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Management of Chronic Intractable Constipation in Children

Kerur, Basavaraj*; Kantekure, Kanchan; Bonilla, Silvana; Orkin, Bruce§; Flores, Alejandro F.||

Journal of Pediatric Gastroenterology and Nutrition: December 2014 - Volume 59 - Issue 6 - p 754–757
doi: 10.1097/MPG.0000000000000535
Original Articles: Gastroenterology

Background: Chronic intractable constipation (CIC) is a debilitating disease that is challenging to manage. Treatment options in children include medications, enemas, and surgical management in selected cases.

Method: We reviewed medical records of pediatric patients diagnosed as having CIC at Tufts Medical Center from 2005 to 2012. Demographic variables, diagnostic procedures, and medical and surgical outcomes were collected. Clinical outcome was defined using the Rome III criteria.

Results: A total of 14 patients were included in the study (10 boys). The age range was 10 to 21 years. All of the patients had the diagnosis of CIC. Eleven patients had cecostomy placement. During the follow-up period, 10 patients underwent total abdominal colectomy with ileorectal anastomosis, 1 had total colectomy with ileostomy, and 1 had partial colectomy with colorectal anastomosis. Successful clinical outcome was reported in 7 patients with 3 patients reporting persistent fecal incontinence. Colonic motility studies were performed on 12 patients (colonic neuropathy in 11 patients and normal study in 1 patient). Defecography was consistent with isolated pelvic floor dysfunction in 1 patient, abnormal motility and anatomy in 1 patient, pelvic floor dysfunction and abnormal motility in 2 patients, and found abnormal motility only in 5. Defecography study was normal in 5 patients. All of the patients with abnormal colonic manometry underwent a surgical procedure.

Conclusions: Anorectal manometry, colonic manometry, and defecography help in understanding the pathophysiology of defecation disorders in children. The majority of patients with abnormal colonic manometry underwent TAC-IRA. There was no statistical correlation between individual investigations (anorectal manometry, colonic manometry, and defecography) with surgical intervention (P > 0.35). TAC-IRA may be safe and useful intervention in a subset of patients when other treatment options have failed.

*Pediatric Hospitalist Division, Floating Hospital for Children

Department of Pathology and Laboratory Medicine, Tufts Medical Center

Division of Pediatric Gastroenterology, Floating Hospital for Children, Boston, MA

§Department of Colorectal Surgery, Rush University Medical Center Chicago, IL

||Division of Gastroenterology, Hepatology, and Nutrition, Boston Children's Hospital, Boston, MA.

Address correspondence and reprint requests to Basavaraj Kerur, MD, Pediatric Hospitalist Division, Floating Hospital for Children, 800 Washington St, Boston, MA 02111 (e-mail:

Received 26 September, 2013

Accepted 7 August, 2014

The authors report no conflicts of interest.

Constipation is a common problem in childhood. It accounts for 3% to 5% of pediatrician visits and 10% to 25% of referrals to gastroenterologists (1). A vast majority of children with constipation respond to medical management that includes medication and behavioral modification (2). A subset of children do not respond to medical management. These children are classified as having chronic intractable constipation (CIC) or chronic constipation refractory to medical management. Management of CIC in children is challenging and includes medication, administration of antegrade continence enemas (3), and surgical management in selected cases (4). We described the clinical outcome of a group of pediatric patients with CIC who failed to improve and underwent surgical procedures such as total abdominal colectomy with ileorectal anastomosis (TAC-IRA) and subtotal colectomies (5), with the majority of the patients experiencing successful outcome at follow-up.

Children with intractable chronic constipation should be evaluated thoroughly with physiological and radiological tests before considering surgical options because these tests may predict clinical outcomes. Defecography provides structural and functional evaluation of the rectum and anal canal, and is commonly included in the workup of chronically constipated adult patients (6). Defecography is not always included in the workup of childhood CIC, often for technical reasons. The role of defecography findings in the management of childhood CIC is unclear.

The purpose of this study was to evaluate the role of defecography and other tests included in the workup of childhood CIC and to explore the relation between investigation types and surgical outcomes.

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This was a retrospective study. We reviewed the medical records of pediatric patients diagnosed as having CIC treated at Tufts Medical Center between 2005 and 2012. Demographic variables, diagnostic procedures, medical management, surgical management, and postsurgical outcomes were collected. Clinical outcome was defined using the Rome III criteria for functional constipation as either success or failure. This study was approved by the institutional review board at Tufts Medical Center, Boston. Statistical analysis was done with IBM SPSS Statistics version 19 (IBM, Armonk, NY). Cohen κ test was used to define agreements between each defecographic finding and each result of other tests, and phi correlation was used to find any correlation between investigations done and surgical intervention.

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A total of 14 patients were included in the study (10 boys). The age range was 10 to 21 years. All of the patients had the diagnosis of CIC. In addition, 6 patients had the following coexisting conditions: spinal bifida (1 patient), celiac disease (1 patient), gastroesophageal reflux disease (2 patients), and behavioral problems (2 patients). The majority of the patients in this study had a surgical intervention during the follow-up course.

Findings on anorectal manometry included normal rectoanal inhibitory reflex in 10 patients, low resting pressure of internal anal sphincter in 1 patient, and incomplete relaxation of IAS in 1 patient. Anorectal study was not performed in 2 patients. Gastric motility study using solid meal was performed in 7 patients, and 3 had abnormal anorectal study with delayed emptying time. Colonic manometry studies were performed on 12 patients; colonic neuropathy was seen in 10 patients and normal study in 2 patients. Barium enema study was normal in 4 patients, 4 patients showed moderate retention of stool, and 2 of these had redundant sigmoid colon. Rectal biopsies showed normal ganglion cells in all of the patients. Colonoscopy and rectal biopsies were normal in all of the patients.

Thirteen patients underwent defecography. Defecography was consistent with isolated pelvic floor dysfunction in 1 patient, abnormal motility and anatomy in 1 patient, pelvic floor dysfunction and abnormal motility in 2 patients, and isolated abnormal motility in 5 patients. Five patients had normal defecography.

Eleven patients underwent cecostomy, 1 patient underwent subtotal colectomy, and 2 patients did not have cecostomy before TAC-IRA. At follow-up, 10 patients had TAC-IRA, 1 had total colectomy with ileostomy, and 1 had partial colectomy with colorectal anastomosis. All of the patients were followed up for 1 to 5 years following surgery. Of the 10 patients with ileorectal anastomosis (IRA), 3 had incontinence on follow-up. One incontinent patient underwent ileostomy. Seven patients were continent with frequent bowel movements (Table 1).



There was no correlation of defecography results with either colonic manometry results (κ 0.37) or anorectal manometry results (κ 0.46). There was no statistical significant association among defecography, colonic manometry, and anorectal manometry with surgical intervention (P > 0.35). The majority of patients with abnormal colonic manometry underwent TAC-IRA.

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This was a case series of pediatric patients who underwent TAC-IRA for intractable constipation. A PubMed search for IRA for constipation in children yielded no articles. CIC is an extremely troublesome disorder that significantly reduces quality of life. Refractory constipation is a difficult symptom to manage. It is often frustrating for patients, parents, and health care providers because many medical treatment options fail. All of our patients had constipation during infancy or early childhood. These patients were medically managed and followed up almost for a decade before undergoing surgical intervention. Symptoms of constipation may be influenced by secondary conditions, either organic or behavioral.

In our study, 11 patients had abnormal colonic manometry suggestive of colonic neuropathy. All 11 patients underwent a surgical intervention. Nine of these patients initially underwent cecostomy. Colonic manometry is used in the evaluation of chronic constipation in children (7). Standards for colonic manometry in children have been established (8). Older children have an average of 6 to 10 high-amplitude propagating contractions (HAPC) per day whereas younger children (<5 years) have more frequent HAPCs. Diverting colostomy or ileostomy is recommended in patients with absent of HAPCs (1). Of the 9 patients with cecostomy and abnormal colonic manometry, 7 patients underwent IRA and 1 underwent colectomy with ileostomy. Colonic manometry is the preferred test for differentiating children with anorectal retention from those with slow colonic transit. In our case series colonic manometry was the most important study before surgical intervention, because most patients with surgical intervention had abnormal colonic manometry.

Defecography is indicated to rule out a variety of conditions that could contribute to the presenting symptoms, such as paradoxical contraction of the puborectalis muscle, a rectocele, rectoanal intussusception, and complete external rectal prolapse (9,10). Because their clinical significance remains a matter of debate, there is general agreement that the results of contrast radiography should not be relied upon exclusively for treatment decisions because the clinical significance of the results is largely debated (11,12). Many authors consider conventional defecography to be superior to magnetic resonance imaging for the evaluation of CIC before surgery because horizontal positioning of the patient during magnetic resonance imaging could influence pelvic floor physiology and the dynamics of defecation (13).

In our case series, an abnormal defecography correlated with abnormal colonic motility studies; this relation was seen in 8 patients. One patient had abnormal defecography with normal colonic motility. Three of the patients with normal defecography had neuropathy by colonic manometry studies. Defecography is often performed before surgical intervention because it evaluates both the morphological and dynamic factors of defecation.

The balloon expulsion test is a reliable form of anorectal manometry (14). Anorectal manometry assesses anorectal sensitivity and motility during straining and is the preferred test to rule out obstructive constipation (15,16). Commonly measured parameters include resting anal pressure, squeezing pressure, rectoanal inhibitory reflex, rectal sensations (first sensation, maximum tolerable volume), rectal compliance, and rectal and anal pressure during attempted defecation (straining). The results of anorectal manometry vary with age and sex; normal values come from a cohort of healthy individuals (15,17–19). Although anorectal manometry provides information before surgical intervention, in our case series 2 patients had abnormal anorectal manometry. These 2 patients also had abnormal motility and defecography. Anorectal manometry is useful in assessing patients for pelvic floor rehabilitation program before any surgical intervention.

Rectal biopsy and gastric motility did not contribute to decision making regarding surgical intervention of CIC. Biopsy is, however, always recommended to rule out Hirschsprung disease.

No single investigation alone is capable of determining disease type or need for surgical intervention. Anorectal manometry, colonic motility, and defecography are complementary procedures for the assessment of children with CIC. These 3 tests are recommended in the evaluation of children with CIC, before any surgical intervention.

This is the largest series of pediatric patients who underwent TAC-IRA in the literature to date. Until recently, surgical intervention for pediatric chronic constipation consisted primarily of colostomies and colectomies with ileostomy. TAC-IRA has been the standard surgical treatment in adult patients with colonic inertia, normal anorectal function, and lack of evidence of generalized intestinal dysmotility (20). Significant morbidity in both the early and late stages of the disease process must be balanced against present disability. Although TAC-IRA has been studied in adult patients, there is little information about this procedure in the pediatric population. Ten children in our series underwent TAC-IRA; 2 as the primary procedure, 5 after failure of both medical intervention and surgical intervention (cecostomy), and 3 at patient request. Several adolescents did not want to continue using a cecostomy owing to cosmetic concerns. TAC-IRA was safe without morbidity, except for 1 case of suspected postsurgical infection. All of the patients were followed up for at least 1 year and had good outcomes without further constipation as defined by Rome III criteria. Seven of 10 patients were continent with frequent bowel movements (3–6/day). Three patients had incontinence and 1 underwent subsequent ileostomy. The present literature on adult patients with TAC-IRA suggests that this procedure is an effective surgical intervention with acceptable morbidity and fever as adverse effects for patients with CIC. The majority of patients were satisfied with the procedure and the frequent stooling that resulted (21–23).

The major limitations of the study are small sample size, retrospective study design, and a variable number of study results that were not available for each patient. We could not gather detailed information about stool frequency and soiling before surgical intervention. We also did not have follow-up after TAC-IRA beyond 2 years.

In this study, all of the patients who underwent a surgical intervention for constipation had abnormal colonic manometry. This study found no statistical agreement among anorectal manometry, colonic manometry, and defecography. We propose that when used together these 3 tests will identify children who may require surgical intervention. TAC-IRA is a safe method of surgical intervention for appropriately selected pediatric patients when other surgical options have failed.

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colorectal surgery; constipation; defecography; motility; pediatric surgery

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