Reoperations in Hirschsprung disease

Background

We sought to identify causes of preventable complications related to operations for Hirschsprung disease.

Methods

We reviewed the cases of 51 patients with Hirschsprung disease who underwent a primary procedure elsewhere, had a complication, and were referred for reoperation.

Results

Thirty-five patients had 1 failed operation, 10 had 2, and 6 had 3. Initial operations were Soave (20), Duhamel (15), Swenson (5), transanal endorectal (4), myectomy (3), unknown (3), and laparoscopic Swenson (1). Thirty-one patients presented with a stoma. Patients without a stoma (20) had fecal impaction (8), recurrent enterocolitis (6), and fecal incontinence (6). None had both enterocolitis and incontinence. Reoperation was performed posterior sagittally (40) or transanally (5). Indications included stricture (21), megarectal Duhamel pouches (12), fistulae (11 [8 rectocutaneous, 2 rectourethral, and 1 rectovaginal]), pouchitis (2), and retained aganglionic bowel (8). After reoperation, 14 were continent, 11 had a stoma (8 permanent), 6 had voluntary bowel movements but soiled occasionally, 6 received rectal irrigations to avoid enterocolitis, 6 were incontinent but clean with bowel management, and 2 were lost to follow-up.

Conclusion

Stricture, megarectal pouch, fistula, and retained aganglionic bowel are preventable complications. Enterocolitis is partially preventable but can occur after a technically correct procedure. Fecal incontinence is a preventable complication likely because of anal canal damage.     

Redo pull-through in Hirschsprung's [corrected] disease for obstructive symptoms due to residual aganglionosis and transition zone bowel

Background

Reoperations in Hirschsprung disease may be required for residual aganglionosis or transition-zone bowel found at the distal pull-through. We aimed to review the management of patients who had this complication and offer suggestions on how to avoid it.

Methods

Ninety-three patients with Hirschsprung disease were referred to our institution with recurrent problems after a pull-through done elsewhere. All required reoperations with a variety of indications, and of these, 25 had residual aganglionosis/transition-zone histology. This was the only indication for redo in 16 children.

Results

Children (range, 2-17 years) presented 6 to 66 months after the initial pull-through. The predominant symptoms were enterocolitis (n = 9 [56%]), constipation (n = 7 [44%]), failure to thrive (n = 5 [31%]), and impaction (n = 4 [25%]). The rectal biopsy performed as part of their post pull-through work up showed hypertrophic nerves (n = 16), absent ganglion cells (n = 6), and normal ganglion cells (n = 10). The original frozen-section biopsy, determining the level of the pull-through, only sampled the seromuscular layer in 3 children, leading to misdiagnosis. Reoperations involved a transanal resection (n = 15) and a posterior sagittal approach (n = 1). In all cases, obstructive symptoms were resolved, and no patient has had recurrent enterocolitis.

Conclusion

Patients' post pull-through with recurrent obstructive symptoms may have residual aganglionosis or transition-zone bowel. Reoperation can result in the resolution of these symptoms. A full-thickness biopsy at the time of the initial pull-through to include the mucosa and submucosa may increase the possibility of identifying hypertrophic nerves.     

先天性巨结肠术后便秘复发再手术37例临床分析

目的总结先天性巨结肠术后便秘复发的原因及再手术的治疗经验。方法分析37例先天性巨结肠术后便秘复发再手术患儿的临床资料。结果37例患儿术后便秘复发的主要原因为肠管切除不足14例,合并肠神经发育不良6例,闸门综合征5例,其他原因12例。再手术后无1例死亡;31例(83.8%)获6个月至11年的随访。术后15例排便1～2次/d,7例1次/2d,6例1次/3d;1例Rehbein术者(该术式本身保留了过多的病变直肠)和1例Soaves术者(再手术后未及时行肛管扩张)每周排便1～2次,仍间断使用缓泻剂;1例Soaves术者术后有粪液污裤现象。均无大便失禁者。结论先天性巨结肠便秘复发的主要原因为肠管切除不足;复发后再手术效果良好。     

Clinical outcome in children after transanal 1-stage endorectal pull-through operation for Hirschsprung disease

Background/Purpose

Recently, the transanal 1-stage pull-through operation has been widely used in Hirschsprung disease (HD), and it is obviously superior to traditional approach in early term for its noninversion. However, the procedure is relatively so new that it makes assessment of the functional outcome and stooling patterns difficult. The aim of this study was to evaluate the clinical outcomes of the transanal 1-stage endorectal pull-through operation in the management of rectosigmoid HD.

Methods

Fifty-eight children (39 boys and 19 girls) aged 12 months to 13 years (mean, 2 years) who underwent transanal 1-stage endorectal pull-through operation for HD were followed up from 6 to 24 months. Clinical outcome was assessed by interviews and questionnaires. All patients had an aganglionic segment confined to the rectosigmoid area which was confirmed by the preoperative barium enema and postoperative pathological examination.

Results

Forty-six patients had satisfactory results without complications. In all the children, the mean stool times were 1 to 2 per day; only 4 had mean stool times of 8 to 10 per day. Postoperative soiling was present in 9, constipation in 5, and HD-associated enterocolitis in 3. There were no incontinence, cuff infection, anastomotic leak, and mortality in any of the patients. In the 12 symptomatic patients, there were 4 children with length of aganglionic segment less than 30 cm, and 8 had 30 cm or more. In the 46 asymptomatic patients, 42 had length of aganglionic segment less than 30 cm, and 4 had 30 cm or more. There was a significant difference between the group with less than 30 cm and the group with 30 cm or more of aganglionic segment. For statistical analysis, the Fisher exact test showed P < .05.

Conclusions

The transanal 1-stage endorectal pull-through is a feasible and safe procedure in children with rectosigmoid HD. The clinical outcome is satisfactory. A gradual recovery could be noted in the stooling patterns along with the time after surgery. The younger the patient operated on and the shorter the aganglionic segment, the lower do the stooling disorders occur and the faster does the stooling function recover.     

Case report of a skip segment Hirschsprung’s disease: A real phenomenon

Introduction and importanceHirschsprung’s disease is a congenital anomaly that results from an incomplete craniocaudal migration and maturation of intestinal ganglion progenitor cells leading to distal intestinal aganglionosis. Skip segment Hirschsprung’s disease is an extremely rare phenomenon. We report a case involving only the small bowel with confirmed colonic ganglionosis.Case presentationA case report of a 14-month-old with a skipped segment involving the distal 50 cm of the small bowel associated with colonic ganglionosis is presented. A current review of the literature is discussed.Clinical discussionOur patient had persistent obstructive symptoms despite undergoing a technically good, ganglionic pull-through operation at an outside institution. A laparoscopic-assisted pull-through might have documented a small bowel wall diameter discrepancy.ConclusionAlthough rare, skip segment Hirschsprung’s disease is a real phenomenon that paediatric surgeons should be aware of and could involve small and large bowels.     

Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not

Purpose

This study was conducted to determine the effect of age at diagnosis and length of ganglionated bowel resected on postoperative Hirschsprung-associated enterocolitis (HAEC).

Methods

Children who underwent endorectal pull-through (ERPT) between January 1993 and December 2004 were retrospectively reviewed. t Test, analysis of variance, Kaplan-Meier, and Cox's proportional hazards analyses were performed.

Results

Fifty-two children with Hirschsprung disease (median age, 25 days; range, 2 days-16 years) were included. Nineteen (37%) had admissions for HAEC. Proportional hazards regression showed that HAEC admissions decreased by 30% with each doubling of age at diagnosis (P = .03) and increased 9-fold when postoperative stricture was present (P < .01), after controlling for type of ERPT, trisomy 21, transition zone level, and preoperative enterocolitis. Thirty-six children, with age at initial operation less than 6 months, were grouped based on length of ganglionated bowel excised (A [5 cm] and B [>5 cm]). No significant difference in the number of HAEC admissions during initial 2 years post-ERPT was seen between groups A (n = 18) and B (n = 18). The study had a power of 0.8 to detect a difference of 1 admission over 2 years.

Conclusions

Children diagnosed with Hirschsprung disease at younger ages are at a greater risk for postoperative enterocolitis. Excising a longer margin of ganglionated bowel (>5 cm) does not seem to be beneficial in decreasing HAEC admissions.     

Questioning the failure of neural crest cell migration theory in Hirschsprung’s disease: A case report and literature review

IntroductionSegmental aganglionosis (the absence of ganglions) is a rare presentation of Hirschsprung’s disease, whereby only limited segment/segments of aganglionic bowel is interposed between segments of innervated bowel, or “skip’’ area of normal innervations is present within an area of aganglionosis.Presentation of caseWe reported a case of a 3 day old male newborn who presented with failure to pass meconium along with progressive abdominal distension. There were skip lesions present in between. Mikulicz double barrel enterostomy was carried out, which was followed by an uneventful postoperative period. Four months later, the patient was admitted for levelling biopsies which revealed the absence of ganglions in the terminal ileum as well as in the rectosigmoid junction. But the ganglions were present in between and proximal to the terminal ileum where the previously dilated small bowel segment was resected. This presentation was contradicted the most accepted migration theory of Hirschsprung’s disease.DiscussionAs seen in our case, and in21 other cases published between 1954–2016, we highly recommend that leveling/mapping biopsies should definitely include the cecal pole and the small bowel segments proximal to the ileocecal valve as well as the multilevel colonic biopsies down till the rectum.ConclusionReporting of these cases brings out interesting questions with respect to the pathogenesis and serves to highlight the existence of several variants within the spectrum of Hirschsprung’s disease.     

成人先天性巨结肠症的诊断和外科治疗

目的 探讨成人先天性巨结肠症的诊断和外科治疗。方法 回顾性分析1985年5月至2005年5月收治的成人先天性巨结肠症10例患者的临床资料。结果 男性7例，女性3例。年龄14．40岁。10例均有便秘史，均经钡灌肠检查确诊，5例肛门直肠测压均未引出直肠肛门抑制反射。无神经节细胞段在乙状结肠远端和直肠2例，直肠7例，未明1例。手术方法：乙状结肠造口1例，Ikeda法6例，Rehbein法2例，改良Swenson法1例。根治手术后排便功能：优7例，良1例，差1例。结论 成人先天性巨结肠症的诊断主要依据便秘史、钡灌肠检查和／或肛门直肠测压。拖出型手术是有效的手术治疗方法，手术方式应个性化。     

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.     

A novel corrective pullthrough surgery in a mouse model of Hirschsprung's disease

Background/Purpose

The study aimed to develop a mouse model of post-pullthrough Hirschsprung's disease that will allow investigation of mechanisms that cause postoperative complications.

Methods

We developed a novel microsurgical pullthrough operation on Balb/C mice and evaluated its effect on growth rate and stooling pattern. Histologic assessment of the pullthrough colon was performed. The pullthrough operation was then performed on Ednrb−/− mice that have aganglionic megacolon and Ednrb+/+ littermate controls, and the outcomes compared.

Results

The Balb/C pullthrough group had 97% survival at 1 week and 70% survival at 2 weeks. Body weight of the pullthrough animals declined 15% in the first week after surgery and subsequently normalized. The stooling pattern showed consistently softer stools in the pullthrough group, but no difference in frequency compared to controls. Histopathologic analyses 4 weeks postoperatively showed well-healed coloanal anastomoses. Two-week survival after pullthrough surgery in Ednrb−/− and Ednrb+/+ mice was 50.0%, and 69.2%, respectively (P = NS). Increased mortality in the Ednrb−/− mice was related to the technical challenge of performing microsurgery on smaller-sized mice with poor baseline health status.

Conclusions

Our microsurgical pullthrough operation in mice is feasible and allows systematic investigations into potential mechanisms mediating post-pullthrough complications and poor long-term results in mouse models of Hirschsprung's disease.     

Advances and Pitfalls in the Diagnosis of Hirschsprung Disease

Surgical pathology for Hirschsprung disease (HSCR) occasionally is difficult, especially for those who encounter the disorder infrequently. This article reviews pathologic features of HSCR, considers various specimens the pathologist is required to evaluate, and discusses useful ancillary tests. Potential diagnostic pitfalls are highlighted, and helpful hints are provided to successfully navigate challenging situations. Finally, the article looks forward to new ancillary tests on the horizon and future topics for HSCR research.     

Redo-endorectal pull through following various pull through procedures in Hirschsprung's disease

Background The purpose of this study was to analyse the outcome of redo-endorectal pull through in Hirschsprung's disease following different original pull through procedures. In the past, redo-endorectal pull through was mainly performed following endorectal pull through, but not following the Duhamel procedure. We present the outcome of eight patients after redo-endorectal pull through, including five who underwent Duhamel pull through as original procedure. Materials and methods Between 2002 and 2004, eight patients underwent redo-endorectal pull through following the Duhamel procedure (five), Rehbein procedure (one) and endorectal pull through (two). A retrospective study was performed to evaluate the clinical course after redo-endorectal pull through, reviewing inpatients' and outpatients' charts and performing standardised interviews. Results Four of eight patients had normal stool pattern after redo-endorectal pull through. In two patients mild and in another two patients severe chronic constipation occurred after redo-surgery. Constipation-associated incontinence was noted in four patients, which is terminated after initiation of laxative treatment in three. Enterocolitis occurred in one patient and recurrent ileitis in another child with total colonic aganglionosis. No impairment of bladder function was observed after redo-endorectal pull through. Conclusion Redo-endorectal pull through proved to be a safe technique and feasible even after prior Duhamel pull through, resulting in good clinical outcome. Parts of this study have been presented at 123. Kongress der Deutschen Gesellschaft für Chirurgie, 2–5 Mai 2006, Berlin.     

Persistent bowel dysfunction after surgery for Hirschsprung’s disease: A neuropathological perspective

Hirschsprung’s disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.     

One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children

BACKGROUND: The surgical management of Hirschsprung's disease (HD) has evolved from the original 3-stage approach to the recent introduction of minimal-access single-stage techniques. We reviewed the early results of the transanal Soave pullthrough from 6 of the original centers to use it. METHODS: The clinical course of all children with HD undergoing a 1-stage transanal Soave pullthrough between 1995 and 2002 were reviewed. Children with a preliminary stoma or total colonic disease were excluded. RESULTS: There were 141 patients. Mean time between diagnosis and surgery was 32 days, and mean age at surgery was 146 days. Sixty-six (47%) underwent surgery in the first month of life. Forty-seven (33%) had the pathologic transition zone documented laparoscopically or through a small umbilical incision before beginning the anal dissection. Mean blood loss was 16 mL, and no patients required transfusion. Mean time to full feeding was 36 hours, mean postoperative hospital stay was 3.4 days, and 87 patients (62%) required only acetaminophen for pain. Early postoperative complications included perianal excoriation (11%), enterocolitis (6%), and stricture (4%). One patient died of congenital cardiac disease. Mean follow-up was 20 months; 81% had normal bowel function for age, 18% had minor problems, and 1% had major problems. Two patients required a second operation (twisted pullthrough, and residual aganglionosis). One patient developed postoperative adhesive bowel obstruction. CONCLUSION: To date, this report represents the largest series of patients undergoing the 1-stage transanal Soave pullthrough. This approach is safe, permits early feeding, causes minimal pain, facilitates early discharge, and presents a low rate of complications.     

The contrast enema for Hirschsprung disease: predictors of a false-positive result

Purpose

To examine predictors of a false-positive (FP) result on contrast enema (CE) for the diagnosis of Hirschsprung disease (HD).

Methods

Retrospective analysis, over a 5-year period (1999-2004), of infants (<6 months of age) with suspected HD undergoing rectal biopsy following abnormalities identified on CE (transition zone [TZ], abnormal rectosigmoid ratio, microcolon, retained contrast, or mucosal irregularity).

Results

One hundred twenty-nine patients underwent rectal biopsy following an abnormal CE. The FP rate was 48.5% (66 with HD). Age below 30 days (OR, 3.4; 95% CI, 1.1-10.3), female sex (OR, 3.4; 95% CI, 1.6-7.3), and absence of TZ (OR, 6.3; 95% CI, 2.6-15.3) were independently associated with an increased risk for FP on multiple variable logistic regression. A history of bilious emesis decreased the probability of FP (OR, 0.2; 95% CI, 0.06-0.5).

Conclusions

Transition zone, sex, age, and bilious emesis are important predictors of FP in those with suspected HD and CE abnormalities. With 100% incidence of FP, infants younger than 30 days with neither bilious emesis nor a TZ and female infants younger than 30 days with these features may represent a subpopulation in whom rectal biopsy can be avoided.     

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.     

肛门直肠局部注射肉毒毒素A治疗先天性巨结肠症

目的探讨局部注射肉毒毒素A治疗短段型先天性巨结肠症的可行性。方法对8例经病理组织学和钡灌肠诊断证实为短段型先天性巨结肠患儿,在截石位3、6、9钟位,将肉毒毒素A注射入肛门内括约肌和直肠肌层内,总剂量1.5U/kg。治疗前及治疗后1个月、1年分别作肛门直肠测压,治疗后3～6个月复查钡灌肠,以后每年钡灌肠复查1次。结果所有患儿治疗后均恢复良好,无术中和术后并发症。1年内所有患儿均能自主排便,未发现腹胀、便秘。有3例1年后再次出现便秘(3～5d排便1次)和轻度腹胀症状,但经扩肛后均迅速缓解。随访1个月时,肛门直肠静息压8例均较术前降低,1年后复查6例仍低于术前。术后3～6个月复查钡灌肠,8例扩张结肠均较术前明显缩小。结论肉毒毒素A注射治疗短段型巨结肠症是一种安全、可靠的新方法,其损伤小,近期疗效良好;但作用机制和远期疗效有待进一步研究。     

Development of a standardized definition for Hirschsprung's-associated enterocolitis: a Delphi analysis

Purpose

The reported incidence of Hirschsprung's-associated enterocolitis (HAEC) is extremely variable. A standardized definition would permit comparison of different studies and provide an interpretable outcome measure for future prospective studies in patients with Hirschsprung's disease.

Methods

The Delphi method is a technique for achieving consensus among a panel of experts. A list of 38 potential criteria from the history, physical examination, radiologic studies, and pathologic specimens was made available to pediatric surgeons and gastroenterologists who have contributed to the literature on Hirschsprung's disease. Each expert ranked the diagnostic importance of each item using a Likert scale. In subsequent surveys, the same process was used, but the means and SDs from previous rounds were included as a way of influencing the experts toward consensus. Cronbach's α was used after each round to measure variability among the experts. Once consensus was reached, an overall “HAEC score” was developed by assigning a value of 1 or 2 to each item that was considered important by the expert panel. The score was then validated by circulating 10 clinical cases to the panel and asking if each represented HAEC or not.

Results

Twenty-seven experts completed the survey. Cronbach's α increased from 0.93 after the first round to 0.97 after the second. Criteria receiving the highest scores were diarrhea, explosive stools, abdominal distension, and radiologic evidence of bowel obstruction or mucosal edema. Eighteen items were included in the score. During the validation process, the score agreed with the experts in 9 of the 10 case scenarios.

Conclusion

The most important clinical diagnostic criteria for HAEC were identified from a larger pool of potential diagnostic items through a consensus approach using the Delphi method. A score was developed and validated and can now be used as a standardized and reproducible outcome measure for future studies in children with Hirschsprung's disease.     

Hirschsprung disease and fecal incontinence: diagnostic and management strategies

Purpose

Ideally, fecal incontinence after operative management for Hirschsprung disease should not occur. If it does, it presents a formidable challenge. The purpose of this study was to describe the causes of fecal incontinence and present our algorithm for its treatment.

Methods

We reviewed 68 patients with Hirschsprung disease and fecal incontinence referred to us after surgery at other institutions. Patients were evaluated by contrast enema and by an examination under anesthesia to look specifically for the integrity of the anal canal. They were designated as having a dilated colon and constipation or a nondilated colon and a tendency to diarrhea based on their stooling pattern and the appearance of the contrast enema. Medical management was started that included laxatives for those patients with a dilated colon and constipation. For those with a nondilated colon and tendency to diarrhea, the management included loperamide, pectin, and a special dietary regimen (constipating diet, 3 meals per day, and no snacks). Those patients who responded to medical management were retrospectively considered to have been pseudoincontinent. Those who did not respond were considered truly incontinent. The truly incontinent group was treated with enemas alone for those with a dilated colon, or enemas, loperamide, pectin, and a constipating diet for those with a nondilated colon and tendency to diarrhea.

Results

Fifty-six patients had true incontinence and 12 had pseudoincontinence. Of the true incontinent group, 27 had a dilated colon and 29 had a nondilated colon. Five of these patients had a damaged or absent anal canal (anastomosis at the anal skin) and all of them had true incontinence. In the dilated colon group with true incontinence, 23 (85%) patients were clean after treatment. In the nondilated colon group with true incontinence, 23 (79%) were successfully treated. All patients in the pseudoincontinent groups had no soiling after treatment. Of 55 in the truly incontinent group, 39 (70%) had had an endorectal (Soave type) pull-through.

Conclusion

Fecal incontinence after operative management of Hirschsprung disease represents a serious problem. Poor surgical technique may be a contributing factor in some of the cases. Successful management depends on the appropriate evaluation, which determines whether the incontinence is true or pseudo, and the type of colon the patient has. Each category can be well treated, leading most of the time to a clean child.     

Stabilization period after 1-stage transanal endorectal pull-through operation for Hirschsprung disease

Purpose

The aim of this study was to evaluate postoperative outcomes and to find the period required for normal stooling pattern after the 1-stage transanal endorectal pull-through operation (TERPT).

Method

The authors retrospectively reviewed the clinical data and postoperative courses of 61 patients who had the aganglionic bowel confined to rectosigmoid and underwent TERPT between 2001 and 2007.

Results

Thirty-three patients (54.1%) were neonates, and 56 patients (91.8%) were less than 6 months old at operation. The mean age at TERPT was 90 ± 216 days, and the mean body weight at TERPT was 4.5 ± 2.8 kg. The average operating time was 189 ± 49 minutes, and mean length of bowel resection was 11.1 ± 3.2 cm. The mean postoperative hospital stay was 8.0 ± 3.6 days. Postoperatively, 5 (8.2%) patients were considered as failure of TERPT because of persistent problems in defecation. Fifty-six (91.8%) patients finally had normal stooling patterns and normal findings in abdominal radiography after 9.4 ± 6.2 weeks of the mean postoperative stabilization period. Neonatal cases had significantly longer postoperative stabilization periods than nonneonatal cases (11.3 ± 6.9 weeks vs 7.3 ± 4.6 weeks, P = .016). The postoperative stabilization period significantly decreased by age at operation as the patient's age increased (P = .018).

Conclusion

Clinical outcomes after TERPT are satisfactory, but a postoperative stabilization period is required for a normal stooling pattern to develop. The outcome of TERPT should consider a postoperative stabilization period.