52 CHAPTER
Anus
Amit Merchea, David W. Larson
OUTLINE
Disorders of the Anal Canal
Pelvic Floor Disorders
Common Benign Anal Disorders
Less Common Benign Anal Disorders
Neoplastic Disorders
DISORDERS OF THE ANAL CANAL
The anal canal remains an area of medicine and surgery plagued
by obscurity and limited provider knowledge. Many conditions
are in fact common and benign, but some lead to incapacitating
interference with the patient’s daily life. Provider limitations in
both experience and traditional knowledge often lead to misdiag-
nosis or maltreatment. Therefore, attempts to improve our fund
of knowledge of the anatomy and function of the anal canal
and the basic physiology of the pelvic floor should facilitate accu-
rate diagnosis and management of both common and rare
conditions.
Anatomy
The anal canal extends from the anorectal ring at the most distal
aspect of the rectum to the skin of the anal verge and is approxi-
mately 4 cm in length. The internal and external sphincter muscles
along with the pelvic floor structures contribute significantly to
the regulation of defecation and continence. The anus is bounded
by the coccyx posteriorly, the ischiorectal fossa bilaterally, and the
perineal body and either vagina or urethra anteriorly.
The sphincter apparatus of the anal canal consists of the inter-
nal and external sphincters and can be considered as two tubular
structures overlying each other. The circular muscle layer of the
rectum continues distally to form the thickened and rounded
internal sphincter, which terminates approximately 1.5 cm below
the dentate line, just cephalad to the external sphincter (inter-
sphincteric groove). The external sphincter is elliptical and sur-
rounds the internal sphincter superiorly and is continuous with
the puborectalis and levator ani muscles (Fig. 52-1). The perineal
body is formed by the constitution of the external sphincter,
bulbospongiosus, and transverse perineal muscles anteriorly. The
paired levator ani muscles form the bulk of the pelvic floor, and
their fibers decussate medially with the contralateral side to fuse
with the perineal body around the prostate or vagina.
The internal sphincter is tonically contracted independent of
voluntary control. It receives innervation by the autonomic
nervous system. The external sphincter, under voluntary control,
1394
is innervated by the inferior rectal branch of the internal pudendal
nerve and perineal branch of the fourth sacral nerve. Thus, the
loss of bilateral S3 nerve roots (either surgically or otherwise) will
result in incontinence. If all sacral nerve roots on either side are
sacrificed, normal function is preserved. Similarly, if S1-S3 remains
intact on only one side, the patient would maintain anorectal
control.
Distal to the anal verge, the perianal skin becomes anoderm,
which is a modified squamous lining without hair. At the dentate
line, the squamous epithelium transitions to columnar epithe-
lium; this region is referred to as the anal transition zone. Proximal
to this area, the lining becomes exclusively gastrointestinal colum-
nar epithelium.
Physiology
The process of defecation and maintenance of continence consti-
tute the principal function of the anus. Various factors affect our
ability to effectively achieve these functions—the coordinated
sensory and muscular activities of the anus and pelvic floor, the
compliance of the rectum, and the consistency, volume, and
timing of the fecal movements are all critical to avoidance of fecal
incontinence or defecatory disorders.
As the external sphincter contracts, the anal canal lengthens.
With straining, it shortens. The internal anal sphincter imparts
the resting pressure (~90 cm H2O). Squeeze pressure, generated
by external sphincter contraction, more than doubles resting pres-
sure. The pressure differential between the rectum and anal canal
(low to high) is the principal mechanism that provides continence.
The anorectal angle is the angle between the anal canal and the
rectum. This angle is approximately 75 to 90 degrees at rest and
becomes more obtuse, straightening with straining and evacua-
tion. The ability of the puborectalis to relax and to allow this
straightening of the angle facilitates defecation.
Diagnostic Evaluation of the Anus
Anorectal disorders are common. Therefore, basic principles
including careful history and physical examination should occur
before elaborate testing.

Conjoined
longitudinal
muscle
Deep
External
sphincter Superficial
muscle Subcutaneous
Internal
sphincter muscle
Anal
margin
FIGURE 52-1 The anal canal musculature and pelvic floor muscles are depicted.
History
It is important to elicit symptoms of pain, bleeding, discharge
(purulent or fecal), and any alteration of bowel habits (frequency,
consistency). Other past medical, social (including sexual), and
family history may be relevant in diagnosing anorectal disease.
Bleeding is among the most common presenting symptoms
of diseases of the anus and large bowel. Specific details about the
nature of the bleeding can help localize the source in the alimen-
tary tract. Blood that drips, is separate from stools, and is bright
red is usually seen with rectal outlet bleeding, as from internal
hemorrhoids. Blood on toilet tissue may be associated with
minor hemorrhoidal disease but also with anal fissure, although
anal fissure is typically accompanied by severe pain at defecation.
Passage of clots or melena may indicate a more proximal source
of bleeding. One must always give consideration to evaluation
of the more proximal bowel to exclude serious conditions, such
as cancer. This should be of prime importance when initial
anorectal examination cannot confirm a bleeding source, when
the patient has greater than average risk of cancer, or when bleed-
ing does not resolve promptly after initiation of appropriate
treatment.
Anal or rectal pain occurring during or immediately after stool-
ing is another common presenting complaint. Severe pain during
defecation, often described by patients as passing glass, is usually
associated with anal fissure. Pain, either with or without defeca-
tion, that is throbbing in nature is most often seen with an abscess
or poorly draining fistula. Patients may also complain of purulent,
mucoid, or feculent discharge. A deep-seated rectal pain unrelated
to defecation often characterizes proctalgia fugax or levator ani
syndrome. This is characterized by painful episodes of short dura-
tion (<20 to 30 minutes) that are often relieved by walking, warm
baths, or other maneuvers.
CHAPTER 52 Anus 1395
Levator ani
muscle
Rectum
Puborectalis
muscle
Anal transition
zone
Anoderm
Anal
verge
Physical Examination
Adequate visualization of the external anal anatomy and the anal
canal by anoscopy requires appropriate positioning of the patient
(either left lateral or, more commonly, prone jackknife) and good
lighting. Skin tags, excoriations, scars, and any changes in color
or appearance of perianal skin should be easily recognized. A
patulous anus may indicate incontinence and possibly prolapse.
Inspection while straining (even with the patient on the commode)
may help differentiate the presence of hemorrhoids from rectal
prolapse. A careful and systematic digital examination allows the
identification of any palpable abnormalities of the anal canal.
Finally, the resting tone and strength of the squeeze pressure of
the anal sphincter can be assessed.
Anoscopy or proctosigmoidoscopy (flexible or rigid) after
enema preparation enables visualization of the anus, rectum, and
left colon. Mucosal inflammation is identified by loss of the
normal vascular pattern with erythema, granularity, friability, and
even ulcerations. Gross lesions (polyps or carcinoma) should be
readily identifiable. Biopsy specimens of any suspicious findings
should be obtained for histologic diagnosis.
Other investigations, such as stool culture, specialized imaging
(ultrasound, magnetic resonance imaging [MRI], defecography),
and specialized physiologic testing, may be helpful adjuncts and
should be obtained as clinically appropriate.
PELVIC FLOOR DISORDERS
Incontinence
Fecal incontinence is defined as the recurrent uncontrolled passage
of fecal material for at least 1 month in an individual with a
developmental age older than 4 years. The reported prevalence of
fecal incontinence varies considerably. It has been estimated to
1396 SECTION X Abdomen
occur in 2% to 15% of noninstitutionalized adults.1 Fecal incon-
tinence can have a significant impact on people’s lives, causing
physical discomfort, embarrassment, and social isolation. Further-
more, the financial costs and caregiver burden are substantial with
real risk of underreporting.
Clinical Evaluation
Obtaining a complete medical history and thorough physical
examination is imperative in determining the cause of the fecal
incontinence, which will ultimately guide therapy. Defining the
extent of the incontinence can be accomplished by a simple
history—major incontinence represented by complete loss of solid
stool, and minor incontinence by occasional staining or seepage.
Mucus seepage from prolapsing hemorrhoids or large secretory
villous polyp, urgency from colitis or proctitis, and overflow
incontinence from fecal impaction may be inappropriately con-
fused with true incontinence. The severity of the incontinence
should be established by assessing the patient’s control of flatus
and liquid and solid stool and by the impact of symptoms on
quality of life. Numerous scoring systems for fecal incontinence
exist and can help quantify the problem objectively (Table 52-1).
The cause of fecal incontinence is often multifactorial with
combinations of both anatomic and physiologic dysfunctions.
Anatomic defects in the sphincter may be the result of trauma
from previous surgical procedures, impalement, or obstetric inju-
ries. Moreover, physiologic changes may occur from these ana-
tomic changes, over time or directly, that may lead to sphincter
dysfunction, decreased rectal compliance, or decreased sensation.
All of these issues may contribute to or have an impact on the
degree of incontinence even years after the inciting events.
The most common nonanatomic causes of fecal incontinence
should include associated gastrointestinal disorders, such as diar-
rhea, which can aggravate continence.2 A physical examination
may reveal anatomic abnormalities that account for the inconti-
nence, such as the presence of prolapse, hemorrhoids, or abscess/
fistula. A proper digital rectal examination can highlight a weak
resting tone and squeeze pressure or a patulous anus and the pres-
ence of scars, defects, deformities, or keyhole abnormalities.
Endoscopy remains critical to exclude proctitis, impaction, neo-
plasia, or other rectal mucosal abnormalities that may be contrib-
uting factors.
To confirm physical examination findings, the use of focused
diagnostic tests may be required. Anorectal manometry confirms
the extent of impairment of the internal and external sphincters.
Manometry may identify asymmetry, suggesting anatomic defects
amenable to repair. Balloon expulsion testing during manometry
may demonstrate impairments in rectal sensation. Endoanal ultra-
sound or MRI may also be employed to detect structural defects
of the anal sphincters, rectal wall, and puborectalis muscle.
TABLE 52-1 Cleveland Clinic Fecal Incontinence Score
TYPE NEVER RARELY
Solid 0 1
Liquid 0 1
Gas 0 1
Pad use 0 1
Quality of life impact 0 1
From Jorge JM, Wexner SD: Etiology and management of fecal incontinence. Dis Colon Rectum 36:77–97, 1993.
Responses are scored and summed. A score of 0 indicates perfect continence, 20 is complete incontinence; rarely, <1/month; sometimes, >1/
month; usually, >1/week; always, >1/day.
Treatment
Medical management. Medical management is the initial
option for patients with mild incontinence that may not signifi-
cantly affect quality of life in patients without a reparable ana-
tomic abnormality. Medications to slow transit, to decrease
frequency, and to increase stool consistency can be used. Biofeed-
back training uses noninvasive methods to strengthen the anal
musculature and to improve sensation. Nearly 90% of patients
note improvement in quality of life.3 A bowel management
program, including antidiarrheal medications (loperamide),
bulking agents (methylcellulose), and anticholinergic agents
(hyoscyamine), has been successful for some patients.4 Medical
management can also be complementary to surgical therapy and
may be carried out before or after surgery to optimize surgical
results.
Surgical repair. Surgical options range from the traditional
anatomic approaches, such as sphincter repair, to newer tech-
niques like sacral nerve stimulation and the artificial bowel
sphincter. For patients with intractable symptoms or failure of
other therapeutic measures, colostomy remains a definitive cure.
For defined anatomic defects, the most common surgical approach
is the sphincteroplasty, in which the separated muscle ends are
dissected, reapproximated, and sutured. This can be done in an
overlapping fashion or by simply reapproximating the sphincter
(Fig. 52-2).5 The overlapping sphincteroplasty is associated with
low rates of morbidity and mortality and reasonable rates of short-
term success, with good to excellent results achieved in 55% to
68% of patients.6 Long-term results of sphincteroplasty have dem-
onstrated a deterioration of continence over time, with only 40%
having good to excellent control at a median follow-up of 10
years.7
The use of injectable materials for the treatment of fecal incon-
tinence has recently gained attention. In this procedure, a bulking
agent (silicone, collagen, carbon microbeads, or dextranomer–
hyaluronic acid) is injected into the intersphincteric space to
augment the internal anal sphincter. There are limited data for
these approaches; however, a single randomized controlled trial
did demonstrate short-term improvement in 50% of patients
undergoing injection with dextranomer–hyaluronic acid (Solesta;
Salix Pharmaceuticals, Raleigh, NC).8
More complex approaches to treatment of fecal incontinence
include the dynamic graciloplasty, sacral nerve stimulator, and
artificial bowel sphincter. The use of the gracilis muscle as a flap
encircling the anal canal is reserved for patients in whom the bulk
of the anal sphincter is missing and requires complete reconstruc-
tion. Results of graciloplasty have been favorable, with the major-
ity of patients maintaining continence and the ability to defer
defecation at 5 years.9 Sacral nerve stimulation has been tradition-
ally used in patients in whom the anal sphincter is intact but there
SOMETIMES USUALLY ALWAYS
2 3 4
2 3 4
2 3 4
2 3 4
2 3 4
 CHAPTER 52 Anus 1397

A B C
FIGURE 52-2 Overlapping Sphincteroplasty. A, An anterior curvilinear incision is made on the perineum
between the anus and vagina. The disrupted ends of the external sphincter are dissected free, and the
muscle ends are overlapped and reapproximated with suture. A levatorplasty is also concurrently performed.
B, Digital rectal examination can judge for appropriate tightness of the repair. C, The incision is then reap-
proximated. Some surgeons may leave drains or a small gap in the closure to allow drainage.

is an associated neurologic injury or poor innervation. A prospec-
tive multicenter trial demonstrated success rates of up to 85% at
2 years with a decrease in the number of incontinent episodes per
week by approximately 70%.10 More recent investigation has been
conducted to determine its effectiveness in the setting of a defined
anal sphincter defect (<180 degrees); many patients have noted
significant improvement in these settings.11 With increasing use
of sacral nerve stimulation, the use of an artificial bowel sphincter
has been limited. Complications associated with its use are erosion,
infection, and obstruction. A review had demonstrated little
change in the rate of device explantation or infection in spite of
novel surgical techniques and approaches to placement.12 An
algorithm for the treatment of fecal incontinence is presented
(Fig. 52-3).
Prolapse of the Rectum
Pathogenesis and Clinical Presentation
Prolapse of the rectum, or procidentia, is an uncommon problem
(incidence of 0.25% to 0.4%) characterized by eversion of the
rectum through the anus.13 The prolapse may be complete, char-
acterized by protrusion of all layers of the rectal wall, or partial,
characterized by a mucosal prolapse only. Risk factors that increase
the risk of prolapse include female sex, age older than 40 years,
multiparity, vaginal delivery, prior pelvic surgery, chronic strain-
ing, dementia, and pelvic floor anatomic defects.
The symptoms of early prolapse may be vague, including dis-
comfort or a sensation of incomplete evacuation during defeca-
tion, bleeding, and seepage. Many patients have a long history of
constipation and straining. When prolapse is complete, protru-
sion of the rectum is generally obvious. In patients with occult
prolapse, a feeling of pressure and sensation of incomplete evacu-
ation may be the only symptoms.
Preoperative Evaluation
The preoperative assessment should focus on defining the extent
of the prolapse; the presence of associated conditions, such as
constipation and pelvic floor dysfunction; and other potentially
related complications, such as incontinence. Any of these factors
may influence management. Nearly 50% of patients have
constipation, and the majority have fecal incontinence.14 If the
presence and extent of the prolapse are not readily apparent,
examination while straining on the commode may make it more
evident. Complete prolapse demonstrates full-thickness rectal
protrusion with concentric rings (Fig. 52-4); this should be dif-
ferentiated from prolapsed hemorrhoids, which demonstrate
radial folds. Associated complex pelvic floor abnormalities (cysto-
cele, enterocele, rectocele, vaginal vault prolapse) can be assessed
by dynamic pelvic floor MRI; identification of these additional
pathologic processes may alter the surgical approach.
Complete lower gastrointestinal tract evaluations should be
performed as indicated. On endoscopy, a solitary rectal ulcer 6 to
8 cm anteriorly may be evidenced by redness or ulceration of the
anterior rectal wall. Additional tests can be ordered but have
limited value and are not typically required. Manometry identifies
the presence of sphincter damage but does not predict recovery.
Despite the ability of pudendal nerve terminal motor latency to
predict a high risk for postoperative anal incontinence, it rarely
influences the management. In patients with severe constipation,
colonic transit studies may be valuable; although it is exceedingly
rare, these patients may respond better to a more extensive colonic
resection.
Surgical Correction
Surgical repair of rectal prolapse may be conducted through a
perineal approach (Delorme or Altemeier procedure) or an
abdominal approach (rectopexy with or without resection and
mesh fixation). Insufficient data exist to definitely demonstrate
which surgical approach is superior.15,16 The perineal approach is
less morbid for the patient but has a higher recurrence rate; thus,
it is best suited for patients with high operative risk and limited
life expectancy. Abdominal approaches (laparoscopic, robotic, or
open) are preferred for younger patients, particularly those with
constipation or associated pelvic floor disorders.
Perineal procedures. For patients with a short (3 to 4 cm)
prolapse, a mucosal sleeve resection with muscularis plication
(Delorme procedure) is ideal (Fig. 52-5). Recurrence rates associ-
ated with this procedure range from 10% to 15%. Mortality
and major morbidity are low, approximately 1% and 14%,
1398 SECTION X Abdomen

Clinical evaluation
including digital rectal
examination

Mild Incontinence Severe Incontinence

Nonoperative
Biofeedback Sphincter Defect?
Treatment

If no improvement,
Fiber, bulking agents,
consider injectables or Yes No
loperamide
SECCA Procedure

Overlapping Sacral Nerve

Sphincteroplasty Stimulator

Artificial Bowel
Sphincter

Fecal Diversion

FIGURE 52-3 Algorithm for treatment of fecal incontinence.

respectively.17 Improvement in incontinence is observed in as

FIGURE 52-4 Full-thickness rectal prolapse. The prolapsed rectal wall
appears as concentric folds circumferentially. With prolapsed hemor-
rhoids, these concentric rings are not seen. (Courtesy Mayo Foundation
for Medical Education and Research, Rochester, Minn.)
many as 69% of patients. Prolapse recurrence is common and
is likely underestimated because this procedure is performed
in patients with limited life expectancies and therefore short
follow-up.
The Altemeier procedure (perineal rectosigmoidectomy)
involves a full-thickness rectal resection, starting just above the
dentate line. The bowel and associated mesentery are resected.
Because the pelvic cavity is entered, care must be taken to avoid
injury to the small bowel. A full-thickness anastomosis is com-
pleted once the prolapsed bowel is resected. For patients with
incontinence, a levatorplasty may be added to the resection. Long-
term results are similar to those described for the Delorme
procedure.18
Abdominal procedures. The abdominal approaches include
rectal mobilization to the pelvic floor, with or without anterior
resection (dependent on the presence of constipation), followed
by rectopexy of the rectum to the sacral promontory, with or
without mesh. Preservation of the lateral stalks is believed to
improve function but results in a greater risk for recurrence. If
resection and anastomosis are being performed, they should be
performed high rather than low in the rectum; this decreases the
risk of anastomotic leak. Once it is completely mobilized, the
rectum is retracted cephalad out of the pelvis and secured with
sutures at the level of the sacral promontory. Resection with rec-
topexy is generally completed in those patients with symptoms of
constipation and is associated with low recurrence rates (0% to
9%). Constipation has been shown to improve in up to 50% of
 CHAPTER 52 Anus 1399

FIGURE 52-5 Delorme Repair. Mucosal sleeve resection is performed and followed by muscle plication,
anastomosing the proximal mucosal resection site to the distal mucosa, proximal to the dentate. (Courtesy
Mayo Foundation for Medical Education and Research, Rochester, Minn.)

patients. Minimally invasive (laparoscopic or robotic assisted) Incontinence and Biofeedback

techniques have been described; however, meta-analyses have yet
to identify a significant improvement in function or recurrence
rates with this approach compared with more traditional open
techniques.16,19 The universal benefits of minimally invasive
approaches (reduced pain, shorter return of bowel function,
decreased length of stay) are still evident.
Rectopexy with mesh fixation, and no resection, avoids the
risks for resection and anastomosis with low recurrence rates. The
mesh can be placed anteriorly or posteriorly. Complications can
result, however, from the presence of a foreign body, and symptoms
of constipation are often aggravated. In one described technique
of anterior mesh fixation, the rectum is minimally mobilized in
an effort to avoid autonomic nerve injury and to decrease the risk
of increased constipation and poor function postoperatively.20 In
this technique, the right lateral peritoneum is incised overlying
the sacral promontory and extended along the rectum toward the
pouch of Douglas. Denonvilliers fascia is incised, opening the
rectovaginal septum. No rectal mobilization or lateral dissection
is performed. A piece of permanent mesh is fixed to the ventral
distal rectum and to the sacral promontory using nonabsorbable
sutures. Care is taken to not place the rectum on traction. The
incised peritoneum can be closed over the mesh, protecting it from
the abdominal viscera. Reviews of this technique have demon-
strated a low morbidity with less than 5% risk of recurrence.21
Incontinence secondary to pudendal nerve damage from chronic
stretching may improve after prolapse repair, except in those
patients in whom the lateral stalks are divided. Although the
majority of patients report excellent satisfaction with results, the
most common reasons for dissatisfaction include persistent con-
stipation or incontinence. The role of biofeedback for treating
persistent postoperative incontinence or for preventing recurrent
prolapse in patients with pelvic floor dysfunction has not been
well established. However, it can be beneficial to some patients,
and it is noninvasive.
Rectocele
Clinical Evaluation
Rectocele, like sigmoidocele and enterocele, is associated with a
posterior vaginal defect. Symptoms include a vaginal bulge or
prolapse of the anterior rectal wall into the vagina, obstructed
defecation, and, in many cases, the need to digitally compress the
vagina or to digitize the rectum or perineum to evacuate. The
cause of rectocele is likely to be multifactorial as it is often associ-
ated with a number of other pelvic floor disorders, including
constipation, paradoxical muscle contraction, and neuropathies
or anatomic disorders from vaginal childbirth.22 A careful recto-
vaginal examination will reveal the size of the defect where the
rectum prolapse extends to the vagina.
1400 SECTION X Abdomen
Defecography (fluoroscopic or magnetic resonance) can dem-
onstrate dynamic information on rectal emptying. A rectocele is
diagnosed if the distance between the line of the anterior border
of the anal canal and the maximal point of bulge of the anterior
rectal wall into the posterior vaginal wall is more than 2 cm. It is
the most useful test for understanding the relevance of the recto-
cele in the defecation process and for identification of additional
pelvic floor abnormalities.
Treatment
Treatment, when the rectocele is symptomatic, initially involves
the optimization of bowel function through diet, fiber supple-
mentation, and good bowel habits. Nonsurgical therapies include
the use of pessaries and biofeedback. A pessary has been associated
with resolution of prolapse symptoms, but many of these patients
are older and have less severe prolapse. Biofeedback has met with
limited success, providing only partial relief in most patients.23
Surgical treatment. Patients should be considered for surgical
correction if the rectocele is larger than 2 cm or the patient has
to digitize the vagina or rectum to assist with defecation. Approach
to surgical repair is through a transvaginal, transperineal, or trans-
anal technique. All repairs can be completed with or without mesh
and may include a levatorplasty. Symptomatic improvement has
been observed in 73% to 79% of properly selected patients. A
newer technique, the stapled transanal rectal resection, has been
investigated for treatment of rectocele associated with internal
rectal prolapse causing obstructed defecation. This procedure uses
two circular staplers, one anteriorly to resect the rectocele and a
second posteriorly to resect the mucosal prolapse. Whereas many
patients note improvement in their symptoms, this has been asso-
ciated with a high rate of fecal urgency and potential for anal
stenosis.24
COMMON BENIGN ANAL DISORDERS
Hemorrhoids
Presentation and Evaluation
Hemorrhoids are normal, vascular tissue within the submucosa
located in the anal canal. They are thought to aid in anal conti-
nence by providing bulk to the anal canal. They are typically
located in the left lateral, right anterior, and right posterior quad-
rants of the canal. Hemorrhoids can be external or internal; the
differentiation is based on physical examination. External hemor-
rhoids are distal to the dentate line and are covered with anoderm;
these may periodically engorge, causing pain and difficulty with
hygiene. Thrombosis of these external hemorrhoids results in
severe pain. Internal hemorrhoids are characterized by bright red,
painless bleeding or prolapse. Internal hemorrhoids are stratified
into four grades that summarize their severity and influence treat-
ment options (Table 52-2). The patient may report dripping or
squirting of blood in the toilet. Occult blood loss resulting in
anemia is rare, and other causes of anemia, such as a more
TABLE 52-2 Internal Hemorrhoids Grading
GRADE SYMPTOMS AND SIGNS
First degree Bleeding; no prolapse
Second degree Prolapse with spontaneous reduction
Third degree Prolapse requiring manual reduction
Fourth degree Prolapsed, cannot be reduced
proximal colorectal lesion, should be investigated. Prolapse of
hemorrhoidal tissue may occur, extending below the dentate line;
many of these patients complain of mucus and fecal leakage and
pruritus.
The physical examination should include inspection of the
anus, digital rectal examination, and anoscopy. Examination
during straining may make prolapse more evident (Fig. 52-6).
Digital examination should focus on anal canal tone and exclusion
of other palpable lesions, especially low rectal or anal canal neo-
plasms. Given that most patients confuse numerous other anorec-
tal symptoms for hemorrhoidal disease, it is imperative that other
anorectal diseases be considered and excluded. Anoscopy is gener-
ally sufficient to arrive at the correct diagnosis, but complete
endoscopic evaluation of the proximal bowel should always be
considered to exclude proximal mucosal disease, particularly neo-
plasia, if the extent of hemorrhoidal disease is incongruent with
the patient’s symptoms, the patient is due for colonoscopic sur-
veillance, or the patient has risk factors for colon cancer, such as
a family history. Depending on the extent of the hemorrhoidal
disease and the patient’s symptoms, treatment can either be non-
surgical or involve formal hemorrhoidectomy.
Treatment
Nonoperative management. Dietary modifications including
fiber supplementation and increasing fluid intake may improve
symptoms of prolapse and bleeding. Bulking of the stool permits
a soft, formed stool for avoidance of excessive straining and
promotion of better bowel habits. Bleeding symptoms are
generally reduced during a period of weeks with the use of fiber
supplementation alone. Patients with prolapse of internal plus
external hemorrhoids will generally benefit from additional
interventions.
First-, second-, and some third-degree internal hemorrhoids
can usually be treated with office procedures. Results are typically
more favorable for lower degree hemorrhoids. Rubber band liga-
tion remains one of the simplest and widely used office-based
procedures for treatment of hemorrhoids. Sedation is not required,
and ligation is carried out through an anoscope, using a ligator
(Fig. 52-7). Although rare, severe perineal sepsis has been reported
after rubber band ligation; thus, patients should be instructed to
be aware of delayed or increasing pain, inability to void, or fever.
Patients with larger hemorrhoids are likely to be better served by
a surgical approach, which is more durable and effective. In
patients undergoing one or more bandings, relief of symptoms
was noted in 80%, with failure predicted in patients who required
four or more bandings.25 Relative contraindications to banding
include immunocompromised patients (chemotherapy, HIV/
AIDS), presence of coagulopathy, and patients taking anticoagula-
tion or antiplatelet medications (excluding aspirin products).
Sclerotherapy involves the injection of a low volume (3 to
5 mL) of a sclerosant (e.g., 3% normal saline) into the internal
hemorrhoid. This technique has good short-term success, but the
hemorrhoidal disease tends to recur in longer follow-up. The
benefit to this technique is its application in patients with bleed-
ing tendency or who are taking anticoagulants that cannot be
stopped. Infrared and laser coagulation involves the use of light
energy to cause coagulation and necrosis, causing fibrosis of the
submucosa in the region of the hemorrhoid.
Surgical treatment. Hemorrhoidectomy is a durable option
and has the best long-term results. It should be considered when-
ever patients fail to respond to more conservative repeated
attempts to treat the disease. Hemorrhoidectomy should
 CHAPTER 52 Anus 1401

A B

C D

E
FIGURE 52-6 Hemorrhoids. A, Thrombosed external. B, Internal, first-degree internal as seen through
anoscope. C, Internal prolapsed and reduced spontaneously, second degree. D, Internal prolapsed, requiring
manual reduction, third degree. E, Inability to be reduced, strangulated, fourth degree. (Courtesy Mayo
Foundation for Medical Education and Research, Rochester, Minn.)

be considered in patients who continue to present with severe
prolapse and require manual reduction (grade III) or cannot be
reduced (grade IV); for those hemorrhoids complicated by stran-
gulation, ulceration, fissure, or fistula; and for those patients who
have symptomatic external hemorrhoids. Patients with throm-
bosed external hemorrhoids may ideally undergo excision in the
office, during the period of maximum pain (generally the first 72
hours). In this case, the thrombosed external hemorrhoid should
be excised, not incised, as this may increase the risk of rethrom-
bosis (Fig. 52-8). Those patients presenting after the period of
maximum pain are best served by supportive measures. In patients
presenting with complex internal or external hemorrhoids, opera-
tive hemorrhoidectomy can be performed as an outpatient
procedure.
Closed (Ferguson) hemorrhoidectomy consists of simultane-
ous excision of internal and external hemorrhoids (Fig. 52-9).
With use of a large anoscopic retractor, such as the Fansler, an
elliptical incision is made encompassing the complete hemor-
rhoidal column. Care must be taken to not excise excessive
amounts of tissue and to ensure that sufficient anoderm is
1402 SECTION X Abdomen
FIGURE 52-7 Rubber Band Ligation. The internal hemorrhoid is identified proximal to the dentate line;
the area of proposed banding should be pinched to test for sensation before banding. The hemorrhoid is
drawn into the ligator by use of either forceps or the suction device of a suction ligator. The band is then
placed. (Courtesy Mayo Foundation for Medical Education and Research, Rochester, Minn.)
FIGURE 52-8 Thrombosed external hemorrhoids should be excised,
not incised, after the area has been infiltrated with local anesthetic.
There is no need to close the excision site. (Courtesy Mayo Foundation
for Medical Education and Research, Rochester, Minn.)
preserved to avoid the complication of anal stenosis. The excision
site is then closed with a continuous absorbable suture. The open
(Milligan-Morgan) hemorrhoidectomy differs in that the excision
site is not closed and left open. Postoperative complications
include urinary retention (in up to 30% of patients), fecal incon-
tinence (2%), infection (1%), delayed hemorrhage (1%), and
stricture (1%). Patients typically recover quickly and are able to
return to work within 1 to 2 weeks.
Other techniques involving the application of ultrasonic (Har-
monic Scalpel; Soma, Bloomfield, CT) or electrical energy (Liga-
Sure; Covidien, Boulder, CO) devices have been applied to the
operative treatment of hemorrhoids. Both methods remove the
excess hemorrhoidal tissue, with minimal lateral thermal injury in
the hope of decreasing postoperative pain and edema. Various
studies have investigated their efficacy and have demonstrated
decreased postoperative pain and analgesic use in these groups
compared with traditional techniques, with similar short-term
success rates.26,27
Stapled hemorrhoidopexy is a technique that results in excision
of a circumferential portion of the lower rectal and upper anal
canal mucosa and submucosa with a circular stapling device (Fig.
52-10). The procedure is completed by first reducing the hemor-
rhoidal tissue into the anal canal. A purse-string suture is placed
3 to 4 cm above the dentate line, ensuring that all the redundant
tissue is incorporated circumferentially. Overly aggressive place-
ment of the sutures may inadvertently incorporate the vaginal wall
anteriorly, resulting in a rectovaginal fistula. In addition, if the
suture is placed too close to the dentate line, it could result in
severe and intractable pain.
Results of stapled hemorrhoidopexy are varied. A meta-analysis
of randomized controlled trials comparing this with conventional
hemorrhoidectomy found that the stapled procedure was safe but
on long-term follow-up was associated with a higher rate of recur-
rence and reoperation (Fig. 52-11).28
Anal Fissures
Presentation and Evaluation
An anal fissure is a linear ulcer usually found in the midline, distal
to the dentate line (Fig. 52-12). These lesions are typically easily
seen by visual inspection of the anal verge with gentle spreading
of the buttocks. Location may vary; most fissures are identified in
the posterior midline, and anterior midline fissures are still more
common that lateral fissures. Other associated findings include a
sentinel tag at the distal portion of the fissure and a hypertrophied
anal papilla proximal to the fissure. Fissures occurring in the
lateral positions should raise the possibility of other associated
diseases, such as Crohn’s disease, tuberculosis, syphilis, HIV/
AIDS, or carcinoma. Anal fissure most often is manifested with
 CHAPTER 52 Anus 1403

A
B

C
FIGURE 52-9 Closed Hemorrhoidectomy. A, An elliptical incision is made surrounding the hemorrhoidal
tissues, and these are excised from distal to proximal. B, Care is taken to preserve the sphincter muscle.
C, The feeding vascular pedicle at the proximal point is sutured and the defect closed with a running absorb-
able suture. In the open technique, this excision site is not closed with suture. (Courtesy Mayo Foundation
for Medical Education and Research, Rochester, Minn.)

A B

C
FIGURE 52-10 Procedure for Prolapsed Hemorrhoids. A, Circumferential grade IV hemorrhoids.
B, The stapling device has an obturator that is placed in the internal canal to aid in reduction of tissue and
placement of purse-string suture in the mucosa above the dentate line. C, Stapling device demonstrating
circumferential excision of anal canal and hemorrhoid mucosa.

excruciating anal pain (because of its location extending onto the
very sensitive anoderm) with defecation and bleeding.
Patients typically describe a preceding episode of constipation.
Digital and anoscopic examination may result in severe pain and
is not necessary if the fissure can be visualized. Examination under
anesthesia, with or without biopsy of the fissure, or endoscopic
examination should be performed if it is refractory to medical
management.
Pathogenesis
The cause of anal fissure is likely to be multifactorial. The passage
of large and hard stools, low-fiber diet, previous anal surgery,
1404 SECTION X Abdomen

Hemorrhoids

External Internal

Thrombosed Nonthrombosed Grade I Grade II Grade III Grade IV

Dietary 1. Diet 1. Diet

>72 hours <72 hours 1. Excision/PPH
modification 2. Banding 2. Banding 1. Excision/PPH
2. Banding
3. Excision

Dietary
Consider Consider excision
modification,
surgical if: large, poor
pain control,
excision hygiene, painful
sitz baths

FIGURE 52-11 Algorithm for the treatment of hemorrhoids. PPH, procedure for prolapse and
hemorrhoids.

considered, including topical nitric oxide (e.g., nitroglycerin),

FIGURE 52-12 Posterior midline anal fissure. (Courtesy Mayo Founda-
tion for Medical Education and Research, Rochester, Minn.)
trauma, and infection may be contributing factors. Increased
resting anal canal pressures and reduced anal blood flow in the
posterior midline have also been postulated as causes. These pos-
sibilities have led to the introduction of several medical approaches.
Treatment
Given that a hypertonic sphincter and large, hard stools may
contribute to anal fissures, most medical therapies are directed to
achieve the goals of relaxation of the anal sphincter without
causing fecal incontinence, passage of soft and formed stools,
and relief of pain. Many pharmacologic agents have been
calcium channel blockers (e.g., diltiazem, nifedipine), and botu-
linum toxin injections.
Nonsurgical therapy is safe and often effective, with limited
side effects, and should be the first-line therapy for anal fissure.
However, a subset of patients may benefit from upfront surgical
intervention, and the treatment should generally be individualized
(Fig. 52-13).
Medical management. Medical therapies for acute anal fis-
sures (those presenting within 6 weeks of symptom onset) are
effective. Medical management includes topical and oral pharma-
cotherapy in addition to diet modification and bulking agents.29
Topical therapy is popular, given its fewer side effects compared
with oral therapies.
Topical nitrates (0.2% to 0.4% nitroglycerin) or calcium
channel blockers (0.2% nifedipine or 2% diltiazem) are com-
monly prescribed. A meta-analysis compared nonsurgical treat-
ments and found that nitroglycerin was significantly better than
placebo (49% versus 36%; P < .0009) in healing anal fissures, but
there was a 50% late recurrence rate. Calcium channel blockers
were equally effective but exhibited fewer side effects.29
Temporary chemodenervation of the internal anal sphincter
can be achieved by injection of botulinum toxin (Botox). This
results in relaxation of the internal anal sphincter and is believed
to promote increased blood flow to the affected anoderm, allow-
ing the fissure to heal. Success is variable, with reported rates of
60% to 80% being achieved. Up to 10% of patients may develop
temporary incontinence to flatus, with rare temporary fecal incon-
tinence. Presently, there is no agreed on standard dose, site of
injection, or number or timing of injections in the administration
of botulinum toxin. Our practice is to inject 20 units of botuli-
num toxin into the internal anal sphincter on each side of the
fissure. In patients who are nonresponders to diet modification
and topical pharmacotherapy, such as topical nitroglycerin or
calcium channel blockers, and who wish to avoid surgery, botu-
linum injection may be a reasonable alternative treatment.
 CHAPTER 52 Anus 1405

Location of
Fissure?

Midline Lateral

Nonoperative Operative Consider, Crohn’s

treatment treatment disease, TB, syphilis,
HIV/AIDS, cancer, etc

Topical nitrates,
Botulinum toxin Lateral internal
calcium channel
injection sphincterotomy
blockers

Failure to heal?

Perform examination
under anesthesia,
consider biopsy and
evaluation of sphincter

Hypotonic Hypertonic
sphincter sphincter

Consider fissurectomy Consider repeated

with advancement flap sphincterotomy

FIGURE 52-13 Algorithm for the treatment of anal fissure.

Surgical management. Patients with severe or chronic fissures Internal sphincter

and those who have failed to respond to medical therapy may
benefit from surgery. Lateral internal sphincterotomy remains the
operation of choice and has been shown to be superior to all other
medical therapies, anal dilation, or fissurectomy.30 Lateral internal
sphincterotomy can be carried out by the closed or open (Fig.
52-14) technique, depending on the surgeon’s preference. There
is no significant difference between these techniques for rate of
healing or rate of incontinence. In terms of the extent of sphinc-
terotomy, studies have investigated whether sphincterotomy to
External sphincter
the level of the dentate line is superior to sphincterotomy to the
fissure apex. Those with sphincterotomy to the level of the dentate FIGURE 52-14 Lateral Internal Sphincterotomy. A large operating
had a higher rate of fissure healing, with no statistical difference anoscope is placed into the anal canal. A small incision is made along
the intersphincteric groove. The mucosa is elevated from the sphincter,
in rate of incontinence.30 The risk of incontinence with sphinc-
and the underlying internal sphincter is elevated off the external sphinc-
terotomy is not negligible. A meta-analysis demonstrated an ter. This is divided either to the level of the dentate line or to the proxi-
overall rate of incontinence of 14%. Incontinence of flatus mal extent of the fissure. (Courtesy Mayo Foundation for Medical
occurred in 9% of patients. Incontinence of liquid or solid stool Education and Research, Rochester, Minn.)
occurred in 2% of all patients.31 It is important to evaluate for
any preexisting incontinence before undertaking surgical inter-
vention so as not to further compromise sphincter function.
1406 SECTION X Abdomen
Supralevator
Ischioanal
Perianal
FIGURE 52-15 Anatomic locations and classification of perianal/perirectal abscesses.
In patients with chronic or recurrent fissure with hypotensive
anal sphincter, fissurectomy with endoanal advancement flap is an
alternative surgical approach.
Anorectal Suppuration
The most common cause of anorectal suppuration is nonspecific
cryptoglandular infection. Other less common although not nec-
essarily rare causes include Crohn’s disease and hidradenitis sup-
purativa. The abscess represents the acute manifestation and the
fistula the chronic sequela.32
Anorectal abscesses generally originate in the intersphincteric
space. The infectious process may remain isolated within the
intersphincteric space, or it may extend vertically upward or
downward, horizontally, or circumferentially. Abscesses are gener-
ally classified into perianal, ischiorectal, intersphincteric, and
supralevator (Fig. 52-15).
Clinical Presentations of Various Types of Abscesses
An intersphincteric abscess is limited to the primary site of origin.
These abscesses may be asymptomatic; however, classically, the
patient will present with pain out of proportion to physical exami-
nation findings. Downward extension results in a perianal abscess,
exhibited by a tender swelling at the anal margin.
Upward extension may result in a supralevator abscess. These
abscesses may be difficult to diagnose because the patient may
complain of vague pelvic or anorectal discomfort, and there is a
lack of external manifestations. Supralevator abscesses may also
result from pelvic infectious processes, such as diverticulitis. In
rare instances, these may extend downward into the ischiorectal
or intersphincteric space.
Horizontal spread may traverse the internal sphincter inter-
nally into the anal canal or externally across the external sphincter
into the ischiorectal fossa, forming an ischiorectal abscess. These
abscesses may become large as external physical examination find-
ings may be subtle, and the infection may initially or insufficiently
Intersphincteric
Submucosal
be treated with only antibiotics, allowing expansion to the roof of
the fossa or through the pelvic floor into the supralevator space.
Patients may complain of pain and fever before an obvious fluctu-
ant mass is detected. A complex horseshoe abscess may result if
the infectious process spreads circumferentially from one side to
the other of the intersphincteric space, supralevator space, or
ischiorectal fossa.
Treatment
Acute anorectal abscesses should be incised and drained at the
time of diagnosis. If the abscess is superficial and simple, this can
most often be done under local anesthesia in the office setting. In
the presence of immunosuppression (AIDS, diabetes mellitus,
chemotherapy), systemic symptoms (fever), or more complicated
large abscesses, definitive treatment should be undertaken in the
operating room under anesthesia. The location and method of
drainage are determined by the location of the abscess.
For a perianal abscess, a simple skin incision is adequate. The
incision should be kept as close as possible to the anal verge,
without injury to the underlying sphincter muscle, to minimize
the length of any potential fistula that may form. Intersphincteric
abscesses are drained into the anal canal by dividing the internal
anal sphincter at the level of the abscess. The cause of a supraleva-
tor abscess must be determined before drainage; supralevator
abscesses, if not the result of upward extension of an ischiorectal
abscess, should be drained into the lower rectum and upper anal
canal or transabdominally if the result of an abdominal source.
Ischiorectal abscesses should be drained through an appropriate
incision through the skin and subcutaneous tissue overlying the
infected space (Fig. 52-16). If the abscess is deep within the fossa,
needle localization of the purulent material may help guide the
surgeon for the optimal location of the skin incision. Care should
be taken to avoid vigorous débridement of the abscess cavity as
there exists a low risk of injury to the inferior rectal branch of the
internal pudendal nerve. Abscesses that are not adequately treated

NO!
NO!
FIGURE 52-16 Sites of Drainage for Abscesses. It is important
that the location of the incision is appropriate to avoid creation of
extrasphincteric or suprasphincteric fistulas. (From Parks AG, Gordon
PH, Hardcastle JD: A classification of fistula-in-ano. Br J Surg 63:1–12,
1976.)
Counterdrainage
Counterdrainage
External
sphincter muscle
Dentate line Internal sphincter
muscle (cut)
FIGURE 52-17 Modified Hanley procedure for draining horseshoe
abscesses. (From Gordon PH: Anorectal abscesses and fistula-in-ano.
In Gordon PH, Nivatvongs S, editors: Principles and practice of surgery
for the colon, rectum, and anus, ed 2, St Louis, 1992, Quality Medical,
p 232.)
may result in a devastating and sometimes lethal necrotizing infec-
tion of the perineum. In these situations, wide débridement of
infectious and necrotic tissue is necessary, often combined with
broad-spectrum antibiotics.
Recurrent abscesses may result if there has been inadequate
drainage, presence of a fistula, or underlying immunosuppression.
In this setting, examination under anesthesia may be warranted
after imaging studies of the abdomen or pelvis (e.g., MRI or
computed tomography [CT]) have been obtained. Horseshoe
abscesses result from circumferential spread of the infectious
process. A modified Hanley procedure that drains the deep post-
anal space and lateral extensions of the abscess should be per-
formed. A posterior midline incision is made extending from the
subcutaneous portion of the external sphincter over the abscess to
the tip of the coccyx, separating the superficial external sphincter
and unroofing the deep postanal space and its lateral extension
(Fig. 52-17). Lateral incisions can be made and setons placed to
drain any anterior extensions of the abscess.
CHAPTER 52 Anus 1407
BOX 52-1 Parks Classification of Fistula
in Ano
Intersphincteric: The fistula is confined to the intersphincteric plane.
Trans-sphincteric: The fistula traverses the external sphincter, communicating
with the ischiorectal fossa.
Suprasphincteric: The fistula extends cephalad over the external sphincter and
perforates the levator ani.
Extrasphincteric: The fistula extends from the rectum to the perianal skin,
external to the sphincter apparatus.
From Parks AG, Gordon PH, Hardcastle JD: A classification of
fistula-in-ano. Br J Surg 63:1–12, 1976.
45% 30%
Type 1 Type 2
20% 5%
Type 3 Type 4
FIGURE 52-18 Fistulas and their relationship to the sphincter appara-
tus: type 1, intersphincteric; type 2, trans-sphincteric; type 3, supra-
sphincteric; type 4, extrasphincteric. (From Parks AG, Gordon PH,
Hardcastle JD: A classification of fistula-in-ano. Br J Surg 63:1–12,
1976.)
Antibiotics and abscess culture have a limited role in the treat-
ment of uncomplicated anal suppuration but should be consid-
ered in patients with immunosuppression, systemic illness, or a
history of prolonged and frequent antibiotic use.
Ancillary imaging studies (CT scan, MRI, endoanal ultra-
sound, fistulography) may have some utility in select cases, but
treatment is generally based on clinical findings. These studies
may be helpful to guide management in patients with complex or
recurrent disease.
Fistula in Ano
Fistula in ano can develop in approximately 40% of patients
during the acute phase of sepsis or even be discovered within 6
months of initial therapy. Fistulas are categorized by the Parks
classification (Box 52-1 and Fig. 52-18).
Clinical Presentations
The most common anal fistula is an intersphincteric fistula. Most
frequently, the fistula traverses directly downward to the anal
margin. In some cases, however, the track may travel upward in
the rectal wall (high blind track), with or without a perineal
opening.
1408 SECTION X Abdomen
In trans-sphincteric fistulas, the track travels across the external
sphincter and into the ischiorectal fossa, terminating at the peri-
neal skin. If it passes through the muscle at a low level, it is
uncomplicated and treatment is relatively simple; however, if it
involves the upper two thirds of the sphincter, repair is more
complicated. Care must be taken in probing these fistulas to avoid
inadvertent puncture of the lower rectum, creating an iatrogenic
extrasphincteric fistula. Suprasphincteric fistulas are rare and can
be difficult to treat. With these fistulas, the trajectory is above all
the muscles of importance to continence. Furthermore, the fistula
may have an additional extension into the pelvis, parallel to the
rectum (high blind track).
Finally, an extrasphincteric fistula is also rare and often results
from iatrogenic injury. It travels from the perineal skin to the
rectal wall above the levator ani. The track is completely outside
the sphincter apparatus. Treatment often involves the need for a
colostomy.
Treatment
Adequate and appropriate treatment is dependent on correct clas-
sification of the fistula and identification of the internal and
external openings, the course of the track, and the amount of
sphincter muscle involved. Surgical treatment remains the primary
modality of treatment for noninflammatory bowel disease–related
fistulas. Examination under anesthesia allows a complete anorectal
examination and appropriate classification of the disease. Digital
examination may reveal a palpable nodule in the wall of the anal
canal, an indication of the primary opening. A probe can be
placed gently from the external skin opening to the internal anal
canal opening.
Management of simple fistulas (those with minimal involve-
ment of the sphincter complex—low trans-sphincteric fistulas,
intersphincteric fistulas) is often done by laying the track open by
fistulotomy. Depending on the length or depth of the track, it
may be marsupialized to promote healing. Recurrence and incon-
tinence rates are generally low but heavily dependent on operator
judgment and experience. Goodsall’s rule (Fig. 52-19) is useful
for predicting the anatomy of simple fistulas. If direct probing
cannot identify the internal opening, injection of a mixture of
methylene blue and hydrogen peroxide may help identify it.
Complex fistulas often require more than simple fistulotomy
to resolve. A variety of techniques exist for their treatment, which
again is dependent on the classification and accurate identification
of the internal opening. For fistulas that involve more than 30%
of the sphincter, those distal to the dentate line, or high trans-
sphincteric fistulas, a draining seton (suture or elastic) is placed
loosely through the track to facilitate drainage of any sepsis and
to preserve the sphincter. This may be converted to a cutting
seton, which is periodically tightened at regular intervals to slowly
cut through the involved sphincter muscle and to promote fibrosis
to avoid disruption of the sphincter and the resulting inconti-
nence. Success rates are variable and have ranged from 60% to
100%; many patients ultimately experience some level of incon-
tinence, more often to flatus than to liquid or solids.
Other methods of fistula closure in the setting of complex
disease include the use of biologic material to promote the closure
of fistulas without division of any sphincter muscle (fibrin glue or
porcine-derived fistula plug). Both products promote healing of
the track by providing an extracellular matrix that serves as a scaf-
folding, allowing ingrowth of host tissue for incorporation and
remodeling. Fibrin glue contains human pooled plasma fibrino-
gen and thrombin. The fibrin glue is injected into the anal fistula
Posterior
Transverse
anal
line
A
B Anterior
Long. ant. fistula
FIGURE 52-19 Goodsall’s Rule. The predicted relationship of inter-
nal and external fistula orifices is depicted. The internal opening is
marked A. The long anterior fistula is often an exception to the rule.
(From Schrock TR: Benign and malignant disease of the anorectum. In
Fromm O, editor: Gastrointestinal surgery, New York, 1985, Churchill
Livingstone, p 612.)
track starting from the internal opening and filling the track from
internal to external. A comprehensive review of the literature has
reported that successful fibrin glue injection results are varied and
range from 14% to 60%.32
A bioprosthetic fistula plug is also available to serve as a matrix
for tissue ingrowth and to obliterate the fistula track. It is inserted
into the fistula and then secured at the internal opening. The
external opening is widened and left open to allow drainage. Over
time, tissue will grow into the plug and replace the matrix, oblit-
erating the fistula track. There are limited data about the success
of this approach, but its low morbidity and low risk make it an
attractive option in patients with complex fistulas.
High fistulas or other persistent fistulas may be treated by a
sliding advancement flap made of mucosa, submucosa, and cir-
cular muscle to cover the internal opening. Success rates are again
variable, ranging from 13% to 56%, and some patients still report
changes in continence, even though the sphincter is not
violated.32
Finally, a newer technique, ligation of the intersphincteric
fistula track, has been developed (Fig. 52-20). This involves dis-
section in the intersphincteric plane for identification of the fistula
track and ligation of it to obliterate the communication with the
anal canal. This approach limits risk to the sphincter mechanism
and has shown promise with success rates of 60% to 94%.32
Pilonidal Disease
Pilonidal infections and chronic pilonidal sinuses are usually
found in the midline of the sacrococcygeal region of young hirsute
men. The incidence of disease is approximately 26 per 100,000
population. The presence of hair in the gluteal cleft seems to play
a central role in the pathogenesis of this disease. This is consistent
with the observation that pilonidal disease rarely occurs in those
with less body hair. Other risk factors include obesity, local
trauma, sedentary lifestyle, deep natal cleft, and family history.
Diagnosis is generally a clinical one; patients may present with a

A B
FIGURE 52-20 Ligation of intersphincteric fistula track. This procedure preserves the sphincter and mini-
mizes any risk of incontinence.
chronic inflammation or a sinus with persistent drainage. Acutely,
there may be an abscess or multiple complex subcutaneous tracks.
Treatment
Acute management. Acute presentation often involves a painful
fluctuant abscess or a draining infected sinus. Both are generally
managed in the office with simple treatments; more definitive
surgical procedures are reserved for patients who develop a recur-
rence. Abscesses can be drained with use of local anesthesia. The
abscess extends to either side of the midline cleft, and incision and
drainage should be performed down to the subcutaneous tissues,
off the midline to allow complete drainage and to lower wound
complications. The surrounding skin should be shaved weekly to
prevent the reintroduction of hair. Laser hair removal can also be
considered to accomplish effective, long-lasting hair removal.33
Recurrent or chronic disease develops in 10% to 15% of patients
and may eventually require more extensive surgical management.
Surgical management. The simplest approach for chronic pilo-
nidal disease is midline excision and primary closure. An alternative
approach involves marsupialization, whereby the areas of midline
pits and sinuses are excised and the wound is reduced in size by
suturing the wound edges to the fibrous base of the wound. This
has been shown to decrease time for complete wound healing but
requires meticulous wound care until the incision has healed. A
systematic review compared healing by primary versus secondary
intention and found that recurrence rates were the lowest with
healing by secondary intention but faster with primary closure.
There was no difference in surgical site infections. In addition,
when closure was performed, those who underwent off-midline
closure had better outcomes than those closed in the midline.34
In patients with recurrent disease who have undergone mul-
tiple prior surgical interventions, flap-based procedures, such as a
V-Y advancement flap, rhomboid flap, Z-plasty, Bascom cleft lip
repair, or Karydakis flap, may be beneficial.
Limited comparative studies of the various flap-based proce-
dures exist. A rhomboid fasciocutaneous flap that serves to flatten
the gluteal cleft has been shown to have lower recurrence rates
compared with a V-Y advancement flap. A Karydakis flap and
rhomboid flap are essentially equivalent in published series. In
most practices, the complex flap closures are reserved for patients
with refractory disease for whom previous simple measures have
failed.
CHAPTER 52 Anus 1409
C
LESS COMMON BENIGN ANAL DISORDERS
Rectovaginal Fistula
A rectovaginal or anovaginal fistula is a communication between
the rectum or anus and vagina. Rectovaginal fistulas may be con-
genital or acquired through trauma, inflammatory bowel disease,
irradiation, neoplasia, infection, or other rare causes. The most
common cause is obstetric-related trauma from unsuccessful
repair of third- or fourth-degree perineal laceration or pressure
necrosis secondary to prolonged labor. Patients complain of
passage of gas, feces, mucus, or blood through the vagina. In the
setting of traumatic injuries, anal manometry and endoanal ultra-
sound may demonstrate the severity of the underlying sphincter
defect and help guide the surgical approach.
Surgical Repair
Before embarking on surgical repair of rectovaginal fistulas, it is
important to consider the underlying disease, size of the fistula,
presence of active inflammation, and severity of symptoms. Small,
low-output fistulas may close spontaneously. However, those asso-
ciated with inflammatory bowel disease, radiation therapy, or
underlying infection may resolve only with medical or surgical
therapy. High rectovaginal fistulas (upper third of the vagina)
generally require a transabdominal approach, whereas low recto-
vaginal fistulas can be repaired transvaginally, transrectally, or
transperineally.
An endorectal advancement flap, sphincteroplasty, and trans-
perineal procedures can all be employed for the repair of a truly
low-lying fistula (anovaginal fistula).35 An endorectal advancement
flap consists of a flap of rectal mucosa, submucosa, and underlying
internal anal sphincter muscle that is advanced to cover the primary
opening in the rectum or anus. The flap is best suited for the
initial repair or for patients without an underlying sphincter defect.
The transperineal approach (perineoproctotomy) converts the rec-
tovaginal fistula into a fourth-degree tear. The tissues are then
reapproximated in a normal anatomic fashion with the internal,
external, and levator muscles in discrete layers. This should be
reserved for patients with preexisting sphincter defects for whom
other more conservative approaches have failed.35
For high rectovaginal fistulas, a transabdominal approach is
often required. This approach requires mobilization of the recto-
vaginal septum, division of the fistula, and subsequent closure of
1410 SECTION X Abdomen
the rectal and vaginal defects. Placement of a viable pedicle of
tissue between the two structures may help augment the repair
and promote healing. In some cases, no rectal resection is neces-
sary. In the setting of severe prior radiation exposure, inflamma-
tory bowel disease, or neoplasia, rectal excision is required. A low
anterior resection or coloanal anastomosis may be possible, pre-
serving the sphincters and allowing normal evacuation.35
Sexually Transmitted Diseases and Acquired
Immunodeficiency Syndrome
Multiple partners and anal receptive intercourse increase the risk
for transmission of sexually transmitted disease (STD), which can
be bacterial, viral, or parasitic in origin (Box 52-2). The site and
manner of infection dictate the symptoms of presentation.
Clinical Presentation
Patients with STDs may present with varied but often overlapping
symptoms that may include pruritus, bloody or purulent rectal
discharge, tenesmus, diarrhea, and fever. Proctoscopic examina-
tion may demonstrate proctitis, mucopurulent discharge, ulcer-
ation, and abscesses. Diagnosis is aided by clinical findings based
on physical examination, endoscopy, and cultures.
Patients infected with molluscum contagiosum develop pain-
less flattened, round, and umbilicated dermal lesions. Endoscopy
often demonstrates vesicles, ulcers, and friability. Similar endo-
scopic findings may be seen in herpes or anal warts. The diagnosis
is obtained by cultures, scrapings, or biopsy. Treatment for herpes
infection is with the antiviral acyclovir. Other viral lesions are
generally treated by destruction or excision.36
Parasitic STDs have a greater incidence of systemic symptoms,
such as fever, abdominal cramping, and bloody diarrhea. Ent-
amoeba histolytica characteristically causes hourglass-shaped ulcer-
ations. More diffuse ulceration is seen with Giardia lamblia.
Diagnosis is based on biopsy specimens or scrapings and specific
stains.
AIDS. Anorectal disease affects approximately one third of
patients with HIV infection and can cause significant morbidity.
Pain and bleeding are the most frequent presenting complaints.
The most common findings in this population of patients include
abscesses, fistulas, infectious proctitis, condyloma, anal intraepi-
thelial neoplasia (AIN), and cancer. Herpes, cytomegalovirus, and
Chlamydia spp. are the most typical infectious agents. Examina-
tion should include inspection of the perianal skin, palpation of
BOX 52-2 Sexually Transmitted
Organisms
Bacterial Parasitic
Neisseria gonorrhoeae Entamoeba histolytica
Treponema pallidum Giardia lamblia
Haemophilus ducreyi Cryptococcus spp.
Chlamydia spp. Isospora belli
Shigella flexneri
Campylobacter spp.
Viral
Herpes simplex
Human papillomavirus
Molluscum contagiosum
the lymph nodes (may be enlarged in syphilis or herpes simplex
infection), and visualization of the anal canal. Mucopurulent anal
discharge that is associated with pain may be secondary to gonor-
rhea, chlamydia, or herpes. One should also consider obtaining a
Gram stain or culture as indicated.
Neoplastic disorders are more prevalent in men who have sex
with men, and this is even greater in those who are HIV positive.
These disorders include condyloma, AIN, epidermoid carcinoma,
and Kaposi sarcoma.37 Treatment of anal condyloma is not differ-
ent on the basis of HIV positivity status; however, the recurrence
rates and conversion to malignancy are higher for those who are
HIV positive. In those patients with squamous cell carcinoma,
whether invasive or in situ disease, CD4 count and treatment with
antiretroviral therapy are key adjuncts to success with local exci-
sion or chemoradiotherapy.
Anal condyloma and human papillomavirus (HPV) infections
are considered STDs. However, these infections are discussed
later, with other neoplastic disorders.
Hidradenitis Suppurativa
Hidradenitis suppurativa primarily involves the intertriginous
skin regions of the axilla, inframammary, groin, perianal, and
perineal areas. Hidradenitis has traditionally been considered the
result of occlusion of apocrine glands by keratotic debris. This
occlusion leads to bacterial proliferation, suppuration, and spread
of inflammation to surrounding subcutaneous tissues. Subcutane-
ous tracks and pits develop; the infected tissues ultimately become
fibrotic and thickened. Many bacterial organisms have been iden-
tified, including Streptococcus milleri, Staphylococcus aureus, Staph-
ylococcus epidermidis, and Staphylococcus hominis.
Clinical Presentation
Perianal hidradenitis can be manifested as early acute or late
chronic, severe form; the condition most commonly is first mani-
fested with an inflammatory, painful nodule. The nodules may
spontaneously regress and then recur or rupture and drain. Fistu-
las or sinus tracks may ultimately develop that intermittently
release purulent debris. After repeated interventions, dense scar-
ring and fibrosis may develop. The appearance is classic (Fig.
52-21). In rare cases, squamous malignant neoplasms may also be
FIGURE 52-21 Hidradenitis suppurativa. (Courtesy Mayo Foundation
for Medical Education and Research, Rochester, Minn.)
 CHAPTER 52 Anus 1411

A B
FIGURE 52-22 Perineal Crohn’s disease. A, Crohn’s disease fissures demonstrate deep and shaggy edges,
significant ulceration, and abundant granulation tissue. B, Perianal Crohn’s disease can be complicated by
multiple fistulas and abscesses, complicating treatment and requiring combination surgical and medical
therapy. (Courtesy Mayo Foundation for Medical Education and Research, Rochester, Minn.)

present. To ensure appropriate treatment, hidradenitis must be
differentiated from Crohn’s disease–related fistulas or cryptoglan-
dular infection. Hidradenitis fistulas arise distal to the dentate line
in the anal skin, differentiating them from cryptoglandular fistu-
las, which communicate with the dentate line, and Crohn’s
disease, which may have tracks to the anorectum proximal to the
dentate line.
Treatment
The general goals in treatment are to reduce the extent of disease,
to remove chronic infection/sinuses, and to limit scar formation.
To exclude the possibility of malignancy, liberal biopsies should
be performed. For acute disease, the mainstay of therapy remains
incision and drainage. In an effort to reduce the risk of recurrence,
medical management includes the use of topical clindamycin,
which may have additional benefit in treating secondary infec-
tions. Oral antibiotic treatment, with clindamycin, minocycline,
doxycycline, or amoxicillin–clavulanic acid (Augmentin), may
also have some benefit in acute flares of mild disease.
Sinus tracks that are chronic and superficial can be unroofed
or laid open. Avoiding curettage of the epithelized track may
facilitate rapid healing and minimize scarring. Extensive, deep and
fibrotic disease may require wide excision, although this is associ-
ated with recurrence rates of approximately 50%. Wide excision
may lead to large wounds, which can be managed with delayed
healing and dressing changes, flaps, or skin grafts. Involvement of
a plastic surgeon to assist with closure or wound management is
sometimes necessary. Healing by secondary intention requires
significant wound care, and it can often take weeks to months for
complete healing to be accomplished.
Crohn’s Disease of the Anorectum
Clinical Presentation
Perianal complications of Crohn’s disease can affect approximately
40% of patients. In these patients, the presence of perianal disease
is often predicted by the location and activity of more proximal
gastrointestinal disease. The majority (>90%) of patients with
rectal disease will have perianal disease. This is compared with
approximately 40% of patients with isolated colonic disease,
whereas up to 5% of patients with perianal disease will have no
disease in the proximal gastrointestinal tract.38 Those patients
younger than 40 years and nonwhites are also more likely to
present with perianal disease. Perianal Crohn’s disease may include
fissures, fistulas, and abscesses. Patients may have a varied presen-
tation including pain, swelling, drainage, incontinence, fever, and
bleeding (Fig. 52-22).
Evaluation and Treatment
In evaluating patients with Crohn’s disease, one must determine
if the anal pathologic change is secondary to Crohn’s disease or,
especially in the setting of quiescent disease, a common anorectal
problem unrelated to the underlying disease. It is important that
the examination be complete (including endoscopy). Obtaining
a thorough examination may require anesthesia for diagnostic and
therapeutic purposes, particularly in the patient experiencing pain
that limits an office examination. In the absence of rectal or anal
Crohn’s disease, common anorectal conditions (fissure, fistula, or
abscess) may be treated by standard approaches. The surgeon must
be cautious when treating a Crohn’s disease patient with anorectal
problems; however, undertreatment of symptomatic conditions
should also be avoided. The disease activity can be objectively
measured by the Perianal Crohn’s Disease Activity Index, a scoring
system that evaluates the fistulizing disease in five categories:
discharge, pain, restriction of sexual activity, type of perianal
disease, and degree of induration (Table 52-3).39
Crohn’s disease fissures often are multiple, off-midline lesions.
Although conservative measures, such as sitz baths, stool softeners,
oral analgesics, and nifedipine ointment, may be tried, patients
will often require medical treatment to control the disease.
Sphincterotomy and fissurectomy should be avoided when Crohn’s
disease is present. In the case of anorectal suppuration caused by
Crohn’s disease, a combination of surgical and medical therapy is
advised.40,41 Abscesses should be drained, and the anus and rectum
should be completely evaluated for fistulas or other inflammation.
For fistulas, treatment should be based on the symptoms and
relationship of the fistula to the sphincter complex. Simple, low
to mid fistulas may be treated with fistulotomy or staged with
seton placement. In addition, antibiotics may complement the
treatment. Complex fistulizing disease or fistulizing disease with
extensive proctitis requires drainage with setons and initiation of
immunomodulator therapy, such as infliximab or azathioprine
and mercaptopurine. In severe cases, diversion or even proctec-
tomy is sometimes required. In patients treated with azathioprine
and mercaptopurine, a complete response is seen in nearly 40%
1412 SECTION X Abdomen

Clinical Evaluation
TABLE 52-3 Perianal Crohn’s Disease
Initial assessment should begin with a complete history and physi-
Activity Index
cal examination. The extent and duration of the symptoms, such
ELEMENTS SCORE as bleeding, pain, changes in continence, and weight loss, should
Discharge be documented. The perianal area should be closely examined for
None 0 changes in the normal skin. Digital rectal examination establishes
Minimal mucous 1 tumor location, fixation, and function of the sphincter muscles.
Moderate mucous or purulent 2 Anoscopy or rigid proctosigmoidoscopy can help determine the
Substantial 3 size and relation of the tumor to the dentate line, anal verge, or
Gross fecal soiling 4 anorectal ring. One should also examine for any pathologic
lymphadenopathy. Staging studies investigating for distant disease
Pain or Restriction of Activity include chest, abdominal, and pelvic CT. Pelvic MRI for staging
None 0 purposes is encouraged to determine exact tumor size and nodal
Mild discomfort, no restriction 1 involvement. A positron emission tomography scan should be
Moderate discomfort, some limitation 2 considered in those with T2 or greater tumors or those with posi-
Marked discomfort, marked limitation 3 tive nodal involvement.
Severe pain, severe limitation 4
Anal Margin (Perianal) Tumors
Restriction of Sexual Activity Neoplastic lesions that exclusively involve the skin of the anal
None 0 margin are treated like skin lesions at any other skin site unless
Slight restriction 1 treatment would compromise the sphincter mechanism.
Moderate restriction 2
Marked restriction 3 Condyloma Acuminatum
Unable to engage in sexual activity 4 Condyloma acuminatum (anogenital wart) is caused by HPV. It
remains the most common virally transmitted STD in the United
Type of Perianal Disease States. Viral subtypes, such as HPV-6 and HPV-11, are found more
None 0 commonly in benign warts, whereas HPV-16 and HPV-18 tend to
Anal fissure or mucosal tear 1 be aggressive and are associated with dysplasia and progression to
<3 Perianal fistulas 2 cancer. Acquiring condyloma is directly related to sexual activity
>3 Perianal fistulas 3 and is more common in immunocompromised individuals.
Sphincter ulceration 4 Clinical presentation. Patients may complain of pruritus,
bleeding, pain, discharge, or a palpable mass. Examination typi-
Degree of Induration cally reveals pinkish warts of varying sizes that may coalesce to
None 0 form a mass (Fig. 52-23A). Anoscopy may reveal disease within
Minimal 1 the anal canal. The diagnosis is generally made by direct inspec-
Moderate 2 tion of the perineum and genitals; anoscopy and proctosigmoid-
Substantial 3 oscopy must be performed as the disease often extends intra-anally,
Gross fluctuance/abscess 4 and some patients have disease limited to the anal canal. Histology
From Irvine EJ: Usual therapy improves perianal Crohn’s disease as confirms the diagnosis. High-resolution anoscopy with or without
measured by a new disease activity index. McMaster IBD Study 5% acetic acid may improve detection of disease.
Group. J Clin Gastroenterol 20:27–32, 1995. Diagnostic evaluation. The most important factors contribut-
ing to the successful management of condylomata acuminata are
the extent of disease, presence of underlying contributing condi-
of patients. The use of maintenance infliximab has been associated tions, and risk of malignancy. Condylomas are often found in
with decreased rate of recurrence, fewer hospitalizations, and other sites around the perineum or genitals, and these areas should
fewer procedures performed for fistulizing disease. be thoroughly evaluated; concurrent treatment of nonanal sites
helps limit the risk of relapse. In addition, sexual partners are at
risk for contracting the disease and should also undergo evaluation
and treatment.
NEOPLASTIC DISORDERS Patients with condylomata acuminata should be screened for
There is a wide spectrum of benign and malignant neoplastic any immunologic compromise. This may be secondary to HIV
lesions of the anus. Defining the anatomy of the anal canal is infection, medication related (transplant patients), or oncologic
important in differentiating the appropriate treatment modalities related. Immunosuppression increases the risk of malignancy in
for various lesions. patients with condyloma or undergoing treatment for condyloma,
The American Joint Commission on Cancer (AJCC) has and these patients should be closely observed. HIV testing should
defined three anatomic regions in which anal or perianal squa- be considered if it has not previously been conducted, and tissue
mous cell cancer may be manifested: anal canal, perianal, or skin. samples should be obtained for HPV and AIN evaluation. High-
Anal canal lesions are defined as those lesions that cannot be resolution anoscopy can help locate sites of high-risk lesions.43
visualized or are incompletely visualized by gentle traction of the Treatment. Treatment approaches involve physical or chemical
buttocks. Perianal lesions are completely visible and arise within destruction, immunologic therapies, and surgery. Despite the
5 cm of the anal opening when the buttocks are gently spread. various multimodal therapies, recurrence rates of 30% to 70%
Skin lesions are outside of the 5-cm radius.42 have been reported. Chemical agents include podophyllin,

A B
FIGURE 52-23 A, Perianal condyloma acuminatum. B, Sharp excision of condyloma. (Courtesy Mayo Foun-
dation for Medical Education and Research, Rochester, Minn.)
trichloroacetic acid, and 5-fluorouracil (5-FU). Podophyllin is
cytotoxic and can be irritating to normal skin. Its use should be
limited to those patients with minimal extra-anal disease. Care
must be taken to avoid systemic toxicity. Although it is simple
and inexpensive, the results are often disappointing, with high
recurrence rates reported. Trichloroacetic acid can be used peri-
anally and intra-anally and is less irritating than podophyllin. The
recurrence rate with both agents is much higher than with surgical
excision. Another topical immune therapy is imiquimod, which
creates an inflammatory response that destroys the condyloma.
Current recommendations are for the topical application of
imiquimod 5% cream three times weekly (for 6 to 10 hours). This
can be used as a primary treatment or as an adjunct after initial
resection or destruction of disease. Although it is not currently
approved for intra-anal use, some centers have applied this medi-
cation in this manner with similar results.
Destruction of the condyloma by electrocauterization with a
needle tip cautery is effective and used extensively, and it is often
combined with excision of larger lesions. Carbon dioxide laser is
also an effective tool, but the cost is higher without evidence of
increased efficacy. Excision at the base with small scissors can also
be performed; it is precise, minimizes destruction of the skin, and
can be used on larger lesions (Fig. 52-23B). General or regional
anesthesia is often necessary. All options, however, are associated
with a significant chance of recurrence. Thus, close follow-up of
patients is recommended.
Anal Intraepithelial Neoplasia
There exists variability and confusion in regard to the terminology
describing anal squamous intraepithelial lesions. The AJCC clas-
sifies these lesions as low-grade anal intraepithelial lesions (LSIL)
to include AIN I (low grade) and high-grade anal intraepithelial
lesions (HSIL) to include AIN II-III (moderate or high grade).
The term Bowen disease, which has been ascribed variably to anal
squamous cell carcinoma in situ, AIN II, and AIN III, further
complicates the discussion as there is little if any biologic differ-
ence between these entities. The term Bowen disease should be
abandoned for the more universal term HSIL.
Treatment. Treatment should be individualized to the patient
and disease process. Historically, the recommendation has been
for anal mapping and treatment according to the extent and loca-
tion of the disease. In situ lesions that are unifocal can be managed
with local excision to achieve negative margins. In some cases,
CHAPTER 52 Anus 1413
wounds can be of sufficient size to require wound closure with
more complex means other than primary approximation; various
flap techniques have been previously described. Whereas this
would provide excellent local control, recurrence rates remained
high.44 More frequently and in a less morbid approach, high-
resolution anoscopy with acetic acid to aid in lesion visualization
has allowed directed destruction. Perianal disease can be treated
with topical application of imiquimod, as described earlier, which
has been associated with complete clinical and histologic clearance
of AIN in some reports. Multifocal anal canal disease is usually
focally ablated. All patients should be closely observed for recur-
rence and for invasive disease. Interval of follow-up should be
determined on the basis of grade of dysplasia, extent of disease,
and presence of immunosuppression. Patients who are immuno-
compromised may have an increased risk for development of
invasive disease.
Verrucous Carcinoma
Verrucous carcinoma is also referred to as giant condyloma acu-
minatum or Buschke-Löwenstein tumor. These large lesions are
slow growing and may be complicated by fistulization, infection,
or malignant transformation. Wide local excision is recom-
mended; in rare cases when all disease cannot be removed, abdom-
inal perineal resection (APR) is necessary. Those tumors that
progress to invasive squamous cell carcinoma are associated with
a poor prognosis. However, some may respond favorably to com-
bined chemoradiotherapy.
Squamous Cell Carcinoma
Squamous cell carcinoma of the anal canal is five times more
common than perianal squamous cell carcinoma. Cancers of the
anal margin (those occurring within 5 cm of the squamous muco-
cutaneous junction) that are small (T1) and well differentiated
may be treated by wide local excision. Any anal margin lesions
that are greater than T1 or node positive should be treated like
primary squamous cell cancers of the anal canal with definitive
chemoradiation (see later). Patients with stage IV metastatic
disease may benefit from cisplatin-based chemotherapy with or
without radiation therapy.
Paget Disease
Extramammary Paget disease of the anus is a rare intraepithelial
adenocarcinoma. Paget disease more commonly is manifested in
1414 SECTION X Abdomen

FIGURE 52-25 Basal cell carcinoma of anal margin. (Courtesy Mayo

Foundation for Medical Education and Research, Rochester, Minn.)

FIGURE 52-24 Perianal Paget disease. (Courtesy Mayo Foundation for

Medical Education and Research, Rochester, Minn.)

older patients (usually in the seventh decade of life) and is often

seen in areas of high density of apocrine sweat glands.45 Over time,
these can develop into an invasive cancer of the underlying apocrine
glands. The most common presenting symptom is intractable
itching, and some patients with long-standing symptoms are mis-
diagnosed with pruritus ani. The typical appearance is an eczema-
tous, well-demarcated plaque with occasional ulceration and
scaling (Fig. 52-24). Histology demonstrates the presence of peri-
odic acid–Schiff–positive (because of significant mucin) Paget cells
(large, vacuolated cytoplasm with eccentric nuclei), confirming the
diagnosis. Extramammary Paget disease is associated with an
underlying invasive carcinoma in 30% to 45% of patients. However,
visceral malignant neoplasms may be seen in up to 50% of patients.
Treatment is based on the local extent of the disease and pres-
ence or absence of invasion. Limited, noninvasive disease is best
managed by wide local excision and closure of the defect primarily
or with V-Y advancement flaps. Recurrences can generally be
FIGURE 52-26 Squamous cell carcinoma of anal canal. (Courtesy
treated with re-excision as long as disease remains noninvasive. In
Mayo Foundation for Medical Education and Research, Rochester,
patients medically unfit for surgery and with noninvasive disease, Minn.)
other techniques can be used, such as topical 5-FU, imiquimod,
cryotherapy, or argon beam laser therapy. Close observation in
these cases is advised, and biopsies for symptoms are recom- Many patients are initially misdiagnosed and ultimately present
mended.45 For patients with an invasive component, consider- late. Some have reported only 10% of patients being diagnosed
ation should be given to radical resection with APR. Five-year with tumors confined to the epithelium and subepithelial tissues.
disease-specific survival ranges from 50% to 70% of all patients AJCC staging for anal canal cancers is based on the size of the
with extramammary Paget disease. tumor and local invasion of adjacent organs or structures (Table
52-4).42 A tumor that is 2 cm or smaller is designated T1, larger
Basal Cell Carcinoma than 2 cm but not more than 5 cm is T2, and larger than 5 cm
Basal cell carcinoma is a rare type of anal tumor. In gross appear- is T3. Any size tumor that invades a local structure is designated
ance, these lesions are characteristic for pearly borders, with T4. Staging of disease includes CT of the chest, abdomen, and
central depression (Fig. 52-25). In most cases, these tumors can pelvis and pelvic MRI. Positron emission tomography scanning
be treated by wide local excision, reserving APR for extensive should be considered for larger (T2 or greater) tumors or any
lesions or those involving the sphincter mechanism. node-positive disease. HIV testing and checking a CD4 count
should also be considered if indicated.
Malignant Anal Canal Neoplasms Historically, treatment consisted of surgery alone or radiation
Squamous Cell Carcinoma therapy alone. Epithelial or subepithelial tumors were locally
Squamous cell carcinoma of the anal canal typically is manifested excised, and more advanced lesions underwent APR. The intro-
as a mass, sometimes with bleeding and pruritus (Fig. 52-26). duction of multimodality therapy combining chemotherapy and
 CHAPTER 52 Anus 1415

TABLE 52-4 TNM Staging Classifications completely.47 Patients with persistent disease up to 6 months after
treatment generally require APR. Those who have local recurrence
for Anal Malignant Neoplasms
are also recommended for APR. In the setting of isolated inguinal
Primary Tumor (T) node recurrence, groin dissection is generally required with con-
TX Primary tumor cannot be assessed sideration for radiation therapy to the inguinal node basins if no
T0 No evidence of primary tumor prior radiotherapy was given. Up to 50% of patients treated with
Tis Carcinoma in situ (Bowen disease, high-grade squamous salvage APR can expect a 5-year cure. This is compared with
intraepithelial lesion [HSIL], anal intraepithelial approximately 27% of patients treated with salvage radiation and
neoplasia II-III [AIN II-III]) concurrent cisplatin-based chemotherapy who can expect cure.
T1 Tumor 2 cm or less in greatest dimension In those patients presenting with anal squamous cell carcinoma
T2 Tumor more than 2 cm but not more than 5 cm in greatest in the setting of HIV infection, disease severity (CD4 count and
dimension use of antiretroviral therapy) has a significant impact on success
T3 Tumor more than 5 cm in greatest dimension of standard chemoradiation. The current consensus is that stan-
T4 Tumor of any size that invades adjacent organ(s) (e.g., dard protocols for chemoradiotherapy should be attempted,
vagina, urethra, bladder*) regardless of HIV status, and that medical management of the
patient’s HIV infection be optimized. The 2-year survival rates for
Regional Lymph Nodes (N) HIV-positive patients have been reported to be the same as for
NX Regional lymph nodes cannot be assessed HIV-negative patients, 77% and 75%, respectively.48
N0 No regional lymph node metastasis
N1 Metastasis in perirectal lymph node(s) Melanoma
N2 Metastasis in unilateral internal iliac and/or inguinal Melanoma of the anal canal is a rare tumor that can be manifested
lymph node(s) as a mass, pain, or bleeding. Tumors can be amelanotic and are
N3 Metastasis in perirectal and inguinal lymph nodes and/or sometimes incidentally found during histopathologic examination
bilateral internal iliac and/or inguinal lymph nodes of tissue obtained from an unrelated procedure, for example, a
hemorrhoidectomy specimen. Outcomes for patients with anal
Distant Metastasis (M)
melanoma are poor, with 5-year survival rates dependent on the
M0 No distant metastasis
extent of disease: 32%, 17%, and 0% for local, regional, and
M1 Distant metastasis
distant disease, respectively.49 Extent of surgery has not been
Stage Grouping shown to correlate with long-term outcomes. It remains contro-
0 Tis N0 M0 versial whether the optimal surgical approach involves wide local
I T1 N0 M0 excision or APR. Whichever approach is chosen, obtaining R0
II T2 N0 M0 resection remains a significant predictor for the best survival rates:
T3 N0 M0
19% 5-year survival for R0 cases and 6% 5-year survival for cases
IIIA T1 N1 M0
with involved margins.
T2 N1 M0
Adenocarcinoma
T3 N1 M0
T4 N0 M0
Adenocarcinoma of the anal canal is rare and thought to arise
IIIB T4 N1 M0
from the columnar epithelium of canal ducts. Differentiating
distal rectal cancer from true anal canal cancer may be difficult,
Any T N2 M0
and it is not necessary as the treatment for both is the same. These
Any T N3 M0
patients should be treated with multimodality therapy consisting
IV Any T Any N M1
of chemoradiotherapy and APR. This approach is superior to local
From Edge S, Byrd D, Compton C, et al, editors: AJCC cancer staging excision50 and radiation therapy alone.
manual, ed 7, New York, 2010, Springer.
*Invasion of the rectal wall, perirectal skin, subcutaneous tissue, or
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