Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus

Background/Purpose

In this study, the patients operated on for anorectal malformations (ARM) were evaluated in terms of segmental (SCTT) and total colonic transit times (TCTT) and clinical status according to Krickenbeck consensus before and after treatments.

Methods

Forty-one patients with ARM (28 males/13 females) older than 3 years (median age, 7.7 years; range, 3-25) who had no therapy before were assessed for voluntary bowel movements (VBM), soiling (from 1 to 3), and constipation (from 1 to 3), retrospectively. Distribution of the patients were rectourethral fistula (17), perineal fistula (PF; 8), vestibular fistula (VF; 8), cloaca (3), rectovesical fistula (1), rectovaginal fistula (1), pouch colon with colovestibular fistula (1), no fistula (1), and unknown (1). The patients ingested daily 20 radiopaque markers for 3 days, followed by a single abdominal x-ray on days 4 and 7 if needed. The results were compared with the reference values in the literature.

Results

Mean follow-up period was 36 months (range, 1-108.5 months). All patients but 1 had soiling in different degrees. Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). The longest TCTT and rectosigmoid SCTT were found in group 3 (69.5 and 35.2 hours, respectively). Group 1 had long SCTT in rectosigmoid but normal TCTT (27.8 and 47.4 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT, and there was no significant difference between them. After the appropriate treatment, of the patients, 45% (18/40) had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had unchanged soiling score, and 3 were excluded from the study because of follow-up problems. Half of the patients in group 3 (4 VF, 2 rectourethral fistula, PF) gained VBM without soiling after laxative treatment. Only four of 23 patients had decreased constipation score (2 cloaca, PF, VF).

Conclusions

In this study, ARM patients complaining of constipation with or without VBM had prolonged SCTT in the rectosigmoid region. Percentage of the improvement in soiling scores was more conspicuous than that of constipation scores. The dismal figure observed at the first examination in the assessment of VBM was not associated with an unfavorable improvement with laxative treatment. So, it is suggested that assessment of VBM initially may be deceptive for clinical status.     

Functional outcome after operation for Hirschsprung disease—transanal vs transabdominal approach

Background

It has been hypothesized that the extensive transanal dissection in transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD) can impair the anal sphincters in neonates and thereby cause incontinence. Theoretically, transabdominal endorectal pull-through might have less impact on the sphincters. The aim of this study was to compare functional outcome in HD patients operated with either TEPT or laparotomy-assisted endorectal pull-through (LEPT) with particular focus on soiling and fecal incontinence.

Patients and Methods

Anorectal function in 52 children older than 3 years is reported. The patients were operated for HD with either TEPT (n = 28) or LEPT (n = 24) and followed prospectively. Functional outcome was recorded by standardized interviews. The Krickenbeck criteria were used to classify voluntary bowel movements, soiling, and constipation.

Results

The median age at follow-up was 5.7 years (3.1-13.2) for TEPT and 10.1 years (7.7-16.2) for LEPT. Twenty-nine patients reported soiling at final follow-up. There was no difference in the rate of soiling between children operated with TEPT (54%) or LEPT (58%). Constipation was reported in 11 children (TEPT, 25%; LEPT, 17%).

Conclusions

The functional outcome and in particular the rate of soiling did not differ between patients operated with LEPT or TEPT.     

Laparoscopic treatment of rectovaginal fistulas. Feasibility, technical details, and functional results of a rare anorectal malformation

Introduction

Rectovaginal fistulas are a rare variety of anorectal malformations. Eight patients with this anomaly among 420 children with anorectal malformations were treated in our center. We used a laparoscopic approach in 5 of the children. The aim of this study is to define the feasibility and technical details of laparoscopy in the treatment of these patients as compared with those treated by posterior sagittal anorectoplasty (PSARP) and to evaluate the preliminary results.

Methods

Three patients were treated with a PSARP starting February 2000 (group 1 [GI]). The laparoscopic approach (laparoscopic treatment [LT]) was used in the next 5 girls starting in November 2002 (group 2 [G2]). All patients were operated on by the authors. In LT cases, we used 3 ports. The rectum was dissected laparoscopically, and the fistula was transected. An assisted anorectal pull-through was then performed using a minimal perineal incision. Clinical features, age at operation, associated anomalies, ratio/sacrum (sacral ratio [SR]), distal colostogram, operative findings, complications, urinary continence, voluntary bowel movements, constipation, soiling, and requirements of bowel management program were analyzed. Patients older than 3 years and 6 months and with more than 3 months after colostomy closure were included in the analysis of functional results.

Results

The mean age at the time of operation was 21.8 months. Urogenital anomalies were seen in 6 (75%) of the 8 girls. Two patients had SRs less than 0.6. Only 3 patients in each group were considered for evaluation of functional results. Mean age was 67 months in 3 (G1) and 64 months in 3 (G2) patients. All 6 patients were continent of urine. Only 1 in G1 had an SR less than 0.6. Voluntary bowel movements were present in 1 patient in G1 and 2 in G2. One patient in G1 had severe constipation and grade 2 soiling requiring bowel management program (SR <0.6), and 1 patient in G2 had occasional soiling.

Conclusions

Laparoscopy allowed an optimal view of the pelvis and helped to achieve a low dissection of the fistula. Although the number of patients in this study is few, functional results with LT seem to be comparable to the open PSARP approach when done by experienced pediatric surgeons. The laparoscopic approach may be considered as an option for the treatment of this rare anomaly.     

Outcome of males with high anorectal malformations treated with laparoscopic-assisted anorectal pull-through: preliminary results of a comparative study with the open approach in a single institution

Objective

The aim of this study was to analyze the outcome of males with HARM treated with a laparoscopic-assisted anorectal pull-through compared with the open posterior sagittal approach in a single institution.

Methods

This study includes 32 patients: 17 (9 with a rectoprostatic fistula [RPF] and 8 with a rectovesical fistula [RVF]) who underwent laparoscopic-assisted anorectal pull-through from October 2001 onward and 15 (8 with an RPF and 7 with an RVF) treated by posterior sagittal approach before that date. Patients were reviewed retrospectively but were operated on by the authors and had longitudinal follow-up. Parameters analyzed included associated anomalies, sacral ratio (SR) index, age at surgery, operative time, complications, presence of voluntary bowel movements, constipation, and soiling. A good outcome was determined by absent or grade 1 soiling and a poor outcome result by soiling grades 2 and 3.

Results

Mean age at surgery was 22 and 37.5 months for patients with RPF and RVF, respectively, in the laparoscopic group and 29.2 and 25.7 months in the open group. Operative time was significantly shorter (P < .0036) for the laparoscopic RVF repair compared with the open approach. In patients with RPF, 50% in the laparoscopic (L) and 37.5% in the open (O) approach had an SR below 0.6. Fifty percent of all patients with RVF had an SR below 0.6, making groups comparable in terms of evaluating bowel function. Four patients were excluded in the analysis of functional results. Voluntary bowel movements with previous defecatory sensation were present in 83.l3% (5/6) in L vs 87.5% (7/8) in O patients with RPF and 62.5% (5/8) L vs 50% (3/6) in O patients with RVF. Grade 1 soiling was present in 50% (3/6) vs 62.5% (5/8) of patients with RPF and 37.5% (3/8) vs 16% (1/6) of patients with RVF in the L and O groups, respectively. Soiling grade 2 or 3 was present in 50% (3/6) vs 12.5% (1/8) of patients with RPF and 37.5% (3/8) vs 50% (3/6) of patients with RVF in the L and O groups, respectively. The risk of poor outcome was 61% in the group with SR lower than 0.6 vs 13% in the group with a higher ratio. By stratifying the groups according to type of surgery or anatomical type, these results were maintained.

Conclusion

The laparoscopic approach is a reasonable surgical option for the management of HARM. Laparoscopic approach was less time consuming in patients with RVF without impairing functional results.     

Outcome of patients after single-stage repair of perineal fistula without colostomy according to the Krickenbeck classification

Purpose

The aim of the study is to assess the characteristics and outcome of anorectal malformation (ARM) patients who underwent single-stage repair of perineal fistula without colostomy according to the Krickenbeck classification.

Methods

From 2002 to 2013, twenty-eight males and four females with perineal fistula who underwent single-stage repair without colostomy in our institute were included in this study. Patients with perineal fistula who underwent staged repair were excluded. Demographics, associated anomalies, and operative complications were recorded. The type of surgical procedures and functional outcome were assessed using the Krickenbeck classification.

Results

Six patients had associated anomalies, including two patients with renal, two with cardiac, one with vertebral, and one with limb abnormalities. Thirteen patients underwent perineal operation, and fourteen patients underwent anterior sagittal approach in the neonatal period. One patient underwent anterior sagittal approach, and four patients underwent PSARP beyond the neonatal period. One patient had an intra-operative urethral injury and one a vaginal injury. Complications were not associated with the type of surgical procedure (p = 0.345). All perineal wounds healed without infection. By using the Krickenbeck assessment score, all sixteen children older than five years of age had voluntary control. One patient had grade 1 soiling, and no patient had constipation.

Conclusions

Single-stage operation without colostomy was safe with good outcomes in patients with perineal fistula. The use of Krickenbeck classification allows standardization in assessment on the surgical approach and on functional outcome in ARM patients.     

Antegrade colonic enemas and intestinal diversion are highly effective in the management of children with intractable constipation

Purpose

Intractable constipation in children is an uncommon but debilitating condition. When medical therapy fails, surgery is warranted; but the optimal surgical approach has not been clearly defined. We reviewed our experience with operative management of intractable constipation to identify predictors of success and to compare outcomes after 3 surgical approaches: antegrade continence enema (ACE), enteral diversion, and primary resection.

Methods

A retrospective review of pediatric patients undergoing ACE, diversion, or resection for intractable, idiopathic constipation from 1994 to 2007 was performed. Satisfactory outcome was defined as minimal fecal soiling and passage of stool at least every other day (ACE, resection) or functional enterostomy without abdominal distension (diversion).

Results

Forty-four patients (range = 1-26 years, mean = 9 years) were included. Sixteen patients underwent ACE, 19 underwent primary diversion (5 ileostomy, 14 colostomy), and 9 had primary colonic resections. Satisfactory outcomes were achieved in 63%, 95%, and 22%, respectively. Of the 19 patients diverted, 14 had intestinal continuity reestablished at a mean of 27 months postdiversion, with all of these having a satisfactory outcome at an average follow-up of 56 months. Five patients underwent closure of the enterostomy without resection, whereas the remainder underwent resection of dysmotile colon based on preoperative colonic manometry studies. Of those undergoing ACE procedures, age younger than 12 years was a predictor of success, whereas preoperative colonic manometry was not predictive of outcome. Second manometry 1 year post-ACE showed improvement in all patients tested. On retrospective review, patient noncompliance contributed to ACE failure.

Conclusions

Antegrade continence enema and enteral diversion are very effective initial procedures in the management of intractable constipation. Greater than 90% of diverted patients have an excellent outcome after the eventual restoration of intestinal continuity. Colon resection should not be offered as initial therapy, as it is associated with nearly 80% failure rate and the frequent need for additional surgery.     

Dynamic magnetic resonance imaging of the pelvic floor in children and adolescents with vesical and anorectal malformations

Background

Magnetic resonance imaging (MRI) of the pelvic floor allows better assessment of pelvic pathology and has a potential as an adjunct for therapy planning. In complex congenital malformations of the pelvic floor and continence organs, it plays a major role in assessing urinary and fecal incontinence or constipation, especially when performed as a dynamic investigation such as MRI defecography.

Patients and Methods

Twenty-three patients (3-21 years old) with urinary and/or fecal incontinence or constipation attributable to congenital malformations of the pelvic region presented at our institution. The diagnoses were anorectal malformations (18), bladder exstrophy (2), and cloacal exstrophy (3). All patients underwent static and dynamic MRI of the pelvic floor with rest, squeeze, and evacuation in supine position.

Results

Morphology and function of the pelvic floor and pelvic organs could be demonstrated in each case. The reason for urinary incontinence, fecal incontinence, or constipation could be defined, and an individual therapeutic management concept was made based upon the data obtained by the investigation.

Conclusions

The advantages of this method, in comparison to others, are direct visualization of the pelvic floor muscles and continence organs and their anatomical relationship during different functional actions. Pelvic floor dysfunction is often the reason for fecal and urinary incontinence and can be detected by MRI. Especially in children, minimizing radiation exposure is of major concern. Disadvantages are the costs and long investigation time.     

Single-stage repair for rectovestibular fistula without opening the fourchette

Background

Anorectal malformations are one of the most common congenital defects. This study is conducted to demonstrate new technique for treatment of rectovaginal fistula without disturbing the fourchette through posterior sagittal approach.

Method

All the patients of rectovestibular fistula admitted after the neonatal age were treated with posterior sagittal anorectoplasty without opening the fourchette. The results were evaluated for cosmetic appearance and anal continence.

Result

A total of 40 patients were included in our study. All patients were more than 1 month old. Operative time ranges from 70 to 150 minutes. The cosmetic appearance was good. Anal continence was good in 72% cases and fair in 20% cases. Fifteen percent of patients had minimal constipation and 7.5% patients had mucosal prolapse.

Conclusion

Single-stage repair for vestibular anus through posterior sagittal anorectoplasty without opening fourchette has a good cosmetic appearance and good anal continence.     

Transanal rectosigmoid resection for severe intractable idiopathic constipation

Introduction

Idiopathic constipation is a source of significant morbidity in children. A subset of patients is refractory to medical therapy and requires surgical intervention. We present a novel surgical technique for the management of these patients.

Methods

We reviewed the records of 288 patients with severe idiopathic constipation and soiling. Patients who were refractory to medical management and had a megarectosigmoid underwent a transanal full-thickness rectosigmoid resection with a primary colo-anal anastomosis.

Results

Fifteen patients underwent a transanal rectosigmoid resection. The preoperative contrast enema demonstrated an enormously dilated rectosigmoid in 14. An average of 43 cm (range, 8-98 cm) of rectosigmoid was resected. Of 14 patients with more than 3 months of follow-up, the preoperative laxative dose was 68 mg of senna/d (range, 52-95 mg), which decreased to 8.6 mg postoperatively (P < .001). Nine patients are clean without soiling, 1 is more prone to diarrhea, but is clean. Two patients soil occasionally, but are noncompliant, and 2 were lost to follow-up.

Conclusion

Transanal rectosigmoid resection for medically intractable idiopathic constipation resulted in a dramatic reduction or elimination in laxatives use while preserving continence. It is a useful alternative to surgical options such as other colonic resections, antegrade enemas, and stomas.     

Redo surgery in Hirschsprung disease: what did we learn? Unicentric experience on 70 patients

Purpose

This article is aimed at describing a series of 70 patients who underwent a redo pull-through in the period between January 1991 and January 2007 and to compare them with a series of consecutive patients with Hirschsprung disease who underwent a single pull-through.

Methods

The notes were reviewed, and a comprehensive interview based on a disease-specific questionnaire for continence and quality of life was submitted to all eligible patients. We used a number of consecutive patients who underwent a single pull-through for Hirschsprung disease as control group.

Results

Seventy patients were included. Residual aganglionosis in pulled-through bowel or retained aganglionic rectum represented the indications to reoperation in most cases. Intestinal obstruction represented the leading symptom in more than 60% of patients. We adopted either the endorectal pull-through, the Duhamel, or the Swenson procedure. Thirty-six patients were assessed for long-term outcome. One third experienced complications. Long-term continence was satisfactory in 70%.Enterocolitis, soiling, and perineal excoriations were complained by 30%. Excellent to good perspectives were described by 94%, and excellent to good cosmetic results by 22%. The only significant difference with control group (109 patients) involved cosmetic appearance, whereas soiling, although more frequent, could not be considered significantly increased.

Conclusions

Patients who experience failure of a pull-through must go through multiple procedures to achieve definitive results. Although overall outcome does not significantly differ from that of a single effective pull-through, great efforts should be made to minimize complications and avoid the need for a redo, which requires experienced surgeons and highly committed families for a longer course of the disease.     

Primary anorectoplasty in females with common anorectal malformations without colostomy

Background/Purpose

The objective of this study is to assess the feasibility of primary posterior sagittal anorectoplasty in vestibular fistula without a covering colostomy.

Methods

Girls presenting from July 1997 to July 2005 with vestibular fistula were included prospectively in the study, in a nonrandomized manner, after excluding those with megarectosigmoid and pouch colon. All underwent primary posterior sagittal anorectoplasty after total gut irrigation with normal saline. They were kept nil per oral until the fifth postoperative day. No patient was started on anal dilatation. Patients were assessed for immediate and delayed complications as well as voluntary bowel movements and continence.

Results

A total of 72 patients with an age range of 1.5 months to 8 years (median, 9 months) were studied after excluding 7 with pouch colon and 3 with megarectosigmoid. Of the 72, 3 had undergone previous surgery. Follow-up ranged from 7 months to 8 years. No wound dehiscence or recurrence of fistula was noted. There were 5 mild wound infections. At 1 month postoperative, all patients had 1 to 3 stools per day with no episodes of soiling. None required anal dilatations, laxatives, or enemas.

Conclusions

Primary posterior sagittal anorectoplasty in vestibular fistula can be performed without a covering colostomy provided fecal contamination of the wound can be kept to the minimum in the first postoperative week. We achieve this by thorough total gut irrigation preoperatively and keeping the child nil per oral for the first 5 postoperative days. Continence rates are excellent and postoperative constipation is unlikely if megarectosigmoid and pouch colon are ruled out before surgery. Anal dilatation is not required after surgery.     

Bowel symptoms in children with anorectal malformation — a follow-up with a gender and age perspective

Background

Gender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling.

Purpose

To assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age.

Method

All children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8 years, was analyzed.

Results

Among those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p = 0.005) whereas constipation occurred in 62% of the females and 35% of the males (p < 0.001). No bowel symptoms differed between the females with perineal and vestibular fistulas (p > 0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p = 0.013). Bowel symptoms did not change with age among the females.

Conclusion

Gender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes.     

Robotic-assisted anorectal pull-through for anorectal malformations

Background/purpose

Many reports have addressed the feasibility and safety of using robotic surgery in children. To our knowledge, no published report has described the use of a surgical robot in the repair of anorectal malformations (ARMs).

Methods

Included children underwent robotic-assisted repair of ARMs with rectourethral fistula between April 2006 and March 2010 at King Khalid University Hospital, Riyadh, Saudi Arabia, using the da Vinci Surgical System. Their medical records were reviewed with respect to demographic data, associated anomalies, techniques and operative procedures, complications, outcomes, and follow-up.

Results

Five male infants (mean age, 6.6 months) underwent robotic-assisted repair of ARMs with rectourethral fistula using the Georgeson technique. The fistulae were divided and ligated in 4 patients and was left open in 1. All procedures were successfully completed without conversion to an open technique. One patient developed left-sided epididymo-orchitis postoperatively. All the patients had their colostomy closed. The follow-up ranged from 6 to 36 months. Fecal continence was difficult to assess in 2 patients. Two patients have voluntary bowel movements without soiling. One infant has fecal soiling and is on a laxative/enema for constipation.

Conclusions

Robotically assisted repair of ARMs with rectourethral fistula is feasible and safe. It offers a good alternative to the criterion standard, posterior sagittal anorectoplasty (PSARP), for repair of ARMs with rectourethral fistula. More patients and a longer follow-up period are needed for further evaluation of this novel approach.     

The antegrade continence enema successfully treats idiopathic slow-transit constipation

Background

Antegrade continence enemas (ACEs) are successful for constipation and/or fecal incontinence caused by anorectal malformations or spina bifida but have been thought to be less successful in the treatment for patients with colonic dysmotility. We studied the long-term efficacy of ACE in a large group of patients with idiopathic slow-transit constipation (STC).

Methods

We identified 56 children with an appendicostomy for ACE with radiologically proven STC. An independent investigator (SKK) performed confidential telephone interviews.

Results

We assessed 42 of 56 children (31 boys) of mean age 13.1 years (range, 6.9-25). Mean follow-up was at 48 months (range, 3-118). Mean symptom duration before appendicostomy was 7.5 years (range, 1.4-17.4). Indications for appendicostomy were soiling (29/42), inadequate stool evacuation (7/42), and recurrent hospital admissions for nasogastric washouts (6/42). Both quality of life (Templeton quality of life [P < .0001]) and continence (modified Holschneider continence score [P < .0001]) improved with ACE. Soiling frequency decreased in 32 of 42 (11/32 completely continent). Thirty-seven of 42 children had reduced abdominal pain severity (P < .0001) and frequency (P < .0001). Complications included granulation tissue (33/42), stomal infection (18/42), and washout leakage (16/42). Fifteen of 42 children ceased using the appendicostomy (7/15 symptoms resolved). Thirty-five of 42 families felt that their aspirations had been met.

Conclusions

Antegrade continence enemas were successful in 34 (81%) of 42 children with STC, contradicting views that ACEs are less effective in patients with colonic dysmotility.     

Left colonic antegrade continence enema: experience gained from 19 cases

Purpose

As problems have developed with the right colonic antegrade continence enema procedure (Malone's procedure/Monti's retubularized ileocolostomy), left colonic antegrade continence enema (LACE) procedure, in which retubularized ileum or sigmoid colon is anastomosed into the sigmoid colon, has gained popularity. The aim of the study was to describe our experience with the LACE procedure.

Methods

We retrospectively reviewed 19 LACE procedures that were performed at the Yonsei University College of Medicine Hospital (Seoul, Korea) from March 2001 to March 2005.

Results

Male-to-female ratio was 11:8, with median age of 10 years (range, 3-34 years). Most common diagnosis was meningomyelocele (78.9%, 15/19). The median total follow-up period was 23 months (range, 3-37 months); median antegrade continence enema volume used was 600 mL (range, 250-1500 mL); and median transit time was 30 minutes (range, 15-60 minutes). Patients performed antegrade continence enema with an average of once every 2 days (range, 0.3-3 days). Social continence was achieved in 14 patients (73.7%). Regurgitation of fecal material through stoma was not reported at all in 17 patients (89.5%).

Conclusions

We recommend LACE as the procedure of choice for children with congenital malformations or any other condition predisposing to fecal incontinence or constipation intractable to conventional treatment.     

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.     

The Malone antegrade continence enema procedure: the Amsterdam experience

Background

The Malone antegrade continence enema (MACE) procedure has been previously described as a safe and effective option for the treatment of children with chronic defecation disorders when maximal medical therapy and conventional treatment have failed.

Purpose

To evaluate clinical success, complications, and quality of life of children with chronic defecation disorders with a MACE stoma.

Methods

A retrospective analysis of 23 patients who underwent the construction of a MACE stoma was performed. Preoperative and postoperative data were evaluated. A specific questionnaire was used to assess patient satisfaction.

Results

A significant increase was found in defecation frequency (1.0 [range, 0-4] pretreatment vs 5.5 [range, 0-28] posttreatment per week; P < .006) and a significant decrease in fecal incontinence frequency (10 [range, 0-14] pretreatment vs 0 [range, 0-14] posttreatment per week; P < .034). Postoperative complications of the MACE procedure were fecal leakage (43%), wound infection (52%), and stomal stenosis (39%). A total of 86% of the patients were satisfied with the results of the Malone stoma (n = 21).

Conclusions

The MACE procedure is an effective treatment in children with intractable defecation disorders. Postoperative complications are, however, not uncommon. Further refinement of the technique focused to reduce the complication rate is necessary to expand the application of this approach.     

Quality of life in children with slow transit constipation

Background

Slow transit constipation (STC) causes intractable symptoms not readily responsive to laxatives, diet, or life-style changes. Children with STC have irregular bowel motions associated with colicky abdominal pain and frequent uncontrollable soiling.This study assessed the physical and psychosocial quality of life (QOL) in children with long-standing (≥2 years) STC vs healthy controls.

Methods

Children (aged 8-18) were recruited from gastrointestinal and surgical clinics and a Scout Jamboree. After informed consent was obtained, the questionnaire (Pediatric Quality of Life Inventory) was administered. This consists of parallel child and parent self-report scales encompassing physical functioning, emotional functioning, social functioning, and school functioning. Higher scores indicate better QOL. P value less than .05 was considered statistically significant.

Results

In 51 children with STC (mean, 11.5 years; male/female, 2:1) and 79 controls (mean, 12.1 years; male/female, 1.9:1), Pediatric Quality of Life Inventory QOL score was significantly lower in the STC group (72.90 vs 85.99; P < .0001). In addition, parents of children with STC reported a significantly lower QOL score than their child compared with the child's own report (64.43 vs 72.90; P = .0034). Parents of controls did not (84.25 vs 85.99; P = .12).

Conclusions

Slow transit constipation is a debilitating condition affecting both physical and emotional functioning in children. Parental perception of QOL is significantly worse, highlighting the considerable family impact of constipation and uncontrollable soiling.     

Long-term bowel function and quality of life in children with Hirschsprung's disease

Background/Purpose

Little is known about the quality of life (QOL) of children with Hirschsprung's disease (HD) as they grow older. The purpose of this study was to measure the QOL and bowel function of these children as they mature.

Methods

All children who were surgically treated for HD at British Columbia Children's Hospital, Vancouver, British Columbia, Canada between 1986 and 2003 were invited to participate. Each family was sent 3 previously validated questionnaires exploring current QOL and bowel function.

Results

Fifty-one families participated (49%), with children between the ages of 3 and 21 years. Fecal continence improved significantly with age (P = .04) and was the strongest predictor of QOL scores of all variables in our study. There was no statistically significant difference in QOL scores between children with HD and healthy children, although a clinically relevant impairment in QOL may be present, especially in psychosocial scores.

Conclusions

Fecal continence is an important predictor of overall QOL in children surgically treated for HD. Although continence tends to improve with age, a number of older children still have ongoing continence problems, and they seem to be a group at risk for impaired QOL. Our study indicates that interventions for children with incontinence may offer gains in QOL as well as bowel function.     

Single-stage repair of vestibular and perineal fistulae without colostomy

Purpose

This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute.

Methods

From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study. All patients were assessed for immediate and delayed complications including continence of the neorectum.

Results

From January 2000 to June 2007, 123 patients having vestibular (94) and perineal fistulae (29), age range from 28 days to 10 years, were subjected to primary repair either by the ASARP (34) or PSARP (89) technique. Follow-up period ranged from 3 months to 7 years. Mortality was nil. Constipation (25.68%) was the major long-term problem. Incontinence occurred in 1 patient (1.85%), who also had associated sacral agenesis. A total of 98.15% of patients were continent with stool frequency of 1 to 4 per day. Recurrence of fistula (0.81%), anal stenosis (6.76%), mucosal prolapse (2.70%), and anterior migration of the neoanus (1.35%) were the other major problems. Other minor problems like wound infection, superficial wound dehiscence, transient constipation, and diarrhea, etc, were successfully managed by local wound care, antibiotics, laxatives, enema, anal dilatation, and dietary changes.

Conclusion

Primary anorectoplasty either by PSARP or ASARP is feasible in vestibular and perineal fistulae without covering colostomy. Associated sacral agenesis/hypoplasia, redundant rectosigmoid or pouch colon, and wound infections with dehiscence are the major confounding factors affecting overall outcome. Better outcome in terms of continence can be achieved by careful surgical technique and follow-up along with proper toilet training. Complication rate was greater in cases of vestibular fistula than of perineal fistula, regardless of technique used. Some sort of laxatives and enema are often required. Dilatation of the neoanus for varying periods is also needed.