Duhamel Procedure Research Articles

Short-Pouch and Low-Anastomosis Duhamel Procedure Results in Better Fecal Control and Normal Defecation Pattern

The purpose of this study was to analyze the long-term outcome of the short-pouch and low-anastomosis Duhamel procedure and to evaluate the quality of life after pull-through. We also tried to answer the questions: Does the modified Duhamel procedure produce fecal continence? Is the quality of life correlated to normal bowel function (1-3 per day) without the use of laxatives? Between January 1993 and January 2002, 53 patients, 44 (83 percent) males and 9 (17 percent) females, who underwent a Duhamel procedure were assessed retrospectively. Of the 53 children, 36 (67.9 percent) underwent a primary Duhamel procedure at our institute (Group A). Seventeen (32.1 percent) patients had a primary Duhamel pull-through and subsequently a stoma before a redo modified Duhamel procedure (Group B). The technical modification was creation of a short rectal pouch of 35 mm, achieving a low colorectal anastomosis 0.5 cm from or on the dentate line. All 53 patients were assessed prospectively by a disease-specific questionnaire. The total score provided a single index of the quality of life associated with fecal continence. For Group A (n = 36), the mean period of follow-up was 71.4 +/- 29 months. Constipation was seen in four (11.1 percent) patients. In two (5.6 percent) patients there was a mild degree of soiling. Sensation and urge to defecate was intact in 34 (94.4 percent) children who could wait to go to the toilet until it was socially convenient. For Group B (n = 17), the mean period of follow-up was 73.9 +/- 31.2 months. Constipation occurred in 17 (100 percent), encopresis in 14 (82.4 percent), and enterocolitis in 15 (88.2 percent) after the primary Duhamel procedure. In comparison, there was complete absence of constipation (100 percent), encopresis (100 percent), and complete resolution of enterocolitis (100 percent) after the redo modified Duhamel (P = 0.001). Continence to solid stools after primary Duhamel was seen in 5 (29.4 percent) vs. 17 (100 percent) after redo modified Duhamel. After the redo modified Duhamel all 17 (100 percent) patients have retained the sensation and urge to defecate and 15 (88.2 percent) can wait to get to the toilet until it is socially convenient. In all 53 patients, the quality of life was good in 86.8 percent (46/53) and correlated directly with fecal continence (r = 0.977). There was no correlation between age of patient and fecal continence (r = 0.597) and rate of fecal incontinence did not decrease with age. Direct correlation was seen with the presence of constipation and the incidence of late onset enterocolitis (r = 0.942). Short-pouch, low-anastomosis Duhamel pull-through procedure results in fecal continence and complete emptying. The above procedure has a positive impact on the quality of life in these children.

Long-term outcomes in total colonic aganglionosis: a 32-year experience

Background/Methods A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid–small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

Systematic Review of Surgical Options for Idiopathic Megarectum and Megacolon

A subgroup of patients with intractable constipation has persistent dilatation of the bowel, which in the absence of an organic cause is termed idiopathic megabowel (IMB). The aim of this systematic review was to evaluate the published outcome data of surgical procedures for IMB in adults. Electronic searches of the MEDLINE (PubMed) database, Cochrane Library, EMBase, and Science Citation Index were performed. Only peer-reviewed articles of surgery for IMB published in the English language were evaluated. Studies of all surgical procedures were included, providing they were performed on 3 or more patients, and overall success rates were documented. Studies were critically appraised in terms of design and methodology, inclusion criteria, success, mortality and morbidity rates, and functional outcomes. A total of 27 suitable studies were identified, all evidence was low quality obtained from case series, and there were no comparative studies. The studies involved small numbers of patients (median 12, range 3-50), without long-term follow-up (median 3 years, range 0.5-7). Inclusion of subjects, methods of data acquisition, and reporting of outcomes were extremely variable. Subtotal colectomy was successful in 71.1% (0%-100%) but was associated with significant morbidity related to bowel obstruction (14.5%, range 0%-29%). Segmental resection was successful in 48.4% (12.5%-100%), and recurrent symptoms were common (23.8%). Rectal procedures achieved a successful outcome in 71% to 87% of patients. Proctectomy, the Duhamel, and pull-through procedures were associated with significant mortality (3%-25%) and morbidity (6%-29%). Vertical reduction rectoplasty (VRR) offered promising short-term success (83%). Pelvic-floor procedures were associated with poor outcomes. A stoma provided a safe alternative but was only effective in 65% of cases. Outcome data of surgery for IMB must be interpreted with extreme caution due to limitations of included studies. Recommendations based on firm evidence cannot be given, although colectomy appears to be the optimum procedure in patients with a nondilated rectum, restorative proctocolectomy the most suitable in those with dilatation of the colon and rectum, and VRR in those patients with dilatation confined to the rectum. Appropriately designed studies are required to make valid comparisons of the different procedures available.

The versatility of the umbilical incision in the management of Hirschsprung's disease

Background/Purpose The aim of this report is to describe how the umbilical incision provides a quick and safe alternative to laparoscopy or other abdominal incisions in the management of Hirschsprung's disease (HD). Methods An analysis of 24 patients with HD, who were treated using an umbilical incision as part of their operative management between 1999 and 2003, was performed. Results There were 18 boys and 6 girls (mean age at diagnosis: 16.5 ± 20.9 days). Twenty-one had rectosigmoid HD, and 3 had total colonic HD. Eighteen patients received a 1-stage transanal pull-through with transumbilical colonic biopsies at a mean age of 33.9 ± 25.3 days, and a mean weight of 3.8 ± 1.0 kg. Three patients with rectosigmoid disease had more complicated HD: 2 had a colostomy (1 enterocolitis, 1 extensive colonic dilatation), and 1 had an ileostomy (for perforated cecum). All subsequently underwent transanal pull-through. Three patients with total colonic HD had an ileostomy. All stomas were situated at the umbilicus. One of these patients subsequently underwent a Duhamel procedure via the umbilicus; 1 is still waiting; and 1 died of sepsis. Complications in this cohort included a seromuscular tear of the distal sigmoid at the level of the umbilical incision (1), infection at the umbilical incision requiring antibiotics (2), obstruction (1) and death (1 sepsis, 1 from Ondine's curse). The median time to start full feeds was 24 hours. For postoperative analgesia, 13 patients required acetaminophen only, and 9 patients received low-dose morphine. After a mean follow-up of 7.3 ± 9.7 months, there has been an excellent functional and cosmetic result in the 22 survivors. Conclusions The umbilical incision has all the benefits of a minimal access approach, but is less expensive and requires no specialized equipment or skill. The umbilical incision is an excellent, safe, and versatile alternative to laparoscopy for the treatment of patients with the full spectrum of HD.

Hirschsprung's Disease in Adults: Report of a Case and Review of the Literature

Hirschsprung's disease in the adult is a rare and frequently misdiagnosed cause of long-standing refractory constipation. We report a case of Hirschsprung's disease in a 23-year-old man and review the literature. The patient had a history of chronic constipation that required daily enemas, since early infancy, but he had remained in good health until intestinal obstruction developed. As a subemergency operation, right transverse colostomy was performed, to relieve the constipation. Histological examination, by a biopsy, showed absence of ganglion cells in the myenteric plexus in the rectum. One year later, Ikeda's modification of the Duhamel procedure was successfully performed as definitive surgery. The postoperative course was uneventful, and complete resolution of the symptoms without complications has been confirmed by a 22-year follow-up. A review of 229 cases of adult Hirschsprung's disease in the literature suggested that the Duhamel procedure is the operation of choice because of the lower postoperative morbidity rate and better functional outcome.

Laparoscopic-assisted approaches for the definitive surgery for Hirschsprung’s disease

The surgical management of Hirschsprung's disease has progressed from a two- or three-stage procedure to a primary operation over the last 25 years. More recently, definitive surgery for Hirschsprung's disease has been performed using minimally invasive techniques. The Swenson, Duhamel and Soave endorectal pull-through procedures have all been reported using minimally invasive approaches. The endorectal dissection has become the dominant minimal access procedure because of the ease and reliability in performing this technique and the excellent results obtained. Although a transanal endorectal pull-through can be performed without laparoscopy, the laparoscopic-assisted transanal endorectal pull-through is a much more versatile technique and allows early biopsies to determine the extent of aganglionic and dysfunctional bowel before ablation of the rectum and mesocolon. The authors use a laparoscopic-assisted transanal pull-through for aganglionosis of the left and transverse colon. Total colon aganglionosis or aganglionosis of the ascending colon is managed by a laparoscopic-assisted Duhamel procedure which provides a better reservoir in patients with a short or absent colon.

Results of a mechanical duhamel pull-through for the treatment of Hirschsprung’s disease and intestinal neuronal dysplasia

Background/purpose The authors analyzed the results of a modified entirely mechanical Duhamel pull-through for the treatment of Hirschsprung’ disease or type B intestinal neuronal dysplasia. The aim of the follow-up was to evaluate results of a Duhamel procedure entirely performed with the use of staplers. Methods Fifty-six patients were followed up to detect complications and outcome. Patients were evaluated clinically, and, when symptoms were present, a suction biopsy with histochemical analysis, an x-ray contrast enema, or an endoscopy was performed. Results Early complications developed in 4 cases (7%) including a case of leakage (1.8%). No patient experienced recurrence. During a mean follow-up of 49 months, further complications occurred in 16 other patients: 4 adhesive obstructions requiring surgery, 3 strictures, 5 chronic bleeding, 4 fecaloma formation, 2 urinary problems, and 13 postoperative enterocolitis. Four patients (7%) had more than 3 passages of liquid stools per day, and 3 of them who had an ileal pull-through had constant soiling. Thirty-seven children (68.5%) had 1 to 2 bowel movements per day and were continent. Seven (13%) had less than 3 bowel movements per week. Three children who underwent total colectomy were incontinent (5.5%). Three more children (5.5%) have not yet reached the age for continence. Forty-nine parents (87%) judged the outcome satisfactory. Conclusions Follow-up of mechanical Duhamel pull-through showed an acceptable long-term outcome. An increased risk of incontinence is present in case of ileal pull-through, whereas constipation is more frequent after partial colon resection, regardless of the type of intestinal neuronal malformation.

Laparoscopic Duhamel Procedure: Management of 55 Cases

Introduction: Hirschprung disease (HD) is a common cause of neonatal intestinal obstruction and chronic constipation. Advances in instrumentation and in laparoscopic experience have made laparoscopic correction possible in recent years.Methods: Medical records of children who underwent a laparoscopic Duhamel (LD) procedure from February 1995 to June 2002 in our institution were reviewed. Operative technique was always the same, using 4 ports (5, 5, 10, and 12 mm) and wall suspension. We recorded birth weight, clinical features, age and weight at procedure, morbidity, and followup.Results: Fifty-five children, 46 boys and 9 girls, with a median birth weight of 3046 g, were operated on. They ranged in age from 25 days to 8 years (mean, 13 months). Four cases of enterocolitis and 16 cases of sepsis necessitated 20 colostomies and 5 ileostomies (total colonic aganglionosis). Average operative time was 140 minutes (range, 100–330 minutes). Four conversions were necessary. There were no deaths. There was 1 anas...

Can the Swenson, Duhamel, and Soave-Boley Procedures Be Applied in an Era of Minimally Invasive Surgery? The Results of an Experimental Study

Background: The purpose of this experimental study was to explore if, at this point in modern technology, the predominant techniques used for the treatment of Hirschprung disease (Swenson, Duhamel, and Soave-Boley) can be achieved laparoscopically, without laparotomy. Methods: Sixteen male pigs, weighing 15-25 kg each, were included in our trial experiment. We developed an experimental model and laparoscopically applied the rectosigmoidectomy (Swenson technique), the endorectal pull-through (Soave-Boley technique), and the retrorectal pull-through (Duhamel procedure) as well as the transanal mucosectomy of the rectum. We then recorded the techniques and the outstanding characteristics that evolved during their laparoscopic application. Results: The experimental animals had an uneventful postoperative course. The coloanal anastomosis was free of tension, airtight, and without stenosis regardless of the procedure used, in all the animals. Feeding was started following postoperative normal bowel movements. Median followup was three months and stool output was normal. Conclusions: In the surgical treatment of Hirschsprung disease the abdominal stage of the various operations can be carried out laparoscopically. The operating time of transanal mucosectomy can be significantly diminished if devascularization of the bowel is performed laparoscopically. The transanal mucosectomy of the rectum according to Georgeson is a modification of the Soave-Boley technique and it is the chosen mode of treatment for patients undergoing endorectal anastomosis.

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.

Hirschsprung’s Disease in Adults: the Duhamel Procedure

Hirschprung’s disease is a rare condition in the adult. We present three patients with Hirschsprung’s disease who successfully underwent the modified Duhamel procedure. The diagnosis was suspected on the basis of the typical medical history of lifelong constipation. The diagnosis was confirmed by anorectal manometric studies and/or full thickness rectal biopsy. The relief of the marked preoperative abdominal distention was complete and the bowel movements were active and smooth. Two out of three had perfect faecal control and continence. The Duhamel-Martin procedure seems a safe and reliable technique in the treatment of Hirschsprung’s disease.

Does transitional zone pull-through in Hirschsprung’s disease imply a poor prognosis?

Purpose: Accurate frozen section interpretation of intraoperative biopsies is critical to the success of the Duhamel procedure. Errors during sampling or interpretation may result in an abnormal pull-through. The authors’ aim was to determine the incidence and outcome of transitional zone pull-through (TZPT). Methods: Ninety-six children who underwent a Duhamel procedure between January 1987 and May 2002 were followed up prospectively. The outcome of 18 children with TZPT was compared statistically with that of a cohort of 58 patients with a ganglionic pull-through. Results: The incidence of TZPT was 18.8%. Concordance rate between frozen and paraffin-section analyses was 88.5%. The incidence of enterocolitis ( P = .003) and intractable constipation ( P = .02) was found to be significantly higher in TZPT. There was no significant difference in continence ( P = .34), rectal sensation ( P = .35), and control ( P = .53). Five (27.8%) TZPT patients required a revisional surgery for failure of conservative management. The incidence of stoma placement and revision was significantly less in the ganglionic group (3.4%, P = .007). Conclusions: Up to one third of patients with TZPT will require revisional surgery for intractable symptoms. Vigilance is warranted in view of the greater risk of enterocolitis. Continence is not significantly affected.

Primary pull-through for Hirschsprung’s disease

The first report of a successful primary pull-through for Hirschsprung’s disease using the endorectal pull-through (ERPT) was by So et al. (J. Pediatr. Surg. 15 (1980) 470; J. Pediatr. Surg. 33 (1998) 673). Subsequently, because of the simplified nature of this approach and the potential for cost savings, several groups have reported excellent results with this procedure. In addition to the ERPT, both the Duhamel and Swenson procedures have been performed in a one-stage fashion. More recently, primary laparoscopic approaches for each of these techniques have been utilized. The purpose of this review is to discuss the technique of primary pull-through, the peri-operative management and a summary of clinical results.

Improved quality of life by combined transplantation in Hirschsprung's disease with a very long aganglionic segment

Background/Purpose: The treatment of children with Hirschsprung's disease beginning in the proximal jejunum remains a challenge for the pediatric surgeon. These patients need a definitive parenteral nutrition, which could lead to a liver impairment. The goal of this work is to assess the quality of life after combined liver, intestine, and right colon transplantation. Methods: This is a retrospective study of 3 patients. Data regarding symptomatology, radiographic and operating findings, postoperating recovery, and quality of life were analyzed and compared with the quality of life before the transplantation. Results: The suspicion of a very long intestinal aganglionosis should be derived from the intestinal biopsies. Three combined liver, intestine, and right colon transplantation operations have been performed. The immunosuppression included steroids, tacrolimus, and azathioprine. An abdominal pull-trough (Duhamel procedure 2, Swenson procedure 1) was performed from 6 to 24 months after the transplantation. The follow-up after the transplantation ranges from 2 to 6 years. These 3 patients are completely off total parenteral nutrition with bowel movements 2 to 3 times a day. Two patients are continent day and night, and one is continent during the day only. Conclusions: Intestinal transplantation is feasible with good results even when a liver impairment needs a combined intestine and liver transplantation. The right colon transplantation, in our experience, does not impair the results. The quality of life after the transplantation is better than before. J Pediatr Surg 38:422-424. Copyright 2003, Elsevier Science (USA). All rights reserved.

Anal achalasia after pull-through operations for Hirschsprung's disease -- preliminary experience with topical nitric oxide.

Following definitive pull-through for Hirschsprung's disease (HD), a minority of patients develop constipation, incontinence and enterocolitis. The cause for these symptoms in some of the patients is believed to be "internal sphincter achalasia" related to abnormal innervation of the sphincter, with an absent anorectal relaxation reflex. Transanal myectomy, posterior sagittal rectal myectomy, anal dilatation and even intrasphincter injection of botulinum toxin have been used to solve this problem. Nitric oxide (NO) has been identified as the chemical messenger of the intrinsic non-adrenergic, non-cholinergic pathway mediating relaxation of the normal internal anal sphincter when applied topically. To evaluate the effect of topical isosorbide dinitrate (DTN) applied to the anus and its role in the management of patients with HD after pull-through who have ongoing difficulties in stool evacuation. Four children, aged 2, 5, 7 and 13 years, who all underwent the Soave operation for Hirschsprung's disease, were assessed. Three patients had recurrent episodes of enterocolitis, and all had symptoms of difficulty in rectal/colonic evacuation. Conservative treatment of repeated anal dilatation under anaesthesia had failed to improve symptoms. A rectal myectomy and conversion of Soave to Duhamel procedure had been done in 2 without significant improvement in symptoms. In all patients, ano-rectal manometry was performed before and after application of DTN paste (1 mg/kg/day in two separate doses) which was continued for a minimum period of 3 weeks. Results. Marked symptom improvement was noted in all 4 children. On manometric assessment the median maximum pressure (pre-DTN application) was 165 mm Hg (range 96 - 250), the median sphincter length 2.7 cm (range 2.3 - 3.1) and the high-pressure zone (HPZ) median length 1.6 cm (range 1.2 - 2.1). After application of DTN paste, the maximum pressure dropped by a median of 88 mm Hg (range 46 - 90), total sphincter length shortened to a median of 2.1 cm and the HPZ by a median total length of 1.4 cm (range 0.01 - 0.9). In addition, vector volume was reduced by a median of 59 % (range 40.5 - 77). From these results, it is evident that DTN paste is not only an adjunct in the investigation, but can also be used as a temporary form of treatment of obstructive symptoms in Hirschsprung's disease patients.

Total colonic manometry as a guide for surgical management of functional colonic obstruction: Preliminary results

Background/Purpose: Functional colonic obstruction (pseudo-obstruction) encompasses a broad group of motility disorders. Medical management of colonic pseudo-obstruction is complex and often fails, leading to surgical referral. In most cases (excepting Hirschsprung's disease) the surgeon is unable to precisely localize the area of functional obstruction. Total colonic manometry can directly measure intraluminal pressures and contractile function along the entire length of the colon. The authors propose that total colonic manometry can be used by the pediatric surgeon to guide the timing and extent of surgical therapy in refractory functional colonic obstruction. Methods: Four patients were evaluated for functional colonic obstruction. All underwent barium enema and rectal biopsy with a diagnosis of Hirschsprung's disease in one patient. All patients underwent colonoscopy and total colonic manometry. Manometric tracings were obtained while fasting, after feeding, and after pharmacologic stimulation both preoperatively (n = 4) and postoperatively (n = 3). Results: Total colonic manometry identified an abrupt end of normal peristalsis in 2 of the non-Hirschsprung's patients (one in the proximal colon and one in the transverse colon). Medical therapy failed in both of these patients, and they underwent diverting ostomy proximal to the loss of normal peristalsis. The third non-Hirschsprung's patient essentially had normal manometry and was able to have her colon decompressed successfully on a laxative regimen. Repeat manometry after colonic decompression showed return of normal peristalsis in 2 of these patients and continued abnormal peristaltic activity in the third. Definitive surgical intervention based on the results of total colonic manometry was performed on the latter. All 3 patients achieved normal continence. A fourth patient had Hirschsprung's disease confirmed by rectal biopsy and underwent a 1-stage neonatal modified Duhamel procedure, which was complicated by postoperative functional obstruction. Manometry showed a lack of peristaltic function beginning in the right colon. An ileostomy was performed, and timing of ileostomy closure was guided by the return of normal colonic peristalsis seen on manometry. Conclusions: These initial cases show the utility of total colonic manometry in the management of colonic pseudo-obstruction syndromes. In addition to its diagnostic utility, direct measurement of colonic motor activity can be valuable in deciding the need for and timing of diversion, the extent of resection, and the suitability of the patient for restoring bowel continuity. In Hirschsprung's disease, total colonic manometry can potentially be used to determine suitability for primary neonatal pull-through versus a staged approach. J Pediatr Surg 36:1757-1763. Copyright © 2001 by W.B. Saunders Company.

Long-term follow-up of redo pull-through procedures for Hirschsprung's disease: Efficacy of the endorectal pull-through

Background/Purpose: The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome. Methods: From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-through at another hospital and 2 patients with an unsuccessful pull-through from C.S. Mott. All redo pull-throughs (n = 19) were performed in these patients, and their clinical courses are reviewed. Results: Twelve patients required reoperation secondary to a mechanical problem with their first pull-through. The other 7 patients had evidence of residual segments of dilated colon leading to functional failure of their initial operation including 5 patients with documented aganglionic bowel present at the second pull-through. Ten of the patients requiring reoperation initially had an endorectal pull-through (ERPT), 5 had a Duhamel procedure, 3 had a Swenson procedure, and 1 had a Rehbein procedure. Choice of revision was an ERPT in 8 patients in whom an adequate rectal cuff could be developed. Additional redo procedures included a Duhamel in 8 patients and a Swenson in 3 patients. Follow-up ranges from 3 months to 23 years (mean, 13.8 years). There were no deaths in the series, and 1 patient required a third pull-through. All patients who are not neurologically impaired and are over age 3 are continent except one (94%). Stools per day range from 1 to 10 (mean, 3.2). Conclusions: Redo pull-through operations for Hirsch-sprung's disease appear to be as effective as primary procedures in terms of continence and stooling frequency. Distinct from other series, we found an ERPT to be the procedure of choice if an adequate rectal cuff was present. J Pediatr Surg 35:829-834. Copyright © 2000 by W.B. Saunders Company.

Diagnostic difficulties in neuronal intestinal dysplasia and segmental colitis

An 11-month-old girl with a prolonged history of bloody, mucoid diarrhea is presented. Although the initial diagnosis given by the rectosigmoid biopsy obtained during laparotomy was neuronal intestinal dysplasia, accompanying findings including mixed inflammatory cell infiltration of the mucosa and submucosa with mucosal ulcerations suggested nonspecific colitis. The subsequent biopsy specimen that was obtained after performing colostomy and treating with broad-spectrum antibiotics and rectal irrigations showed improvement in the structure of ganglion cells and submucous and myenteric plexuses. Although the mucosal ulcerations and inflammatory reaction improved, the colonic stricture persisted, so the Duhamel procedure was performed, and the patient had an uneventful outcome. It is claimed that inflammatory disease of the rectosigmoid colon of unknown etiology and neuronal intestinal dysplasia have occurred together in the current case or that one disease might cause the other in time.

Laparoscopic Duhamel procedure. Management of 30 cases.

Between February 1995 and June 1998, 30 laparoscopic Duhamel pull-through procedures were performed in our department. Our main aim was to prove the feasibility of the laparoscopic abdominal Duhamel procedure for different localizations of Hirschsprung disease. We used one camera port and three working ports. The sigmoid colon and posterior rectum were mobilized laparoscopically. A standard posterior colo-anal anastomosis was fashioned and a stapler was used for the anterior anastomosis. The top of the rectum was then closed by endo stapler under laparoscopic vision. Thirty patients underwent laparoscopic surgery for this procedure. Three laparoscopic procedures were converted because of technical difficulties. The operative time was 100-330 mn. Oral feeding was started at a mean postoperative time of 2.5 days. Mean postoperative hospitalization was 9 days. Early postoperative complications included 1 anastomotic leak, 1 retrorectal abscess, 2 urinary infections, and 1 evisceration (after conversion). No enterocolitis or enterocolitis-like symptoms were noted. All patients now have daily spontaneous bowel movements. The laparoscopic Duhamel procedure can be performed safely, giving good results.

Long-term outcome after Hirschsprung's disease: Patients' perspectives

Background : The results of treatment of Hirschsprung's disease (HD) are not uniformly successful, and the parents' and child's interpretation of the outcome may be different from that of the surgeon. Methods: One hundred seven children treated for HD over 22 1 2 years were reviewed retrospectively. Adequate clinical data regarding functional outcomes were available in 78, of which, an additional questionnaire was completed by 69. Follow-ups were divided into type of pull-through (PT), age at time of PT (<4 months and >4 months) and age at last follow-up (<5 years, 5 to 15 years and >15 years). Degrees of constipation and incontinence were determined using standard scoring systems. Results: The median age at presentation was 9 days (range, 1 day to 9.4 years), and 41% presented within the first 72 hours of life. Aganglionosis extended to the rectosigmoid region in 75%, proximal to the splenic flexure in 11%, and total colon in 6.5%. Treatments included a Soave procedure in 57, Duhamel in 31, Swenson in 10, and sphincterotomy-myectomy in two. No surgical treatment or only a decompressing enterostomy was performed in two and three children, respectively, and two underwent a PT elsewhere. Complications related to the enterostomy occurred in 39 patients. Postoperative enterocolitis and bowel obstructions occurred in nine and 13 patients, respectively. Staple-line fusion, anastomotic stenosis, and anastomotic leak occurred in nine, 17, and two patients, respectively. Seven children died, only one directly related to the PT. Aside from more constipation associated with the Duhamel procedure, functional outcomes were not significantly different among the types of PTs. No differences were found between patients who underwent a PT at less than 4 months of age and those at greater than 4 months of age. Significantly, however, fecal continence was relatively poor in those less than 15 years of age (50% with only fair to poor continence), but improved markedly once the child reached later adolescence (8%, P < .0002). Correspondingly, the negative impact on the child's social life was much greater in the younger age groups compared with later adolescence (50% of those aged 5 to 15 years v 18% of those >15 years; P = .007). The effect on the families' lives mirrored this. Finally, only 64% of patients were interpreted as having “normal” stooling habits, yet 90% of parents were moderately or very satisfied with their child's outcome, with no differences found among the three sets of comparison groups. Conclusions: The outcomes for HD are not always as good as surgeons may perceive; long term follow-up is important. With time, most children significantly improve with respect to fecal continence, but this may not be until later adolescence. In the meantime, the impacts on their social and family lives may be significant. Despite high complication rates, often poor continence, and relative infrequency of normal stooling habits, most parents are satisfied with their child's outcome and adapt to their functional abnormalities along with them.