Acute and Chronic Megacolon

Stephen B. Hanauer, MD
Arnold Wald, MD
Corresponding author
Arnold Wald, MD
Section of Gastroenterology and Hepatology, University of Wisconsin
School of Medicine and Public Health, 600 Highland Avenue, H6/516
CSC, Madison, WI 53792-5124, USA.
E-mail: axw@medicine.wisc.edu
Current Treatment Options in Gastroenterology 2007, 10:237–247
Current Medicine Group LLC ISSN 1092-8472
Copyright © 2007 by Current Medicine Group LLC

Opinion statement
Megacolon, defined as dilation of the abdominal colon, may occur acutely or in a
chronic form. Acute megacolon that occurs in association with severe inflamma-
tion of the colon is known as toxic megacolon, whereas acute megacolon without
obvious colonic disease is known as Ogilvie’s syndrome. The pathophysiology and
management of toxic megacolon, Ogilvie’s syndrome, and chronic megacolon in
adults differ significantly, and it is critically important to distinguish among these
entities. Toxic megacolon is a medical emergency that requires coordinated inten-
sive medical and surgical management. In addition to vigorous resuscitation with
fluids, electrolytes, and blood products, medical treatment consists of parenteral
corticosteroids, broad-spectrum antibiotics, and close monitoring of the patient.
Surgical intervention is required if there is no improvement, or deterioration after
12 to 24 hours of intensive medical management, or if there is evidence of colon
perforation. Ogilvie’s syndrome usually occurs in hospitalized patients with seri-
ous underlying medical or surgical illnesses. Management is directed at prevent-
ing ischemia and perforation of the distended colon. Supportive therapy includes
nasogastric suction, correction of fluid and electrolyte imbalances, stopping po-
tentially aggravating medications, and decompressing the colon with a rectal
tube and positional changes. Intravenous neostigmine is the only pharmacologic
agent of proven efficacy; colonoscopic decompression is an alternative in patients
who do not respond to neostigmine or who have conditions that contraindicate
its use. Daily oral administration of polyethylene glycol electrolyte solutions ap-
pears to decrease the relapse rate after initial decompression is achieved. Chronic
megacolon in adults represents advanced colon failure that does not respond to
pharmacologic stimulation. Goals of therapy are to cleanse the colon, prevent im-
paction, and minimize stool volume and gas buildup. For patients with disabling
symptoms, surgical exclusion of the colon, decompression and antegrade enemas
via cecostomy, or subtotal or segmental resection may be palliative.

Introduction

Megacolon may be defined as dilatation of the
abdominal colon (defined as > 9 cm in diameter) in
the absence of a mechanical obstruction. It may be
associated with a megarectum (defined as > 6 cm
diameter at the pelvic brim) or may occur alone.
Acute megacolon may occur in patients with severe
inflammatory bowel disease or infectious colitis, or in
patients without obvious colonic disease; the former
is known as toxic megacolon, whereas the latter is
nontoxic megacolon or Ogilvie’s syndrome. Chronic
megacolon may occur congenitally (ie, Hirschsprung’s
disease) or may be acquired. It is important to dis-
tinguish among these entities, as the pathophysiology,
evaluation, and management differ significantly.
238 Colon and Anorectum

Treatment
Toxic megacolon
• Both ulcerative colitis and Crohn’s disease have the potential to develop
severe, fulminating, or toxic colitis [1], which is a medical emergency re-
quiring coordinated intensive medical and surgical management. Patients
with fulminant colitis have transmural inflammation that can result in
circular smooth muscle paralysis, precipitating dilatation [2••]. Toxic
megacolon refers to acute nonobstructive dilatation of the colon arising
as a complication of any severe inflammatory colitis, including ulcerative
colitis [3], Crohn’s disease, amebic colitis, pseudomembranous colitis,
and other infections (shigella, salmonella, Chagas’ disease, and cytomeg-
alovirus [CMV]) [4]. Toxic megacolon has been reported to complicate
1% to 13% of all ulcerative colitis cases [5] and 2% to 3% of Crohn’s
colitis cases. Although mortality in early series was as high as 25%
(reaching 50% if colonic perforation occurred), early recognition and
management of toxic megacolon have substantially lowered mortality to
as low as 2% in experienced centers. Factors associated with increased
mortality include older age (> 40 years), colonic perforation, and delay
of surgery. Colonic perforation, whether free or localized, is the greatest
risk factor leading to increased morbidity or death.

Predisposing factors
• Severe disease activity is the most important predictor for the develop-
ment of toxic megacolon, which more commonly occurs in extensive
colitis than proctitis or proctosigmoiditis. Occasionally, limited right- or
left-sided segmental colitis [5] has been associated with toxic megacolon.
• Toxic megacolon typically occurs early in the course of ulcerative colitis,
usually within the first 5 years of disease; approximately 25% to 40%
of cases present with the initial attack. The onset of toxic megacolon
has been temporally linked to diagnostic examinations such as barium
enemas or colonoscopy, suggesting that manipulation of the inflamed
bowel or vigorous laxative preparation or electrolyte imbalance may
exacerbate fulminant colitis toward toxic megacolon.
• Drug therapies such as diphenoxylate atropine sulfate (Lomotil; Pfizer,
Inc., New York, NY), loperamide, and other inhibitors of colonic motil-
ity (eg, opiates and narcotics) have been implicated in the development
of toxic megacolon through their inhibition of colonic muscle function
in the setting of severe transmural disease. Electrolyte and acid-base dis-
turbances are also risk factors (eg, severe potassium depletion secondary
to severe diarrhea, corticosteroid therapy, or both is known to inhibit
colonic motility). Despite early speculation regarding their potential
to induce toxic megacolon, corticosteroids and adrenocorticotropic
hormone are no longer implicated as precipitating factors [2••,3,6].
Nevertheless, corticosteroids may suppress signs of perforation, thereby
delaying surgery.
• CMV infection may contribute to fulminant colitis or toxic megaco-
lon [7,8]. There are no controlled trials regarding the utility of treating
CMV, and in the absence of systemic manifestations of CMV (eg, fever,
hepatitis), no treatment is necessary [2••,6]. There are a few reports of
successful interventions targeting CMV if identified in colon biopsies.
 Acute and Chronic Megacolon Hanauer and Wald 239

Table 1. Criteria for diagnosis of toxic megacolon

1. Radiographic evidence of colonic dilatation
2. At least 3 of the following:
a. Temperature > 101.5°F
b. Heart rate > 120 beats/min
c. White blood cell count > 10.5 (× 109/L)
d. Anemia
3. At least 1 of the following:
a. Dehydration
b. Mental status changes
c. Electrolyte disturbances
d. Hypotension

Clinical features
w
Toxic megacolon usually occurs in the setting of chronic inflammatory
bowel disease and is characterized by greater than 10 bowel movements
per day, rectal urgency, continuous bleeding, abdominal pain, abdominal
distention, fevers, weight loss, and dehydration [9]. On physical examina-
tion, patients present with fever, tachycardia, abdominal tenderness and
mild distention, tympany, and decreased bowel sounds [10]. Table 1 sum-
marizes the most accepted clinical criteria for toxic megacolon, based on
signs, symptoms, and diagnostic abnormalities [11]. The clinical picture
typically progresses to abdominal distention. Impaired consciousness and
lethargy are ominous signs. Occasionally in chronically treated patients,
a paradoxic decrease in stool frequency with passage of only bloody
discharge may be an ominous sign. Thereafter, clinical signs of toxemia,
including temperature greater than 101.5°F and tachycardia, develop as
abdominal pain and distention become progressive, whereas bowel sounds
diminish or cease. On physical examination, peritoneal irritation, includ-
ing rebound tenderness and abdominal guarding, suggests transmural
inflammation with serosal involvement, even in the absence of free perfo-
ration. Conversely, peritoneal signs may be minimal or absent in elderly
patients or those receiving high-dose or prolonged corticosteroid therapy.
In such patients, loss of hepatic dullness may be the first clinical indication
of colonic perforation. Mental status changes such as confusion, agitation,
and apathy occasionally are noted. Leukocytosis (white blood cell count
> 10,500 cells/mm3) with a left shift, anemia, hypokalemia, and hypoalbu-
minemia are common laboratory findings [11]. The degree of metabolic
alkalosis correlates with severity of colitis [2••].

Diagnosis
w
A plain abdominal radiograph may show features of fulminant colitis
such as wall thickening with islands of edematous mucosa surrounded
by deep ulcerations [10,12]. The presence of colonic dilatation greater
than 5.5 cm is predictive of the evolution to toxic megacolon [10].
w
Plain films of the abdomen usually are sufficient to suggest the diagnosis,
revealing loss of haustration with segmental or total colonic dilatation
[13]. Clinical studies have demonstrated a strong correlation between
colonic dilatation and deep ulceration involving the muscle layers. The
magnitude of dilatation may not be severe, averaging 8 to 9 cm (normal is
< 5 to 6 cm), although colonic diameter may reach 15 cm before rupture.
240 Colon and Anorectum

Maximal dilatation can occur in any part of the colon. Accompanying

mucosal thumbprinting or pneumatosis cystoides coli reflects severe trans-
mural disease. Free peritoneal air is an immediate indication for surgery.
Infrequently, retroperitoneal tracking of air from a colonic perforation
may produce subcutaneous emphysema and pneumomediastinum without
pneumoperitoneum. In patients with severe colitis, small bowel ileus may
herald toxic megacolon [14] and is a poor prognostic sign for medical
success [15]. Discrepancies between physical and radiographic findings
may exist. Abdominal distention by physical examination may be mini-
mal despite massive colonic dilatation. Conversely, peritoneal signs in the
absence of free air or dilatation should not be ignored.
w
A limited proctoscopic examination or flexible sigmoidoscopy with
minimal air insufflation may be performed safely to evaluate the mucosa
for pseudomembranes or ischemia [16]. Examination generally shows
extensive ulceration with friable, bleeding mucosa. In patients whose
initial presentation of inflammatory bowel disease is severe colitis,
biopsies should be performed to evaluate for Crohn’s disease and to rule
out acute, self-limited colitis. In those with an exacerbation of known
disease, biopsies help to exclude Clostridium difficile or CMV infection
[4]. Although this is controversial, more extensive endoscopic examina-
tions [17] generally are contraindicated [2••]. If performed, the presence
of severe colitis (deep-penetrating ulcers) in conjunction with clinical
features of severe disease is a poor prognostic sign [10,17–19]. Stool
analysis for amebiasis, C. difficile, Escherichia coli O157:H7, campylo-
bacter, salmonella, and shigella infection should be part of the diagnostic
work-up [2••,6].

Medical management
• A team approach with early management and continuous assessment by
both groups is vital, not only to determine whether surgery is indicated,
but also to support critically ill patients pre- and postoperatively. Early
recognition and institution of therapy by an experienced team can alter
the outcome of this life-threatening illness [2••,6,20,21].
• Resuscitative measures include vigorous fluid, electrolyte, and blood
replacement to maintain the serum hematocrit above 30%. Intravenous
fluid and electrolytes should be ordered to restore previous losses while
continuing to replenish ongoing losses from diarrhea, fever, and third-
spacing of fluids [2••]. Oral intake should be discontinued if colonic
dilatation is recognized in a patient with toxic manifestations. Patients
with nausea and vomiting or significant abdominal pain also should be
on complete bowel rest. Anticholinergic and narcotic agents should be
discontinued immediately. In the presence of small bowel ileus, a na-
sogastric tube usually is placed; despite a lack of clear evidence for the
placement of long intestinal tubes, they are advocated by some. Patient
repositioning from front to back or prone knee-elbow position may
redistribute colonic air and assist in decompression [22]. Rarely, patients
with dilatation in the absence of toxic signs or symptoms may benefit
from rectal tube decompression.
• Parenteral nutritional support to correct malnutrition and electrolyte
and acid-base balance (including repletion of phosphate, calcium, and
magnesium) should be initiated. Although severe hypokalemia may
not be present, total body potassium depletion is common and can be
exacerbated by glucocorticoids such that resuscitative measures should
include adequate potassium replacement [2••].
• Aminosalicylates, a mainstay of maintenance therapy and of the treat-
 Acute and Chronic Megacolon Hanauer and Wald 241

ment of mild to moderate disease, have no role in the treatment of toxic

colitis [2••,6,20,21].
• Broad-spectrum antibiotics with adequate gram-negative and anaero-
bic coverage should be administered without delay once transmural
inflammation or toxic megacolon is suspected [11]. Antibiotics should
be continued until the patient stabilizes over several days to a week, or
through the initial postoperative period. Whether antibiotics help avert
progression of toxic megacolon is not known.
• Corticosteroids have long been used in the management of fulminant or
toxic ulcerative or Crohn’s colitis [2••,6,21]. Generally, most patients
with inflammatory bowel disease will have been receiving corticosteroids
before toxic megacolon developed, in which case they should be con-
tinued. There is no evidence that corticosteroids precipitate or worsen
outcome in toxic megacolon, although they can mask signs of perfora-
tion or peritonitis. In cases of toxic megacolon with infectious etiologies,
corticosteroids should not be used.
• There is no general agreement regarding which corticosteroid preparation
or dose should be administered. Usual doses employed range between
40 and 80 mg of methylprednisolone (often 1 mg/kg in Europe) or
400 mg of hydrocortisone provided in divided doses or continuous infu-
sion [2••,6,20,21,23••]. Prednisone, 25 mg intravenously every 6 hours,
and prednisolone sodium phosphate also have been used successfully. In
the United States, hydrocortisone, 100 mg every 6 hours, and methylpred-
nisolone, 6 to 15 mg every 6 hours, are available for intravenous adminis-
tration. There is no advantage to doses greater than 60 mg of methylpred-
nisolone daily [23••]. Although a continuous infusion of corticosteroids
may be beneficial to maintain steady plasma levels, a recent trial did not
identify differences between twice-daily intravenous dosing and continu-
ous infusions in the setting of severe (not toxic) colitis [24].
• The response to corticosteroids in the setting of severe fulminant colitis
has remained constant for the past several decades [23••], with approxi-
mately 75% of patients responding [16,23••,25]. The short-term prog-
nosis for corticosteroids can be predicted as early as 24 hours. Persis-
tence of greater than nine stools per day, an albumin less than 3 mg/dL,
or a pulse rate greater than 90 beats/min was predictive of treatment
failure [10]. Patients with greater than eight stools per day and a C-reac-
tive protein (CRP) greater than 4.5 mg/dL by day 3 had an 85% likeli-
hood of requiring colectomy [16] or cyclosporine therapy. Continuation
of intravenous corticosteroids beyond 7 to 10 days does not provide any
additional benefits [23••,26] and may increase morbidity and surgical
risks [27]. In addition, ex-smokers have a worse prognosis [28]. Mecha-
nisms of steroid resistance have not been fully elucidated [29].
• If no improvement, or deterioration occurs after 12 to 24 hours of in-
tensive medical management for toxic megacolon, surgical intervention
is required. Evidence of colonic perforation is an unequivocal indication
for emergent surgery. If physical signs of perforation are absent, 12- to
24-hour radiographic surveillance is necessary. Perforation is associ-
ated with severe complications, including peritonitis, extreme fluid and
electrolyte imbalance, and hemodynamic instability. Early recognition of
perforation should lessen morbidity or mortality. Other indications for
emergent surgery include signs of septic shock, multiorgan dysfunction
[2••], and imminent transverse colon rupture (diameter > 12 cm) [11].
Hypoalbuminemia, persistently elevated CRP or erythrocyte sedimenta-
tion rate, small bowel ileus, and deep colonic ulcers are poor prognostic
factors for successful medical therapy [15,16,30].
242 Colon and Anorectum

• Cyclosporine A, administered as an intravenous continuous infusion,

either alone [31] or in combination with corticosteroids, has not been
evaluated in clinical trials for patients with toxic megacolon but has
been effective in treating severe ulcerative colitis [26], with response
rates of up to 85% to 92% [32]. However, due to the acuity and severity
of illness in patients with toxic megacolon, use of cyclosporine has rare-
ly, if ever, been advocated in this setting. If employed, failure to improve
to less than eight stools per day or persistence of CRP elevation after 3
days of cyclosporine is predictive of the need for colectomy [16]. Careful
daily monitoring for serious side effects of nephrotoxicity, infection, and
seizures must be done when using cyclosporine [33].
• Similarly, infliximab, a chimeric anti-tumor necrosis factor monoclonal
antibody, has been shown to be effective as outpatient therapy for patients
with moderate to severe ulcerative colitis [34] but has not been evaluated
in the setting of toxic megacolon, aside from a single case report.
• Patients who improve are then transitioned to oral prednisone at the same
daily dose used to achieve clinical remission. They may be discharged
from the hospital when tolerating a low-residue diet, with formed stools
without blood or rectal urgency. Premature discharge is doomed to failure
and readmission. Aminosalicylates are added as a maintenance therapy
once patients are tolerating oral steroids and a full diet. The long-term
prognosis after hospitalizations for fulminant or toxic colitis requiring
corticosteroid therapy is not as promising as once considered [35–37].

Surgical management
• In the absence of improvement after 12 to 24 hours of intensive medical
management, or if deterioration occurs, surgical intervention is required.
Some physicians view early surgery for toxic megacolon as the conser-
vative approach, noting that delay of operative therapy may promote
higher mortality.
• The surgical management of toxic megacolon must be individualized for
each patient. The type of operation is dependent on the clinical condi-
tion of the patient and the experience of the surgeon [38,39]. Early
intervention to reduce mortality must be balanced against the potential
for intensive medical management to control the inflammatory process
and complications, thereby potentially preventing the psychosocial and
medical stigmata of colectomy.
• In the setting of toxic megacolon, most surgeons prefer a limited ab-
dominal colectomy with ileostomy, leaving the rectosigmoid as a mucous
fistula or the rectum alone, using a Hartmann procedure. This approach
limits a lengthy pelvic dissection in acutely ill patients while allowing
for the option of a subsequent restorative, sphincter-saving procedure
(ileoanal anastomosis). In patients with indeterminate colitis or Crohn's
disease, preservation of the rectum may provide the opportunity for an
eventual ileorectal or ileoanal anastomosis to preserve anal continence
after temporary diversion and pathologic review of the colectomy speci-
men [1]. Rarely, “blow-hole” colotomies may be useful in highly selected
individuals with poor operative prognoses.

Acute nontoxic megacolon

• Acute nontoxic megacolon (Ogilvie’s syndrome) usually occurs in hospital-
ized patients who have serious underlying medical or surgical illnesses. The
most common clinical settings are orthopedic injuries, obstetric and ab-
dominal surgeries, systemic infections, and neurological illnesses. In surgical
 Acute and Chronic Megacolon Hanauer and Wald 243

Figure 1. Algorithm for management of acute colonic pseudo-

obstruction. PEG—polyethylene glycol.
Conservative management for 24 hours

Success No improvement or cecum

> 12 cm or distention > 3 days

Intravenous neostigmine

PEG electrolyte Success No improvement

solution orally

Colonoscopy

Signs of ischemia No improvement

or perforation

Tube cecostomy
Surgical resection

patients, the mean onset of symptoms and signs occurs 5 days postopera-
tively. The pathogenesis is poorly understood; the most widely held theory
involves an imbalance between relatively excessive sympathetic and insuf-
ficient parasympathetic regulation of colonic motor activity. Among the risk
factors for the development of postoperative Ogilvie’s syndrome are older
age, obesity, immobility, and use of patient-controlled analgesia [40].

Clinical manifestations and diagnostic methods

• In the typical case, progressive abdominal distention develops some-
time between the third and fifth postoperative day, often occurring
with lower abdominal pain and, in approximately one half of all cases,
nausea and vomiting. Only a minority of patients will have hypoactive
or absent bowel sounds, and some continue to pass flatus. The presence
of peritoneal signs is worrisome for perforation or ischemia, but this is
uncommon in the early stages of the illness.
• Plain abdominal films should be obtained to establish the diagnosis.
Typically, as the patient generally is supine, dilatation often is most pro-
nounced in the transverse and ascending colon and cecum, with relatively
less distention in the left colon and rectum. Typically, haustral markings
are preserved, in contrast to their absence in toxic megacolon. If obstruc-
tion must be excluded, an abdominal CT scan is preferred; a single-
contrast gastrografin enema instilled to the level of colonic dilatation is
a secondary option. This is to minimize the risk of cecal perforation if
colonoscopy is performed in the presence of a mechanical obstruction.
• The initial evaluation also includes assessing for signs of ischemia and
perforation, as well as the degree and duration of colonic distention.
• The presence of peritoneal signs, leukocytosis, and fever are worrisome
signs of possible perforation or ischemia. Although a cecal diameter
greater than 12 cm is considered a significant risk factor for perforation,
there is no clear linear relationship between complications and cecal
diameter. However, the longer the duration of distention, the greater the
risk is thought to be.
244 Colon and Anorectum

Nonsurgical treatment
• Nonsurgical treatment options include supportive therapy, neostigmine,
and colonoscopic decompression (Fig. 1). Supportive therapy includes
nasogastric suction, correction of fluid and electrolyte imbalances,
discontinuation of potentially offending medications, rectal tube decom-
pression, and frequent changes of position with efforts to minimize the
supine position. Medications to avoid or discontinue, if possible, include
narcotic analgesics, anticholinergics, calcium channel antagonists, and
serotonin type 3 antagonists [41].
• Intravenous neostigmine is the only pharmacologic agent of proven
efficacy, with response rates of greater than 80% and a low recurrence
rate [42,43]. The dose is 2 mg administered intravenously over 3 to 5
minutes with electrocardiogram, vital signs monitored for 30 minutes,
and atropine available at the bedside. Contraindications include signs of
ischemia or perforation, pregnancy, serum creatinine greater than
3 mg/dL, active bronchospasm, and bradycardia. The mean time to
response in the original report was 4 minutes [43]. If the patient fails
to respond to two separate doses, or if there is a prompt recurrence of
colonic distention, colonoscopic decompression is advised.
• Colonoscopic decompression remains an effective alternative in patients
who do not respond to neostigmine or who have conditions that con-
traindicate its use [44]. An overall success rate in achieving a critical
reduction in cecal diameter of 70% has been reported, but recurrence
rates are as high as 40%, and it is not without risk in acutely ill patients
[45]. Some have advocated the placement of a decompression tube in the
right colon after colonoscopy, but it is debatable whether it significantly
reduces the recurrence rate [45]. An alternative approach is the adminis-
tration of polyethylene glycol (PEG) electrolyte orally or via nasogastric
tube following colon decompression by pharmacologic or mechanical
means. In a recent randomized controlled trial, PEG electrolyte solution,
29.5 g daily, significantly increased the sustained response rate compared
with placebo after successful therapeutic intervention [46•].
• In patients who fail to respond to the previously described interventions
or who develop ischemia or perforation, surgery may be necessary. In
patients without ischemia or perforation, the placement of a tube cecos-
tomy may be performed surgically for decompression and administra-
tion of PEG electrolyte solution [47]. Surgical resection for ischemia or
perforation is associated with high morbidity and mortality rates, mak-
ing early recognition and intervention of Ogilvie’s syndrome optimal.

Chronic megacolon
• Chronic megacolon in adults is an uncommon condition that gener-
ally is associated with constipation. Two main groups are recognized:
1) a congenital absence of myenteric and submucosal ganglia of
varying portions of the distal colon always affecting the internal anal
sphincter, which denotes Hirschsprung’s disease; and 2) an acquired
disorder either due to known causes (ie, Chagas’ disease), associated
with neurological disorders or diseases of intestinal smooth muscle,
or (most commonly) an idiopathic form. Some studies of idiopathic
megacolon suggest that there is a severe disintegration of the enteric
nerves in some cases [48], whereas others have focused on atrophy
of the collagenous connective tissue membrane of the myenteric
plexus and muscularis propria that abolishes peristalsis and permits
unlimited distention of the colon [49]. Thus, chronic megacolon is a
 Acute and Chronic Megacolon Hanauer and Wald 245

decompensated colon and should not be managed as would chronic

constipation alone. It will not respond to pharmacologic stimulation
the way acute colonic pseudo-obstruction does.

Clinical manifestations and diagnostic tests

• Evaluation includes exclusion of a mechanical obstruction with colo-
noscopy (if the patient is of screening age) or a water-soluble contrast
enema. Medications that inhibit colonic motility should be stopped or
replaced. If there is significant exposure to traveling or living in South
America, serologic screening for Chagas’ disease should be obtained.
• Very uncommonly, Hirschsprung’s disease will not be diagnosed until
adulthood [50]. The diagnosis should be considered, especially in a male
with constipation since childhood and in the absence of fecal inconti-
nence. In such patients, a barium enema should be performed in an un-
prepared colon and only to the level at which colonic dilatation is seen.
The insertion catheter then should be removed to look for the transition
zone between dilated and nondilated colon and rectum. The diagnosis
must be confirmed with suction biopsies or full-thickness biopsies of the
nondilated rectum, using either acetylcholinesterase stains or to demon-
strate absence of myenteric and submucosal nerve elements.

Treatment: general approach

w
Regardless of underlying etiology, management is symptomatic and
palliative. The management of Hirschsprung’s disease is primarily
surgical and based upon the extent of the aganglionic segment. Ano-
rectal myectomy has been advocated as an initial approach to adults
with Hirschsprung’s disease because of its low morbidity and technical
ease of performance. However, it may be difficult to identify patients
who require myectomy only from those who will benefit from both
myectomy and anterior resection. Within the last decade, laparoscopic
surgical correction has replaced open surgery in these patients.
• For most patients with chronic megacolon, a nonsurgical approach
is effective. Stool retention is not uniformly seen in all patients with
chronic megacolon. If large amounts of stool are present, the patient
should undergo thorough cleansing of the colon with high-colonic
water enemas, water-soluble contrast enemas, and, if tolerated, large
volumes of PEG electrolyte solutions. Once accomplished, a colonic
maintenance regimen may be implemented.
• Fiber supplements and osmotic agents such as lactulose and sorbitol
should be avoided, as these increase stool volume and gas production.
Rather, fiber restriction with small amounts of PEG solutions (8.5 g/d) will
reduce stool buildup. Large-volume, warm-water enemas or oral colonic
lavage with PEG electrolyte solutions may be administered once or twice
weekly to empty the colon regularly. Diets should be designed to avoid
gas-producing foods that will increase distention and discomfort. Stimu-
lant laxatives and enterokinetic agents are unlikely to be effective in view
of failure of the enteric nervous system and colonic smooth muscle.
• If conservative measures fail, there are several surgical options, de-
pending upon anorectal function (Table 2). These include ileostomy
with colonic exclusion, subtotal colectomy with ileorectal anastomosis
if anorectal function is preserved, or decompressive cecostomy with
periodic antegrade enemas if distention is uncomfortable [49]. If
subtotal colectomy with ileorectal anastomosis is considered, exclusion
246 Colon and Anorectum

Table 2. Surgical options for chronic megabowel

Condition Surgical options
Isolated megacolon Subtotal colectomy with ileorectal anastomosis*
Diverting loop ileostomy†
Megacolon and megarectum Colectomy with ileoanal anastomosis*
Diverting loop ileostomy†
Decompressive cecostomy with periodic
antegrade enemas
Segmental megacolon Segmental resection
*If anorectal function is normal.
†
If anorectal function is abnormal.

of a more generalized intestinal pseudo-obstruction predicts a more

optimal outcome. In selected cases, segmental resection of isolated
areas of dilated bowel may be appropriate.

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