Guideline for the Management of Pediatric Idiopathic Constipation and Soiling FREE

A MULTIDISCIPLINARY team from the University of Michigan Medical Center in Ann Arbor was assembled to develop an evidence-based guideline for the diagnosis and treatment of idiopathic constipation and soiling in children. The multidisciplinary team included members from the departments of general pediatrics, pediatric gastroenterology, pediatric surgery, child psychiatry, nursing, and clinical affairs who were clinicians with an interest or expertise in idiopathic constipation and soiling or had experience with guideline development. Chronic constipation and soiling are common and affect 1% to 3% of children. Unfortunately, symptoms often precede diagnosis for years.¹ The impetus for development of this guideline came from general and developmental and behavioral pediatricians at this institution who believe that primary care physicians can provide earlier diagnosis thorough evaluation and management of this medical problem and recognize appropriate points of referral. The team members recognized that primary care physicians are in a unique position to identify medical problems in a timely way because they see children routinely for health supervision visits.

A recent report² suggests that symptoms of hard, painful stools and withholding may occur as early as 1 year of age and herald later problems with constipation and soiling that may take years to remedy. One goal of this guideline is to promote earlier detection, effective intervention, and referral if needed by providing concise recommendations to primary care physicians. The multidisciplinary team members also recognized that the styles and preferences for management of childhood constipation and soiling vary by discipline and that this might be confusing to primary care practitioners who seek to provide the most effective frontline care. Thus, the second goal of this guideline is to provide recommendations for the evaluation and management of childhood constipation and soiling that are evidence based where possible and coordinated across disciplines. This guideline is intended to be a useful reference for primary care physicians in a busy practice and is not designed to provide an exhaustive review of diagnostic evaluations, medical treatments, or behavioral programs that would be of interest to the subspecialist working with children in whom primary care management failed. A complete explanation of the process for guideline development is documented elsewhere.³

The team members generated the following 3 questions as a focus for the goals of developing this guideline: (1) What is the best path to early, accurate diagnosis?, (2) What are the best methods for adequate clean-out? and (3) What approaches promote patient and family compliance with management?

Literature was identified using a MEDLINE search from January 1975 through January 1998 that included the following terms: constipation, soiling, encopresis, pediatric(s), diagnosis, treatment, randomized controlled trial, clinical trials, controlled clinical trial(s), and review. Bibliographies of relevant articles were scanned for additional references. The abstracts of articles identified were reviewed independently by team members. If several articles on the same topic were identified, randomized controlled trials were reviewed preferentially over observational, quasi-experimental study designs or reviews. During this process, however, it became apparent that, at this time, randomized controlled trials are few in this clinical area. Therefore, several review and experience articles by experts were used. Twenty-five articles were identified, critically reviewed, and summarized for team members in evidence tables that identified the reference, study design, sample size, purpose, and results. (See Table 1 for example of evidence table. Full tables are available from the authors on request.) Any disagreements between recommendations from the literature review were settled by consensus opinion of team members. An outcome of this process was interdisciplinary compromise regarding those areas of management for which strong evidence for one approach vs another was not available. Where there were several approaches documented in the literature, these are stated. Agreement between the team members was nearly universal on each point of recommendation.

Recommendations are depicted in an algorithm along with supporting tables and text. Key recommendations are classified in terms of the level of evidence based on study design, with the evidence grade presented in parentheses: randomized controlled trials (A); controlled trials, no randomization (B); observational studies (C); expert opinion (D); and consensus of guideline panel (E).

Constipation with or without soiling is common. It affects 3% of preschool-age children and 1% to 2% of school-age children. It is equally distributed by sex at toddler age. In the school-age child, the disorder is more prevalent in boys (D).¹ Longer duration of symptoms before diagnosis has been associated with poorer long-term outcome, defined as continued soiling. Current studies indicate that time from onset of symptoms to diagnosis is usually 1 to 5 years. Some experts suggest that earlier diagnosis and effective management might improve outcomes (C).^{1 ,4}

Stool frequency declines with age. Although the frequency of stool production varies during infancy and early childhood, children have on average more than 4 stools produced per day during the first weeks of life, 2 per day by 4 months, and 1 per day by 4 years of age. Stool frequency ranges from 3 per day to 3 times per week for 96% of children aged 3 to 4 years.⁵ Constipation is a symptom that has been defined by a frequency of less than 3 stools produced per week. However, stool consistency and occurrence of pain at defecation are also important characteristics. For example, stool that is hard and painful to pass may also define constipation, even if the frequency is 3 or more times per week (D).^{1 ,6} Soiling is the involuntary passage of stool and is often associated with fecal impaction. Encopresis is the voluntary or involuntary passage of formed, semiformed, or liquid stool into a place other than the toilet at regular intervals (at least once per month) after 4 years of age (D).⁷ A goal of this clinical guideline is to address the diagnosis and management of pediatric idiopathic constipation and soiling for the primary care physician. Encopresis without constipation is not specifically addressed.

Chronic constipation in children often follows an acute stool problem that was not managed adequately (D).⁵ Several factors increase the risk of infrequent, hard stools in children, such as diet replete with constipating foods and low in fiber-rich foods, insufficient time or routine for regular toileting, and painful stool passage. Stool withholding and retention may occur in the attempt to avoid painful defecation. In one study,² 63% of children with constipation and soiling had painful defecation that began before 3 years of age (C). Some children may also have an altered rectosphincteric reflex, in which case a stool bolus in the rectum elicits sphincter and pelvic floor contraction rather than the normal relaxation response during the attempt to defecate. Stool retention and impaction stretch the rectum and colon. Impaction over time is associated with several physiologic consequences, ie, diminished sensory threshold at the rectum and weakened rectal and sphincter musculature (C).⁸ Encopresis or soiling is a consequence of these physiologic alterations. This soiling of stool reflects overflow of liquid feces from an impacted rectum for most children. A few children may present with soiling without a history or signs and symptoms of constipation, which should alert the practitioner to an underlying gastrointestinal, neuromuscular, or behavioral disorder (Table 2).

Figure 1 presents an algorithm for the diagnosis and management of idiopathic constipation and soiling in children. Up to 95% of children referred for evaluation of constipation receive a diagnosis of idiopathic constipation (C).⁵ Constipation may be defined according to stool frequency, character, and ease of passage. Children younger than 3 years most often present with pain at defecation, impaction, and withholding; children older than 3 years most frequently present with soiling, impaction, and withholding (C).²

A careful history and physical examination excludes other diagnoses in the differential for most patients (Table 2). Attention to the pattern of stooling during infancy assists practitioners in recognizing Hirschsprung disease—which occurs in 1 in 5000 children—and is diagnosed in 40% of patients by 3 months, in 61% by 12 months, and in 82% by 4 years of age (C).⁹

The following symptoms are commonly found in children with idiopathic constipation and soiling: stool frequency less than 3 times per week, painful defecation, withholding, hard stool, soiling, and abdominal discomfort. However, other important symptoms can include stool caliber and length ("clogs the toilet"), pattern and time of soiling (eg, after school), anorexia, abdominal discomfort often relieved by stool passage, enuresis, and urinary tract infection. Medications or environmental exposures that may contribute to constipation should be elicited, and developmental history may suggest concurrent developmental delay, autism, or other behavioral disorders. Although children frequently present with low self-esteem or other behavioral concerns, these symptoms are improved for most of them with education and management for the constipation and soiling (C).^{10 - 11}

The results of abdominal examination are positive for stool impaction in about half of patients (C).¹² Firm, packed stool in the rectum has a positive predictive value of more than 84% for the diagnosis of idiopathic constipation, and thus, rectal examination is helpful to include (C).¹³ Most experts suggest that studies are usually not needed for diagnosis but to consider an abdominal plain film if the results of rectal examination are negative for impaction.^{5 ,14} The results of rectal examination may be negative if the child has recently evacuated stool. It is important to weigh the potential benefit of information gained by rectal examination against the potential risk of causing further trauma and pain for the child (E). Take time to explain the procedure and provide methods to promote relaxation (deep breaths) and choices to help the child feel in control. For some children, deferring the examination and assessing response to initial management may be indicated. Other key points that aid in considering the differential diagnoses include anal placement and occurrence of fissures and neurologic examination, including the presence of abdominal and cremasteric reflexes, anal wink, and lower-extremity deep-tendon reflexes to evaluate for underlying neurologic dysfunction.

On average, half to two thirds of patients recover. Recovery rate is higher for children who begin intervention earlier (C).¹²

Experts highlight the importance of explaining the physiologic basis of soiling to the child and caretakers with 2 goals: to alleviate blame and to enlist cooperation. Most experts also agree that education improves compliance with the treatment plan (D),^{9 ,14 - 15} and recommend using age-appropriate or developmentally appropriate drawings or play to explain the physiologic changes that occur as a consequence of chronic constipation, including a diminished ability to recognize the need to stool or that soiling has occurred. Explain that this condition is common and is multifactorial in origin for most children. (An educational handout for parents is available from the authors.)

There are many effective choices for disimpaction described in the literature, including high-dose mineral oil (98% effective clean-out) (C)¹⁶ ; combination approaches using enema, suppository, and oral laxatives (D)¹⁷ ; and exclusive use of enemas (mineral oil, saline, hypertonic phospate) (D) (Table 3).¹⁸ Metabolic abnormalities associated with the provision of hypertonic phosphate enemas in young children and individuals with intestinal (Hirschsprung) or renal (renal failure) abnormalities have been reported (D).¹⁹ Based on this report, concerns raised by others, and the experience and consensus of this guideline panel, specific caution is given on the use of hypertonic enemas in children (E). We recommend that, if used, they be limited to 1 to 2 per day, with attention to ensuring full expulsion. Furthermore, parents should be instructed to contact their physicians if full expulsion does not occur; appropriate monitoring and support is needed because hypertonic dehydration is a risk. Currently, there is insufficient evidence to strongly support one clean-out method over another. The consensus of this guideline panel is that oral regimens are effective and may be the method of choice for children because they avoid further trauma to the anal area (E). Child and family compliance with the disimpaction phase may be aided by discussing the options with child and parents and involving them in the choice (E).

The purposes of the maintenance phase of therapy are to promote regular stool production and prevent reimpaction so that bowel rehabilitation can occur (Table 4). Most experts recommend a goal of 1 to 2 soft stool productions per day and the alleviation of withholding. A combination of behavioral training and laxative therapy affords earlier remission for most subjects (A).^{17 ,20} Although the importance of dietary education has not been determined separately, most experts include this as a component of the maintenance phase of therapy (D).^{5 - 6 ,14}

Below is an outline of behavioral training points provided in the parent education handout. (Handout is available on request from the authors.)

Commonly, increasing dietary fiber increases the frequency of bowel movements (D).²¹ Ongoing dietary advice is required to maintain an increase in the patient's fiber intake (C).²² High fiber intake does not adversely affect biochemical and anthropometric indicators of nutritional status (C).²³

Stool softeners or laxatives are often required to promote regular production of soft stools. There are many choices and insufficient evidenced-based literature to support one product over another. Dosage recommendations for some common products are provided in Table 4. Product and absolute doses vary by author in the literature. In general, medications should be titrated in amount to give 1 to 2 soft stool productions daily. Give mineral oil cold, mixed with chocolate milk, ice cream, soda, or juice, or with a tasty treat after intake to reduce aversion to product consistency.

Experts report that children with idiopathic constipation and soiling usually require maintenance therapy for 6 to 24 months. Most experts suggest frequent follow-up to assess and promote patient compliance and adequacy of treatment. There are no studies to suggest specific follow-up times. However, a plan to follow up at 1 to 2 weeks to ensure effective clean-out, then 1 month, 3 months, and 3- to 6-month intervals thereafter to ensure an effective maintenance phase, is commonly considered to be a fair and average approach by this guideline panel (E).

A trial of weaning laxatives at 6-month intervals (while maintaining the progress achieved with regular toileting) is suggested by our panel in accordance with the recommendations of national experts (D).¹² Recovery is defined as 3 or more stool productions per week with no soiling. Stool frequency of less than 3 per week with or without soiling indicates the need to continue with the maintenance program. In 1 study,¹ 50% of patients discontinued taking laxatives by 1 year and an additional 20% were weaned by 2 years (D). Others have reported 70% recovery (>3 stool productions per week) at 6 years from diagnosis (C)¹ and 48% recovery (defined as >4 stool productions per week) at 1 and 5 years from diagnosis (C).^{4 ,24 - 26}

Failure of treatment (continued soiling, reimpaction) despite reported compliance suggests the need to review education and other treatment components (D).¹² Repeated failure despite good compliance suggests the need to reconsider the differential diagnoses.

Noncompliance with the treatment regimen suggests the need to first review the education components and treatment goals. Repeated noncompliance may indicate the need for further evaluation of child and family psychosocial and psychological factors and subsequent referral to a developmental behavioral pediatrician or child psychiatrist if indicated (E).

A clinical care algorithm and guideline for the evaluation and management of children with idiopathic constipation and soiling are presented for use by primary care physicians. The guideline was developed based on a literature review (evidenced based where possible) and consensus by a multidisciplinary panel. Review of the literature found few randomized controlled studies for key components of management. However, as clinical studies that address these points become available, new evidence can be incorporated and recommendations updated. This guideline was recently disseminated to physicians at the University of Michigan (available at http://www.med.umich.edu/i/oca/practiceguides/) and will be updated biannually.

Accepted for publication August 26, 1998.

We appreciate the review and comments by L. Wild, BSN, and D. Kemp, MSN, and the secretarial support of Mary Dluzon and Janis Beard.

Reprints: Barbara Felt, MD, University of Michigan, 300 N Ingalls St, Ann Arbor, MI 48109-0406 (e-mail: truefelt@umich.edu).

Editor's Note: While you might quibble about minor aspects of the recommendations, this clinical pathway to exit the colon of chronically constipated children is a breath of fresh air.—Catherine D. DeAngelis, MD