1316 PART VII ABDOMEN

circumferential.76 Rectal prolapse and mucosal ectopia were

Rectal Prolapse much more common before the era of the PSARP procedure.77
------------------------------------------------------------------------------------------------------------------------------------------------
Rectal polyps may be a leading point for a prolapse.
Rectal prolapse is a relatively common, usually self-limiting The diagnosis of rectal prolapse is usually based on history.
condition in children. The peak incidence is between 1 and Most commonly a rosette of rectal mucosa is noted after defeca-
3 years of age. Prolapse can be either partial or complete. In tion. The child complains that something comes out of the anus.
partial prolapse the rectal mucosa protrudes only about 1 to Usually the prolapse reduces spontaneously but must be some-
3 cm from the anal verge with characteristic radiating folds times reduced manually. In mild forms the prolapse comes out
from the center of the anal aperture. In complete prolapse, occasionally following major straining or during diarrheal ill-
the full thickness of the rectum is involved; 5 cm or more ness. The problem is more annoying and worrisome for the
of the rectum protrudes, and the prolapse is distinguished patient and parents if the prolapse occurs after every defecation.
by the circular folds of the mucosa (Fig. 104-2). There is sig- Usually the prolapse cannot be provoked when the child is
nificant controversy as to whether rectal prolapse in children brought to consultation. Rectal examination is indicated to
is partial or complete. rule out rectal polyps and ulcers. If there is a history of rectal
bleeding, colonoscopy may be necessary to look for higher
polyps or other lead points. Dynamic defecography is war-
PATHOGENESIS AND DIAGNOSIS
ranted at least when a prolapse is associated with rectal ulcer,
The vast majority of patients suffering from rectal prolapse do suggesting intussuscepting prolapse of the sigmoid colon or
not have any predisposing factors. The children suffering from accompanying enterocele.
idiopathic rectal prolapse are usually otherwise healthy. The
role of constipation as an etiologic factor is controversial; only
3% of patients suffering from severe chronic constipation have
TREATMENT
rectal prolapse.30
Several organic conditions predispose to rectal prolapse. In acute prolapse, reduction may occur spontaneously on
Cystic fibrosis is associated with rectal prolapse.73 More than standing up. If not, the prolapse must be reduced as soon
a fifth of the patients with cystic fibrosis develop rectal pro- as possible. The parents often rush the child to a hospital
lapse.74,75 The neuropathic causes of complete rectal prolapse when the prolapse appears for the first time. The tip of the her-
excluding myelomeningocele are rare. Nevertheless, paralysis niated bowel can usually be gently pushed into the anus.
of the levator ani with raised intra-abdominal pressure leads to If edema has formed, a gentle squeezing pressure may be
procidentia and prolapse. In ectopia vesicae there is wide required. Reduction technique must be taught to the parents.
separation of the symphysis pubis and the puborectalis mus- There is spontaneous cure in most cases of recurrent pro-
cle, and this wide hiatus predisposes to prolapse of the pelvic lapse.78 In many cases the prolapse reduces spontaneously.
organs including the rectum. Besides severe malnutrition, In cases without spontaneous reduction the parents can
connective tissue diseases (e.g., Ehler-Danlos) and behavioral reduce the prolapse gently if appropriately instructed. Accom-
disorders (e.g., Asperger) predispose to rectal prolapse. panying constipation is treated with laxatives when present.
Iatrogenic full-thickness rectal prolapse may occur follow- Local transanal treatments such as injections of the prolapse,
ing pull-through operations for high anorectal anomalies. multiple linear thermocauterization to the mucosa, excision of
Much more common is mucosal prolapse that is usually not 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 laparo-
scopic suspension of the rectum to anterior sacrum with rou-
tine 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,80–83
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.
FIGURE 104-2 Rectal prolapse in a 2-year-old boy. In patients with mucosal prolapse treatment involves excision
 CHAPTER 104 OTHER DISORDERS OF THE ANUS AND RECTUM, ANORECTAL FUNCTION 1317

of the mucosal ectopia and reconstruction of a skin-lined anal stools streaked with bright-red blood. Anal fissure is often
canal with a local skin flap. Patients with a complete prolapse but not necessarily associated with constipation, which is
require a repeat posterior reconstruction of the levator funnel caused by fear of painful defecation.
and external sphincter complex, as well as rectal suspension. The diagnosis is made by direct inspection. The typical
longitudinal tear distal to the dentate line can be visualized
by retracting the perianal skin gently away. No further diag-
nostic modalities are necessary. The most common location
Anal Fissure of idiopathic anal fissure is posterior midline, but especially
------------------------------------------------------------------------------------------------------------------------------------------------
in infants it may be found anywhere in the anal circumference.
Anal fissure is a longitudinal tear or ulcer in the distal anal In female infants a common site of anal fissure is the anterior
canal epithelium extending to the anal verge. Most acute midline. A sentinel pile or skin tag at the area of the fissure
fissures heal spontaneously within a few weeks, but a pro- is associated with chronic or subchronic fissure. Atypical
portion become chronic. Anal fissure is the most common fissures may be multiple and often off the midline and are
cause of hematochezia in childhood, and it is one of the most commonly large and irregular. Atypical appearance of fissure
common lesions to consider in the differential diagnosis of should initiate further investigations including biopsy,
anal pain. Anal fissures are common, although their exact cultures, and colonoscopy to rule out Crohn disease, immu-
incidence in children is unknown. nodeficiency states, tuberculosis, venereal infection, and
malignancies.
PATHOGENESIS
TREATMENT
Pathogenesis of idiopathic anal fissure is still incompletely
understood, and it may differ in adults and children.86 Anal Most idiopathic anal fissures in children heal without any
fissures in childhood are often associated with secondary specific treatment in a few months.89,90 Only symptomatic
constipation due to painful passage of stools. The classic con- fissures require treatment. If fissure is associated with consti-
cept of mechanical tear caused by hard stools as a primary pation and/or painful defecation, stool softening with dietary
causative factor may be too simple and outdated. However, de- modification and bulk laxatives is indicated. Lubricants ease
liberate avoiding of defecation does cause rectal distension painful passage of stools. The goal is to interrupt the vicious
and leads to decreased rectal sensation, which in turn, results circle of painful defecation, fecal retention, hard stools, and
in infrequent, bulky, and hard stools that prevent healing of prevention of healing of fissure. As expected, most fissures re-
fissure. Fear of painful defecation may lead to fecal retention spond promptly to stools softening and heal in several
and gives rise to a vicious circle. weeks.89 Hematochezia stops when fissure heals. Occasion-
There is a widely accepted theory on the pathogenesis of ally a child presents with typical history after the symptoms
anal fissure in adults.86,87 According to this theory increased have disappeared and the fissure has healed. Initially, these
internal sphincter pressure and muscle spasm lead to im- patients may be treated expectantly unless no abnormal
paired tissue perfusion and finally epithelial ulceration. Spasm clinical signs are present and hematochezia has not recurred.
of internal anal sphincter is so severe that the pain caused by Botulinum toxin injection into sphincter muscles in order
fissure is thought to be due to ischemia. The most common to overcome increased pressure is a novel treatment for
site of idiopathic anal fissure is posterior midline, which is less chronic fissures. Quick and effective healing has also been
vascularized than other areas of the anal canal. Anal canal rest- reported in children.91 We use botulinum toxin injections into
ing pressure is increased in patients with anal fissure. Decrease the internal part of the sphincter complex with a dose of
of anal canal pressure after surgical or pharmacologic sphinc- 15-25 U, depending on the patient’s age, into each of the four
ter relaxation is accompanied with improved perfusion of quadrants. Usually healing occurs in several weeks and injec-
anoderm and healing of chronic fissures. Currently, it is un- tions may be repeated in refractory cases. After encouraging
known whether this theory also applies to pediatric patients. initial results in adults, several recent randomized placebo-
In children a vast majority of idiopathic anal fissures heal controlled trials have assessed efficiency of topical glyceryl
without any specific therapy. This may be due to relatively trinitrate in anal fissure in children.89,90,92,93 Two studies
better tissue perfusion of the anal canal, greater regenerative reported faster healing of fissures and relief of symptoms in
capacity in general, or different pathogenesis in children than children treated with glyceryl trinitrate,90,93 whereas no ben-
in adults. An unhealed fissure may become inflamed due to efit was found in one.89 Few children experienced temporary
bacterial infection and chemical and mechanical irritation. incontinence, and none reported headache during glyceryl
As a result of long-standing inflammation, chronic anal fissure trinitrate treatment.89,90,93 Taken together, topical glyceryl
may have hypertrophied anal papilla proximally and a sentinel trinitrate for anal fissures is only marginally better than
skin tag distally. This kind of chronic anal fissure is only rarely placebo.92
seen in children and should raise the suspicion of underlying Surgical therapies reported for treatment of anal fissure
Crohn disease.88 in children include fissurectomy, anal dilatation under general
anesthesia, and lateral internal sphincterotomy.94,95 Lateral
DIAGNOSIS subcutaneous sphincterotomy also appears to be an effective
procedure in children.94 Fissure cure rates (80%) after fissur-
Anal fissure may occur in any age. Typical age of presentation ectomy combined with laxatives are comparable with simple
is around 2 years. Most often anal fissures present with bright laxative therapy.89,95 Anal dilatation causes unpredictable
red rectal bleeding that may be associated with painful defe- degree of sphincter damage and should be avoided. In adult
cation. The child may cry with bowel movements and have patients, lateral sphincterotomy is associated with an