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27 The Pediatric Patient
419
Symptoms are similar to adults including bleeding, prolapse, itching, pain, blood-streaked stools, and a bulge or
dilated venous plexus at the anal orifi ce. Treatment in children is usually conservative with diet modifi cation, increased
liquid intake, and sitz baths. Laxatives can also be helpful,
and glycerin suppositories work well for younger children.
The vast majority of hemorrhoids resolve with medical
treatment for constipation alone. Operative intervention is
reserved for the rare failure and consists of the procedures as
adults [
12 ].
Anal Fissure
Key Concept : Anal fi ssure presentation and treatment is similar to adults .
While anal fi ssures can occur at any age, they most commonly present around 2 years old [ 12 ]. Symptoms are simi-
lar to adults, with rectal pain, bleeding, streaked stools, as
well as crying with defecation. The lesions in infants may be
found anywhere, although they are more common in the posterior midline. Females, similar to adults, more commonly
have anterior midline fi ssures [ 12 ]. If the lesions are off the
midline or are multiple, consider a workup for Crohn’s disease or a culture and/or biopsy to exclude malignancy, tuberculosis, venereal disease, or immunodefi ciency in the
appropriate setting. Recognize, however, that most anal fi ssures are secondary to constipation and will heal spontaneously with a simple focus on maintaining adequate fl uid
intake along with a proper bowel regimen, and that more
intensive will only be required in a small subset of patients.
Similar to adults, glyceryl trinitrate 2 % (GTN) has been
associated with improvements in fi ssure healing and symptom relief compared with lidocaine or placebo in both the
short term (10 days) and at 8 weeks [ 12 ]. Healing rates have
been up to 84 %, with symptomatic relief reported in
91–94 % with GTN [ 12 – 15 ]. Botulinum toxin can also be
used for resolution of anal fi ssure, though most data consists
of case reports. Keshtgar showed in their study that 4 children who underwent treatment for anal fi ssure with transcutaneous botulinum toxin had resolution of their lesion [ 16 ].
Finally, successful use of lateral subcutaneous sphincterotomy has also been described in children, with a complete
resolution of all fi ssures in small series [ 12 , 17 ].
Our approach is as follows: Asymptomatic lesions are
likely to resolve and should be managed expectantly with
dietary and fl uid management. If intervention is necessary,
an initial trial of topical therapy (lidocaine, GTN) should be
used. Recurrences can be treated medically (GTN is not used
or botulinum toxin), with surgery reserved for continued
failures. You should remember that any surgical intervention
involving a small child or infant should be done so with the
most extreme caution , as incontinence and anal stenosis are
reported in up to 30 % [ 12 ].
Rectal Prolapse
Key Concept : Rectal prolapse in children is most commonly
secondary to constipation , and the majority will resolve with
treatment directed towards proper bowel habits . Surgery
should be reserved only for refractory cases with severe
symptoms .
Rectal prolapse is a common problem in children, with a
peak incidence in the fi rst year of life and most occurrences
taking place under 4 years old around the time of toilet training [ 18 – 20 ]. Prolapse in this cohort is a symptom of an
underlying condition, usually constipation, but can also be
caused by increased abdominal pressure (i.e., chronic cough),
acute or chronic diarrhea, parasites, neoplastic disease of the
rectum, malnutrition, cystic fi brosis, or pelvic fl oor weakness [ 12 ]. While the exact etiology is unknown, the rectosig-
moid in children with prolapse tends to be ~1.5-fold longer
than their normal counterparts [ 21 ].
Most cases are mild and typically resolve spontaneously
shortly after the rectum prolapses [ 18 , 19 ]. You should use
manual reduction prior to the onset of edema for an acute
prolapse that does not reduce on its own. Firm steady pressure may be necessary to decrease the swelling and allow
reduction. Importantly, perform a digital examination to
ensure the reduction is complete, and exclude any distal
source of pathology. Another helpful tip in children is to
approximate the buttocks with adhesive tape for several
hours to reduce recurrence [ 18 ]. Roughly 90 % of rectal pro-
lapse cases in children under the age of 4 will resolve with
these simple steps alone and rarely continues after 6 years
old [ 18 ]. Parents should be instructed to use stool softeners
and/or laxatives, while avoiding prolonged straining and
ensuring the use of a proper toilet size for their child to prevent recurrent episodes. We also tell parents to promptly
reduce a prolapse if it were to reoccur and to bring the child
in for reevaluation.
Children over 4 years of age are more likely to have neurologic (i.e., spinal cord lesions) or muscular defects and
require intervention. You should also consider screening for
cystic fi brosis (CF), as up to 11 % of children with rectal prolapse will have CF [ 22 ]. Indications for surgery include fail-
ure of conservative measures, continued prolapse after 1 year
despite nonoperative therapy, greater than 2 episodes requiring manual reduction, or chronic pain, bleeding, and perianal
excoriation from the recurrent prolapse [ 18 ]. The simplest,
most benign, and still effi cacious, intervention is sclerosing
injections. Several different agents have been used with effi cacy rates ranging from 83 to 100 %, depending on the sclerosant (i.e., 30 % saline, 5 % phenol in almond oil, ethanol
70 %) and number of treatments [
should be used with caution, as reported complications
include temporary fecal incontinence, temporary limping,
bleeding, perirectal infl ammation, urinary retention, necrosis
of the rectal mucosa, and abscess formation [
23 – 28 ]. However, this
23 , 28 ].
420
T. Tanner et al.
Both perineal and abdominal approaches are available for
refractory cases; however, similar to adults, no single operation has been shown to have signifi cantly better results [
22 ]. Although the Thiersch procedure is not used much in
20 ,
adults anymore, it may be ideal for select children with prolapse secondary to weak pelvic fl oor muscles or those associated with cystic fi brosis [
18 ]. A less invasive procedure
somewhat unique to children (and not used extensively anymore) is linear cauterization, which has a reported success
rate up to 94 % [ 29 ]. Some institutions also advocate a com-
bination of these techniques, with linear cauterization used in
conjunction with sclerotherapy injection and a Thiersch procedure [ 20 ].
Although you may not have much experience in any of
these, other procedures you may hear include posterior sagittal transsphincteric rectopexy, Ekehorn’s rectosacropexy
(placing a U-shaped suture through the rectal ampulla and
tying the suture outside at the sacrococcygeal junction), and a
transcoccygeal rectopexy with puborectalis plication. Success
and recurrence rates remain highly variable in limited series
[ 30 – 32 ]. More commonly used procedures in adults, such as
the Altemeier perineal proctosigmoidectomy, have also been
associated with good results in the pediatric population.
Laparoscopic approaches such as suture rectopexy, posterior mesh rectopexy, and resection of the sigmoid colon with
or without rectopexy have also been associated with good
outcomes in children [ 21 ]. Recurrence rates are ~5 %, with
failures perhaps higher in children with cystic fi brosis [ 33 ].
In general, we do not use prosthetic mesh initially in the
pediatric population. Adding a sigmoid resection with rectopexy in children has been also controversial, although it has
been shown to be safe, eliminates the risk of volvulus, and
has a low morbidity and low recurrence rate [ 21 ]. Finally,
similar to adults, the addition of a resection is preferred in
those with intractable constipation and prolonged transit
studies.
Constipation
Key Concept : Similar to adults , constipation therapy should
be guided by a thorough history and physical examination ,
with initial dietary , fl uid , and nonoperative management .
Select diagnoses with severe nonresponsive symptoms may
be considered for operative intervention .
Constipation is a common problem in childhood, affecting 7–30 % of children [ 34 ]. You should remember that
constipation is a symptom rather than a diagnosis, manifesting with painful defecation, diffi culty with passage of
stools, or decreased stool frequency [
lem, responsible for ~3 % of visits to outpatient clinics and
up to 20 % of pediatric gastroenterological complaints [
When not treated adequately, constipation can lead to
35 ]. It is a major prob-
35 ].
Table 27.1 Rome III criteria for pediatric functional constipation
Two or fewer defecations per week
At least one episode of fecal incontinence per week
Stool retentive posturing
Painful or hard bowel movements
Large diameter stools that could obstruct the toilet
Presence of large fecal mass in the abdomen or rectum
With permission from Mugie et al. [
Publishing Group
Table 27.2 Features suggestive of organic causes of constipation [
Organic causes
History: delayed passage of meconium (>48 h after birth), early
onset of symptoms (<6 months of age), bilious vomiting, bloody
stools, ribbon like stools, delayed or abnormal development, no
withholding, no response to conventional treatment, and
extraintestinal symptoms (fever, fatigue, eczema, bladder disease,
neurological disturbances, oral ulcerations)
Physical exam: failure to thrive, fever, absent cremasteric refl ex,
abdominal distension, abnormal position of anus, perianal fi stula,
absent anal wink, tuft of hair on spine, deep sacral dimple,
decreased lower extremity strength/tone/refl ex, asymmetry of the
lumbosacral region or fl attening of the gluteal muscles/lower limb,
spina bifi da, and explosive diarrhea after rectal exam
Sexual abuse
History: smear feces
Physical exam: extreme fear during anal inspection or rectal
examination, anal scars, fi ssures, and hematomas
With permission from Burgers and Di Lorenzo [
38 ]. Copyright © 2011 Nature
38 ]
39 ]
psychological problems, low self-esteem, withdrawal, and
social isolation [ 36 – 38 ].
Evaluation
Key Concept : History and physical examination guide most
of the evaluation , with ancillary testing used mostly for
directed fi ndings , diffi cult cases , or those who have nonresponsive severe constipation .
Functional constipation is the underlying reason in more
than 95 % of children [ 39 ], with the diagnosis confi rmed by
having two or more of the symptoms listed in Table 27.1
[ 38 ]. A thorough history and physical examination is typi-
cally suffi cient to rule out most pathological causes, and
extensive additional testing prior to medical and dietary
treatment is unnecessary (Table 27.2 ) [ 38 ]. Similar to adults,
a comprehensive history should include the frequency and
character of the stools, withholding behavior, timing of
incontinence, straining, and pain with defecation. Children
who present with constipation under 6 months of age, especially who have delayed passage of meconium greater than
48 h after birth, or those who present with abdominal distention and refusal to feed are concerning symptoms for
27 The Pediatric Patient
421
Table 27.3 Causes of constipation in children
Idiopathic constipation (most common)
Dietary
Inadequate fl uid/fi ber intake
High milk intake
Dietary protein allergy
Cow’s milk allergy
Anal / rectal disorders
Anal stenosis, anterior ectopic anus
Anal fi ssure (acute, chronic)
Perianal group A streptococcal infection
Eosinophilic proctitis
Megarectum
Neurogenic abnormalities
Sacral dysgenesis (anorectal anomaly)
Myelomeningocele (spina bifi da)
Spinal cord abnormalities/trauma
Static encephalopathy
Tethered cord
Hirschsprung’s disease
Intestinal neuronal dysplasia
Cerebral palsy
Endocrine and metabolic disorders
Hypothyroidism
Hypercalcaemia
Renal tubular acidosis
Diabetes mellitus
Hypokalemia
Vitamin D intoxication
Medications
Opioids
Anticholinergic agents
Antidepressants
Phenytoin
Phenothiazine
Abnormal defecation dynamics
Fear and withholding habit
Pelvic fl oor muscle dyssynergia
Other
Anorexia nervosa
Sexual abuse
Scleroderma
Cystic fi brosis
Celiac disease
With permission from Keshtgar et al. [
35 ] © 2004 by W.B. Saunders
Hirschsprung’s disease, anal stenosis, or ectopic anus
(Table 27.3 ) [ 35 , 39 ]. Other concerning symptoms that
should prompt you to consider a more extensive workup are
accompanying urinary tract infections, weight loss, neuromuscular development, and psychological or behavioral
problems (Table
27.2 ) [ 39 ].
In addition to a complete abdominal and perineal exami-
nation, differences in the examination for pediatric patients
should involve looking for other signs of congenital syndrome anomalies (i.e., VACTERL) and ensure there is fecal
impaction or anal stenosis [
13 ]. A fecal mass either found in
the abdomen or rectum is present in 30–70 % of children with
constipation [ 16 ]. Investigations for children may include a
basic metabolic panel to exclude electrolyte imbalance and
thyroid or celiac panels to work up patients with severe unremitting constipation. You should note that most laboratory
investigations rarely uncover an underlying disease [ 35 , 38 ].
Plain abdominal x-rays are seldom helpful to assess fecal
retention, and actually correlate poorly between symptoms
and pathology, leaving more open to misinterpretation [ 39 ,
40 ]. For children over 12 months of age, anorectal manome-
try can be extremely valuable when Hirschsprung’s disease is
in the differential diagnosis. Failure of relaxation of the internal sphincter is an absolute indication for rectal biopsy and
has a high positive predictive value for the absence of ganglion cells [ 35 ]. Furthermore, in any infant or other patient in
whom Hirschsprung’s disease is suggested, a rectal biopsy is
the gold standard for diagnosis. You should remember that
short-segment Hirschsprung’s disease may manifest later in
childhood (or even adult life) with constipation, and work up
those select patients accordingly. While ultrasound has been
suggested to look at fecal load in the colon, it really is not
very useful. This is similar to the colon transit studies, where
some authors feel it is helpful [ 37 ]. One could argue that in
children with normal transit time, surgery is not typically
indicated [ 41 ]. Others suggest it aids in determining the point
at which normal peristalsis is lost [ 39 , 42 ]. However, we
rarely fi nd it useful in the primary workup for constipation.
Finally, though the indication for endoscopy for primary constipation symptoms is rare, high- risk or concerning symptoms should direct the need accordingly.
Treatment
Key Concept : Use medical management and dietary changes
as fi rst - line therapy — they will work most of the time .
Years of experience and expert opinion guide most of
constipation management. We fi nd that the most useful fi rst
step is often helping the patient and family to understand the
problem and enlist their cooperation and compliance in what
is often a trial and error period [
an association between not only low-fi ber intake and constipation but also a positive family history, obesity, and low
levels of parental education with an increased prevalence of
childhood constipation [ 38 , 43 ]. The American Academy of
Pediatricians suggests a balanced diet that includes whole
grains, fruits, and vegetables as the fi rst-line treatment of
constipation; however, when comparing fi ber supplementation to placebo, there was no signifi cant difference in defecation frequency or resolution of symptoms [
36 , 38 ]. Interestingly, there is
36 ]. We feel there
422
T. Tanner et al.
is very little downside, so we agree with this initial approach,
along with ensuring adequate fl uid intake and physical activity [
44 ]. Children with a developmental age of at least 4
years should also be instructed to have dedicated toilet time
for 5–10 min after each meal (three times per day) to encourage regular toileting [
45 ].
If you encounter children who have severe constipation
marred by fecal impaction and/or overfl ow incontinence,
disimpaction can be accomplished using either oral or rectal
therapies [
44 – 48 ]: high-dose PEG 3350 (1.5/g/kg/day, max
dose of 100 g/day) orally for outpatient use and milk of
molasses enemas (mixed 1: 10 mL/kg with maximum of
500 mL) followed with maintenance PEG 3350 have been
compared with relatively similar results beyond the fi rst day
[ 47 ]. In general, enemas relieve symptoms faster than the
oral treatment, but either approach is feasible. Other authors
have shown that PEG with electrolytes is more effective than
suppositories, rectal enemas, or even manual evacuation
[ 49 ]. If you would like to avoid rectal therapy or if oral dis-
impaction fails, you may need to take the child to the operating room for disimpaction under general sedation.
Maintenance treatment must be initiated immediately and
may be necessary for several months. You should discontinue this treatment only after there are regular bowel movements for several weeks without effort and the child is toilet
trained [ 34 , 44 ]. Tailored therapy is best and you can choose
from a variety of laxatives. Unlike adults, laxatives cannot
be assumed to work in a similar manner in children due to
the fact that the etiology for childhood constipation is typically different than that of their adult counterparts [ 36 , 38 ].
PEG with electrolytes is our preferred agent and has been
shown to be more effective in achieving treatment success
compared to other laxatives (lactulose and milk of magnesia)
[ 50 – 52 ]. There is also evidence suggesting that mineral oil is
effi cacious, though chronic use should be avoided, as it has
been associated with vitamin defi ciencies [ 52 ]. Other agents
such as probiotics may be helpful in the treatment of functional constipation, but there is very little data available, and
we withhold recommendation. Behavioral therapy alone or
biofeedback should not be the sole treatment of constipation
in children; however, there is some evidence that behavioral
intervention plus laxative therapy improves continence in
this age group [ 38 , 44 ].
Surgery: Sphincter Procedures, Antegrade Continence Enema, and Stoma
Key Concept : Several surgeries somewhat unique to children
are available for symptomatic severe constipation that is
nonresponsive to medical management .
Surgical options for constipation depend in large part on
the underlying etiology. Procedures include anal dilation,
intra-sphincteric injection of botulinum toxin, antegrade
continence enema (ACE), excision of colon and/or megarectum and diversion, and reconstructive proctocolectomy
35 , 53 ]. While (as previously stated) transit studies and
[
total colonic manometry don’t play a major role in the initial
evaluation of constipation, we do use them prior to performing a defi nitive surgery. We fi nd its major use is to ensure the
distal- most segment of the colon or remaining rectum will
have a functional motility pattern that will effectively allow
for the passage of stool [
42 ]. We also recommend a period
of inpatient or close home observation to document objective evidence in order to help identify misreporting and
exaggerating symptoms and thus avoiding unnecessary
operations [ 53 ].
Anal dilation and myomectomy of the internal anal sphincter have been performed with the thought that children with
constipation have hypertrophy of their internal anal sphincter,
and anal dilation would decrease sphincter tone and allow for
painless defecation [ 35 ]. Unfortunately two double-blinded
randomized controlled trials have shown that anal dilation
does not benefi t children with chronic constipation [ 35 ], and
we do not generally advocate its use. Myomectomy of the
internal anal sphincter has been associated with weakening of
the sphincter in subsequent years, and again, we are not strong
proponents. Botulinum toxin sphincter injection has reported
rates of symptom resolution in up to 94 %, without the longterm problems [ 16 ]. While this is generally well tolerated,
~20 % may require repeat injection [ 16 ].
Antegrade enemas are a therapeutic option for children
who have intractable constipation or fecal incontinence and
have failed either (a) a maximal medical management or (b)
a sphincter procedure [ 35 , 38 , 53 , 54 ]. Originally described
by Malone in 1990 [ 54 ], subsequent modifi cations include
an appendicostomy placed in an open or laparoscopic fashion, the cecum or rectum reconstructed to create a continent
conduit, or a percutaneous cecostomy tube or button placed
laparoscopically or open (Fig. 27.2 ) [ 35 , 54 ]. The concept
remains the same: to deliver antegrade enemas to clean out
the entire colon to reduce soiling, with reported success rates
of 52–92 % [ 53 ]. Each procedure has their own complica-
tions including stenosis, prolapse, skin infections, granulation tissue formation, leakage, and prostheses dislodgment
[ 16 , 19 ]. Results do not seem to differ in the complications or
outcomes between a right and left ACE [ 55 ]. Ultimately,
most children with idiopathic constipation can progress to a
point they use no oral medications, and the stoma can be no
longer used or taken down.
Patients with Hirschsprung’s disease should be managed by the appropriate surgical therapy, which is most
often a pull-through procedure that can be performed open
or laparoscopically (Figs. 27.3 , 27.4 , and 27.5 ) and is one
or several stages. One rare, but obviously signifi cant,
complication you may see is in children with chronic
27 The Pediatric Patient
423
Fig. 27.2 Plain fi lm of an antegrade cecostomy tube in place
Fig. 27.4 Mobilized aganglionic sigmoid-rectum pulled through the
anus. We have determined the level where ganglion cells are present
(pictured here close to the anus). After transecting the aganglionic segment, a hand-sewn coloanal anastomosis is performed above the dentate line (Courtesy of David, Gourlay, MD)
Fig. 27.3 Abdominal view at the beginning of laparoscopic-assisted
pull-through procedure. The transition from dilated/normal sigmoid
colon ( right on the image) to contracted/aganglionic colon ( left on the
image). We will typically perform a few submucosal biopsies to confi rm the presence of ganglion cells (Courtesy of David Gourlay, MD)
constipation; a small percentage may develop dilation of
the rectum and sigmoid (megarectum). Resection of the
dilated segments may be performed in the most extreme
cases that have failed all other options, including ACE
[ 53 , 57 ]. Unfortunately, this rarely results in a complete or
immediate cure, as children can still have fecal incontinence or constipation and may still need further resection
[ 38 ]. Yet, almost all will be able to reduce their laxative
need, and roughly half will be able to eliminate their laxative requirement altogether [ 56 ]. Finally, when combined
with the physiological manometric data, the need for per-
Fig. 27.5 Abdominal view of the completed pull-through (Courtesy of
David Gourlay, MD)
manent stomas secondary to failure for these procedures
can be reduced [ 20 ].
For those patients with severe problems that are nonresponsive and signifi cantly affect their quality of life, a stoma
can be performed. In this case, we prefer to use a temporary
diverting loop ileostomy in hopes the child will wish to consider alternative treatments [ 16 ]. Finally, there are case
reports of a restorative proctocolectomy performed in this
424
T. Tanner et al.
fi nal group with generally good results; however, we have
little experience with this and feel it should not be undertaken lightly [
It is important to manage expectations accordingly. You
should let all parties known that 1 year after the commencement for the treatment of constipation, the problem can persist in 50 % of children with another 50 % of those children
still suffering from constipation 5 years later [ 34 , 58 ].
57 ].
Incontinence
Key Concept : Fecal incontinence in children encompasses both
encopresis ( the expulsion of a normal bowel movement in inappropriate places in someone 4 years or older developmentally )
and soiling ( involuntary leakage of small amounts of stool ),
with the difference arising from the quantity of feces lost .
Kids with fecal incontinence have signifi cant social consequences (like adults) such as restriction of social activities,
as well as an increase in behavioral problems, and lower educational levels than expected when compared to their continent counterparts [ 59 – 62 ]. There are fi ve main groups of
children with fecal incontinence: constipation with fecal
retention and overfl ow incontinence, functional nonretentive fecal soiling, anorectal malformations, spinal problems, and sphincter damage or dysfunction [ 59 , 60 ]. The
pathophysiology and treatment strategies differ for each subgroup. While we understand that you may not be the one
primarily managing these patients, we will attempt to give
you a brief overview and some general evaluation and management tips.
Functional Retentive Overfl ow Incontinence
Key Concept : Functional fecal retention with overfl ow incontinence is the most common cause of fecal soiling . Treatment
should be aimed at treatment of constipation .
Constipation can lead to a vicious cycle that begins with
painful defecation and ends in voluntary withholding and
accumulation of stool in the rectum [ 16 ]. With chronicity, the
rectum may become dilated, and sensation is gradually lost;
therefore, when the softer stool arrives from the more proximal colon, it cannot be accommodated and leaks around the
bolus of hard stool [ 46 ]. Because of the lack of sensation, this
passage of soft stool is not sensed until the incontinence has
occurred. This functional problem is typically self- limiting
but requires aggressive treatment of constipation [ 59 , 60 ].
Functional Non-retentive Fecal Soiling
Key Concept : This is a subgroup of patients who lack a history or physical exam fi ndings consistent with constipation
or functional fecal retention , but who have evacuation of
large bowel movements in their underwear at least once a
week . Treatment should be aimed at toilet training and positive feedback .
Confounding things, these children often have daily
bowel movements in the toilet with near complete evacuation of stool and normal colonic transition studies [
Historically this was considered a manifestation of emotional disturbances in school-aged children; however, studies
have not shown any serious psychological disorders, and
treatment with psychotherapy alone has not been effective
[ 63 ]. Treatment for these children should involve parental
education and encourage defecation 5 min after each meal, a
rewards system, and usage of a diary with avoidance of accusatory toilet training and guilt [ 59 ]. Laxatives have been
shown to worsen outcomes, and functional non-retentive
fecal soiling should be treated differently from retentive soiling [ 64 ]. Involvement of a mental health professional may be
benefi cial [ 59 ]. Resolution of the symptoms occurs in only
30 % of the children after 2 years and in 70 % of patients
after 4 years with the problem persisting into young adulthood in up to 22 % of the population [ 65 ].
59 ].
Anorectal Malformations
Key Concept : These patients present with a wide range in
abnormalities , depending on the level of malformation and
associated defects , with the management and outcomes often
contingent on the malformation . However , a trial at medical
therapy is still warranted in this group .
These malformations can be subdivided into three categories: [ 1 ] low anomalies, when the rectum has descended
below the level of the puborectalis—typically without a fi stula to the genitourinary tract and either with or without a
cutaneous perineal fi stula; [ 2 ] intermediate anomalies
located at or just below the puborectalis muscle—often having a fi stulous connection to the distal genitourinary tract;
and [ 3 ] high anomalies with the rectum ending above the
puborectalis (which may or may not be developed)—with a
fi stula to the urinary tract or vagina with no perineal opening
(Fig. 27.6 ) [ 59 ]. Dependent upon the degree of pelvic fl oor
development, there is great variation in the functional outcomes after repair of these malformations [ 60 ]. Generally,
lower malformations have good functional outcomes,
although they still suffer from occasional incontinence, constipation, an inability to control fl atus, or sexual dysfunction
[ 59 ] . Patients with lower defects mostly have voluntary
bowel movements; however, up to 30 % can still experience
soiling. Contrast this with patients with high anomalies,
where 30 % may experience voluntary bowel movements
and up to 90 % experience soiling [ 59 , 66 ]. Major advances
in pediatric surgery in recent decades have allowed for
improved anatomic reconstruction primarily due to the
27 The Pediatric Patient
425
Fig. 27.6 Imperforate anus in an infant with no external opening
Fig. 27.7 PSARP procedure demonstrating the recto-urethral fi stula
and catheter in the urethral opening
69 ]. However, presence of severe sacral defects is one of
[
the most important negative prognostic factors for continence [
59 ]. These patients (i.e., incontinence from anorectal
malformations with minor defects) are ideal for biofeedback, as they are more likely to respond. On the downside,
this is time consuming, requires full cooperation from
patients, and may not be helpful in patients with hypoplastic
sphincters [ 70 ]. Therefore, medical treatment should fi rst be
used to modify the consistency of stool, with antidiarrheals
for loose stools and enemas for those patients that have constipation and overfl ow incontinence [ 59 , 60 ]. The enemas
may be given retrograde or in an antegrade fashion via an
ACE, which tends to be more effective than retrograde enemas [ 59 , 60 ]. Many patients undergo a second surgery for
their incontinence; however, long-term outcomes are no better and may be worse than those who only had one reconstruction [
71 ].
In patients where the anal canal is not surrounded by the
sphincter complex, a PSARP has been advocated. The longterm outcomes are variable in this cohort, with a small group
of patients having complete resolution of their symptoms
and most patients still relying on other measures for continence [
72 ]. These patients may also get fecal soiling due to
intractable constipation and overfl ow incontinence. They
may also have a resulting megarectum either from distal
obstruction or inadequate treatment of constipation. Although
invasive, resection of this dilated portion of bowel can lead to
improvement of symptoms of constipation and overfl ow
incontinence. This should be considered only in children
who have a functional internal anal sphincter, good anal
sphincter tone, and no signs of neuropathy [ 73 ].
Fig. 27.8 Completed imperforate anus anastomosis with restoration of
intestinal continuity to the perineum
posterior sagittal anorectoplasty (PSARP) for higher anorectal anomalies (Figs. 27.7 and 27.8 ) [ 59 , 67 ]. These patients
have improved outcomes, with up to half of patients experiencing full continence and only ~20 % having frequent soiling [ 59 , 67 ], although they may still suffer from severe
problems with constipation [ 66 ].
Remember that three elements are necessary for continence: functional sphincters, normal rectosigmoid motility,
and intact sensation [ 59 , 66 ]. Children with anorectal mal-
formations who have subsequent defecation disorders
should undergo both colonic and anorectal manometric testing to tailor their treatment [ 68 ]. It’s not surprising that
patients who have a functional internal anal sphincter have
higher anal resting tone and better outcomes in continence
Spinal Pathology
Key Concept : The degree of bowel functional disorders from
spinal pathology depends on the level of the defect . Although
medical therapy is again a good fi rst - line therapy , many will
require a surgical procedure .
The most common causes of neuropathic bowel in children are spina bifi da and myelomeningocele. The most common type of spina bifi da affects the lumbosacral region
causing bowel and bladder dysfunction [ 59 ]. Typical anal
changes for these patients are poor voluntary sphincter function, poor colonic motility (specifi cally the left colon), and
poor anorectal sensation [ 60 ]. However, the involuntary
internal sphincter function is usually spared [ 60 ]. More than
50 % of patients with myelodysplasia suffer from fecal
incontinence [ 60 ]. High lesions affect voluntary sphincter
function, sensation, and colonic motility, and these patients
are prone to fecal retention [ 60 ]. In patients with lower
lesions, the functional problem is that patients cannot control
their bowel emptying and stools evacuate spontaneously
without warning; however, patients can rectally stimulate
426
T. Tanner et al.
themselves or strain and have some degree of defecation
control [
60 ]. Dietary changes and toilet routines should be
established; however, enemas are frequently necessary in
these patients, and many of these patients can benefi t from an
ACE procedure [ 60 ].
Sphincter Damage
Key Concept : The age of the patient , degree of the defect , and
presence of any associated nerve damage will determine the
need for surgery and eventual outcome .
Partial sphincter injuries are often expected to scar and
heal spontaneously, while other more serious defects will
require reconstruction of the sphincters. For example,
patients who have undergone a pull-through for
Hirschsprung’s disease have incontinence rates reported as
high as 50 % [ 74 ]. This is primarily due to the partial sphinc-
terotomy incorporated into some of the pull-through procedures, as well as the large degree of sphincter stretching in
others. It is important to note that these are not necessarily
the fi nal outcomes, especially in the infant population. The
incidence of incontinence decreases with age, and many
have complete resolution by the time adulthood is reached
[ 75 ]. Therefore, patients with continence problems after a
pull-through operation can usually be treated conservatively
at fi rst. However, if severe problems persist in the bowel, we
prefer retrograde or antegrade enemas [ 59 ].
Fecal incontinence associated with mild or moderate perineal trauma should raise concern for sexual abuse and should
prompt a thorough workup including psychotherapists to
prevent further physiological or psychological injury (if
present) [ 59 ]. Finally, penetrating trauma or impalement
injury can cause severe sphincter damage and should undergo
immediate primary repair and protecting colostomy when
necessary (Fig. 27.9a, b ) [ 59 , 76 ].
a
b
I n fl ammatory Bowel Disease (IBD)
Key Concept : Treatment of IBD in children follows many of
the same principles as adults .
Similar to adults, treatment of IBD in kids is focused on
modulating the immune response, suppressing infl ammation, preserving bowel mucosa and intestinal length. This
may be particularly true in children with Crohn’s disease
(CD), due to repeated patterns of fl ares and remission, thus
yielding a high risk for complications and an overall malabsorptive state over a longer period of time. In children, this
malabsorptive state can rapidly result in malnutrition and
poor overall growth [ 77 , 78 ]. Indeed, growth itself (i.e., bone
age) can be used as a marker of disease [ 77 ].
IBD can affect children in any age group, although the average age at diagnosis is 12.5 years. Twenty percent of children
Fig. 27.9 ( a , b ) Traumatic sphincter injury
with IBD are diagnosed before age 10, and <5 % are diagnosed prior to the age of 5 [ 79 ]. Unfortunately, a defi nitive
diagnosis of IBD in the pediatric population can be more challenging than in adults. Furthermore, up to 15 % will have the
fi nal diagnosis of CD or ulcerative colitis (UC) changed during
the course of the disease [ 80 ]. This is a factor that needs to be
heavily considered prior to performing any rectal reconstructions after total colectomy and proctectomy in children.
In children, both CD and UC present with varying degrees
of abdominal pain, diarrhea, rectal bleeding, weight loss,
27 The Pediatric Patient
427
anemia, as well as derangements in growth, nutritional status, and psychosocial development [
children with IBD are also impacted in less obvious ways,
such as the ability to participate in recess or organized sports.
Children with UC and CD have an impaired aerobic and
anaerobic exercise capacity [
obesity rates nearing 33 % in American children, 20 % of
patients with CD are classifi ed as obese or overweight,
whereas the rates in children with UC approached that of the
general population [
84 , 85 ].
83 ]. Furthermore, in light of
81 , 82 ]. The lives of
Anorectal Crohn’s Disease
Key Concept : Patients who are diagnosed with CD before the
age of 6 tend to progress along a more benign course . In
contrast , children presenting between the ages of 6 and 17
have more aggressive disease and an increased propensity
for developing abscesses and perianal fi stulas .
Pediatric perianal CD can have a varying course [ 86 ]; and
anywhere from 10 to 47 % of newly diagnosed Crohn’s
patients will present with perianal disease at the time of initial diagnosis [ 87 , 88 ]. Ultimately, upwards of 60 % of chil-
dren may have perianal disease including skin tags, fi ssures,
fi stulas, and abscesses (Fig. 27.1 ) [ 88 , 89 ]. While the major-
ity of children will eventually have some manifestation of
perianal CD, most will resolve with medical therapy alone
[ 87 ]. In contrast to adults, in a study of 325 children, only
12 % required an operation for the anorectal component of
their disease [ 88 ]. Medical treatment of perianal CD varies
widely, with agents such as metronidazole, alone or in addition to 6-mercaptopurine (6-MP), demonstrating benefi t [ 88 ,
90 ]. The combination of azathioprine and 6-MP for at least 6
months has demonstrated a 40 % fi stula closure rate, 67 %
improvement in perianal drainage, and overall improved disease activity indices [ 91 ]. Infl iximab has also had some suc-
cess in decreasing the signs and symptoms of perianal
disease in those children with moderate to severe CD [
When using infl iximab, you should ensure that there are no
undrained abscesses, as an ongoing infection is a contraindication to its use. Topical tacrolimus has also been shown to
be effective in treating IBD-related fi stula-in-ano, with the
same caveat that there is not an underlying deep space infection [
4 ]. Consequently, pelvic MRI has been shown to be a
useful imaging adjunct in defi ning or ruling out underlying
pelvic abscesses in children, as well as the extent and location of fi stulas [ 93 ]. Endoscopic ultrasound is also useful in
defi ning fi stulizing perianal CD and can provide important
anatomical information prior to any attempted surgical ther-
94 ]. If simple fi stulas do not resolve with medical ther-
apy [
apy alone, a fi stulotomy or seton may be used depending on
the extent of muscle involved. Extensive, complex fi stulas
likely will need both medical optimization and seton placement [
90 ].
92 ].
Fortunately, most perianal disease in children follows a
generally benign course and will not become extensive [ 88 ,
89 , 95 , 96 ]. However, a minority will progress to develop
very complicated fi stulas, while others go on to become
highly destructive perianal disease (HDPD). In a retrospective review of 350 pediatric patients, 8 had complicated fi stulas such as recto -urethro-perineal, rectovaginal, rectolabial,
and perineal fi stulas. In that same study, six developed HDPD
with deep perineal ulcerations, undermining of the perineum,
and perirectal tissue with a signifi cant exudate. Worth noting,
two children progressed so far as fecal incontinence [ 89 ]. As
you can see, perianal CD can be quite diffi cult to treat and
may not respond well to any treatment. Their course is hallmarked by repeated abscesses requiring multiple incisions
and drainages, setons, and extensive trials with medical therapy, and even fecal diversion may be necessary [ 96 , 97 ].
Crohn’s Colitis
Key Concept : Don ’ t be fooled by Crohn ’ s colitis in children .
Ensure the proper diagnosis between CD and UC in young
patients presenting with colitis prior to embarking on extensive , irreversible , surgical therapy .
Pediatric patients presenting with IBD prior to age 5 are
more likely to present with isolated colonic Crohn’s disease
or Crohn’s colitis, adding to the confusion over a proper
diagnosis [ 86 ]. Crohn’s colitis can be more diffi cult to distin-
guish from UC in children than in adults, yet the importance
of the diagnosis distinction is no less important. Growth failure is more common in children with Crohn’s colitis than
their counterparts with UC, as is the presence of perianal disease [ 98 ]. In one study of 70 children, 13 % had their diag-
nosis changed from UC to CD or IC after colectomy [ 99 ].
Performing an ileal pouch anal anastomosis (IPAA) in the
setting of CD will certainly lead to more complications [ 100 ,
101 ]. Similar to adults, these can include chronic pouchitis,
pouch fi stulas, and even pouch failure [
ease is also associated with worse outcomes after IPAA
[
101 ]. For these reasons, the diagnosis of Crohn’s colitis is a
contraindication to IPAA, and if a colectomy is warranted for
separate clinic reasons, an ileorectal anastomosis would be
the suggested non-diverting alternative [ 102 ]. Finally, while
a child with Crohn’s colitis may experience temporary benefi t or remission from a colectomy, the disease is very likely
to recur within the small intestine [ 98 ].
101 ]. Perianal dis-
Ileocolic Crohn’s Disease
Key Concept : Resection or stricturoplasty for isolated ileocolic disease appears to have equivalent outcomes .
Although CD can affect any part of the gastrointestinal
tract in children, the most common location remains the
428
T. Tanner et al.
terminal ileum and right colon [ 99 , 103 ]. With isolated active
disease there is some controversy as to the proper treatment.
In 11 pediatric patients who underwent resection of their isolated disease at the terminal ileum and right colon, zero had
evidence of recurrence at a mean of 2.6-year follow-up
(maximum 7 years) [ 104 ]. Others have reported no differ-
ence in relapse or recurrence following resection of the disease versus stricturoplasty [
children, it was found that those who had surgery within 3
years of their initial diagnosis derived a protective benefi t
from the need for later immunosuppression. They also tended
to catch up in height and weight better [ 106 ]. While it is dif-
fi cult what to make of these fi ndings, for those children that
ultimately do need surgery for CD, the open, laparoscopic,
and laparoscopic-assisted approaches have all been proven
to be safe [ 107 – 109 ].
105 ]. In a separate study of 130
Chronic Ulcerative Colitis
Key Concept : UC in children presents with an aggressive
nature more often than in adults , with over one - third eventually requiring a colectomy .
While CD in children tends to present quite similarly to
adults, pediatric UC generally has a much “more severe phenotype” than UC in adults [ 110 ]. Children present with more
extensive and widespread disease and have higher rates of
acute exacerbation [ 110 – 112 ]. Remarkably, upwards of
80 % will present with a pancolitis [ 103 ]. In a population
comparison study, the pediatric adolescent patients with UC
incurred the highest total hospital costs compared to other
age groups, largely secondary to their number of inpatient
hospitalizations [ 113 ].
Further highlighting this issue, 45 % of children with UC
will become steroid dependent, and more than 30 % of children will fail steroid management [ 110 , 111 ]. An average of
30–40 % of pediatric UC patients will ultimately need a colectomy; however, ranges from 10 to 60 % have been reported
[ 111 , 114 , 115 ]. The most common reason children with
chronic UC undergo surgery is for refractory bleeding [ 99 ].
In the elective setting, a total proctocolectomy with ileal
pouch anal anastomosis can be performed. One must be cautious of children on infl iximab, as the complication rate is
nearly twice that as compared to children not on infl iximab.
It is recommended to wait at least 8 weeks after the last dose
of infl iximab or perform a colectomy with end ileostomy as
the fi rst operation [ 116 ]. A 3-stage operation would also be
the recommended course in the setting of an acute exacerbation that required an urgent operation and even more so in
the setting of high-dose steroid therapy [
Short of performing an emergent operation, a few points
regarding their evaluation and course are worth men tioning.
First, while not all laboratory tests are useful in pediatric
117 ].
UC, in two-thirds of children with UC, either CRP or ESR
will be refl ective of their disease. More importantly, if one
of the tests does refl ect disease severity, then it is likely to
continue to predict disease severity throughout the entire
clinical course [
of chronic UC, the Pediatric Ulcerative Colitis Activity
Index (PUCAI) was created. It correlates with the oft-used
Mayo score in adults. The score ranges from 0 to 85 and
includes points for abdominal pain, rectal bleeding, consistency of stools, number of stools per day, nocturnal stools,
and activity level [ 119 ]. In general, a PUCAI score less
than 10 indicates remission, 10–34 mild disease, 35–64
moderate, and greater than 65 points severe disease [ 111 ].
If the score remains greater than 45 on day 3 of treatment
of an acute exacerbation, then steroids will likely fail, and
alternate medical therapy should be initiated [ 120 ]. If the
score is greater than 70 on day 5 of treatment, then both
alternate medical and surgical therapies should be considered [ 120 ]. A clinically signifi cant response to treatment is
usually indicated by a drop in the PUCAI score of at least
20 points [ 111 ].
118 ]. Next, to aid in assessing the severity
Ulcerative Colitis Emergencies
Key Concept : Children with UC present for emergent surgery
just like adults with free perforation , fulminant colitis , toxic
megacolon , and massive hemorrhage . In the emergent setting , the procedure of choice is a subtotal colectomy with end
ileostomy .
Emergent subtotal colectomy and end ileostomy for UC
in children allow for all the advantages seen in adults [ 121 ].
Removal of the majority of disease, shorter operative time
without pouch creation, and allowance for subsequent medical optimization prior to a defi nitive or restorative operation
have all been demonstrated in children as well [ 122 ].
Fortunately, it is rare for pediatric patients to require a colectomy in the urgent or emergent setting. Reported rates have
varied between 1.5 and 13 % [ 123 – 125 ].
Polyposis Syndromes
Key Concept : Polyps in children occur in the setting of familial and genetic syndromes and should be worked up
accordingly .
Intestinal polyps are less prevalent in children than
adults. The most common presenting complaints are abdominal pain and gastrointestinal bleeding [ 126 ]. Of the inher-
ited polyposis syndromes, familial adenomatous polyposis
(FAP) is most common and affects about 1 child in every
10,000 [ 127 ]. In children with FAP, polyps will usually
begin to appear at the age of 16 and will progress to hundreds