Symptoms,diagnosis, and therapy of neuronal intestinal dysplasia masked by Hirschsprung's disease

Twenty-four cases of concomitant Hirschsprung's disease (HD) and neuronal intestinal dysplasia (NID) are presented. The clinical picture is characterized by the early and acute onset of HD symptoms. The diagnosis is established by means of rectal and colonic biopsies. Open biopsies during laparotomy should be taken without injury to the mucosa. Early surgical therapy consists of extended resection of the aganglionic segment and the colon affected by NID up to the splenic flexure. Complications are imminent if the aganglionosis masks the symptoms of NID and, accordingly, only the aganglionic segment is resected. As an alternative, postponed resection of the aganglionic segment alone is proposed once the NID-affected bowel develops functional maturation. In children who do not show an improvement of colon dysmotility, however, extended resection is recommended at the age of 4 years. In follow-up studies of colon motility, functional colon sonography is used.Offprint requests to:} G. Pistor     

Laparoscopic colonic mapping of dysganglionosis

 Retention of a proximal aganglionic segment or the unrecognized coexistence of other dysganglionoses may jeopardize the definitive surgical treatment of Hirschsprung's disease (HD). To assess the extent of the disease and/or the presence of other dysganglionoses without an additional laparotomy, we developed a laparoscopic-assisted technique to perform colonic full-thickness biopsies. After creation of a pneumoperitoneum, a 5-mm laparoscope is inserted in the supraumbilical area and a working 10/12-mm port is placed in the left iliac fossa. The sigmoid/descending colon is grasped and pulled through the abdominal wall and a full-thickness biopsy done. The same procedure is applied to the transverse and ascending colon. Rectal and colonic biopsy specimens were studied using enzyme histochemical methods. Over the last year, five children aged 7 months to 12 years with dysganglionosis underwent laparoscopic-assisted mapping of the colon. Previous rectal suction biopsies were diagnostic of HD in three patients and suspicious of hypoganglionosis in two. Proximal full-thickness biopsies revealed: normal colon in two cases of HD; coexistent type B intestinal neuronal dysplasia up to the descending colon in the other case of HD; and hypoganglionosis up to the ascending colon in the two patients with suspected hypoganglionosis. The procedures were performed easily, the patients being discharged after 36 to 48 h with no complications. Four children have already undergone pull-through procedures with resection of the affected colon in adhesion-free abdominal cavities and did not develop constipation or enterocolitis. Laparoscopic-assisted mapping of the entire colon is a simple, safe, and effective procedure that may contribute to improving the outcome of intestinal dysganglionosis by better characterization of the disease. Accepted: 27 October 2000     

Hirschsprung's disease and intestinal neuronal dysplasia – a frequent association with implications for the postoperative course

Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration of normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated. Accepted: 25 April 1999     

Hirschsprung’s disease: the “Swiss roll” technique revisited

Purpose  During pull-through for Hirschsprung’s disease (HSCR), the assessment of innervation is mainly based on the presence of ganglion cells when conventional Hematoxylin and Eosin (HE) staining is used. In hypoganglionosis, the evaluation is difficult. We adapted a standardized methodology for the examination of resected bowel after HSCR surgery, using the technique described by Moolenbeek on rodent intestine and later by Meier-Ruge in children. We have analysed the entire innervation of surgically resected bowels and compared the results with the follow up of patients. Methods  Three longitudinal strips of colon were harvested from the mesenteric, anti-mesenteric and intermediate part in the whole length of resected colon of six patients with HSCR. Each strip was divided into two parts. One of the contiguous strips was assessed with HE and Hematoxylin–Phloxin–Safran, and the other one with acetylcholinesterase (AChE) histochemistry. We analyzed the distribution of ganglion cells and nerve arrangement along the strips with both techniques and compared the results obtained in the three different regions of the bowel. Results  There was no significant difference in the pattern of innervation circumferentially. There was a correlation between a progressive increase of AChE activity and nerve hypertrophy and a decrease of ganglion cells from the proximal to the distal part of the resected colon in the submucosa and the myenteric plexus. Nerve hypertrophy and AChE-positive reaction in the mucosa were found at the resection border in patients who presented postoperative complications. Conclusions  Simultaneous assessment of nerve cells, nerve fibers and AChE activity is important in the evaluation of the innervation of the bowel segment proximal to the aganglionic zone. The method described is feasible and can be adapted to older children and adults with larger bowels. These results point out the importance of assessing nerve fibers in intraoperative biopsies during pull-through procedures to prevent uncomplete surgical bowel resection.     

腹腔镜下结肠切除Soave直肠内拖出术在肠神经元发育不良症中的应用

目的探讨腹腔镜下结肠切除、Soave直肠内拖出术治疗肠神经元发育不良症（IND）的可行性和中期随访结果。方法对25例IND和先天性巨结肠合并IND（HaIND）的患儿在腹腔镜下行Soave、结肠切除术，其中IND17例，HaIND8例。所有病例通过钡灌肠及延迟拍片、直肠黏膜活检、直肠肛管测压、腹腔镜浆肌层或全层活检明确诊断及病变范围。采用一个观察孔，2～4个操作孔。根据病变范围在腹腔镜下分别游离降结肠、横结肠、升结肠及系膜，会阴部手术按改良Soave方法。对患者手术方法、病变范围的确定、术后并发症和临床结果进行评估。结果25例中，10例行左半结肠切除，15例行结肠次全切除，按Deloyers法将升结肠逆时针转位270°下拖。行左半结肠切除术的患儿术前24h钡剂残留部位均在降结肠远端以远肠管，行结肠次全切除术的患儿术前24h钡剂残留在降结肠近端以近的肠管。平均手术时间分别为145min和188min，平均失血40ml。术后并发症：吻合口瘘2例，重度肛周皮肤红肿、糜烂11例，肠粘连1例，小肠结肠炎2例。术后平均随访4年，行左半结肠切除术的患儿，术后3个月内排便恢复正常；行结肠次全切除术的患儿术后3个月大便每日4～10次，12～24个月每日大便2～3次。2例出现污粪，无便秘复发和大便失禁病例。结论腹腔镜下结肠切除、Soave术治疗IND和HaIND安全、可行，创伤较小，术后并发症少，术后中期随访疗效满意。术前钡灌肠24h延迟拍片钡剂残留部位可以预测结肠切除的近端位置。     

MYOELECTRICAL ACTIVITY IN EXPERIMENTAL AGANGLIONOSIS OF THE COLON IN THE RAT

ABSTRACT. Experimental aganglionosis of the colon was produced in rats by an experimental "aganglionosis producing" procedure. Radiological examination of the aganglionic colon showed a narrow segment distal to a dilated megacolon. Histologically, a transverse section of the aganglionic segment showed 3-4 ganglia in contrast to 32-40 ganglia per section in the normal colon. The myoelectrical activity of the normal colon presented two fast activities, a fast activity with a frequency of 25-40 cycles per sec superimposed over a medium-fast activity of 4-7 cycles per sec. However the aganglionic colon showed only the fast activity with complete absence of the medium-fast activity. Thus the experimental aganglionosis produced a characteristic alteration in the myoelectrical activity of colon. This confirms our earlier findings in children with Hirschsprung's disease. It also suggests that the causative mechanism for the production of a narrow segment in Hirschsprung's disease may not be the hyperactivity or the absence of any specific neuronal mechanisms as proposed earlier.     

Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients

A prospective study of 141 consecutive patients with intestinal neuronal malformations is presented. The single malformation of the autonomic nervous system that always required surgical intervention was aganglionosis. Giant ganglia, reduced parasympathetic tone, immature ganglia, and hypogenetic or heterotopic nerve cells were seen in all forms of malformations. However, the incidence in specific malformations was variable. Multiple giant ganglia were identified in all patients with intestinal neuronal dysplasia (IND) type B, but also in various other malformations. Heterotopic nerve cells in the myenteric plexus were seen in the proximal segment of 15 of 74 patients (20.3%) with aganglionosis and 5 of 9 patients (55.6%) with hypoganglionosis. A significant impact on symptoms was found for IND type B: 34 (45.9%) of 74 children with aganglionosis had associated IND type B, and these children more frequently developed ileus (P < 0.001) and more often needed a second resection (P < 0.05) compared to those with isolated aganglionosis. This indicates an additive effect of both malformations, and therefore, in these patients an extended resection should be carried out.Twelve of 67 patients (17.9%) without aganglionosis needed resection for untreatable constipation. This included 7 of 9 children with hypoganglionosis, both patients with heterotopia of the myenteric plexus, 1 of 20 with isolated IND type B, and 2 of 12 with reduced parasympathetic tone. None of the patients with immaturity, heterotopia of the submucous plexus, or mild dysganglionosis required surgery. Six children (8.9%) without aganglionosis underwent sphincteromyotomy and 2 with IND type B had a temporary colostomy. At follow-up (mean 2.4 ± 1.4 years), the outcome in patients with resected aganglionosis was better than in patients who had resections for other malformations; 49 (69%) of 71 patients with aganglionosis were asymptomatic compared to 4 (33.3%) of 12 with other malformations (P < 0.05). It is concluded that some intestinal malformations have a relevant clinical impact. However, the severity of symptoms in the individual patient may not be explained by specific histochemical findings from a limited number of mucosal biopsies. The pathognomonic histochemical criteria of isolated IND typeB — immaturity, reduced parasympathetic tone, heterotopia of the submucous plexus, and mild dysganglionosis —rarely require surgical therapy and should be treated conservatively.     

Proximal segment histology of resected bowel in Hirschsprung's disease predicts postoperative bowel function.

The authors present an overview of 101 patients operated for Hirschsprung's disease by Rehbein procedure. Special attention is directed to the histomorphological findings in the proximal segment of the resected bowel specimens. A strong link was confirmed between these specific features and postoperative bowel function. A histologically regular proximal bowel segment generally predicted good postoperative bowel function with a constipation rate of 10.5%. In patients with intestinal neuronal dysplasia (IND) of the proximal segment the overall clinical result remained unchanged, although the constipation rate rose to 17.8%. Postoperative bowel function was seriously affected in cases characterized by proximal segment hypoganglionosis with a constipation rate of 23.1% and an additional 7.7% with encopresis. Finally, the distinct group of children with aganglionosis of the proximal segment followed a complicated postoperative course with secondary bowel resections and recurrent episodes of enterocolitis. In addition, the authors state their general observation that histological findings become less important whenever a more extensive resection than left hemicolectomy is required. Discussing the results, guidelines are given to further patient treatment once the particular proximal segment histology is diagnosed.     

Whole-mount NADPH-diaphorase histochemistry is a reliable technique for the intraoperative evaluation of extent of aganglionosis

Multiple seromuscular biopsies at three levels (narrow segment, transitional zone, and dilated segment) were taken and investigated intraoperatively to determine the extent of aganglionosis. Using the whole-mount preparation technique, circular muscle fibers were separated from the specimens. After a short prefixation, the muscle fibers were stained by the NADPH-diaphorase technique and were examined within 20–25 min. A fine and dense neuronal meshwork was observed between circular muscle fibers in the normal and ganglionic part of the bowel. In contrast, there was a complete lack of NADPH-diaphorase-positive fibers in the circular muscle of aganglionic colon. In the transitional zone, NADPH-diaphorase-positive fibers were markedly reduced compared to the ganglionic region. The density of these fibers increased and attained normal levels in the proximal bowel above the transition zone. These results suggest that whole-mount NADPH-diaphorase histochemistry is a three-dimensional technique suitable for the intraoperative evaluation of extend of aganglionosis. The technique is sufficiently rapid to be used in conjunction with routine frozen sections to assist in the diagnosis and in selecting the optimal level of resection at the time of pull-through operation.     

Assessment of the colon innervation with serial biopsies above the aganglionic zone before the pull-through procedure in Hirschsprung's disease

Different types of colonic dysganglionosis, and in particular intestinal neuronal dysplasia (IND) have been blamed for certain postoperative complications after surgery for Hirschsprung's disease (HD). We prospectively assessed colon innervation above the aganglionic zone (AZ) before proceeding to pull-through (PT) in order to rule-out IND as a cause of those complications. We first used a two-stage procedure (TSP): Full-thickness biopsies were harvested above the AZ and a colostomy was established during a first stage. Biopsies were assessed postoperatively with conventional acetyl-cholinesterase (AChE) histochemistry and immunohistochemistry for protein gene product 9.5 (PGP 9.5) and antigen CD56 (CD56). Biopsies were repeated after 6 months if IND was found. When the innervation was normal, the PT was performed during a second stage. Since having refined a rapid AChE reaction, we now use a single-stage procedure (SSP). Biopsies are harvested above the AZ and assessed intraoperatively with rapid AChE staining, proceeding to PT during the same stage when the innervation is normal. Four patients underwent the TSP; 3 had normal innervation above the AZ and subsequently underwent PT. In 1 patient serial biopsies revealed IND-like dysganglionosis; 9 months later, the innervation was normal in repeat biopsies and PT was undertaken. Eleven patients underwent the SSP. Biopsies were normal in 9 but showed unclassifiable forms of dysganglionosis in 2. As these changes were not typical for IND, all patients underwent PT in the same stage. Both patients had a poor outcome of bowel function that required a colostomy in 1 and daily saline irrigations in the other. IND was found in repeat biopsies made during the colostomy in the 1st patient and markedly hypertrophied nerves in the submucosa as well as ectopic nerve cells in the lamina propria in the proximal border of the pulled-through colon in the other. All 13 other patients have normal bowel function. The assessment of colon innervation above the AZ before proceeding to PT allows safer surgical treatment of HD. Intraoperative AChE staining is reliable, but due to the size and number of the biopsies, IND might be overlooked. Non classifiable dysganglionosis should thus be taken into account in the diagnosis and follow-up of the patients, as it may be clinically significant.     

Late study results following megacolon operations

The basic pathophysiologic disturbance in Hirschsprung's disease is a functional obstruction caused by defective intramural nerve supply and by internal anal sphincter achalasia. Therapy consists in resecting the dysganglionic bowel segment. In each case however an aganglionic segment of different length and an internal analsphincter with a different degree of achalasia remain in situ. Therefore the postoperative results are dependant on an equilibrium between the proximal normal innervated colon and the length and function of the remaining aganglionic and achalic parts of the rectum and anal canal. In about one third of all patients with Hirschsprung's disease disturbances of this equilibrium postoperatively lead to enterocolitis, encopresis, or chronic constipation. Five years later however the authors could observe enterocolitis in only 7.3% chronic constipation in 9.5% and encopresis in 13.9% of their operated patients. With increasing time after operation there is a growing tendency towards the spontaneous regeneration. Therefore, the prognosis of Hirschsprung's disease is very good: about 90% of all cases can be cured.     

Functional disorders of distal colon in children (author's transl)]

Functional disorders of the distal part of the colon in neonates and infants should be diagnosed by X-ray investigations (defecograms), double-suction biopsies (Erlanger children suction biopsy apparatus), anorectal and sigmoidal manometry and coloscopic functional investigations. 90% of all disorders of the distal colon can be diagnosed by manometric studies, in 40% X-ray investigations will lead to an exact diagnosis, in 12% double suction biopsies and in 10% coloscopic investigations establish the right diagnosis. The double suction biopsy is important for the exclusion of aganglionosis. Either a conservative or a surgical treatment is necessary, depending on the primary lesion. Wash outs, sphinctertraining, toilet training are necessary in cases of prolonged constipation following sigmaresection with restmegacolon, in cases of rectal inertia syndrom, and in cases of overflow incontinence. A pressure reducing operation (a sphincteromyotomy) is indicated in ultrashort aganglionic segments, in special cases of elongated sigma, in cases of prolonged constipation after sigma resection and with disturbed reflex mechanism, in cases of sphincter inhibition syndrome, and in children with paradox reflex mechanism. A resection is indicated in Hirschsprung disease, mechanical obstruction of the bowel, very large and severe cases of sigma elongatum and in cases of overflow-incontinence.     

Hirschsprungs disease with congenital hypothyroidism

We report a female newborn baby who presented with vomiting and abdominal distension on day 21 of life. Examination revealed facial puffiness, open posterior fontanelles, dry skin, cold peripheries and prominent abdominal veins with visible peristalsis. Barium enema revealed dilated proximal colon, empty rectum, funnel like transition zone between proximal dilated and distal constricted bowel. Serum TSH level was >;150 uIU/mL. Biopsy revealed aganglionic segment suggesting Hirschsprungs disease, an unusual association with congenital hypothyroidism.     

Intestinal aganglionosis: a histologic and acetylcholinesterase histochemical study

Varying results have been reported with the use of acetylcholinesterase (AchE) staining to diagnose Hirschsprung's disease in rectal suction biopsy. We analyzed the histology and AchE staining of rectal biopsies from 10 patients with documented intestinal aganglionosis and 57 patients with ganglionic bowel. The results show that histologic identification of submucosal ganglion cells is reliable in excluding Hirschsprung's disease and that the absence of ganglion cells in an adequate suction biopsy is highly suggestive of intestinal aganglionosis. Four AchE staining patterns were recognized; the staining patterns overlap in some patients who have and some who lack ganglion cells. The AchE staining pattern did not correlate with sex or age of the patients, or with the length of the aganglionic segment. The acetylcholinesterase stain is not a reliable method of making or excluding a diagnosis of intestinal aganglionosis except when AchE-positive fibers are increased in both the lamina propria and muscularis mucosae. This AchE staining pattern occurred in 6 of our 10 patients with Hirschsprung's disease. In addition, eight segments of aganglionic colon were studied that included 2 cases of total colonic aganglionosis in which hypertrophic, AchE-positive nerve fibers were absent in all layers of bowel wall. This last finding suggests that an abnormality in the preganglionic cholinergic fiber or extrinsic neuron is involved in the pathogenesis of this unusual form of total colonic aganglionosis.     

Ultrastructural demonstration of abnormal multiaxonal Schwann-cell units in Hirschsprung's disease

The pathophysiology of Hirschsprung's disease is not fully understood. Using light microscopy we have previously demonstrated the absence of a unique Schwann-cell antigen in the circular muscle of aganglionic colon identified by D₇ monoclonal antibody. In an attempt to characterise the morphological changes in neuronal cells at subcellular level, we studied innervation patterns in normal and aganglionic colon by electron microscopy. The most striking observation on ultrastructural serial examination of the entire resected specimen of colon from patients with Hirschsprung's disease was the presence of grossly swollen monoaxonal or oligoaxonal Schwann cell units with loss of cellular contents in the circular muscle of aganglionic colon. The extent of subcellular changes in Schwann cells and axons corresponded with a diminution of immunoreactivity with a panel of neuronal cell antibodies. These ultrastructural findings suggest that degenerative changes in Schwann cells and axons within the circular muscle coat of aganglionic segment may be a significant factor in the pathogenesis of Hirschsprung's disease. Offprint requests to: P. Puri     

Hirschsprung's disease: alpha-naphthylesterase activity for enzyme-histochemical evaluation of the extent of the aganglionic segment during surgery

The authors present their experience with the histotopochemical alpha-naphthylesterase reaction for intraoperative evaluation of the length of the aganglionic segment in Hischsprung's disease (HD). The activity of nonspecific esterases (Davis and Ornstein technique [1]) was used to demonstrate the presence or absence of intramural ganglion cells in seromuscular intestinal biopsies taken at various levels in 50 patients with the preoperative diagnosis of HD; in 44 cases the biopsies were taken during a radical HD operation (endorectal pull-through, Soave technique). In the remaining 6 cases, which presented with intestinal obstruction, the biopsies were taken during laparotomy. The authors consider this histochemical technique a reliable, quick, simple, and economical method for intraoperative evaluation of the length of the aganglionic segment. It is also useful for confirming the diagnosis of HD and is of great help in the study of so-called pseudo-HD. Offprint requests to: P. Dodero     

Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung’s disease

Background and objective

The cardinal diagnostic sign of congenital aganglionic megacolon, or Hirschsprung’s disease (HD), is an aganglionic segment of the distal colon or rectum. To determine the surgical planning of a radiological transition zone (TZ) in HD, this study investigated the association between a radiological TZ and the bowel resection length.

Methods

A prospective observational study was conducted in children (n?=?192) with suspected HD determined by radiological TZ on contrast barium enema, and who underwent pull-through operations. The bowel resection length was ≥10 cm above the proximal radiological TZ levels and confirmed by intraoperative frozen sections. In the contrast enema, the presence and level of a radiological TZ were recorded. Correlation of the TZ features with ganglion cells assessed by immunostaining of neuronal nuclei (NeuN) and the odds ratio were calculated.

Results

The sensitivity and specificity for diagnosing HD by the presence of a radiological TZ were 86.9 and 92.1%, respectively; Youden’s index was 79.0%. The positive and negative predictive values were 91.7 and 87.6%. The kappa value indicating an association between TZ and HD was 0.776 (P?<?0.05). The correlation rate between a radiological TZ and the pathological results was 88.5% in the rectosigmoid colon and 44.4% in the descending colon, and was higher in children older than 3 months (85.3%) than in infants (69.0%).

Conclusion

A preoperatively determined radiological TZ has potential value to identify the length of resected bowel in patients with HD, and it also has a high predictive value for diagnosis of HD.

     

Interstitial cells of Cajal are normally distributed in both ganglionated and aganglionic bowel in Hirschsprung’s disease

Surgery for Hirschsprung's disease is often complicated by post-operative bowel motility disorders. The impact of intestinal neural histology on the surgical outcome has been previously studied, but no information is available concerning the influence of the distribution of interstitial cells of Cajal (ICC) on these complications. These cells are considered to be pacemakers in the gastrointestinal tract. The aim of this study was to assess the distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease and confront these results with the clinical outcome. Using immunohistochemistry for light microscopy, we compared the pattern of distribution of ICC in the proximal segment of resected bowel in Hirschsprung's disease with that in normal colon. We correlated these results with the corresponding neural intestinal histology determined by CD56 and the protein gene product 9.5 immunohistochemistry. The distribution of ICC in the proximal segment of resected bowel is identical to that of normal colon, regardless of normal or abnormal colon innervation. ICC distribution does not seem to contribute to post-operative bowel motility disorders in patients operated for Hirschsprung's disease     

先天性巨结肠微创化手术治疗研究

目的探讨先天性巨结肠微创化手术治疗的方法。方法回顾性分析2004年1月至2007年1月本院收治的68例先天性巨结肠患儿的病例资料。年龄2个月至3岁，其中短段型12例，普通型40例，长段型10例，全结肠型6例。手术方式包括直肠肌条切除术、单纯经肛门结肠拖出术、腹腔镜辅助下巨结肠根治术。结果7例行直肠肌条切除术，35例行单纯经肛门结肠拖出术，20例行腹腔镜辅助下巨结肠根治术，6例行开腹手术。均治愈出院，出院后随访4个月至4年，63例排便正常，5例仍存在便秘。结论进一步规范儿童先天性巨结肠的微创治疗，制定儿童先天性巨结肠微创化治疗标准是提高儿童先天性巨结肠疗效的保证。     

Postoperative obstructive symptoms are common after Rehbein's procedure for Hirschsprung's disease.

Rehbein's procedure for Hirschsprung's disease has been questioned because several centimetres of aganglionic bowel are left behind, considered to result in obstructive symptoms. However, the reported outcome is similar to that after operations with other techniques. The purpose of this study was to review our experience of Rehbein's procedure during an 8-year period, focusing particularly on obstructive symptoms. We retrospectively studied 44 patients operated with Rehbein's procedure for biopsy-verified Hirschsprung's disease from October 1993 to October 2001. Three patients with total colonic aganglionosis and two patients who underwent total colectomy as a result of severe enterocolitis finally had a distal ileo-rectal anastomosis. Four (9 %) patients had a sphincteromyectomy before the Rehbein procedure. The patients' bowel function was evaluated by a questionnaire and an interview carried out 27 to 123 (mean 82) months after the operation. Two patients could not be traced and one patient, with total colonic aganglionosis, had an ileostomy at follow-up. Nineteen (46 %) of the 41 patients were treated with oral laxatives or enemas (12 patients), required repeated Botox injections (4 patients), or had undergone sphincteromyectomy (9 patients) due to obstructive symptoms. Eleven (27 %) of the 41 evaluated patients had soiling at least once a week at follow-up. Eleven (29 %) of 38 patients with a colorectal anastomosis had good bowel function without additional therapy. In conclusion, Rehbein's procedure for the treatment of Hirschsprung's disease is often complicated by obstructive problems.