Critical evaluation of the Hirschsprung-associated enterocolitis (HAEC) score: A multicenter study of 116 children with Hirschsprung disease

Objective

To identify the optimal clinical criteria to diagnose Hirschsprung-associated enterocolitis (HAEC) in children with Hirschsprung disease (HSCR).

Primary pull-through with modified Duhamel technique: 1 institution's experience

Background/Purpose

Primary pull-through for Hirschsprung disease has been reported to give comparable surgical outcomes to staged operations with less morbidity. The aim of this study was to review the early experiences in our institution with the primary pull-through option.

Methods

Medical records of 50 consecutive patients with Hirschsprung disease who underwent a modified Duhamel pull-through operation from 2001 to 2004 were retrospectively reviewed. For analysis, cases were divided into 2 groups: primary pull-through (group I, 30 patients), and patients with a previously established colostomy who underwent a staged operation (group II, 20 patients). Comparisons were made regarding operating time, operative morbidity, and medium-term functional outcomes.

Results

There was no difference between the groups with respect to median age and weight at time of surgery, sex ratio, presenting symptoms, and extent of aganglionosis. Seventy-four percent of the procedures were performed on infants (group I, 21 [70%] of 30 patients; group II, 16 [80%] of 20 patients). Acute postoperative complications were experienced by 4 (13.3%) of 30 patients in group I and 4 (20%) of 20 patients in group II (P <.05). Postoperative enterocolitis occurred in 11 (36.7%) of 30 patients in group I and in 7 (41.2%) of 17 patients in group II.

Conclusions

Our experience indicates that the primary pull-through using a modified Duhamel technique gave neither difference in functional outcomes nor medium-term complications compared to a staged procedure. Considering a sparing of colostomy and a potential economic benefit, the procedure should be considered in selected patients.     

腹腔镜辅助改良Soave术治疗成人先天性巨结肠症

目的 评价腹腔镜辅助改良Soave术治疗成人先天性巨结肠症（HD）的临床价值.方法 回顾性分析华中科技大学同济医学院附属协和医院2005年3月至2009年12月间行腹腔镜辅助改良Soave术的28例术前诊断为成人HD患者的临床资料.结果 本组28例患者均成功实施了腹腔镜辅助改良Soave术,无中转开腹.手术时间135～185（165±12）min,术中出血量50～250 ml,无一例术中输血.术后病理诊断示：19例符合HD,9例符合先天性巨结肠类缘病.术后直肠肌鞘感染2例,肛门口轻度污粪3例,平均住院时间（17.5±1.0）d.术后随访无排粪失禁及便秘复发.结论 腹腔镜辅助改良Soave术治疗成人HD安全、有效.     

Modified Soave procedure through the posterior sagittal approach for Hirschsprung's disease

Purpose

The objective of this study is to describe the surgical technique and clinical results of a modified Soave procedure through the posterior sagittal approach (PSAP) for Hirschsprung's disease.

Methods

The patient was placed in a prone jackknife position. The rectum was approached via PSAP keeping the external sphincter intact. The rectum was isolated and then divided approximately 2 cm from the dentate line. The rectum and sigmoid were freed up to the normal segment. The aganglionic and ganglionic segments were confirmed intraoperatively by frozen biopsy. The rectal mucosectomy was started 0.5 to 1 cm proximal to the dentate line, leaving 1 cm of the muscular cuff. The colon was then pulled through the anus. The aganglionic segment and transitional zone were removed. Coloanal anastomosis was fashioned 0.5 to 1 cm proximal to the dentate line.

Results

Ninety-six patients were operated on from January 2000 to July 2002, using the described technique. Patient ages ranged from 2 to 36 months (median age, 8½ months). The aganglionic segment was located in the rectum in 56 patients and in the sigmoid colon in 40 patients. The average length of bowel resected was 20 cm (range, 10-35 cm). Combined laparotomy was performed in 2 patients and combined laparoscopy surgery in 1 infant. There were no operative or postoperative deaths. Anastomotic leakage occurred in 2 patients. Spontaneous defecation occurred in all patients.

Conclusions

Posterior sagittal approach provides excellent exposure of the operative field and decreases the major complications seen with the abdominal approach. The modified Soave procedure can easily be performed with PSAP.     

Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung's disease

Purpose

The aim of this study was to determine the morbidity and medium-term functional outcome of the Duhamel operation and laparotomy and transanal endorectal coloanal anastomosis (TECA) for Hirschsprung’s disease (HSCR).

Methods

The study populations were 34 consecutive children who underwent the Duhamel operation (or Lester Martin modification) and 37 who had the TECA. Demographic details were obtained by case note review, and functional outcome was determined by a combination of outpatient interview, questionnaire, and telephone enquiry.

Results

There was no difference between the groups with respect to age, gender, and length of aganglionic segment. Seventy percent presented as neonates (Duhamel, 24 of 34; TECA, 26 of 37). A single-stage primary pull-through was performed in 17 of 37 children in the TECA group, and in 1 of 34 from the Duhamel group. There was a single perioperative death in the Duhamel group and an unrelated, late death in the TECA group. Postoperative enterocolitis was seen in 13 of 37 TECA children and in a single child from the Duhamel group. A stricture of the pull-through segment was seen in 7 of 37 children after TECA and required temporary diversion in 2 of 9. Late division of a rectal spur was required in 6 of 33 Duhamel children. Requirement for late myectomy was the same in both groups (Duhamel 3 of 33, TECA 4 of 37). Complications requiring stoma formation occurred in 5 of 37 after TECA and 2 of 33 after the Duhamel operation. Two children from the TECA group and 1 from the Duhamel group remain diverted. One child from each group required a re-pull-through procedure. Two patients were lost to follow-up in the TECA group, leaving 34 children in this group and 33 in the Duhamel group in whom functional outcome could be assessed. Functional outcome was similar in the 2 groups.

Conclusions

TECA and Duhamel procedures have similar medium-term functional outcomes. TECA has a high incidence of postoperative enterocolitis and transient stricture formation but is suitable for single-stage neonatal treatment of HSCR.     

Duhamel operation vs. transanal endorectal pull-through procedure for Hirschsprung disease: A systematic review and meta-analysis

Objective

To compare treatment outcomes in children with Hirschsprung's disease who underwent treatment using the Duhamel or TERPT surgical procedures.

Hirschsprung-associated enterocolitis develops independently of NOD2 variants

Backgroud/Purpose

Hirschsprung-associated enterocolitis (HAEC) represents a cause for significant pre- and postoperative morbidity and mortality in Hirschsprung disease (HD). Although multiple studies on HAEC have been performed and several mechanisms have been presumed, the pathogenesis of this condition remains unclear. As changes in colonic mucosal defense are key factors suggested in both Crohn's disease (CD) and HAEC pathogenesis, the aim of the current study was to investigate genetic alterations in the most important susceptibility gene for Crohn's enterocolitis (NOD2) to see whether carriers of polymorphisms within the NOD2 gene are predisposed to the development of HAEC.

Methods

Genotyping for the NOD2 variants in exon 4 (p.Arg702Trp [rs2066844]), exon 8 (p.Gly908Arg [rs2066845]), and exon 11 (p.1007fs [rs2066847]) was performed in 52 white children with HD (41 boys, 11 girls), 152 healthy controls, and 152 children with CD (onset of disease <17 years; mean, 11.8 years). Seventeen patients with HD (32.7%) were carriers of a RET germline mutation, 35 children (67.3%) had short segment disease, and 17 (32.7%) had long segment disease.

Results

Ten children (19.2%) with HD were heterozygous carriers of at least one NOD2 variant vs 17 (11.2%) in the healthy control group and 69 (45.4%) in the CD cohort. Hirschsprung-associated enterocolitis was observed in 7 children (13.5%), with 4 having short segment HD and 3 with long segment HD; but none of them were carriers of NOD2 variants.

Conclusion

Our study shows that NOD2 variants described to be causatively associated with CD do not predispose to the development of HAEC. As data on the molecular basis of HAEC are limited, the distinct mechanisms involved in the pathogenesis of this complication remain unclear.     

Transanal endorectal pull-through in children with Hirschsprung's disease—technical refinements and comparison of results with the Duhamel procedure

Purpose

Transanal endorectal pull-through (TEPT) has drastically changed the treatment of Hirschsprung's disease (HD). A short follow-up of children submitted to TEPT reveals results that are similar to the classic transabdominal pull-through procedures. However, few reports compare the late results of TEPT with transabdominal pull-through procedures with respect to complication rates and the fecal continence. The aims of the present work are to describe some technical refinements that we introduced in the procedure and to compare the short and long-term outcome of TEPT with the outcomes of a group of patients with HD who previously underwent the Duhamel procedure.

Methods

Thirty-five patients who underwent TEPT were prospectively studied and compared to a group of 29 patients who were treated with colostomy followed by a classical Duhamel pull-through. The main modifications introduced in the TEPT group were no preoperative colon preparation, operation conducted under general anesthesia in addition to regional sacral anesthesia, use of only one purse-string suture in the rectal mucosa before transanal submucosal dissection, and no use of retractors and electrocautery during the submucosal dissection.

Results

The most frequent early complications of TEPT group were perineal dermatitis (22.8%) and anastomotic strictures (8.6%). The comparison with patients who underwent Duhamel procedure revealed no difference in the incidence of preoperative enterocolitis, the patients of the TEPT group were younger at the time of diagnosis and of surgery, they had shorter operating times, and they began oral feeding more quickly after the operation. The incidence of wound infection was lower in the TEPT group. Moreover, the TEPT and Duhamel groups showed no difference in the incidences of mortality, postoperative partial continence, and total incontinence. Although the incidences of complete continence and postoperative enterocolitis were not different, a tendency to the increased incidence in the TEPT group was observed.

Conclusions

This study further supports the technical advantages, the simplicity, and the decreased incidence of complications of a primary TEPT procedure when compared to a classical form of pull-through. Some technical refinements are described, and no preoperative colon preparation was necessary for the patients studied here. The results show that the long-term outcomes of the modified TEPT procedure are generally better than those obtained with classical approaches.     

The problematic Soave cuff in Hirschsprung disease: Manifestations and treatment

Purpose

Following a Soave pull-through for Hirschsprung disease (HD), some children struggle with obstructive symptoms. We hypothesized that these symptoms could result from a functional obstruction of the pull through caused by the Soave cuff, and that cuff resection might improve bowel emptying.

Methods

We reviewed patients referred to our center from 2008 to 2012 with obstructive problems following a Soave pull-through for HD (CCHMC IRB # 2011–2019). Only patients with an obstructing Soave cuff were analyzed. Patients with other reasons for obstruction (anastomotic stricture, transition zone, aganglionic segment) were excluded.

Results

Thirty-six patients underwent reoperation at our center for obstructive symptoms after an initial Soave pull-through. Seventeen of these patients had a Soave cuff only as the potential source of obstruction. Pre-operative symptoms included enterocolitis (10), constipation (6), and failure to thrive (1). Nine patients (53%) required irrigations to manage distension or enterocolitis pre-operatively. 14/17 patients (82%) had a palpable cuff on rectal exam. Eight patients (47%) had radiographic evidence of a cuff demonstrated by distal narrowing (4) or a prominent presacral space (4). Four children (23%) underwent excision of the cuff only. Thirteen (76%) had removal of the cuff and proximally dilated colon [(average length 7.2 cm) (12 performed transanally, and five needed laparotomy as well.)] Post-operatively, episodes of enterocolitis were reduced to zero, and need for irrigation to treat distension was reduced by 50%. Nine patients have voluntary bowel movements, and five are clean on enemas. 3/6 patients with pre-operative constipation or impaction now empty without enemas. (Follow up 1–17 months, mean 7 months.)

Conclusions

Recurrent enterocolitis, constipation, or failure to thrive can indicate a functional obstruction due to a Soave cuff when no other pathologic cause exists. Physical exam or contrast enema can identify a problematic cuff. Reoperation with cuff resection can dramatically improve bowel emptying.     

Duhamel procedure: a comparative retrospective study between an open and a laparoscopic technique

Background Few studies are available comparing open with laparoscopic treatment of Hirschsprung’s disease. This study compares a laparoscopic series of 30 patients with a historical open series of 25 patients. Methods The charts of all patients having had a Duhamel procedure in the period from June 1987 through July 2003 were retrospectively reviewed. Open procedures were performed until March 1994. Patients with extended aganglionosis, pre-Duhamel ostomy, or syndrome were excluded from the study. End points were intraoperative complications, postoperative complications, time to first feeding, hospital stay, and outcome at follow-up such as stenosis, enterocolitis, constipation, fecal incontinence, and enuresis. Results Twenty-five patients had an open Duhamel (OD) and 30 had a laparoscopic one (LD). There were no differences in patient characteristics and there were no intraoperative complications in either group. Time to first oral feeds was significantly longer in the OD group as was the duration of hospital stay. No significant differences at follow-up were observed but there was a tendency for a higher enterocolitis rate in the LD group. In contrast, the adhesive obstruction and enuresis rates were higher in the OD group. Cosmetic results were superior in the LD group. Conclusions Except for a significantly shorter hospital stay and shorter time to first oral feeds in favor of LD, no significant differences could be observed. The cosmetic result was not an end point but there was no doubt that it was better in the LD group. Although not statistically significant different, there were no adhesive bowel obstructions in the LD group compared with 3 of 25 in the OD group. Fecal incontinence was not encountered in either group.     

Early adult outcome of the Duhamel procedure for left-sided Hirschsprung disease--a prospective serial assessment study

Purpose

To assess both early adult functional outcome and change in long-term functional outcome over time after the Duhamel procedure (DP) for left-sided Hirschsprung disease (HSCR).

Methods

The study population consisted of 78 children (aged 19.9 ± 3.6 years) who previously underwent objective outcome assessment after DP was performed for HSCR during the period of 1980 to 1991. Inclusion criteria were previous evaluation of functional outcome and either rectosigmoid or left-sided HSCR. Outcome measures were assessed twice within the cohort, in 1997 and in 2005. The primary outcome measure was the Rintala (J Ped Surg. 1995;30:491-494) functional outcome score (FOS; maximum, 20). Controls consisted of 20 age-matched healthy children. Satisfactory functional score was defined as an FOS at or above the 10th percentile of controls (FOS, ≥17). Secondary outcome measures were the operation failure rate (defined by requirement for a stoma or major reoperative surgery), and enterocolitis rates (defined by intention to treat). Consecutive outcome scores were compared by paired t test. Data were expressed as mean ± SD, and P < .05 was considered significant.

Results

Operation failure occurred in 9 (11.5%) of 78. Consecutive FOSs were obtained in 40 (57%) of 69. A satisfactory functional score was observed in 23 (58%) of 40 adults as opposed to 33 (47%) of 70 children 8 years previously (P = .02). Satisfactory outcome (defined by satisfactory functional score and lack of enterostomy or major revision pull-through procedure) was observed in 23 (47%) of 49. Previously, this figure was 34 (44%) of 78. Individual paired FOSs showed a significant improvement with time (1997: 14.9 ± 4.1; 2005: 16.4 ± 2.8; P = .02).

Conclusions

At early adult follow-up, the operation failure rate has not changed from that of the same cohort 8 years earlier. However, a significant improvement in individual FOSs was demonstrated.     

改良Soave术吻合器吻合治疗先天性巨结肠症

目的探讨改良Soave术吻合器吻合根治先天性巨结肠症(HD)的临床价值。方法回顾性分析解放军第477医院2002年1月至2010年12月期间采用改良Soave术经肛门直肠黏膜与近端肠管端端吻合器吻合治疗的26例HD患者的临床资料。结果改良Soave术吻合器吻合26例患者均顺利完成手术,无吻合口漏、直肠肌鞘感染及小肠结肠炎并发症发生。2例术后出现大便次数增多,分别于经口服收敛药物后15d及30d恢复正常。轻度污粪1例。所有病例均获得随访,随访时间3～60个月,平均32个月,无排粪失禁及便秘发生。结论改良Soave术吻合器吻合,治疗年长儿童及成人HD安全、有效。     

三个月内婴儿巨结肠症腹腔镜Soave改良根治术探讨

目的:总结腹腔镜Soave改良根治术治疗3个月内婴儿巨结肠症的临床经验。方法:为45例巨结肠患儿施行腹腔镜Soave改良根治术并对术后发生并发症的21例进行分析。结果:45例均治愈出院,术后随访3个月至4年,患儿生长发育良好,食欲及大小便正常,无污粪。结论:腹腔镜下巨结肠Soave改良根治术对婴儿打击小,创伤轻,手术风险低,安全系数高,手术效果好。在腹腔镜辅助下可将巨结肠患儿的手术年龄提前至新生儿和小婴儿期,早期治愈有利于患儿身心健康及发育。     

Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC)

PurposeThis study aims to characterize risk factors for Hirschsprung-associated enterocolitis (HAEC). We hypothesize that earlier pull-through surgery is associated with lower risks of developing postoperative HAEC.MethodsA comparative study of 171 Hirschsprung patients treated from 1990 to 2017 was performed. Patients without HAEC were compared to patients with preoperative and/or postoperative HAEC. Results are presented as median [IQR] or frequency (%). Pearson's χ² test and Wilcoxon rank sum test were performed with a significance level at p < 0.05. Multivariable logistic regression analysis was used to adjust for potential confounders. A subanalysis was done to evaluate laparoscopic, laparotomy, and transanal surgeries.ResultsRisk of developing preoperative HAEC was significantly associated with congenital malformations (OR 2.63 [1.11, 6.24]; p = 0.02). Birth weight was lower in patients with preoperative HAEC (OR 0.48 [95% CI 0.25, 0.93]; p = 0.03). On regression analysis, intestinal obstruction after surgery was significantly associated with postoperative HAEC (OR 8.2 [3.18, 21.13]; p < 0.0001). Patients with earlier pull-through surgery did not have a lower risk of developing postoperative HAEC.ConclusionsTiming of surgery does not seem to be associated with a higher risk of developing pre- and postoperative HAEC. Predisposing factors for preoperative HAEC included associated malformations and lower birth weight, whereas intestinal obstruction was found to be associated with postoperative HAEC.Type of studyTreatment study.Level of evidenceLevel III.     

Murine model of Hirschsprung-associated enterocolitis II: Surgical correction of aganglionosis does not eliminate enterocolitis

Background

Hirschsprung disease (HD) results from aganglionosis of the colon, is linked to acute and chronic enterocolitis (known as Hirschsprung-associated enterocolitis) despite successful corrective surgery, and can lead to bacteremia and even death. The genetic and molecular mechanisms underlying these disorders are largely unknown.

Methods

We developed a microsurgical corrective pull-through procedure in mice, and applied that to Ednrb^−/− mice, which manifest aganglionic megacolon that is very similar to HD. Wild-type littermates (Ednrb^+/+) also underwent identical surgery. At prespecified time points postoperatively, mice were sacrificed, and histopathologic analyses of intestinal inflammation were performed. Mice of both genotypes were sacrificed after the postoperative recovery period to determine if corrective surgery itself caused inflammation. Stooling patterns were assessed as well to determine if intestinal function normalized after surgery.

Results

There was no difference in histopathological enterocolitis scores after recovery from surgery. Stooling patterns in Ednrb^−/− and Ednrb^+/+ mice were similar postoperatively, suggesting normalization of intestinal function. However, with time, approximately 40% of Ednrb^−/− mice developed clinical illness consistent with enterocolitis. No control (Ednrb^+/+) mice developed clinical enterocolitis. Histopathological enterocolitis scores in the 40% of Ednrb^−/− mice that developed clinical enterocolitis postoperatively were significantly worse than those of healthy postoperative Ednrb^−/− mice. In contrast, none of the Ednrb^+/+ control mice exhibited postoperative long-term inflammation.

Conclusions

Microsurgical pull-through operation in Ednrb^−/− mice produces a mouse model that closely resembles key features of Hirschsprung-associated enterocolitis, enabling controlled study of genetic and molecular mechanisms in Ednrb^−/− mice and other genotypes that produce similar phenotypes.     

腹腔镜辅助Soave根治术治疗新生儿先天性巨结肠(附73例报告)

目的:探讨腹腔镜辅助Soave根治术治疗新生儿先天性巨结肠的临床疗效及安全性。方法:回顾分析2002~2010年应用腹腔镜辅助Soave根治术治疗73例新生儿先天性巨结肠的临床资料。结果:73例手术均获成功,患儿腹胀、便秘等临床症状消失,大便每天5~19次,腹部切口均愈合良好。术后小肠结肠炎6例,肛周皮炎23例,污粪5例,无复发、肠粘连、吻合口瘘、结肠回缩等并发症发生。术后随访,随小儿年龄增长,大便次数逐渐减少,术后3个月大便呈糊状,6~12个月后大便接近正常,排便功能优良率98%。结论:腹腔镜辅助Soave根治术治疗新生儿先天性巨结肠具有患儿创伤小、操作简单、安全、有效、并发症少等优点,特别是对长段型巨结肠、全结肠型巨结肠优势明显。     

A comparison of the effectiveness of the Soave and Martin procedures for the treatment of total colonic aganglionosis

Purpose

Total colonic aganglionosis (TCA) is a relatively uncommon and severe condition managed by pediatric surgeons. Several procedures exist for the treatment of TCA. However, there is no current consensus on a superior operative procedure. The objective of this article is to evaluate the comparative effectiveness of the Soave procedure and the Martin procedure in the treatment of TCA with respect to preoperative data and postoperative outcome.

Methods

In the period from January 2001 to June 2008, we recruited 29 patients with TCA who were treated with the Martin procedure (14) or the Soave procedure (15). Relevant data were collected from the medical charts kept in the hospital library. The follow-up study used a detailed questionnaire that was answered by patients either by telephone or directly in the clinic. In the clinic, data, including age, body weight before operation, intraoperative blood transfusion, anastomotic leakage, postoperative enterocolitis, durations of any postoperative fever, days in hospital, and a range of functional outcomes, were particularly recorded to enable a comparison of the effectiveness of the Soave procedure and the Martin procedure.

Results

A male predominance has been described for patients with TCA, with a male-to-female ratio of approximately 6.25:1. In the Soave group, time between ileostomy and the definitive procedure, body weight before the definitive procedure, and the operative age were less than those in the Martin group (P < .05). In patients who underwent the Martin procedure, the average duration of postoperative fever and days in hospital were significantly more than those in the Soave group (P < .05). In the Soave group, the incidence of intraoperative blood transfusion (46.7%), abdominal wound infection (6.67%), and anastomotic leakage (0) was significantly less (P < .05) than those in the Martin group (92.8%, 28.5%, and 14.3%, respectively). In the follow-up study, 7 patients in the Martin group had enterocolitis, and 4 of them had severe enterocolitis leading to multiple hospitalizations. In contrast, in the Soave group, only 2 patients had enterocolitis, and 1 was ameliorated after the therapy of anus dilation in the clinic. During the 4 weeks after the operation, all patients had an increased frequency of defecation, with soiling in the perianal area. At 3 months after the operation, there were significantly more patients with normal defecation in the Martin group compared to the Soave group (P < .05). Six months later, 11 (78.6%) patients in the Martin group and 8 (61.5%) patients in the Soave group had normal defecation and the difference was still significant (P < .05).

Conclusion

Patients with TCA managed with the Soave procedure had fewer operative complications compared with those of patients who underwent the Martin procedure. However, the patients managed with the Soave procedure took longer to establish normal defecation. Although each procedure has advantages and disadvantages, the Soave procedure is promising for the treatment of TCA, especially with regard to postoperative complications. A long-term follow-up study is needed.     

Hirschsprung's enterocolitis, prostaglandins, and response to cholestyramine

An infant with Hirschsprung's enterocolitis developed a fulminant secretory diarrhea unresponsive to all conventional therapy until cholestyramine was administered. A 12-fold decrease in prostaglandin E (PGE) levels in the colostomy fluid was documented in response to cholestyramine therapy. It is postulated that increased PGE activity, enterotoxin, and bile acid malabsorption may be involved in the enterocolitis of Hirschsprung's disease.