10-Year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeck classification

Background

Anorectal malformations (ARMs) affect 1 in 4000 to 5000 births. The Krickenbeck conference developed a classification based on anatomical and functional criteria to better compare treatment outcome.

Aim

The aim of this study is to evaluate the functional outcome in patients 10 years following standardized surgical treatment of ARM related to the Krickenbeck classification. .

Methods

Anatomical anomalies were classified as above. Children and carers were followed closely in a multidisciplinary clinic. Data were collected using a functional outcome questionnaire for a minimum of 10 years after surgical reconstruction. Outcome measurements were related to the Krickenbeck classification.

Results

There were 53 children in the study group (29 male, 24 female). Krickenbeck anatomy: perineal fistula, 36%; vestibular fistula, 26%; rectourethral fistula, 36%; rectovesical fistula, 2%.All children were treated by posterior sagittal anorectoplasty.In children with perineal fistula, continence was achieved in 90%. Grade 2 constipation was noted in 21%. One child had a Malone antegrade continence enema (MACE) procedure.In children with vestibular fistula, continence was achieved in 57%. Grade 3 constipation was noted in 28%. One child had grade 1, and one child had grade 2 soiling. Two children had a MACE procedure.In children with rectourethral fistula, continence was achieved in 58%. One child had grade 3 soiling. Grade 3 constipation was found in 42% of children and grade 2 constipation in 1 child. A MACE procedure was performed in 36%.The only child with a bladder neck fistula had a MACE procedure for intractable soiling.

Conclusions

The outcome for patients with ARM is related to the severity of the anomaly. The uniform application of the Krickenbeck classification should allow rational comparison of treatment outcome.     

Antegrade enemas for defecation disorders: do they improve the colonic motility?

Purpose

The aim of the study was to describe the changes in colonic motility occurring after chronic antegrade enema use in children and young adults.

Methods

Colonic manometry tracings of patients who had used antegrade enemas for at least 6 months and were being evaluated for possible discontinuation of this treatment were retrospective reviewed.

Results

Seven patients (median age of 12 years, range 3-15 years) met our inclusion criteria. Four patients had idiopathic constipation, 2 had tethered cord, and 1 had Hirschsprung disease. Colonic manometry before the use of antegrade enemas showed dysmotility in 6 (86%) children, mostly in the distal colon. None of the patients underwent colonic resection between the 2 studies. All the patients had colonic manometry repeated between 14 and 46 months after the creation of the cecostomy. All patients with abnormal colonic manometry improved with the use of antegrade enema with a complete normalization of colonic motility in 5 (83%) patients.

Conclusion

Use of antegrade enema alone, without diversion or resection, may improve colonic motility.     

Normal and abnormal embryonic development of the anorectum in rats

Background

The embryologic and pathologic aspect of anorectal malformation (ARM) remains poorly understood. There is no universally accepted theory to explain anorectal embryology and the abnormal development that produces ARM. The aim of this study was to observe the developmental processes of anorectum in rats and to explore the abnormal embryonic development that leads to ARM.

Methods

Rat embryos with ARM were obtained by treating pregnant rats with administration of ethylenethiourea (ETU). Normal rat embryos and embryos with ARM from gestational days 12.5 to 20 were sectioned serially and sagittally and stained with H & E. The relevant structure including cloaca and urorectal septum (URS) were examined in a temporospatial sequence.

Results

Characteristics of anorectum development in ARM rat embryos treated by ETU were as follows: (1) URS never fused with cloaca membrane. (2) Dorsal cloacal membrane was maldeveloped. (3) Cloacal configuration was abnormal. (4) Tail groove never appeared. All type of ARM was the rectourethal fistula and common cloaca in ETU-treated rat embryos and was discernible on gestation day 16.

Conclusions

Absence of the tail groove and maldevelopment of the dorsal cloacal membrane, cloacal configuration, and urorectal septum are likely to be responsible for the formation of ARM. Failure of fusion of the URS with cloacal membrane is the immediate reason for rectourethral fistula or common cloaca in ETU-treated rats.     

Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review

Background

The purpose of this study is to review the long-term clinical outcome in patients surgically treated for total colonic aganglionosis (TCA).

Methods

The hospital records of 58 patients with TCA from 2 centers were retrospectively analyzed. These patients were then followed up by personal or telephonic interviews using a standardized questionnaire.

Results

Of the 58 patients, 36 were male and 22 were female. Thirty-eight patients presented in the neonatal period. A total of 13 (22.4%) presented with enterocolitis, 43 (74.1%) with intestinal obstruction, and 2 (3.5%) with severe constipation. All patients had disease involving the entire colon up to at least the terminal ileum. Two patients had total intestinal aganglionosis and died after ileostomy. Various pull-through procedures performed in 56 patients included Soave in 16, Swenson in 5, and Duhamel in 35 patients.Postoperatively, 31 (55.4%) patients had enterocolitis. At long-term follow-up (2-31 years), 3 patients had opted for permanent ileostomies because of intractable incontinenece or recurrent enterocolitis. Eleven patients were too young to assess bowel function or unavailable for follow-up. Of the remaining 42 patients, 22 had normal bowel control and 20 were soiling. At 5 years, patients had an average of 5.2 bowel movements per day, which reduced to a mean of 3.4 per day at the age of 15 years.

Conclusion

Patients with TCA continue to have long-term problems with bowel control, although many improve with time.     

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.     

Long-term functional sequelae of sacrococcygeal teratoma: a national study in The Netherlands

Background

Long-term functional sequelae after resection of sacrococcygeal teratoma (SCT) are relatively common. This study determines the incidence of these sequelae associated clinical variables and its impact on quality of life (QoL).

Patients and methods

Patients with SCT treated from 1980 to 2003 at the pediatric surgical centers in the Netherlands aged more than 3 years received age-specific questionnaires, which assessed parameters reflecting bowel function (involuntary bowel movements, soiling, constipation), urinary incontinence, subjective aspect of the scar, and QoL. These parameters were correlated with clinical variables, which were extracted from the medical records. Risk factors were identified using univariate analysis.

Results

Of the 99 posted questionnaires, 79 (80%) were completed. The median age of the patients was 9.7 years (range, 3.2-22.6 years). There were 46% who reported impaired bowel function and/or urinary incontinence (9% involuntary bowel movements, 13% soiling, 17% constipation), and 31% urinary incontinence. In 40%, the scar was cosmetically unacceptable. Age at completion of the questionnaire, Altman classification, sex, and histopathology were not risk factors for any long-term sequelae. Size of the tumor (>500 cm³) was a significant risk factor for cosmetically unacceptable scar (odds ration [OR], 4.73; confidence limit [CL], 1.21-18.47; P = .026). Long-term sequelae were correlated with diminished QoL.

Conclusion

A large proportion of the patients with SCT have problems with defecation, urinary incontinence, or a cosmetically unacceptable scar that affects QoL. Patients who are at higher risk for the development of long-term sequelae cannot be clearly assessed using clinical variables.     

The morbidity of constipation in patients with anorectal malformations

Background

Constipation in anorectal malformations (ARM) is extremely common, particularly in the lower types. Failure to adequately treat it can lead to significant morbidity.

Methods

From our series of over 2000 patients with ARM, we reviewed 398 with good prognosis for bowel control and a tendency toward constipation; rectoperineal fistula (63), rectovestibular fistula (114), rectobulbar urethral fistula (104), imperforate anus with no fistula (46), rectal atresia or stenosis (9), and cloaca with a common channel below 3 cm (62). Those lost to follow-up, not yet toilet-trained (<3 years old), or with poor prognostic features were excluded. We compared morbidities in patients we operated on and managed primarily (group A, n = 268) to those managed at other institutions who suffered from constipation or incontinence and were referred to us for treatment (group B, n = 130). Those we managed primarily were subjected to an aggressive senna-based laxative program, started after their primary repair or after colostomy closure.

Results

Morbidities associated with constipation were higher in the referral group and included fecal impaction (7.8% vs 38.5%), overflow pseudoincontinence (4.9% vs 33.8%), and megacolon (14.6% vs 54.6%). A loop or transverse colostomy (4.9% vs 9.2%), stoma or anorectal stricture, or a stenotic fistula (2.2% vs 28.5%) were contributing factors. Adequate laxative treatment with, in certain cases, resection of a megarectosigmoid (2.6% vs 23.1%) enabled many pseudoincontinent children to achieve bowel control (reported previously). Unneeded colorectal biopsies (1.9% vs 16.2%), Hirschsprung's-type pullthroughs (0% vs 3.1%), and, in retrospect, unneeded antegrade continent enema procedures (0% vs 3.1%) were higher in Group B. Overall, 19.8% of Group A and 66.2% of Group B experienced constipation-related morbidities.

Conclusion

The morbidity of constipation in ARM includes fecal impaction, megacolon, incontinence, and performance of unneeded surgeries. Inadequate treatment, the type of the original colostomy, and postoperative anal or stomal stricture as well as stenotic fistulae were key contributing factors. Children with ARM and good prognosis for bowel control are at the greatest risk for severe constipation and its consequences. With recognition and aggressive, proactive treatment, we have found that these morbidities can be reduced.     

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.     

Impact of cecostomy and antegrade colonic enemas on management of fecal incontinence and constipation: ten years of experience in pediatric population

Background

In childhood and adolescence, fecal soiling represents a psychologically devastating problem. Physical and emotional distress associated with daily rectal enemas is minimized by the introduction of a cecostomy tube for colonic cleansing with antegrade colonic enemas (ACEs).

Patients and Methods

Over a period of 10 years (1997-2007), we performed “button” cecostomies in 69 pediatric patients with fecal soiling secondary to a variety of disorders; laparoscopic procedures were performed in 40 and open procedures in 29. Mean postoperative follow-up was 4.03 SD ± 1.76 years. Cleansing protocols differed between patients.

Results

We adopted a standardized questionnaire concerning management of incontinence/intractable constipation before and after button cecostomy insertion to assess the long-term impact of ACE on symptom severity and quality of life. Complications included tube dislodgement (n = 9), development of granulation tissue (n = 11), decubitus ulcer (n = 5), and infection (n = 3). Patient/parents satisfaction (appraisal scale 1-3) and improvement of quality of life achieved statistical significance for both (P < .001).

Conclusions

Since button cecostomy and ACE were introduced in our institution as a management option, the treatment of fecal incontinence and intractable constipation significantly improved in terms of efficacy and patient compliance and also resulted in greater patient and parent satisfaction.     

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.     

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.     

Social quality of life for adult patients with anorectal malformations

Background/Purpose

Pediatric surgeons who performed the initial surgery on anorectal malformations (ARM) lose contact with the patients as they become adults. In the present study, we examined 20- to 40-year-old adult patients with a history of surgery for ARM and analyzed them from the points of social quality of life.

Patients and Methods

Twenty-nine patients with ARM, aged 20 to 40, were surveyed by questionnaire or personal interview. Thirteen with high-type and 9 with intermediate-type anomalies underwent abdominoperineal rectoplasty, and 7 with low-type anomalies underwent perineoplasty between 1965 and 1985. Responses were analyzed from the perspectives of bowel, urinary, and sexual functions and social activity.

Results

One third of patients with high- or intermediate-type anomalies occasionally complained of fecal soiling. However, the other patients gained good bowel function and enjoyed occupational or student life without problems. Fecal soiling was the key factor disturbing occupational life, although the problem remained within a socially manageable level. All of the patients with ARM had normal urinary function. Three of the 18 male patients had sexual problems such as erectile or ejaculatory dysfunction because of associated genitourinary anomalies. Nine of the 11 female patients had regular menstruation and the other 2 had irregular menstrual periods. Five female patients were married and 4 of the 5 had children (1-5 children). Modes of delivery were normal vaginal delivery in 8 and cesarean section in 1. Only one of the 5 had a slight sacral anomaly. However, she had no apparent abnormality of bladder function and got through pregnancy and delivery without difficulty.

Conclusion

One third of adult patients with high- or intermediate-type anomalies after abdominoperineal rectoplasty had some problems in bowel function. Fecal soiling was the key factor that disturbed their occupational life. Most of the patients had normal urinary and sexual functions if they did not have associated genitourinary anomalies and enjoyed social activities.     

Effect of intravenous erythromycin on the colonic motility of children and young adults during colonic manometry

Purpose

Erythromycin is successfully used as a gastroduodenal prokinetic agent. Given the limited available treatments for colonic dysmotility, further investigation into erythromycin's effect on colonic motility is warranted. We aimed to study the effect of erythromycin on colonic motility in pediatric patients with recalcitrant chronic constipation/encopresis and other suspected colonic motility disorders.

Methods

Patients referred for colonic manometry were eligible for enrollment. Fasting motility was recorded for 1 to 2 hours, then erythromycin lactobionate (EL), 3 mg/kg, was administered intravenously, and colonic motility was monitored for 1 to 2 hours after erythromycin. Manometry was then continued per routine. The motility index (MI) of pressure tracings at each pressure transducer was calculated for each patient for a period of 15 and 60 minutes before and after EL infusion. Change in MI was compared by Wilcoxon signed rank test.

Results

Twenty patients were enrolled. The most common indication was constipation with encopresis. Seventy percent of patients had normal colonic manometry, and 30% of patients demonstrated a neuropathy. Average MI for the 60-minute period before and after EL infusion were 254 ± 74 mm Hg/h and 253 ± 94 mm Hg/h, respectively (P = .55). Average MI for the 15-minute period before and after EL infusion were 64 ± 23 mm Hg/15 min and 69 ± 32 mm Hg/15 min, respectively (P = .45).

Conclusions

Administration of intravenous EL resulted in no changes in colonic MI in pediatric patients referred for colonic manometry. Further studies on potential colokinetic agents are warranted in this population of patients.     

Transanal one-stage endorectal pull-through for Hirschsprung's disease: a multicenter study

Background/purpose

Transanal endorectal pull-through (TEPT) is the latest development in treatment of Hirschsprung’s disease (HD). This prospective study was designed to evaluate the safety and efficacy of 1 stage TEPT technique in the management of patients with HD.

Methods

One hundred forty-nine children (116 boys and 33 girls) aged 8 days to 14 years underwent 1 stage TEPT procedure over an 18-month period at 5 Egyptian academic pediatric surgical centers and affiliated hospitals. Median follow-up was 12 months (range, 3 to 21 months). These patients were evaluated with regard to age, sex, length of the aganglionic segment, intraoperative details, and postoperative functional results or complications. An electromyogram (EMG), endorectal ultrasound scan, and lower gastrointestinal (GI) motility studies were reserved for patients with postoperative problems with bowel control.

Results

Mean operating time was 120.2 ± 27.8 minutes (range, 60 to 210 minutes). The average length of resected bowel was 26.8 ± 12.4 cm (range, 15 to 45 cm). Thirteen patients required laparotomy because of extension of aganglionic segment beyond the sigmoid colon in 9, tear in the mesenteric vessels in 2, and difficulties in getting to the submucosal plane in 2. Three deaths (2%) occurred 3 days, 4 days, and 4 weeks postoperatively, respectively. Postoperative complications included transient perianal excoriation in 48 patients (30 were <3 months of age), enterocolitis (n = 26), anastomotic stricture (n = 7), recurrent constipation (n = 6), hypoganglionosis at distal end of pulled through segment (n = 2), cuff abscess (n = 3), anastomotic leak (n = 1), adhesive bowel obstruction (n = 1), and rectal prolapse (n = 1). Complete anorectal continence was noted in 35 of 42 (83.3%) children older than 3 years, whereas soiling and frequent accidents still occur in 7, who showed a steady improvement in their continence status.

Conclusions

One-stage TEPT technique is both feasible and safe technique in properly selected children with rectosigmoid HD in all ages. The technique is easily learned and is associated with excellent clinical results.     

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.     

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.     

Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations

Background

Anorectal malformations (ARM) are common congenital anomalies seen throughout the world. Comparison of outcome data has been hindered because of confusion related to classification and asssessment systems.

Methods

The goals of the Krinkenbeck Conference on ARM was to develop standards for an International Classification of ARM based on a modification of fistula type and adding rare and regional variants, and design a system for comparable follow up studies.

Results

Lesions were classified into major clinical groups based on the fistula location (perineal, recto-urethral, recto-vesical, vestibular), cloacal lesions, those with no fistula and anal stenosis. Rare and regional variants included pouch colon, rectal atresia or stenosis, rectovaginal fistula, H-fistula and others. Groups would be analyzed according to the type of procedure performed stratified for confounding associated conditions such as sacral anomalies and tethered cord. A standard method for postoperative assessment of continence was determined.

Conclusions

A new International diagnostic classification system, operative groupings and a method of postoperative assessment of continence was developed by consensus of a large contingent of participants experienced in the management of patients with ARM. These methods should allow for a common standardization of diagnosis and comparing postoperative results.     

Is there a role for surgery beyond colonic aganglionosis and anorectal malformations in children with intractable constipation?

Purpose

The aim of this study was to assess benefit of surgery in the treatment of childhood constipation in children without aganglionosis or anorectal malformations.

Methods

Retrospective chart review and follow-up questionnaire of 19 children (10 girls, age 7.6 ± 3.9 years) who underwent surgery after colonic and anorectal manometry had documented abnormal motility. Children at the time of manometric evaluation had symptoms of intractable constipation a mean of 5.1 ± 2.5 years. Follow-up questionnaires were administered to caregivers a mean of 11.4 ± 9.3 months after surgical intervention.

Results

Proximal colonic abnormalities were seen in 2 patients, abnormalities involving the distal colon and rectosigmoid region in 13 patients, pancolonic abnormalities in 2 patients, and incomplete relaxation of the internal anal sphincter in 2 patients. Surgery led to increase in frequency of bowel movements per week (8.1 v 1.9; P < .005), decrease in soiling episodes per week (2.20 v 4.7; P < .01), and decrease in daily use of medications for constipation (0.8 v 2.3; P < .05). Adverse events included skin breakdown at site of ostomy (9%), fecal incontinence after pull-through procedure (13%), and persistent constipation (4.5%). Parents felt that the symptoms after surgery were completely resolved in 89% of patients.

Conclusions

Surgery may be beneficial in the management children with chronic intractable constipation and documented abnormalities in motility.     

Rectovestibular fistula—rarely recognized associated gynecologic anomalies

Introduction

Vestibular fistulas are the most common anorectal malformations (ARMs) in females. Associated gynecologic defects are rarely mentioned in the literature but may have serious clinical implications if undetected. The definitive repair of the ARM offers an opportunity for diagnosis and treatment of these conditions.

Methods

Two hundred seventy-two patients with vestibular fistula were retrospectively reviewed, with emphasis on gynecologic defects.

Results

Forty-eight patients (17%) had 83 gynecologic anomalies. Fourteen patients had a vaginal septum, all with 2 uterine cervices. All septa were resected at the main repair. Twenty-six patients had no vaginal opening. Twenty of them had absent vagina. Eighteen of those had an absent uterus. Patients with absent vagina underwent vaginal replacement with distal rectum (12), sigmoid (6), and ileum (2). Six patients had a patent upper vagina; 3 reached the perineum after mobilization and 3 required replacement; 2 with sigmoid and 1 with rectum.

Conclusion

Vaginal septa are easily diagnosed and can be resected during the repair of the vestibular fistula. The presence of 2 cervices has important obstetric implications. Absent vagina requires a technically demanding repair, with special preoperative planning. Vaginoscopy or careful inspection should precede surgical reconstruction.