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.     

What happens to children with idiopathic constipation who receive an antegrade continent enema? : An actuarial analysis of 80 consecutive cases

Introduction

There is uncertainty about the prognosis for children with idiopathic constipation who opt for treatment by colonic lavage using an antegrade continent enema (ACE). The aim of this study was to perform an actuarial analysis of the outcomes of the ACE in children consecutively referred to our unit for this procedure, who suffered from idiopathic constipation and who had failed to respond to 3 years of medically supervised conservative management.

Methods

This study is a prospective analysis of the outcomes of 80 children with uncontrolled idiopathic constipation who underwent construction of an ACE by 1 surgeon.

Results

Twelve children were able to stop using their ACE because of resolution of their symptoms. The probability of a child who has idiopathic constipation being able to stop colonic lavage was 0.2, 6.2 years after construction of the ACE. In this group, the estimated mean time to have an ACE reversed was 8.8 years. Twelve children did not achieve satisfactory colonic lavage and either gave up (4) or deteriorated and had alternative treatment for their symptoms (8). The probability of ACE failure is 0.3 at 8.5 years after construction. Girls were significantly more likely to fail than boys, and colonic transit time was significantly longer among children who subsequently required alternative treatment for their symptoms.

Conclusions

Children with idiopathic constipation whose symptoms fail to resolve with medical management and who are treated with an ACE have 0.2 probability of cure, 0.3 probability of failure, and 0.5 probability of having to continue with colonic lavage after 6 years of colonic lavage.     

A pilot study using total colonic manometry in the surgical evaluation of pediatric functional colonic obstruction

Background/purpose

Total colonic manometry (TCM) can directly measure intraluminal pressures and contractile function of the entire colon. The utility of TCM to guide the surgical management of functional colonic obstruction has not been reported.

Methods

Total colonic manometry was performed on all patients referred for surgical evaluation of refractory functional colonic obstruction. Manometric tracings were obtained while fasting, after feeding, and after pharmacologic stimulation.

Results

Nine patients were referred for refractory colonic obstruction. The mean age was 4.8 years, and the mean duration of follow-up was 29 months. Two patients had functional obstruction after repair of Hirschsprung’s disease, and 7 patients had idiopathic functional obstruction. In the idiopathic group, 4 distinct motility patterns were identified: (1) normal colonic motility, (2) dysmotility with massive distension, (3) persistent segmental dysmotility, and (4) global neuropathy/myopathy. Both Hirschsprung’s patients showed globally abnormal motility. Surgical management was guided by TCM results. There was significant improvement in bowel function and weight gain after manometry-guided intervention. An unnecessary laparotomy was avoided in 2 patients.

Conclusions

TCM can be valuable in deciding the need for and timing of diversion, the extent of resection required, and the suitability of the patient for restoring bowel continuity in refractory functional obstruction.     

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.     

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.     

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.     

Colonic manometry as predictor of cecostomy success in children with defecation disorders

Purpose

The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders.

Methods

Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence.

Results

Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility.

Conclusion

Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.     

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.     

Continent urinary reconstruction in rhabdomyosarcoma: a new approach

Purpose

The authors present here 5 cases of continent urinary diversion in rhabdomyosarcoma applying a recently described technique for the Mitrofanoff Principle devised by the authors.

Methods

Two previously irradiated rhabdomyosarcoma patients presenting with residual bladder disease and massive sensitive urinary urgency underwent a transverse colonic reservoir with catheterizable stoma. Two other patients presenting with a Bricker conduit underwent conversion into an ileal reservoir. One patient underwent reconstruction after a cystectomy.

Results

All patients were continent and able to perform continent intermittent catheterization.

Conclusions

The technique proved to be feasible for this group of patients. The authors believe that because of its simplicity, it should be an option of continent urinary diversion when the Mitrofanoff Principle is considered.     

Cecal volvulus: a report of two cases occurring after the antegrade colonic enema procedure

Background

Many children with chronic constipation and fecal incontinence have benefited from the antegrade colonic enema (ACE) procedure. Routine antegrade colonic lavage often allows such children to avoid daytime soiling. This report describes 2 children in whom the ACE procedure was complicated by a cecal volvulus.

Methods

A retrospective review of 164 children with an ACE procedure was conducted. Two instances of cecal volvulus were identified.

Results

The first child presented with abdominal pain and difficulty intubating the ACE site. Over the subsequent day, his pain worsened, and radiographs depicted a colonic obstruction. At laparotomy, a cecal volvulus resulting in bowel necrosis was observed, and resection of the affected bowel and appendix (in the right lower quadrant) and end ileostomy was required. He subsequently had the stoma closed and a new ACE constructed with a colon flap. The second child presented with shock and evidence of an acute abdomen. At laparotomy, a cecal volvulus was noted, and ileocolic resection including the ACE stoma (located at the umbilicus) and an ileostomy and Hartmann pouch was performed. He had a protracted hospital course requiring ventilator and inotropic support. He currently is well and still has an ileostomy stoma.

Conclusions

A high index of suspicion for a potentially life-threatening cecal volvulus should be maintained in children undergoing an ACE procedure who present with abdominal pain, evidence of bowel obstruction, or difficulty in advancing the ACE irrigation catheter.     

Bowel function remains subjectively unchanged after ileal resection for construction of continent ileal reservoirs

Background

Construction of a continent catheterizable urinary reservoir or an orthotopic bladder substitute requires substantial bowel resection, which can cause changes in bowel transit time. The reported incidence of chronic diarrhea after ileocecal resection is about 20%. Studies assessing bowel function after resection of 55-60 cm of ileum without compromising the ileocecal valve are scarce, and long-term results have not been reported.

Objective

Prospective assessment of possible changes in bowel function (eg, stool frequency, diarrhea) and the potential impact on quality of life in patients with resection of small bowel for urinary diversion.

Design, setting, and participants

A total of 82 patients who underwent radical cystectomy, extended lymph node dissection, orthotopic ileal bladder substitution, or heterotopic continent cutaneous urinary diversion with a follow-up >1 yr after surgery were prospectively evaluated. Patients who had a neurogenic bladder disorder, had undergone previous radiotherapy, or had not completed the questionnaire were excluded from the study. The validated Gastrointestinal Quality of Life Index was completed by the patients preoperatively and at 3, 12, and 24 mo postoperatively. Five points concerning bowel function (frequent bowel movement, urgent bowel movement, diarrhea, constipation, or uncontrolled stool loss) were assessed, and the median scores were compared pre- and postoperatively.

Results and limitations

Most patients (≥80%) were rarely or never troubled by frequent or urgent bowel movements, diarrhea, constipation, or uncontrolled stool loss preoperatively. In the case of stool frequency, a remarkable shift from rarely to never was observed postoperatively at 3, 12, and 24 mo. Scores for constipation and uncontrolled stool loss remained unchanged throughout the whole time period. For urgent bowel movements the median preoperative score of 4 decreased to 3 at 3 mo and 12 mo and returned to 4 at 24 mo. For diarrhea the preoperative score of 4 decreased to 3 at 3 mo and 24 mo and remained at 4 after 12 mo.

Conclusions

No relevant changes in bowel movements were found after resection of 55-60 cm of ileum if the terminal ileum and the ileocecal valve were left intact.     

Nuclear transit studies of patients with intractable chronic constipation reveal a subgroup with rapid proximal colonic transit

Aims/Background

Nuclear transit studies (NTS) allow us to follow transit through the stomach and the small and large intestines. We identified children with chronic constipation with rapid proximal colonic transit and characterized their clinical features.

Methods

We reviewed NTS from 1998 to 2009 to identify patients with chronic constipation and rapid proximal colonic transit, defined as greater than 25% of tracer beyond hepatic flexure at 6 hour and/or greater than 25% of tracer beyond end of descending colon at 24 hour. This was correlated with clinical symptoms and outcome from patient records.

Results

Five hundred twenty children with chronic constipation underwent investigation by NTS, and 64 (12%) were identified with rapid proximal colonic transit. The clinical history, symptoms, and outcome in 55 of 64 available for analysis frequently showed family history of allergy (10.9%) and symptoms associated with food allergy/intolerance: abdominal pain (80%), anal fissure (27.3%), and other allergic symptoms (43.6%). Eighteen children were treated with dietary exclusion, with resolution of symptoms in 9 (50%).

Conclusions

Some children with intractable chronic constipation have rapid proximal colonic transit, have symptoms consistent with possible food allergy/intolerance, and may respond to dietary exclusion. The NTS can identify these patients with rapid proximal transit that may be secondary to food intolerance.     

Permanent diversion rates after neoadjuvant therapy and coloanal anastomosis for rectal cancer

Background

The aim of this study was to assess the rate of permanent diversion in patients undergoing coloanal anastomosis after neoadjuvant therapy for rectal cancer.

Methods

We performed a retrospective review of patients with rectal cancer who underwent a total mesorectal excision of a tumor within 9 cm of the anal verge.

Results

There were 201 patients who underwent resection with coloanal anastomosis, with a mean follow-up period of 51 months. The average tumor distance from the anal verge was 7 cm (range, 4-9 cm). Neoadjuvant therapy was administrated in 145 patients, 47 had no radiation, and 9 received radiation postoperatively. Thirty-two patients (16%) had long-term complications including incontinence, fistulas, and strictures. Twenty-five patients (12%) had recurrent disease, 16 of these were local recurrence. The total rate of permanent diversion was 29 (14%). Reasons for diversion included local recurrence in 12 patients (6%), complications in 10 patients (5%), and poor function in 7 patients (3%).

Conclusions

Poor bowel function, late complications, and local recurrence all contribute to permanent diversion after a coloanal anastomosis. Neoadjuvant therapy in conjunction with a total mesorectal excision and coloanal anastomosis leads to acceptably low permanent diversion rates in the vast majority of patients.     

Anal ultraslow waves and high anal pressure in childhood: a clinical condition mimicking Hirschsprung disease

Purpose

Anal ultraslow waves (USWs) have been described in several clinical conditions closely related to chronic constipation associated with high anal pressure; however, USW-related clinical manifestations in childhood are poorly understood. The purpose of this study is to elucidate the clinical relevance of USWs in childhood.

Methods

Manometric recordings of 118 cases including 70 children with constipation and 16 patients with Hirschsprung disease were analyzed.

Results

Ultraslow waves were seen in 4 of 70 children with constipation. None of the controls or patients with Hirschsprung disease exhibited USWs. The 4 patients comprised 2 infants with marked abdominal distension mimicking Hirschsprung disease and 2 children (aged 4 and 8 years) with intractable constipation accompanying hemorrhoid or anal fissure. The manometric findings of the USW-positive patients showed a markedly high anal resting pressure and high frequency of slow waves compared to controls, patients with constipation not accompanied by USWs or patients with Hirschsprung disease.

Conclusion

Children with USWs exhibit symptoms mimicking Hirschsprung disease in infants and chronic intractable constipation in older children. In manometric studies of children, more attention should be paid not only to rectoanal reflex, but also USWs.     

Laparoscopic seromuscular colonic biopsies: a surgeon's experience

Background

Colonic dysmotility is a recognised cause of chronic constipation in children. Colonic dysmotility is better analysed by examination of the colonic muscle than rectal biopsy, which does not examine the defective area and has a low yield. We explored the role of laparoscopic colonic muscle biopsies to investigate children with intractable constipation. The authors describe the technique, its application, and results from a large series.

Methods

A retrospective review was conducted of all patients undergoing laparoscopic seromuscular colonic biopsies (hepatic flexure, mid-transverse colon, splenic flexure, and sigmoid colon) by a single surgeon for the investigation of chronic constipation over a 10-year period. Patient records were reviewed to determine the perforation frequency and management, postoperative recovery time and the frequency of an immunohistochemical abnormality.

Results

One hundred ninety-seven patients (118 boys) were investigated by laparoscopic biopsy during the period. The mean age was 8.0 ± 4.0 years (range, 1.4-22.4). The patients took 28.7 ± 13.6 hours (range, 8-120) to recover, with 37 (19%) having nausea and/or vomiting requiring antiemetics. Most patients (160/197, 81%) were discharged the following day. Six patients (3%) had a mucosal perforation recognised at operation (treated by an Endoloop) with no change in postoperative outcome. Two patients (1%) had an unrecognised mucosal perforation requiring laparoscopic reoperation and Endoloop closure (laparotomy/colostomy not required), with no further sequelae. Eight-six patients (44%) had a specific immunohistochemical neuropeptide anomaly (reduced substance P [84], reduced vasoactive intestinal peptide [2]).

Conclusions

Laparoscopic biopsy is a valuable tool to investigate chronic constipation in children, allowing a pathological diagnosis to be made in many cases. The complications of the procedure are acceptably low with this technique.     

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.     

Prenatal and postnatal ciliated hepatic foregut cysts in infants

Purpose

Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC.

Methods

We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis.

Results

Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery.Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia.

Conclusion

In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.     

Is the outcome of the left colon antegrade continence enema better than that of the right colon antegrade continence enema?

Background/Purpose

The purpose of this report was to review the results of the antegrade continence enema (ACE) procedure and to compare the outcomes of right and left colon ACEs in children.

Methods

Thirty patients who underwent an ACE between 1998 and 2005 were analyzed. Data were obtained based on the following parameters: postoperative soiling, catheter insertion time, colonic washout time, quality of life, and abdominal pain during and after the ACE. Twenty-nine patients were followed for an average of 3.8 years (range, 4 months-7.3 years).

Results

Right colon ACEs were performed in 23 patients, and left colon ACEs were performed in 7 patients. The common complications of the ACE included abdominal pain during and after the ACE (51.7%) and stoma strictures (41.4%). The overall ACE success rate was 24/29 (82.8%; right colon ACE, 18/29; left colon ACE, 6/29). Twenty-three patients (95.8%) believed their quality of life was improved. There were no significant differences in complications or outcomes between the right and left ACEs.

Conclusions

An ACE is an effective treatment for children with fecal incontinence. A left colon ACE has similar efficacy as a right colon ACE in managing fecal incontinence in children.     

Urinary diversion in children with pelvic tumors

Purpose

The aim of this study was to investigate children presenting with malignant pelvic tumors obstructing the upper urinary tract.

Methods

Seventeen children with upper urinary tract obstruction by a malignant tumor were reviewed. A nephrostomy tube or Double J (DJ) stent was inserted into each obstructed urinary system and removed after tumor shrinkage and/or hydronephrosis regression.

Results

There were 9 boys and 8 girls in the study; the mean age and median follow-up were 5.7 years and 2.5 years, respectively. The most common obstructing tumor was rhabdomyosarcoma. Twelve children underwent diversion by nephrostomy tubes and 3 by DJ stents; 2 patients underwent resection of the tumors with ureteroureterostomy. Complications after the insertion of the stents included febrile urinary tract infections (UTI) or pyelonephritis in 4 of the children with DJ stents. In the nephrostomy group, febrile UTI developed in 3 and the tube fell out in 1, and was blocked in another. Of the 17 children, 9 have no evidence of disease, 2 are currently under treatment, and 6 died of cancer.

Conclusions

The prognosis of children with malignant pelvic tumor obstructing the upper urinary system justifies urgent and optimal upper tract diversion, enabling chemotherapy to be started immediately.     

Megarectum after surgery for anorectal malformations

Purpose

Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction.

Methods

Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence.

Results

There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology.

Conclusions

Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.