Other Disorders
of the Anus and
Rectum, Anorectal
Function
Risto J. Rintala and Mikko P. Pakarinen
Anatomy
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The adaptive compliance of the colon with the retentive mechanism of the anorectum is required for fecal continence. The
simple anatomic impedence to the descending fecal mass
occurs because the distal end of the gastrointestinal tract is
1311
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PART VII
ABDOMEN
Sensory Elements
Motor Elements
Rectal wall
and stretch
receptors
Rectal wall
Levator ani
Puborectalis
Puborectalis
Striated muscle complex
Striated muscle
complex
Anal mucosa
Internal sphincter
Deep
Superficial
External
sphincter
Subcutaneous
FIGURE 104-1 The main anatomic components of the rectum
and anus.
Constipation
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Physiology of Defecation
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The defecation sequence is a complex combination of involuntary actions and voluntary accomplishments that are controlled by higher cerebral centers. The rectum fills gradually
by colonic action. The distension of the rectum stimulates
stretch receptors in the rectal wall and levator and induces
an initial contraction of the voluntary sphincter complex,
retrograde emptying of the distal rectum, and relaxation of
the IAS. This causes a sensation to desire to defecate that
increases in intensity as the rectum fills. Stool is temporarily
allowed to contact the sensitive mucosa of the anal canal
DEFINITIONS
The consensus Rome III classification is most commonly used
in the diagnosis of functional constipation in children.25
Functional constipation in children is defined as constipation
not associated with congenital abnormalities, acquired
CHAPTER 104
diseases, or medication. The following criteria apply for children of all ages:
Two or more of the following must exist:
1. Two or fewer defecations in the toilet per week
2. At least one episode of fecal incontinence per week (after
acquisition of toileting skills)
3. History of retentive posturing or excessive volitional stool
retention
4. History of painful or hard bowel movements
5. Presence of a large fecal mass in the rectum
6. History of large-diameter stools that may obstruct the toilet
Both constipation and soiling are common. Soiling has
been reported in approximately 3% of children older than 4
years of age, and constipation accounts for at least 3% of all
medical and 25% of pediatric gastroenterology referrals.2629
More than 50% of constipated children have a familial incidence, and most studies suggest a male predominance. The
reported ratios range between 1.5:1 and 3:1.30,31
Because constipation during the neonatal period, usually
associated with distention and vomiting, is never functional,
anatomic or mechanical obstruction must be suspected. Most
(94% to 98%) full-term and most (76%) preterm normal
babies pass meconium during the first 24 hours after birth.
All normal babies (100% of full-term and 99% of preterm)
have a first stool with the first 48 hours of life.32 During the
first year of life, symptomatic constipation warrants an evaluation and organic causes for constipation should be ruled out.
During infancy, constipation is often initiated after dietary
manipulations, often the change from breast-feeding to bottlefeeding or the introduction of solid foods.33 Specifically, in
children anal fissure or perianal dermatitis with group A Streptococcus causes a vicious cycle of stool withholding and painful
defecation and chronic constipation. A suggested alternative
etiology is cows milk protein intolerance.34 Dietary fiber is
poorly associated with constipation except in older children.35
Constipated parents are more likely to have constipated
children.36 Slow transit constipation is a major problem in
adults and is probably important in a subset of children with
constipation.37,38 Psychologic problems are common in constipated children, but they are secondary to constipation in the
majority of cases.30,39
ACUTE CONSTIPATION
Acute constipation may be secondary to inactivity, changes of
environment or diet, or an anal fissure. Presentation as acute
abdominal pain is common. Acute constipation presenting
with abdominal pain is usually relieved by one single enema.
The management of acute constipation is usually straightforward, especially in infants and toddlers. Adding more water
to the diet and restriction of cows milk intake usually relieve
the symptoms. Older children and those who have an acute
anal fissure require bulk laxatives for a variable period of time.
CHRONIC CONSTIPATION
Persistent constipation, which does not rapidly respond to
dietary manipulation or simple laxative treatment, can be
defined as chronic. A child with chronic constipation commonly presents with fecal soiling. Organic causes that should
be taken into account in the diagnostic workup for chronic
constipation are summarized in Table 104-1.
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TABLE 104-1
Differential Diagnosis of Chronic Constipation: Organic
and Acquired Causes
Hirschsprung Disease and Allied Disorders
Internal sphincter achalasia
Intestinal neuronal dysplasia (hyperganglionosis)
Hypoganglionosis
Congenital Anomalies
Anal stenosis
Anterior perineal anus
Acquired Diseases
Chronic anal fissure
Chronic anal fistula
Crohn disease
Associated with Systemic Disease
Hypothyreosis
Hypercalcemia
Cerebral palsy and other neurologic impairment conditions
Uremia
Psychiatric Disease
Depression
Anorexia nervosa
Primary encopresis
Medication
Anticonvulsive drugs
Psychiatric drugs
Anticholinergic drugs
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PART VII
ABDOMEN
TABLE 104-2
Clinical Features of Severe Functional Constipation
Age at Onset
0-1 yr
1-5 yr
> 5 yr
Boys-to-girls
Previous attempts to treat
Soiling
Unsuccessful toilet training
Pain at defecation
Abdominal pain
Occasional blood in stool
Poor appetite
Wetting
Primary psychopathology
Rectal prolapse
20-25%
70%
10-15%
1-3:1
80-90%
70-75%
70-80%
70-80%
50-60%
25-30%
20-30%
20-30%
15-20%
3%
and less than 0.46 in the male (usual values: newborn, 0.58;
age 4 to 18 months, 0.56) the child should be investigated
carefully, especially if constipation is present. To differentiate
between anterior anus and perineal ectopic anus is often difficult, especially if the anterior position of the anus is associated with stenosis. Final diagnosis may require muscle
stimulation in general anesthesia.
Perianal sensation to rule out neurologic disorders can
be evaluated by stroking the perianal tissue gently with a
cotton-tipped applicator, watching for the anal puckering,
and ascent of the perineum. The childs underwear should also
be examined for soiling.
It is advisable to perform a digital rectal examination at least
once in a child with chronic constipation to rule out organic
obstructing causes. The fecal mass that fills the rectum may
have developed to a fecaloma that can be stone hard. The
rectorectal space must be examined to feel masses within
the hollow of the sacrum. The closing reflex should be seen
when the finger is withdrawn. The absence of either the perineal cutaneous or closing reflexes suggests an underlying
neurologic disorder.
If the clinical history and physical examination do not
suggest organic etiology, a trial of medical treatment can be
initiated. A small infant with a history of neonatal symptoms
and early onset constipation should undergo barium enema
without bowel preparation. An abnormal barium enema
should be followed by rectal biopsy and possibly anorectal
manometry to rule out Hirschsprung disease and allied disorders. In an older child without any history of significant
neonatal constipation a primary barium enema or biopsies
are not necessary44,45 before the onset of medical management. Barium enema and other imaging such as plain abdominal radiography, transit time studies, or magnetic resonance
imaging (MRI) are indicated in patients who have poor
response to appropriate medical treatment or abnormal clinical findings such as neurologic symptoms. Patients who have
a poor response to optimal medical therapy should undergo
also rectal biopsy and anorectal manometry to rule out rare
forms of dysganglionoses such as hypoganglionosis, intestinal
neuronal dysplasias, and internal sphincter achalasia.
CHAPTER 104
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PART VII
ABDOMEN
Rectal Prolapse
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TREATMENT
In acute prolapse, reduction may occur spontaneously on
standing up. If not, the prolapse must be reduced as soon
as possible. The parents often rush the child to a hospital
when the prolapse appears for the first time. The tip of the herniated bowel can usually be gently pushed into the anus.
If edema has formed, a gentle squeezing pressure may be
required. Reduction technique must be taught to the parents.
There is spontaneous cure in most cases of recurrent prolapse.78 In many cases the prolapse reduces spontaneously.
In cases without spontaneous reduction the parents can
reduce the prolapse gently if appropriately instructed. Accompanying constipation is treated with laxatives when present.
Local transanal treatments such as injections of the prolapse,
multiple linear thermocauterization to the mucosa, excision of
redundant mucosa, or insertion of a subcutaneous suture
around the anus are not tested in controlled trials.
Operation is indicated in rare cases with intractable prolapse
and may be considered in patients who are not spontaneously
cured in 12 to 18 months of follow-up. Patients older than
4 years of age require surgery much more often than younger
children. There are several surgical methods that have been
used with success for recurrent prolapse. We prefer laparoscopic suspension of the rectum to anterior sacrum with routine suture closure of the space between the rectum and the
vagina or the urinary bladder in order to avoid development
of enterocele.79 An additional resection of the sigmoid colon
may be performed in intussuscepting prolapse of the sigmoid
colon and in recurrent cases. Laparoscopic approach has been
successful in nearly 20 patients that have required surgery. The
procedure is associated with minimal postoperative pain and
short hospital stay. Patients benefit from laxative therapy during
the early postoperative period. Posterior sagittal approach with
muscle repair and suspension of the rectum to the sacrum,8083
posterior rectal plication,84 and Ekehorn rectosacropexy85
are also reported to be associated with a high cure rate.
Secondary operation is indicated for iatrogenic prolapse
after a pull-through operation in symptomatic patients.
Typical symptoms include bleeding and leak of mucus.
In patients with mucosal prolapse treatment involves excision
CHAPTER 104
Anal Fissure
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1317
PATHOGENESIS
Pathogenesis of idiopathic anal fissure is still incompletely
understood, and it may differ in adults and children.86 Anal
fissures in childhood are often associated with secondary
constipation due to painful passage of stools. The classic concept of mechanical tear caused by hard stools as a primary
causative factor may be too simple and outdated. However, deliberate avoiding of defecation does cause rectal distension
and leads to decreased rectal sensation, which in turn, results
in infrequent, bulky, and hard stools that prevent healing of
fissure. Fear of painful defecation may lead to fecal retention
and gives rise to a vicious circle.
There is a widely accepted theory on the pathogenesis of
anal fissure in adults.86,87 According to this theory increased
internal sphincter pressure and muscle spasm lead to impaired tissue perfusion and finally epithelial ulceration. Spasm
of internal anal sphincter is so severe that the pain caused by
fissure is thought to be due to ischemia. The most common
site of idiopathic anal fissure is posterior midline, which is less
vascularized than other areas of the anal canal. Anal canal resting pressure is increased in patients with anal fissure. Decrease
of anal canal pressure after surgical or pharmacologic sphincter relaxation is accompanied with improved perfusion of
anoderm and healing of chronic fissures. Currently, it is unknown whether this theory also applies to pediatric patients.
In children a vast majority of idiopathic anal fissures heal
without any specific therapy. This may be due to relatively
better tissue perfusion of the anal canal, greater regenerative
capacity in general, or different pathogenesis in children than
in adults. An unhealed fissure may become inflamed due to
bacterial infection and chemical and mechanical irritation.
As a result of long-standing inflammation, chronic anal fissure
may have hypertrophied anal papilla proximally and a sentinel
skin tag distally. This kind of chronic anal fissure is only rarely
seen in children and should raise the suspicion of underlying
Crohn disease.88
DIAGNOSIS
Anal fissure may occur in any age. Typical age of presentation
is around 2 years. Most often anal fissures present with bright
red rectal bleeding that may be associated with painful defecation. The child may cry with bowel movements and have
TREATMENT
Most idiopathic anal fissures in children heal without any
specific treatment in a few months.89,90 Only symptomatic
fissures require treatment. If fissure is associated with constipation and/or painful defecation, stool softening with dietary
modification and bulk laxatives is indicated. Lubricants ease
painful passage of stools. The goal is to interrupt the vicious
circle of painful defecation, fecal retention, hard stools, and
prevention of healing of fissure. As expected, most fissures respond promptly to stools softening and heal in several
weeks.89 Hematochezia stops when fissure heals. Occasionally a child presents with typical history after the symptoms
have disappeared and the fissure has healed. Initially, these
patients may be treated expectantly unless no abnormal
clinical signs are present and hematochezia has not recurred.
Botulinum toxin injection into sphincter muscles in order
to overcome increased pressure is a novel treatment for
chronic fissures. Quick and effective healing has also been
reported in children.91 We use botulinum toxin injections into
the internal part of the sphincter complex with a dose of
15-25 U, depending on the patients age, into each of the four
quadrants. Usually healing occurs in several weeks and injections may be repeated in refractory cases. After encouraging
initial results in adults, several recent randomized placebocontrolled trials have assessed efficiency of topical glyceryl
trinitrate in anal fissure in children.89,90,92,93 Two studies
reported faster healing of fissures and relief of symptoms in
children treated with glyceryl trinitrate,90,93 whereas no benefit was found in one.89 Few children experienced temporary
incontinence, and none reported headache during glyceryl
trinitrate treatment.89,90,93 Taken together, topical glyceryl
trinitrate for anal fissures is only marginally better than
placebo.92
Surgical therapies reported for treatment of anal fissure
in children include fissurectomy, anal dilatation under general
anesthesia, and lateral internal sphincterotomy.94,95 Lateral
subcutaneous sphincterotomy also appears to be an effective
procedure in children.94 Fissure cure rates (80%) after fissurectomy combined with laxatives are comparable with simple
laxative therapy.89,95 Anal dilatation causes unpredictable
degree of sphincter damage and should be avoided. In adult
patients, lateral sphincterotomy is associated with an
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PART VII
ABDOMEN
Perianal Streptococcal
Dermatitis
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CHAPTER 104
1319
Vascular Malformations
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Hemorrhoids
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1320
PART VII
ABDOMEN
Infantile Proctocolitis
Sexual Abuse
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Apart from anal fissure, the most common cause of hematochezia in infants younger than 3 months of age is eosinophilic
proctocolitis. The infantile proctocolitis typically presents at
the age of 3 to 4 weeks with fresh blood streaks mixed in
stools. The stools often contain mucus. Usually there are no
other symptoms, and the growth and development of the
infant is normal.116 Colonoscopy shows colitis that is often
patchy and rarely extends beyond the left colon. Histology
reveals marked eosinophilic infiltrate. Some patients may also
have an elevated eosinophilic count in peripheral blood.
Allergic etiology has been suggested because of these findings
but is found in a minority of cases.117 The condition is selflimiting, and symptoms usually subside within a few weeks.
Diet change has been also reported to be helpful in reversing
the symptoms.118
Proctalgia Fugax
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