucosal plexuses in ganglionic colon in HSCR and healthy controls. Co-expression was also seen in submucosal interstitial cells of Cajal and PDGFRα(+) cells. The density of TPH2 immuno-positive fibers decreased incrementally from ganglionic bowel to transition zone bowel to aganglionic bowel in the myenteric plexus. Expression of TPH2 was reduced in ganglionic bowel in those affected by pre-operative HAEC compared to those without HAEC and healthy controls. However, expression of TPH2 was similar or high compared to controls in the colons of children who had undergone diverting colostomy for medically refractory HAEC. CONCLUSION: Altered TPH2 expression in colonic serotonergic nerves of patients with HSCR complicated by HAEC may contribute to intestinal secretory and motor disturbances, including recurrent HAEC.

The Surgical Treatment of Toxic Megacolon in Hirschsprung Disease.

OBJECTIVES: Enterocolitis remains the most significant cause of morbidity and mortality in Hirschsprung disease (HD). It could progress into toxic megacolon (TM)-acute dilatation of the colon as accompanying toxic complication of Hirschsprung enterocolitis. It is a devastating complication, especially in infants with so far undiagnosed HD. METHODS: A retrospective analysis of medical records of 4 infants with TM was performed. The diagnosis TM was determined on the basis of clinical information (abdominal pain or tenderness, abdominal distension, diarrhea, bloody diarrhea, and constipation), plain x-rays of the abdomen (segmental or total colonic dilation), and the presence of such criteria (fever, high heart rate, increased white blood cell count, C reactive protein, anemia, dehydration, electrolyte disturbances, hypotension). Surgical management and outcome was evaluated by retrospective chart review. RESULTS: The median duration of symptoms characteristic for TM was 3 days. Toxic megacolon was seen as the first manifestation of previously unknown HD in 3 patients; in 1 newborn, the contrast radiograph was suggestive of HD. In all patients, conservative treatment was failed. Three patients were treated with surgical decompression and ileostomy only. In all these cases, severe complications occurred, consequently 2 of them died. In 1 patient, a resection of the transverse dilated colon additionally was performed. This patient had no complications in postoperative period and survived. CONCLUSIONS: Because of the high mortality in patients with TM that were treated medically or with colonic decompression, a resection of massively distended part of the colon should be performed.

Resultados del descenso endorrectal transanal en la enfermedad de Hirschsprun / Results of transanal endorrectal descent in Hirschprung’s disease

OBJETIVO: Presentar nuestra experiencia en el tratamiento de pacientes con enfermedad de Hirschsprung (EH) intervenidos mediante descenso endorrectal transanal (DET). MÉTODOS: Estudio retrospectivo de los DET realizados. Correlacionamos, fundamentalmente, longitud del segmento agangliónico, con complicaciones, necesidad de colostomía y vía de abordaje. RESULTADOS: Entre 2003 y 2012 hemos realizado 73 DET (57 varones), 78,6% diagnosticados en el periodo neonatal. La longitud agangliónica objetivada en el enema se correspondió con los hallazgos quirúrgicos en un 68,9% de los casos; esta correspondencia fue mayor en las formas cortas que en las largas o pancolónicas (80,9 vs. 44%; p < 0,001). Cuatro pacientes precisaron colostomía preoperatoria. La edad media al descenso fue de 6,3 meses (rango 4,5-33,7). El 64,4% eran formas cortas. El abordaje abdominal fue necesario en 10 pacientes (13,6%), 9 de ellos aganglionismos largos o pancolónicos. El 10,9% (8 pacientes) ha presentado enterocolitis postoperatoria, sin relación con la longitud agangliónica ni con la existencia de displasia residual. La suboclusión por bridas se presentó en el 19,2% de las formas largas o pancolónicas. Actualmente, de los pacientes valorables, 5 tienen hábito estreñido y 12 presentan escapes ocasionales. CONCLUSIONES: El DET es la cirugía de elección para pacientes con EH. La mayoría pueden manejarse con «nursing» preoperatorio, sin colostomía. Las pruebas diagnósticas de elección son la manometría y la biopsia rectal por succión. No hemos encontrado correlación entre la existencia de enterocolitis y la longitud del segmento agangliónico, aunque hemos observado una reducción drástica de las enterocolitis postoperatorias, desde la introducción de modificaciones técnicas, como la sonda rectal posquirúrgica y la sección posterior del manguito muscular

OBJECTIVE: To present our experience of patients with Hirschsprung disease (HD) operated by transanal endorrectal descent (TED). METHODS: A retrospective study performed of TEDs. We correlate fundamentally the aganglionic segment length with: Complications, need for colostomy and surgical approach. RESULTS: Between 2003 and 2012 we performed 73 TED (57 men), 78.6% diagnosed in the neonatal period. The aganglionic segment length evidenced in the enema was correlated with surgical findings in 68.9% of cases, being higher in the short forms than in the long segments (80.9 vs. 44%, P<.001). Four patients required preoperative colostomy. The mean age of surgery was 6.3 months (4.5 to 33.7). Short forms represented 64.4% of cases. The abdominal approach was necessary in 10 patients, 9 of these were long/total colonic aganglionic segment. Postoperative enterocolitis presented in 10,9% (9 patients), with no relation with the length of aganglionic or the existence of residual dysplasia. Obstructions occurred in 19.2% of the long form or pancolonic forms. Currently, of the evaluable patients, 5 present constipationand 12 present occasional leakage. CONCLUSIONS: TED is the surgery of choice for patients with HD. The majority can be handled by preoperative «nursing» without colostomy. The diagnostic tests of choice are manometry with suction biopsy. No significant correlation was found between enterocolitis and length of aganglionic segment, although we observed a drastic reduction of postoperative enterocolitis, since the introduction of technical changes, such as the use of postoperative rectal tubes and the posterior section of the cuff muscles

Reoperative surgery for Hirschsprung disease.

Despite most children undergoing a successful pull through for Hirschsprung disease, a small portion of children are left with persistent stooling issues. Most of these stooling issues can be addressed by nonoperative approaches. However, in a small group of remaining children, a reoperation may be necessary. Most children who may need a redo pull-through procedure may have a persistent area of aganglionosis, unremitting enterocolitis, or a torsion or stricture of the pull-through segment. Each of these influences the approach the surgeon must take to correct the presenting problem. The chapter details the diagnostic approach as well as the operative techniques, which best deal with each of these complications.