Constipation in Infants and Children - Evaluation - UpToDate

10/11/2017 Constipation in infants and children: Evaluation - UpToDate
Official reprint from UpToDate®

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Constipation in infants and children: Evaluation
Author: Manu R Sood, FRCPCH, MD

Section Editor: B UK Li, MD
Deputy Editor: Alison G Hoppin, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Sep 2017. | This topic last updated: Apr 17, 2017.
INTRODUCTION — Constipation affects up to 30 percent of children and accounting for an estimated 3 to 5

percent of all visits to pediatricians [1]. The peak prevalence is during the preschool years in most reports.
There is no consistent effect of gender on the prevalence of childhood constipation.
Complaints range from infrequent bowel evacuation, hard small feces, difficult or painful evacuation of large-
diameter stools, and fecal incontinence (voluntary or involuntary evacuation of feces into the underwear, also
known as encopresis) [2,3]. Most but not all children with fecal incontinence have underlying constipation.
Functional constipation is responsible for more than 95 percent of cases of constipation in healthy children
one year and older, and is particularly common among preschool aged children [4]. Although it is common, it
is important to evaluate affected children to identify the few that have organic causes of constipation.
Moreover, children with functional constipation will benefit from prompt and thorough treatment interventions.
Delayed or inadequate intervention may result in stool withholding behavior with worsening constipation and
psychosocial consequences.
The evaluation of an infant or child with constipation will be reviewed here. Related information is available in
the following topic reviews:
● (See "Functional constipation in infants and children: Clinical features and differential diagnosis".)
● (See "Functional fecal incontinence in infants and children: Definition, clinical manifestations and
evaluation".)
● (See "Chronic functional constipation and fecal incontinence in infants and children: Treatment".)
● (See "Prevention and treatment of acute constipation in infants and children".)
EVALUATION — Evaluation of a child with constipation relies primarily on a focused history and physical
examination; further testing is performed if the initial evaluation raises concern for an organic cause of
constipation.
History — The history should focus on features that suggest functional constipation (table 1) and also
assess for features that raise concern for some rare but serious organic causes of the symptom (table 6B).
The history should be obtained from the parents or caretakers, and also from the child, if this is appropriate
for his or her age. The partnership established with the family should continue until the constipation is
resolved, to ensure that symptoms do not progress and become more problematic [5].
In an infant, apparent straining during defecation does not necessarily indicate constipation. If accompanied
by the passage of soft stools in an otherwise healthy infant, this symptom is known as "infant dyschezia" (see
"Functional constipation in infants and children: Clinical features and differential diagnosis", section on 'Infant
dyschezia'). Organic causes of constipation should be considered if the straining behavior is accompanied by
hard stools or if warning signs are present. The most important organic causes of constipation in infants are
Hirschsprung disease and cystic fibrosis. In particular, a history of delayed passage of meconium should
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raise concerns about the possibility of Hirschsprung disease, as more than 90 percent of normal newborns
but only 10 percent of infants with Hirschsprung disease pass meconium within the first 24 hours of life. (See
"Functional constipation in infants and children: Clinical features and differential diagnosis", section on
'Differential diagnosis' and 'Alarm signs' below.)
Alarm signs — Alarm signs from the history that suggest the possibility of organic causes include (table
2) [6,7]:
● Acute signs
• Delayed passage of meconium (first meconium passed after 48 hours of life) (table 3)
• Fever, vomiting, or diarrhea
• Rectal bleeding (unless attributable to an anal fissure)
• Severe abdominal distension
● Chronic signs
• Constipation present from birth or early infancy

• "Ribbon" stools (very narrow in diameter)
• Urinary incontinence or bladder disease
• Weight loss or poor weight gain
• Delayed growth (eg, decreasing height percentiles)
• Extraintestinal symptoms (especially neurologic deficits)
• Congenital anomalies or syndromes associated with Hirschsprung disease (eg, Down syndrome)
• Family history of Hirschsprung disease
Psychosocial and environmental factors — When functional constipation is suspected, particular

attention should be given to psychosocial or environmental factors that may have influenced the child's bowel
activities. These factors include a history of painful evacuation, difficulties with toilet training, stool
withholding, introduction of cow's milk, and diet. Dietary factors that sometimes contribute to constipation
include low fiber content (few fruits or vegetables) and low fluid intake, although these associations are weak.
(See "Functional constipation in infants and children: Clinical features and differential diagnosis", section on
'Etiology'.)
Findings supporting a functional etiology include:
● Onset of constipation coincides with dietary change, toilet training, or painful bowel movement
● Stool withholding behavior (table 4)
● Good response to conventional laxatives
If feasible, parents should prepare a five- to seven-day symptom and dietary history before bringing the child
for the evaluation [8], taking care to record stool frequency, appearance, and any pain involved. The point at
which the constipation was first noted, and any potential relationship with a coincident event, should be
sought.
Constipation and bladder dysfunction — Anorectal and lower urinary tract function are interrelated. As
a result, constipation is often associated with bladder dysfunction, including bladder overactivity (urge),
increased or decreased voiding frequency, and bladder underactivity [9]. This relationship between abnormal
bowel and bladder function is referred to as the bowel bladder dysfunction, also known as dysfunctional
elimination syndrome. Although bladder dysfunction is commonly associated with functional constipation,
neurogenic disorders must also be excluded. Successful treatment of the constipation is an important
component of treating the bladder dysfunction [10]. (See "Etiology and clinical features of bladder dysfunction
in children", section on 'Bowel and bladder dysfunction'.)
Physical examination — The physical examination should include an evaluation of the perianal area,
including the appearance and location of the anus, and sensory and motor function. A digital rectal
examination is included for selected cases (table 1 and table 2) [7]:
External examination
● General – The general physical examination should include assessment of growth and abdominal
distension, and abdominal or pelvic masses. It should specifically evaluate for features of spinal
dysraphism, which has clinical manifestations ranging from benign or asymptomatic to severe
neurologic, genitourinary, gastrointestinal, or musculoskeletal anomalies. Occult spinal dysraphism is
suggested by increased pigmentation, vascular nevi, or hair tufts in the sacrococcygeal area. (See
"Closed spinal dysraphism: Clinical manifestations, diagnosis, and management".)
● Neurologic – The neurologic evaluation of children with chronic constipation should focus on symptoms
and signs suggesting spinal cord and/or autonomic nervous system dysfunction, including:
• Sensory loss or motor weakness

• Abnormal muscle tone
• Abnormal deep tendon reflexes
• A patulous anus
• An absent cremasteric reflex
• Associated bladder dysfunction
Patients with truncal hypotonia (eg, those with Down syndrome) may develop constipation because they
do not efficiently generate abdominal pressure for defecation; this is a form of dyssynergic defecation.
Patients with generalized lower motor neuron dysfunction, suggested by hyporeflexia and hypotonia,
develop constipation because of slow intestinal transit, and the problem may be complicated by fecal
incontinence due to decreased anal sphincter tone. In patients with upper motor neuron dysfunction,
suggested by hypertonia and hyperreflexia, constipation is caused by overactive pelvic muscle
contraction and inability to voluntarily relax the external anal sphincter. Abnormal deep tendon reflexes
(delayed relaxation) are also seen in hypothyroidism, a rare cause of constipation in children. (See
'Differential diagnosis'.)
● Perineum – The perineum should be inspected for abnormalities of anorectal development, which
represent a spectrum from high imperforate anus to anteriorly displaced anus (figure 1) [11]. When the
communication is abnormally close to the fourchette or scrotum, the anus is considered "anteriorly
displaced" or "ectopic". An anteriorly displaced anus is easily overlooked. The abnormality is suggested
by noting that the anal opening is not located in the center of the pigmented area of the perineum
(picture 1A-B). The diagnosis is supported by measuring the Anal Position Index (API), which is defined
as the ratio of the anus-fourchette/scrotum distance to the coccyx-fourchette/scrotum distance.
Measurements are most easily made by placing a strip of clear tape on the longitudinal axis of the
perineum, marking the positions of the coccyx, anus, and fourchette/scrotum, then removing the tape for
measurement against a standard ruler. Normal ratios are discussed elsewhere. (See "Functional
constipation in infants and children: Clinical features and differential diagnosis", section on 'Anorectal
anomalies'.)
Digital anorectal examination — A digital anorectal examination is not routinely necessary for the
evaluation of patients with a typical history and symptoms of functional constipation. This is because the
digital anorectal examination is unpleasant for the child and has only moderate sensitivity and specificity for
detecting or confirming constipation in this group of patients [7]. However, some providers perform a digital
examination in selected cases of suspected functional constipation. The goals of the examination are to
detect a fecal impaction, which would require a "cleanout" approach (initiation of treatment with high doses of
laxatives and/or enemas), and to detect occult blood, which would require further diagnostic testing.
A digital examination is suggested for the following groups of patients [7]:
● Infants with constipation

● Children with symptoms since early infancy
● Infants or children with other alarm signs that suggest organic disease (table 2)
● Children in whom the presence or degree of constipation is unclear (eg, meeting only one Rome IV
criterion)
Findings suggestive of Hirschsprung disease include a tight anal canal with an empty ampulla. There may be
an explosive release of gas and stool after the digital rectal examination (squirt sign or blast sign), which may
relieve the obstruction temporarily. In addition, infants with Hirschsprung disease often have gross distention
of the abdomen and failure to thrive. (See "Congenital aganglionic megacolon (Hirschsprung disease)" and
'Hirschsprung disease'.)
Findings suggestive of functional constipation are a distended rectum that is full of stool. However, lack of
stool does not exclude the possibility of functional constipation.
Testing of the stool for occult blood (guaiac testing, eg, Hemoccult), should be performed in most cases if
stool is available from the digital rectal examination or diaper. This is particularly important in infants with
constipation, in whom subclinical milk protein intolerance (or other food protein intolerance) may present as
constipation (see "Functional constipation in infants and children: Clinical features and differential diagnosis",
section on 'Cow's milk intolerance'). In infants and children with marked abdominal distension or who are ill-
appearing, stools with visible or occult blood may indicate enterocolitis, and the patient requires urgent further
evaluation. (See "Emergency complications of Hirschsprung disease", section on 'Enterocolitis' and
'Hirschsprung disease'.)
DIAGNOSIS OF FUNCTIONAL CONSTIPATION
Diagnostic criteria — A Multinational Working Team developed criteria for functional gastrointestinal
disorders known as the "Rome IV" criteria [12,13]. For children and adolescents with developmental age of
four years or older, functional constipation is defined by the presence of at least two of the following
symptoms occurring at least once per week for at least one month, which are not explained by another
medical condition (table 5):
● Two or fewer defecations per week

● At least one episode of fecal incontinence per week
● History of retentive posturing or excessive volitional stool retention (stool withholding)
● History of painful or hard bowel movements
● Presence of a large fecal mass in the rectum
● History of large-diameter stools that may obstruct the toilet
For infants and toddlers, the criteria are modified to reflect age-appropriate toileting skills.
Exclusion of organic causes — The diagnosis of functional constipation also requires exclusion of organic
causes of the symptom. Organic causes are responsible for fewer than 5 percent of children with constipation
[14,15], but are more common among young infants, and among infants and children presenting with atypical
features or "alarm signs" (table 2) (see 'Alarm signs' above).
The main causes are listed in the table for infants (table 6A), or older children (table 6B) and detailed in a
separate topic review. (See "Functional constipation in infants and children: Clinical features and differential
diagnosis", section on 'Differential diagnosis'.)
Particular attention should be paid to the following causes, which are relatively common or require urgent
diagnosis:
● Common organic causes – Cow's milk (or other dietary protein) intolerance; celiac disease;
hypothyroidism
● Urgent causes
• Infants – Hirschsprung disease, spinal dysraphism, sacral teratoma, infantile botulism
• All ages – Cystic fibrosis, lead poisoning, intestinal obstruction
FURTHER TESTING — In most cases, organic causes of constipation can be excluded on the basis of a
careful history and physical examination. If warning signs of possible organic constipation are present,
focused laboratory and radiographic testing should be performed. In addition, these tests may be appropriate
for patients who fail to respond to a well-conceived and carefully administered intervention program, including
disimpaction, frequent and effective use of laxatives, and behavioral management. (See "Chronic functional
constipation and fecal incontinence in infants and children: Treatment".)
Imaging
● Abdominal radiograph – A plain abdominal radiograph is not indicated for the routine evaluation of
functional constipation [7]. However, it can be helpful to document retained stool when there is
inadequate historical information to determine if the patient has constipation or if the physical
examination is limited by patient cooperation, obesity, or is deferred for psychological considerations. It
should be recognized that abdominal radiographs are inconsistently interpreted by different observers,
are not particularly specific for constipation, and rarely add to the diagnosis if the rectal examination
reveals a large amount of retained stool [7,16,17]. Thus, they are not an essential part of the evaluation
of constipation and should not be used as a substitute for a thorough history and physical examination.
● Barium enema – A barium enema provides supportive evidence for Hirschsprung disease in children
with features suggestive of this disorder, such as early onset constipation from the neonatal period,
especially with delayed passage of meconium, or suggestive findings on anorectal examination. The
study should be performed "unprepped", ie, without measures to remove stool from the rectum (image
1A-B). Some providers use anorectal manometry as the initial investigation or proceed directly to rectal
biopsy. In very young infants, the barium enema may be normal and the diagnosis must be established
by rectal biopsy. (See "Congenital aganglionic megacolon (Hirschsprung disease)" and "Functional
constipation in infants and children: Clinical features and differential diagnosis", section on 'Hirschsprung
disease'.)
● Spine radiographs – Plain films of the lumbosacral spine should be performed for children with
evidence of spinal dysraphism or neurological impairment of the perianal area or lower extremities. If
there is a high suspicion of neurologic dysfunction, magnetic resonance imaging (MRI) should be
considered to investigate the possibility of tethered cord and spinal cord tumors [7,18,19]. (See "Closed
spinal dysraphism: Clinical manifestations, diagnosis, and management".)
Laboratory tests — We suggest laboratory testing at the time of the initial evaluation in patients with signs or
symptoms suggestive of an organic cause of constipation, such as the following clinical situations:
● Celiac screening – For children with failure to thrive or recurrent abdominal pain, perform a complete
blood count and serologic screening for celiac disease (usually IgA antibodies to tissue
transglutaminase). The symptoms of celiac disease may be subtle. Therefore, we have a low threshold
for performing celiac screening in children with constipation, despite limited information about its clinical
utility in this population. (See "Epidemiology, pathogenesis, and clinical manifestations of celiac disease
in children" and "Diagnosis of celiac disease in children".)
● Urine analysis and culture – For children with a history of rectosigmoid impaction, especially in
association with encopresis [20-22], perform a urine analysis and urine culture. This is because fecal
impaction may predispose to urinary tract infections due to the mechanical effects of the distended
rectum compressing the bladder.
● Thyroid stimulating hormone – For children with impaired linear growth and depressed reflexes, or
those with a history of central nervous system disease, we suggest screening for hypothyroidism. A
height velocity less than 5 cm/year (1.6 inches/year) suggests the possibility of growth failure in
prepubertal children. If central hypothyroidism is suspected, the screen should include measurement of
free thyroxine (T4) as well as thyroid stimulating hormone (TSH). (See "Acquired hypothyroidism in
childhood and adolescence".)
● Electrolytes and calcium – For children at risk for electrolyte disturbances (eg, those with metabolic
abnormalities or inability to tolerate adequate fluids), we suggest measuring serum concentrations of
electrolytes and calcium.
● Blood lead level – Screening for lead toxicity should be performed in children with risk factors.
Screening recommendations vary by community. Children at particular risk include those with pica,
developmental disabilities, or a family history of lead poisoning in a sibling, or those living in housing built
before 1950 or housing that recently has been renovated. (See "Childhood lead poisoning: Clinical
manifestations and diagnosis" and "Screening tests in children and adolescents", section on 'Lead
poisoning'.)
This list is not exhaustive and specific laboratory testing may be considered in any patient with an atypical
presentation.
Motility testing — Motility testing is typically considered in patients who have no obvious organic cause of
constipation and who fail to respond to vigorous treatment of functional constipation.
Colon transit studies
● Indications – A colonic transit study (eg, a "Sitzmark" study) is not helpful for the routine evaluation of a
child with constipation because the results rarely alter management [7]. This study is generally reserved
for the secondary evaluation of selected patients in whom the diagnosis is unclear despite a thorough
initial evaluation and trials of treatment. In particular, it may be useful for the following purposes [23]:
• To help distinguish between retentive fecal incontinence (constipation-associated) and non-retentive

fecal incontinence [7].
• To identify children with abnormally slow movement of food residue through the colon, a condition
referred to as "slow-transit" constipation. Slow transit constipation is more likely in children with
onset of symptoms before three years of age, severe symptoms (passing less than one stool a
week), and lack of response to optimal standard therapy with laxatives and behavioral intervention.
• To identify children with stool expulsion disorders, suggesting outlet obstruction.
● Technique – Colonic transit studies can be performed using a variety of protocols [24,25]. One of the
easiest techniques is to have the child swallow a capsule containing 24 radiopaque markers (Sitzmark
capsules) once a day for three days. Plain radiographs are taken on the fourth day (and sometimes also
on the seventh day), and analyzed for the number and location of retained markers [25]. Ideally, any
fecal impaction should be relieved, and laxatives should be discontinued several days prior to performing
the study.
The colonic transit time (CTT) can then be calculated with the following formula:
• For the day 4 films:
Colonic transit time (hours) = # markers remaining x 1.0
(where 1.0 = 72 hours/72 markers ingested)
• For the day 7 films:
Colonic transit time (hours) = # markers remaining x 2.3
(where 2.3 = 168 hours/72 markers ingested)
If capsules containing 20 (rather than 24) radiopaque markers are used, the constant 1.2 is used for the
day 4 calculation, and 2.8 for the day 7 calculation. If the capsules containing radiopaque markers are
not available, segments of number 10 radiopaque nasogastric tube cut into 1 cm lengths can be used as
markers, and the constant adjusted according to the number of markers ingested. Transit time also may
be analyzed by colonic segment, using similar calculations.
● Interpretation – Children with functional constipation tend to have slower CTT than those without
constipation. In one study of adolescents, CTT was 58.3 for those with constipation and 30.2 hours for
those without constipation [25].
The term "slow transit constipation" is used to describe particularly severe delays in CTT (>100 hours)
[26]. Slow transit constipation is a clinical description rather than a disease because it remains unclear
whether this group of children is distinct from those with functional constipation. A few children with slow
transit constipation have disorders associated with colonic dysmotility, which include intestinal neuronal
dysplasia and intestinal neuronal dysplasia type B. We suggest early referral to a gastroenterologist for
patients with this profile. (See "Functional constipation in infants and children: Clinical features and
differential diagnosis", section on 'Slow-transit constipation' and "Chronic functional constipation and
fecal incontinence in infants and children: Treatment", section on 'Treatment failure'.)
A colonic transit study also may help to identify patients with outlet obstruction, manifested by
accumulation of markers in the rectosigmoid area. Patients with outlet obstruction may require a biopsy
to evaluate for Hirschsprung disease or other neuromuscular disorders [14]. However, this pattern also
may be seen in patients with fecal impaction and in those with abnormal responses of the pelvic floor
muscles during defecation. (See "Etiology and evaluation of chronic constipation in adults", section on
'Outlet delay' and "Functional constipation in infants and children: Clinical features and differential
diagnosis", section on 'Differential diagnosis'.)
Anorectal manometry — Anorectal manometry involves placement of a catheter containing pressure-

transducing sensors into the rectum, thereby permitting measurement of neuromuscular function of the
anorectum. The procedure includes measurements of the rectoanal inhibitory reflex (which is absent in
Hirschsprung disease), rectal sensation and compliance, and squeeze pressures. The test is performed
mainly in children with intractable constipation that restricts their lifestyle, or when there is suspicion of
internal anal sphincter achalasia, or Hirschsprung disease [14,20,27-29]. Anorectal manometry also can
identify patients with dyssynergic defecation, which is a functional disorder characterized by the incomplete
evacuation of fecal material from the rectum due to paradoxical contraction or failure to relax pelvic floor
muscles when straining to defecate. The chances of artifact due to catheter movement (which can mimic
rectoanal inhibitory response) are higher in children less than six months of age. Definitive diagnosis of
Hirschsprung disease is made by rectal biopsy. (See "Congenital aganglionic megacolon (Hirschsprung
disease)" and "Functional constipation in infants and children: Clinical features and differential diagnosis",
section on 'Differential diagnosis'.)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics"
and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th
grade reading level, and they answer the four or five key questions a patient might have about a given
condition. These articles are best for patients who want a general overview and who prefer short, easy-to-
read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more
detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want
in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail
these topics to your patients. (You can also locate patient education articles on a variety of subjects by
searching on "patient info" and the keyword(s) of interest.)
● Basics topics (see "Patient education: Constipation in children (The Basics)" and "Patient education:
Hirschsprung disease (The Basics)")
● Beyond the Basics topic (see "Patient education: Constipation in infants and children (Beyond the
Basics)")
SUMMARY AND RECOMMENDATIONS — The following recommendations are consistent with guidelines
developed by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition
(NASPGHAN) and endorsed by the American Academy of Pediatrics (AAP) (available at:
www.naspghan.org) [7].
● Functional constipation is responsible for more than 95 percent of cases of constipation in healthy
children one year and older, It is defined by the presence of at least two of six criteria describing stool
frequency, hardness, size, fecal incontinence, or volitional stool retention (table 5). The symptoms must
be present for one month in infants and toddlers, and two months in older children. The diagnosis also
requires exclusion of organic causes of the symptoms. Functional constipation usually can be diagnosed
based upon the history and physical examination. Key elements of the history and physical examination
are outlined in the table (table 1). (See 'Diagnosis of functional constipation' above.)
● Organic causes are responsible for fewer than 5 percent of children with constipation, but are more
common among young infants; the main causes are listed in the tables (table 6A-B). Particular attention
should be given to patients with alarm signs (table 2) or other elements of the history and physical
examination that raise suspicion for an organic cause (table 2). (See 'Alarm signs' above.)
• Signs and symptoms suggestive of Hirschsprung disease include delayed passage of meconium
(after 48 hours of life), failure to thrive or delayed growth, vomiting, abdominal distension, a tight
anal canal with an empty ampulla, or an explosive expulsion of stool after the digital examination
(squirt sign). Hirschsprung disease should be particularly considered for infants presenting with
constipation during the neonatal period. Other causes of constipation or delayed passage of
meconium in the early neonatal period are listed in the table (table 3). (See "Congenital aganglionic
megacolon (Hirschsprung disease)".)
• Cow's milk may cause constipation in infants and young children. The diagnosis is suggested by
onset of symptoms that coincide with an increase in cow's milk in the diet and is generally confirmed
and treated by substitution of soy or hydrolyzed protein in the formula. (See 'Psychosocial and
environmental factors' above and "Functional constipation in infants and children: Clinical features
and differential diagnosis", section on 'Cow's milk intolerance'.)
• Celiac disease is common and occasionally is associated with constipation. As a result, we have a
low threshold for performing celiac screening in children with constipation, by measuring celiac-
specific antibodies (usually IgA antibodies to tissue transglutaminase). (See 'Laboratory tests'
above.)
● Laboratory and radiographic testing should be selectively performed based upon the history and physical
examination. For the majority of children whose presentation is typical of functional constipation,
laboratory testing is pursued only if the patient fails to respond to a well-conceived and carefully
administered intervention program, including disimpaction, frequent and effective use of laxatives, and
behavioral management. In contrast, laboratory testing should be performed early in patients with signs
or symptoms suggestive of an organic cause of constipation. (See 'Further testing' above.)
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge George D Ferry, MD,
who contributed to an earlier version of this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.
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disorders. role in diagnosis and management. Am J Gastroenterol 2003; 98:1052.
Topic 5906 Version 36.0
GRAPHICS
Evaluation of an infant or child with constipation
History Implications
Was there delayed passage of Suggests Hirschsprung disease
meconium (ie, first meconium
after 48 hours of life)?
Onset of constipation:
Was constipation present More likely to be an organic cause (eg, Hirschsprung disease)
from birth or early
infancy?
Was there a preceding Suggests functional constipation

change in diet or diarrheal
illness?
Was the onset around the Suggests functional constipation

time of toilet training, or
around a precipitating
event?
Were there problems with Suggests functional constipation

toilet training? (eg, child
resistance, fear or late
mastery)
Stool quality and

appearance:
Is defecation painful? Suggests functional constipation
Are the stools hard or Soft stools suggests cause other than constipation (eg, dyschezia in an infant)
soft?
Does the stool form pellets Supports diagnosis of functional constipation

in the diaper or toilet? Do
they clog the toilet?
Is there blood on the stool? Possible anal fissure, which can cause or result from functional constipation
If the child is toilet trained, Indicates fecal incontinence, which is usually due to functional constipation and
does he or she have withholding, leading to fecal impaction
"accidents" in the
underwear?
Does the child have stool Suggests functional constipation, with stool withholding
withholding behavior?
("dance", hide or appear to be
trying not to have a bowel
movement)
Dietary issues:
Is the diet unusual? Are Low fluid intake occasionally contributes to constipation
there sources of fiber? Is
there adequate fluid?
Did the constipation Suggests functional constipation, possibly cow's milk protein intolerance
coincide with a change in
diet? (eg, transition to
solid foods in infants, or to
milk)
Are there underlying medical Consider neurogenic causes of constipation; some congenital syndromes are
problems, congenital associated with Hirschsprung disease (eg, Down syndrome). Congenital
anomalies, abnormal growth, malformations, such as kidney and urinary tract anomalies, also raise concerns for
or developmental delay? anorectal, sacral and spinal abnormalities, which can interfere with defecation.
What treatments have been Informs clinical management
tried, and what was the

response?
Is there a family history of Each of these causes has some familial patterns
functional constipation,
Hirschsprung disease, or
celiac disease?
Physical examination
Abdominal distension Severe distension raises concern for organic disease
Palpable stool mass Consistent with constipation from any cause, but lack of palpable stool does not
rule out constipation
Mass in suprapubic area Common finding in patients with rectal stool impaction, but can also suggest sacral
teratoma
Cutaneous changes in the Suggests spinal dysraphism

lumbosacral area (dimple,
hair tuft, lipoma, or deviation
of the gluteal cleft)
Soiled underwear (fecal In the presence of rectal stool impaction, suggests overflow incontinence and
incontinence) functional constipation
Absent anal wink or Suggests neurologic dysfunction

cremasteric reflex, decreased
lower extremity tone or
strength
Anal fissure or scarring Anal fissures may be a cause or a consequence of functional constipation
Anteriorly displaced anus, or Suggests anorectal anomaly

perianal fistula
Digital rectal examination:
Anal sphincter tone Increased tone suggests Hirschsprung disease, low tone suggest neurogenic
constipation
Size of rectal vault Large vault is consistent with chronic functional constipation
Impacted stool (hard or Soft stool suggests possibility of anorectal dysfunction, including Hirschsprung
soft) disease
Explosive expulsion of stool Suggests Hirschsprung disease

after the examination
(squirt sign)
Graphic 79830 Version 4.0
Causes of constipation in children
Physiologic causes of constipation

Functional constipation* (may have dietary or behavioral triggers, exacerbated by painful defecation and stool
withholding)
Cow's milk or other dietary protein intolerance*
Low dietary fiber*
Slow transit constipation
Inadequate fluid intake (fever, hot weather)
Immobility
Anorexia nervosa
Starvation
Neurogenic causes
Hirschsprung disease ¶ Δ
Cerebral palsy
Myelomeningocele
Spinal cord injury
Closed spinal dysraphism ¶ ◊ (eg, tethered cord, sacral agenesis, split spinal cord malformation
[diastematomyelia])
Sacral teratoma ¶ Δ
Neurofibromatosis
Muscular weakness (may be generalized, as in Down syndrome, or due to abnormal abdominal musculature, Δ as
in prune belly syndrome or gastroschisis)
Infantile botulism ¶ Δ (constipation an early complaint, facial and ocular palsies, poor suck and hypotonia are
other features)
Pseudoobstruction (eg, visceral neuropathies, myopathies, mitochondrial disorders)
Intestinal neuronal dysplasia
Familial or acquired dysautonomia
Duchenne muscular dystrophy
Endocrine and metabolic causes

Cystic fibrosis ¶ (with meconium ileus in neonates, Δ or distal intestinal obstruction syndrome in older children)
Hypokalemia
Lead poisoning ¶
Vitamin D intoxication
Hypo- or hypercalcemia
Hypothyroidism
Diabetes mellitus
Pheochromocytoma
Multiple endocrine neoplasia type 2B (MEN2B)
Polyuria (leading to dehydration)
Juvenile systemic sclerosis (scleroderma) or mixed connective tissue disease
Acute intermittent porphyria
Anatomic causes
Anorectal anomalies (imperforate anus, Δ anteriorly displaced anus)
Intestinal obstruction ¶ (in neonates, consider atresia, webs or volvulus) Δ
Small left colon syndrome Δ §

Other causes
Celiac disease*
Drugs (opiates, anticholinergics, antidepressants, chemotherapy, aluminum containing antacids)
Generalized joint hypermobility
* Relatively common cause.

¶ Prompt diagnosis is important to the outcome.
Δ Generally presents during infancy.
◊ Refer to UpToDate topic reviews on closed spinal dysraphism (spina bifida occulta).
§ Similar findings may occur in infants with Hirschsprung disease.
Data from:
1. Tunnessen WJ. Constipation and fecal retention. In: Signs and Symptoms in Pediatrics, 3rd ed, Lippincott, Williams
& Wilkins, Philadelphia 1999. p.518.
2. Tabbers MM, Dilorenzo C, Berger MY, et al. Evaluation and Treatment of Functional Constipation in Infants and
Children: Evidence-Based Recommendations From ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 2014;
58:265.
3. Kajbafzadeh AM, Sharifi-Rad L, Seyedian SS, et al. Generalized joint hypermobility and voiding dysfunction in
children: is there any relationship? Eur J Pediatr 2014; 173:197.
4. Kraus D, Wong BL, Horn PS, Kaul A. Constipation in Duchenne Muscular Dystrophy: Prevalence, Diagnosis, and
Treatment. J Pediatr 2016; 171:183.
Alarm signs or physical findings that suggest an organic cause of constipation in

children
Symptoms or history Physical findings

Acute signs Severe abdominal distension
Delayed passage of meconium (after 48 hours of life) Pelvic mass (eg, sacral teratoma)
Fever, vomiting, or diarrhea Lower spine abnormalities
Rectal bleeding (unless attributable to an anal Lumbosacral dimple, hair tuft or lipoma, or deviation
fissure) of the gluteal cleft
Severe abdominal distension Anal scars
Chronic signs Anteriorly displaced anus
Constipation present from birth or early infancy Patulous anus
Ribbon stools (very narrow in diameter) Perianal fistula
Urinary incontinence or bladder disease Tight anal canal with empty rectum
Weight loss or poor weight gain Explosive expulsion of stool after digital examination
of the rectum
Delayed growth (eg, decreasing height percentiles)
Absent anal wink
Extraintestinal symptoms (especially neurologic
deficits) Absent cremasteric reflex
Congenital anomalies or syndrome associated with Decreased lower extremity tone or strength
Hirschsprung disease (eg, Down syndrome) Abnormal lower extremity deep tendon reflex:
Family history of Hirschsprung disease absence of delay in relaxation phase
Abnormal thyroid gland
Extreme fear during the anal inspection
Based on information in Tabbers MM, Dilorenzo C, Berger MY, et al. Evaluation and Treatment of Functional Constipation
in Infants and Children: Evidence-Based Recommendations From ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr
2014; 58:265.
Diagnoses to consider in infants and children with delayed passage of meconium
Condition Comments
Hirschsprung Abdominal distension and vomiting are common. On digital examination, typical findings are a
disease tight anal canal with empty rectum, often with an explosive "squirt" of soft stool when the finger
is withdrawn. On contrast enema, a transition zone may be seen, but often is not visible in
newborns.
Intestinal Consider atresia, webs, or volvulus. Obstruction may be present even in infants who pass
obstruction meconium.
Meconium ileus Symptoms often begin on second day of life. Most patients with meconium ileus have cystic
fibrosis.
Meconium plug Caused by colonicdysmotility or abnormal meconium consistency, leading to

syndrome obstipation in the newborn. A contrast enema is both diagnostic and
therapeutic. Some patients with meconium plug syndrome have Hirschsprung
disease.
Functional ileus Occurs in setting of prematurity, sepsis, respiratory distress, pneumonia, or electrolyte
disturbances.
Small left Barium enema shows small-caliber left colon. Increased incidence with maternal diabetes.
colon*
Drugs Magnesium sulfate (MgSO4), opiates, or ganglionic blocking agents.

administered to
mother before
delivery
Hypothyroidism Infants with hypothyroidism also may have prolonged jaundice, lethargy, and low body
temperature.
Other Rare disorders associated with intestinal pseudo-obstruction, including megacystis-microcolon-

intestinal hypoperistalsis (Berdon) syndrome.
* Similar findings may occur in infants with Hirschsprung disease (HD), so affected infants should be observed closely and
evaluated for HD if appropriate.
Data from: Tunnessen WJ. Constipation and fecal retention. In: Signs and Symptoms in Pediatrics, 3rd ed, Lippincott,
Williams & Wilkins, Philadelphia 1999. p.518.
Causes of voluntary stool withholding in infants and children
Painful defecation
Anal fissure
Perianal irritation
Sexual abuse
Hemorrhoids
Change of enviroment
New school, traveling, or other change in routine
Family stress
Improper toilet training
Emotional disturbance
Severe mental retardation
Depression
Data from: Tunnessen WJ. Constipation and fecal retention. In: Signs and Symptoms in Pediatrics, 3rd ed, Lippincott,
Williams & Wilkins, Philadelphia 1999. p.518.
Spectrum of anorectal anatomy in the female
A) Normal anatomy with anus in normal location and perineal body between anus and
vagina. B) Severe anomaly with rectum ending high in the vagina. Arrowheads mark
normal anal location. C) Low anomaly with rectoperineal fistula (fourchette ectopic
anus), anterior to normal anal location. D) Intermediate position of anal opening in the
perineum.
Reproduced with permission from: Hendren WH. Pediatric rectal and perineal problems.
Pediatr Clin North Am 1998; 45:1353. Copyright ©1998 Elsevier.
Rectal shelf in a three-month-old girl with an

intermediate anorectal anomaly
A) The anal opening is excentrically located in pigmented skin of perineum. The

opening is not stenotic. B) To demonstrate the posterior shelf, a surgical
clamp is inserted into the anal opening, pulling outward on the posterior lip of
anorectal canal.
Reproduced with permission from: Hendren WH. Pediatric rectal and perineal
problems. Pediatr Clin North Am 1998; 45:1353. Copyright ©1998 Elsevier.
Nineteen-year-old woman with life-long history of

severe constipation
A) In this 19 year-old woman with a life-long history of constipation, the anus is

clearly anteriorly located, with a short perineal body. Normally, the anus should
be located in the shiny pigmented skin, which is posterior to anal opening in this
patient. B) Barium enema in the same patient. There is marked dilatation of
rectum, extending to the anus. This is the typical appearance of so-called "habit
constipation" or "psychogenic" constipation. However, some of these cases are
probably caused by unrecognized slight anorectal malformation, as in this
case. After anoplasty, this patient developed normal defecation patterns.
Reproduced with permission from: Hendren WH. Pediatric rectal and perineal
problems. Pediatr Clin North Am 1998; 45:1353. Copyright ©1998 Elsevier.
Rome IV criteria for the diagnosis of functional constipation in children
Infants and toddlers up to 4 years old Children with developmental age of at least
At least two of the following present for at least one 4 years
month At least two of the following present at least once per week
Two or fewer defecations per week for at least one month*
History of excessive stool retention Two or fewer defecations in the toilet per week
History of painful or hard bowel movements At least one episode of fecal incontinence per week
History of large-diameter stools History of retentive posturing or excessive volitional stool
retention
Presence of a large fecal mass in the rectum
History of painful or hard bowel movements
In toilet-trained children, the following additional
criteria may be used: Presence of a large fecal mass in the rectum
History of large-diameter stools that may obstruct the toilet
At least one episode/week of incontinence after the
acquisition of toileting skills The symptoms cannot be fully explained by another
History of large-diameter stools that may obstruct the medical condition.
toilet
* In addition, the symptoms are insufficient to fulfill the diagnostic criteria of irritable bowel syndrome.
Data from:
1. Benninga MA, Faure C, Hyman PE, et al. Childhood Functional Gastrointestinal Disorders: Neonate/Toddler.
Gastroenterology 2016.
2. Hyams JS, Di Lorenzo C, Saps M, et al. Functional Disorders: Children and Adolescents. Gastroenterology 2016.
Differential diagnosis of straining in infants
Cause of straining Clinical characteristics
Infant dyschezia Healthy infant zero to nine months of age

Soft stool passed after straining
Constipation Healthy infant

Stools are hard or pellet-like
Anal fissure Healthy infant

May or may not have history of constipation
Fissure identified on inspection of anus
Straining may be caused by voluntary stool withholding
Cow's milk intolerance Healthy infant

Diet contains cow’s milk protein (breast- or formula-fed)
Normal or loose stools with gross or occult blood and/or mucus
Hirschsprung disease Newborn or infant

History of delayed passage of meconium (after 48 hours of life)
Well- or ill-appearing
Constipation or abdominal distension; occasionally diarrhea
Rectal examination may reveal tight sphincter, empty ampulla, and/or
explosive squirt of stool on withdrawal of finger
Anorectal manometry demonstrates absence of rectosphincteric reflex
Ganglion cells absent on rectal biopsy
Internal anal sphincter achalasia Presentation and anorectal manometry similar to Hirschsprung disease as
described above
Ganglion cells present on rectal biopsy
Courtesy of Dr. Manu Sood.
Barium enema in Hirschsprung disease
Barium enema of an infant with Hirschsprung disease, showing the transition

zone (arrow) between the lower aganglionic bowel and the normal colon above.
Courtesy of George D Ferry, MD.
Functional constipation
This unprepped barium enema is compatible with functional constipation. The

rectum is mildly dilated with stool and there is no evidence of obstruction.
Courtesy of George D Ferry, MD.
Contributor Disclosures
Manu R Sood, FRCPCH, MD Consultant/Advisory Boards: Kimberly Clark [diaper product development];
QOL [Malabsorption (Sacrosidase)]. Employment (spouse/partner): AbbVie - No relevant conflict on topic.
Equity Ownership/Stock Options (spouse/partner): AbbVie; Abbott. B UK Li, MD Nothing to disclose Alison
G Hoppin, MD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must conform
to UpToDate standards of evidence.
Conflict of interest policy

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Constipation in infants and children: Evaluation

Author: Manu R Sood, FRCPCH, MD

INTRODUCTION — Constipation affects up to 30 percent of children and accounting for an estimated 3 to 5

● (See "Prevention and treatment of acute constipation in infants and children".)

• Constipation present from birth or early infancy

Psychosocial and environmental factors — When functional constipation is suspected, particular

Findings supporting a functional etiology include:

• Sensory loss or motor weakness

A digital examination is suggested for the following groups of patients [7]:

● Infants with constipation

DIAGNOSIS OF FUNCTIONAL CONSTIPATION

● Two or fewer defecations per week

• Infants – Hirschsprung disease, spinal dysraphism, sacral teratoma, infantile botulism

• All ages – Cystic fibrosis, lead poisoning, intestinal obstruction

Colon transit studies

• To help distinguish between retentive fecal incontinence (constipation-associated) and non-retentive

• To identify children with stool expulsion disorders, suggesting outlet obstruction.

• For the day 4 films:

Colonic transit time (hours) = # markers remaining x 1.0

(where 1.0 = 72 hours/72 markers ingested)

• For the day 7 films:

Colonic transit time (hours) = # markers remaining x 2.3

(where 2.3 = 168 hours/72 markers ingested)

Anorectal manometry — Anorectal manometry involves placement of a catheter containing pressure-

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 5906 Version 36.0

Evaluation of an infant or child with constipation

Was there a preceding Suggests functional constipation

Was the onset around the Suggests functional constipation

Were there problems with Suggests functional constipation

Stool quality and

Is defecation painful? Suggests functional constipation

Does the stool form pellets Supports diagnosis of functional constipation

What treatments have been Informs clinical management

tried, and what was the

Cutaneous changes in the Suggests spinal dysraphism

Absent anal wink or Suggests neurologic dysfunction

Anteriorly displaced anus, or Suggests anorectal anomaly

Digital rectal examination:

Explosive expulsion of stool Suggests Hirschsprung disease

Graphic 79830 Version 4.0

Causes of constipation in children

Physiologic causes of constipation

Cow's milk or other dietary protein intolerance*

Low dietary fiber*

Slow transit constipation

Inadequate fluid intake (fever, hot weather)

Spinal cord injury

Pseudoobstruction (eg, visceral neuropathies, myopathies, mitochondrial disorders)

Intestinal neuronal dysplasia

Familial or acquired dysautonomia

Duchenne muscular dystrophy

Endocrine and metabolic causes

Multiple endocrine neoplasia type 2B (MEN2B)

Polyuria (leading to dehydration)

Juvenile systemic sclerosis (scleroderma) or mixed connective tissue disease

Acute intermittent porphyria

Intestinal obstruction ¶ (in neonates, consider atresia, webs or volvulus) Δ

Small left colon syndrome Δ §

Drugs (opiates, anticholinergics, antidepressants, chemotherapy, aluminum containing antacids)

Generalized joint hypermobility