Chronic intestinal pseudo-obstruction

Gastrointestinal motility disorders

Region With dilatation Without dilatation
Esophagus Achalasia with mega-esophagus Achalasia

Stomach Acute gastric dilatation Gastroparesis

Small bowel Pseudo-obstruction Chronic intestinal dysmotility

Colon Megacolon/pseudo-obstruction Slow transit constipation/colonic inertia

Pseudo-obstruction is a syndrome characterized by signs and symptoms of a mechanical obstruction

of the small or large bowel in the absence of an anatomic lesion that obstructs the flow of intestinal
contents. Pseudo-obstruction may be acute or chronic and is characterized by the presence of
dilation of the bowel on imaging.

When there is evidence of chronic small intestinal motility disorder in the absence of bowel
dilatation, the preferred term is chronic intestinal dysmotility.

Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder that may be due to an underlying
neuropathic disorder (involving the enteric nervous system or extrinsic nervous system), a
myopathic disorder (involving the smooth muscle), or abnormality in the interstitial cell of Cajal (ICC)
Neuropathic, myopathic, or ICC abnormalities may be idiopathic or secondary to another disease.
Approximately half of the cases of CIPO are secondary to neurologic, paraneoplastic, autoimmune,
metabolic/endocrine, and infectious diseases.

Degenerative neuropathies — Neurologic (eg, Parkinson disease and Shy-Drager syndrome) and
metabolic disorders (eg, diabetes mellitus) can affect the extrinsic nerve pathways supplying the gut.
Neuropathic disorders may be complicated by a myopathic stage when the muscle layer is
infiltrated, as in amyloidosis.

CIPO has been reported in association with small cell lung cancers or carcinoid tumors and malignant
thymoma. These patients often have antineuronal nuclear (anti-Hu) antibodies
Immune-mediated pseudo-obstruction associated with neuronal or smooth muscle involvement has
been reported. Scleroderma, dermatomyositis, and systemic lupus erythematosus can alter the
enteric nerves, the smooth muscle cells, and possibly the ICC [10,11].

Chagas disease is the most common infectious cause of CIPO

CIPO in association with radiotherapy and chemotherapy for gynecological cancer

Chronic intestinal pseudo-obstruction (CIPO) is rare

Clinical features — Abdominal pain, bloating, and distension are the most common . These
symptoms may be acute, recurrent, or chronic.●Abdominal pain ●Nausea ●Constipation
●Heartburn/regurgitation ●Fullness ●Epigastric pain/burning ●Early satiety

Acute episodes are characterized by abrupt onset of intense, cramping pain, abdominal distention,
nausea, and vomiting. After the acute episode, patients may be asymptomatic or more often,
continue to experience symptoms due to delayed transit in the proximal (eg, anorexia, early satiety
nausea and vomiting) and/or distal (constipation) gastrointestinal tract. When patients develop
these acute exacerbations, it is important to exclude intestinal volvulus
Patients may have diarrhea due to small bowel bacterial overgrowth. Weight loss results from
impaired intestinal transit, malabsorption due to bacterial overgrowth, and inadequate intake due to
exacerbation of symptoms with food ingestion.

Patients may have symptoms due to the underlying disorder (eg, dysphagia in CIPO related to
Chagas disease, proximal muscle weakness leading to difficulty climbing stairs in patients with
polymyositis/dermatomyositis, bladder dysfunction in neuropathic and myopathic CIPO). Patient
may provide a history of culprit medications (including anticholinergic antidepressants, calcium
channel blockers, and the alpha-2 adrenergic agonists such as clonidine) or a family history of
relatives with a similar clinical presentation (eg, mitochondrial neurogastrointestinal encephalopathy
[MNGIE], familial amyloidosis)

The main physical findings are abdominal distention, abdominal tenderness on palpation (localized
to the epigastric and periumbilical regions or more commonly, over the whole abdomen .Patients
may also have signs of an underlying collagen vascular or neuromuscular disease (eg, proximal
muscle weakness may indicate polymyositis/dermatomyositis; classic skin abnormalities associated
with scleroderma; ptosis, ophthalmoplegia, peripheral polyneuropathy

Measurement of serum electrolytes may reveal hypokalemia and metabolic acidosis if there is
prominent diarrhea. Hypokalemia and metabolic alkalosis may be present if there is prominent
vomiting. Patients may also have hypoalbuminemia due to malnutrition. Rarely, serum vitamin B12
concentrations are low due to bacterial overgrowth. Patients may have an elevated TSH due to
underlying hypothyroidism. Search for auto-antibodies (specific antibodies to glutamic acid
decarboxylase, voltage-gated calcium channels [P/Q subtype], nicotinic acetylcholine receptors, and
voltage-gated potassium channels) may identify an association of an immune-mediated process

Imaging — A plain film of the abdomen in intestinal pseudo-obstruction usually demonstrates air-
fluid levels and/or distended loops of small bowel. In addition, computed tomographic/magnetic
resonance enterography may demonstrate dilated loops and rarely diverticula or pneumatosis
intestinalis. Radiographic testing does not usually provide an etiologic diagnosis of CIPO. An
exception is systemic sclerosis affecting the small intestine, which is characterized by dilated
segments, edema, and abnormal texture and motility of the valvulae conniventes

The diagnosis of chronic intestinal pseudo-obstruction (CIPO) is based on the presence of

longstanding symptoms of mechanical obstruction in the absence of an anatomic cause on radiologic
examination and endoscopy, and evidence of impaired motility.Confirmation of the diagnosis
requires exclusion of mechanical obstruction and other causes of dysmotility by performing imaging
studies, endoscopy, and scintigraphy to assess motility.

Plain radiographs identify air-fluid levels, and contrast imaging (computed tomographic
[CT]/magnetic resonance [MR] enterography) identifies an organic cause of obstruction. MR
angiography should be considered in patients with evidence of obstruction when congenital or
acquired vascular abnormalities are suspected based on enterography. Cine MR imaging may be
used to assess small bowel motility

Upper endoscopy and colonoscopy should be performed to rule out an intraluminal or extraluminal
cause of obstruction. Upper gastrointestinal endoscopy is useful to exclude an aorto-mesenteric
artery compression syndrome, which may be difficult to differentiate on imaging from CIPO due to
the impact of severe dysmotility on this segment of the small intestine (ie, sustained uncoordinated
contractions in the distal duodenum). The duodenal mucosa should be biopsied to exclude celiac
disease.
Motility assessment — In patients in whom CIPO is suspected and there is no evidence of an
intraluminal or extraluminal cause of obstruction on imaging and by endoscopy, the presence of a
motility disorder should be confirmed with scintigraphy.

Scintigraphy — Scintigraphy is the method of choice in the evaluation of gastric, small bowel, and
colon transit. Normal small bowel transit time can vary depending on the methods used. While
interpreting scintigraphy results, it is important to note that delayed colonic transit may cause
delayed small bowel transit. Therefore, it is important to consider gastrointestinal transit in all three
main regions (stomach, small bowel, and colon) before concluding that delayed small bowel transit
defines small intestinal dysmotility. A useful clue to clinically significant small intestinal disease is the
finding of delayed gastric emptying. Thus, when small bowel and colonic transit are delayed and
gastric emptying is normal, the main focus of treatment should be normalization of colonic motor
function and treatment of constipation, rather than attempting to normalize the small bowel transit
alone.

Wireless motility capsule — Small bowel transit in suspected chronic intestinal dysmotility can also
be measured by wireless motility capsule, which assesses small bowel transit time .Evaluation of
intestinal motility using endoluminal image analysis acquired by capsule has also been developed

Chronic intestinal dysmotility and slow transit constipation/colon inertia are similar motor disorders
of the small bowel and colon, which are not associated with dilatation

Once the diagnosis of chronic intestinal pseudo-obstruction (CIPO) is established, the underlying
etiology should be determined. All patients should undergo laboratory testing to identify secondary
causes of CIPO.

In patients with delayed transit on scintigraphy, in whom there is a known underlying disease, no
further investigation is necessary. In patients with delayed transit and no known underlying disease,
manometry should be performed. Autonomic testing is useful in patients with evidence of
neuropathic dysmotility on manometry, but without a known underlying neurologic disorder. Full
thickness biopsy is rarely needed and should be considered in patients with severe dysmotility of
unknown etiology who undergo surgery, in patients with poor postsurgical outcomes, or in patients
with a permanent catheter for enteral or parenteral nutrition.

Laboratory examination can identify secondary causes of CIPO related to potentially curable
diseases. The following tests should therefore be performed in all patients with CIPO: complete
blood count, electrolytes, liver tests, vitamin B12, folate, celiac serologies, thyrotropin (TSH),
serologic testing for herpes simplex virus (HSV), cytomegalovirus (CMV), Epstein-Barr virus (EBV),
erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP).

Antineuronal antibodies (ANNA-1/anti-Hu) should be sought in patients with suspected

paraneoplastic syndrome. In patients with suspected mitochondrial neurogastrointestinal
encephalomyopathy (MNGIE) lactic acid (at rest and during exercise), thymidine phosphorylase
levels in the buffy coat, nucleotide concentrations, and genetic analysis should be performed.

Manometric studies of the esophagus, stomach and small intestine should be performed in patients
with abnormal motility on scintigraphy, but no known underlying disease. Manometry plays a
supportive role in defining the underlying diagnosis, but lacks specificity. Myopathic disorders are
typically associated with low amplitude contractions, whereas in neuropathic disorders, the
amplitude of contractions is typically normal, but the organization of the contractile response is
abnormal. A mechanical obstruction of the intestine typically shows simultaneous, prolonged
contractions at the level of the small intestine. Of note, esophageal manometry may show
ineffective peristalsis in about half the patients with CIPO [57].

Autonomic testing — Autonomic testing is useful in patients with evidence of neuropathic

dysmotility on manometry, but without a known underlying neurologic disorder. Several common
neurologic disorders can affect gastrointestinal motility by altering the parasympathetic or
sympathetic supply to the gut These include [●Brainstem tumors or strokes●Diabetes
mellitus●Spinal cord injury●Multiple sclerosis●Parkinson disease●Autonomic system degeneration

Tests of autonomic function can differentiate a preganglionic or central lesion from a peripheral
neuropathy associated with autonomic dysfunction

Brain and spinal cord magnetic resonance imaging (MRI) is essential in patients in whom a central
lesion is suggested from the history or the results of autonomic testing. A peripheral dysautonomia
requires further screening for a toxic, metabolic, or paraneoplastic process (eg, lead poisoning,
porphyria, or lung cancer, respectively).

Full-thickness intestinal biopsy — Full thickness biopsies should be considered in patients with
severe dysmotility of unknown etiology who undergo surgery for any reason especially in those
refractory to therapy, in patients with poor postsurgical outcomes, or in patients with a permanent
catheter for enteral or parenteral nutrition. Histologic findings may help differentiate myopathic,
neuropathic, or other disorders. Histopathologic techniques also allow for detection of subtle
abnormalities in the enteric nervous system and underlying deficiencies in specific neuropeptides
and neurotransmitters, thereby providing valuable information for diagnosis, prognosis, and
management

TREATMENT — Patients should be managed by a multidisciplinary team including a

gastroenterologist, nutritionist, and transplant surgeon with experience in the treatment of CIPO
Patients with CIPO require supplemental nutritional support. Prokinetics may be useful for acute and
chronic therapy of intestinal pseudo-obstruction. Antibiotics are recommended in patients with
small bowel bacterial overgrowth. Immunomodulator therapy should be reserved for patients with
CIPO due to an established underlying inflammatory neuropathy. Surgery should be performed, if
necessary, to provide access for venting/feeding.

Surgery to resect or bypass localized disease of the small bowel should be avoided. Intestinal
transplantation is indicated in selected patients in whom long-term parenteral nutrition cannot be
initiated or continued safely. Treatment should also be directed at the underlying disease. As an
example, enzyme replacement therapy has been associated with alleviation of gastrointestinal
manifestations in patients with Fabry disease.

Nutritional support — Early intervention with nutritional support is important, particularly for those
who have had recurrent vomiting or reduced oral intake. Small meals consisting of liquid or
homogenized foods are better tolerated than solids. Hypercaloric liquid formulations should be used
in patients with low caloric intake. Oral or enteral nutrition is typically used for neuropathic
disorders or in patients in whom the motility disorder is localized to the stomach and duodenum.
Endoscopic access to the stomach with a jejunum extension tube may provide an effective means for
decompression and enteral nutrition. Parenteral nutrition may be necessary for patients with severe
dysmotility (usually myopathic pseudo-obstruction

Prokinetic agents — Prokinetic agents, particularly erythromycin and cisapride, may be useful for
acute and chronic therapy of intestinal pseudo-obstruction, respectively .
It is appropriate to combine a prokinetic agent with an antiemetic medication such as promethazine
12.5 to 25 mg twice daily (also available in liquid form or suppository) for symptom relief. The
sedative effects of promethazine may be helpful when administered at bedtime. In patients who
cannot tolerate promethazine, the 5-HT3 antagonist ondansetron 4 to 8 mg three times daily may be
used. However, this class of medications delays colonic transit.

Erythromycin — Intravenous erythromycin is effective during acute exacerbations of intestinal

pseudo-obstruction, acting at least in part by stimulation of the motilin receptors. Such patients are
typically hospitalized and require intravenous fluids. Intravenous erythromycin lactobionate at a
dose of 3 mg/kg every eight hours should be continued for at least five to seven days.

Erythromycin has not been very effective for chronic therapy and has only been tried in a small
number of patients. Oral cisapride should not be given to patients receiving erythromycin, since
there is a potential risk of drug interaction leading to significant arrhythmia (torsades de pointes).

Prucalopride — Available in Europe and not in the United States, prucalopride is a 5HT4 receptor
agonist. Prucalopride accelerates transit through the stomach, small bowel, and colon [76].
Prucalopride appears promising in the treatment of CIPO. In contrast to cisapride, prucalopride
appears to have much lower risk of cardiac arrhythmia , even in the elderly. The recommended dose
of prucalopride in patients with CIPO is 2 mg daily in adults and 1 mg daily in those >65 years.
Cisapride — oral cisapride at a dose of 20 mg three times a day was effective in improving gastric
emptying but did not provide symptomatic relief in patients with CIPO . Cisapride has been
associated with a number of drug interactions and fatal cardiac arrhythmias

Metoclopramide — Intravenous metoclopramide is an alternative in patients with an acute

exacerbation of intestinal pseudo-obstruction who cannot tolerate erythromycin. Metoclopramide
may help initiate motility if the patient does not have prior evidence of adverse effects with
metoclopramide and cannot tolerate erythromycin. Thus, metoclopramide may help during acute
exacerbations at doses that are tolerated (usually 10 mg four times daily). A possible history of an
extrapyramidal reaction to this agent should be elicited prior to beginning therapy. There is no
evidence that metoclopramide is effective in the long-term treatment of pseudo-obstruction .

Octreotide — Patients with scleroderma may benefit from subcutaneous octreotide (a long-acting
somatostatin analogue). However, it is important to be aware that when octreotide is administered
during the daytime with meals, it significantly delays gastric emptying and small bowel transit of
solids [87,88], which may be deleterious to patients with CIPO. Therefore, it is recommended that if
octreotide is used in CIPO, it should be administered before bed, at least two hours after the last
meal, and its main purpose is to induce MMCs and thereby reduce the risk of bacterial overgrowth in
patients with CIPO. Symptomatic improvement with octreotide may reflect changes in visceral
afferent function rather than an effect upon transit through the intestine.

Neostigmine — Case reports have suggested that acute exacerbation of intestinal pseudo-
obstruction may respond to treatment with neostigmine (0.5 mg intramuscular or intravenous over
five minutes with cardiac monitoring). If the patient has received mu-opiates, there may also be a
response to subcutaneous methylnaltrexone (0.15 mg/kg). .

Antibiotics — Patients with CIPO who have steatorrhea, vitamin B12 malabsorption, or folate excess
may have bacterial overgrowth and should be treated empirically with antibiotics. Jejunal cultures
are needed if steatorrhea does not respond to empiric antibiotics. Jejunal aspirate is the preferred
test for diagnosing bacterial overgrowth in patients with CIPO as breath tests for bacterial
overgrowth have a high false negative rate in patients with motility disorders. The finding of ≥105
aerobic colony forming units/mL in the jejunal aspirate (normal jejunal concentration is ≤104 aerobic
colony forming units/mL) is consistent with bacterial overgrowth. Antibiotics are rotated with drug-
free intervals of at least 15 days in an attempt to avoid the development of bacterial resistance. In
some patients with severe myopathic disease, it may be necessary to use "rotating" antibiotics (eg,
week on, week off).

A pilot study demonstrated safety of using serial frozen fecal microbiota transplantation, symptom
relief (bloating, pain, and reduced intestinal dilatation on CT imaging) in selected patients with CIPO,
and improvement in patient tolerance of enteral nutrition delivered via a naso-jejunal tube.
Immunomodulator therapy — Case reports have described intestinal pseudo-obstruction in
association with lymphocytic infiltration of the myenteric plexus [1,94] or smooth muscle [95,96].
These observations suggest a potential role for immunomodulator therapy. Immunomodulator
therapy should be reserved for patients with CIPO due to an underlying inflammatory neuropathy
that is established by biopsy or by the presence of antineuronal antibodies (anti-Hu) [59]. The most
common immunosuppressive agents used are methylprednisolone or prednisone starting at 40 to 60
mg daily. In one case report, paraneoplastic pseudo-obstruction in the setting of small cell lung
cancer was reported to respond to rituximab and cyclophosphamide .
Surgery — The role of surgery in the management of CIPO is to provide access to the stomach or
small bowel for venting (decompression to relieve symptoms) and feeding, both of which may be
performed laparoscopically. Enteral feeding may thus facilitate avoidance of total parenteral
nutrition-related complications . Resection of localized disease should be avoided in patients with
CIPO. Clinical experience suggests that even though the disease may appear to be localized, it usually
becomes evident in the remaining bowel, thereby rendering the benefits of a bypass temporary.
Repeated surgery also leads to diagnostic confusion, making it difficult to distinguish CIPO from small
bowel obstruction. Subtotal enterectomy for pseudo-obstruction has been performed for relief of
severe pain associated with markedly distended loops of intestine. In such cases, the patient is
committed to total parenteral nutrition for life as a consequence of surgery. Bypass of dilated
segments has been suggested for patients with megaduodenum. However, in our experience, this
procedure has been ineffective in patients with persistent symptoms.

Pacing of the intestine and electrical stimulation of the stomach or intestine are considered
experimental at this time, although initial results have been favorable. In general, non-transplant
surgical management of CIPO should be avoided, as it is associated with high postoperative
morbidity and mortality rates and frequent re-operation

Percutaneous endoscopic colostomy — Colonoscopic insertion of a gastrostomy tube into the colon
to relieve obstructive symptoms has been described in case reports. While the safety and long-term
efficacy of this approach remain to be established, improvement in symptoms for up to two years
has been reported

Intestinal transplantation — Intestinal transplantation is indicated in patients in whom long-term

parenteral nutrition cannot be initiated or continued safely. Intestinal transplantation for patients
with motility disorders has been performed less frequently than for patients with short bowel
syndrome.

Graft rejection, graft-versus-host disease, and immunosuppression-related lymphoproliferative

disorders are more common with small intestinal transplantation than after other organ transplants
Intestinal transplantation may be life-saving in children, but is indicated only in patients in whom
long-term parenteral nutrition cannot be performed or continued safely including patients who
develop liver complications due to parenteral nutrition, have difficult central line access, or have
poor quality of life and worsening pain despite aggressive medical management [103-105]. Although
autologous stem cell transplantation and liver transplantation have been used on patients with
mitochondrial cytopathy, it is unclear if the benefits outweigh the risks

PROGNOSIS The long-term outcome is often especially poor in children, with 60 to 80 percent
requiring parenteral nutrition and a mortality rate ranging from 10 to 40 percent [110-112].

In adults with CIPO, the vast majority of patients have evidence of nutritional compromise, and
almost one-third require long-term home parenteral nutrition (HPN) . Mortality rates of
approximately 10 percent have been reported in adults, including HPN-related complications in 45 to
80 percent of cases

Acute colonic pseudo-obstruction (Ogilvie's syndrome)

Acute colonic pseudo-obstruction (Ogilvie's syndrome) is a disorder characterized by acute

dilatation of the colon in the absence of an anatomic lesion that obstructs the flow of intestinal
contents.
Acute colonic pseudo-obstruction usually occurs in hospitalized or institutionalized patients in
association with a severe illness or after surgery and in conjunction with a metabolic imbalance or
administration of culprit medication (table 1)

Trauma, especially fractures

Obstetrical surgery, especially involving spinal anesthesia

Pelvic, abdominal, or cardiothoracic surgery

Major orthopedic surgery

Severe medical illness, such as pneumonia, myocardial infarction, or heart failure

Neurologic conditions

Chemotherapy (eg, all-trans retinoic acid, methotrexate, vincristine)

Retroperitoneal pathology, such as malignancy or hemorrhage

One of the above plus metabolic imbalance or medication administration (eg, narcotics,
phenothiazines, calcium channel blockers, alpha-2-adrenergic agonists, epidural analgesics)

the most common predisposing conditions were nonoperative trauma, infection, and cardiac
disease, each of which were associated with 10 percent of cases .In this series, cesarean section and
hip surgery were the most common surgical procedures associated with acute colonic pseudo-
obstruction. 43 percent of patients had intestinal perforation or impending perforation, and 47
percent of patients required laparotomy, whereas conservative management was successful in 50
percent of cases. All patients with a cecal diameter of >12 cm perforated as compared to 3 of 17
patients with a diameter of <9 cm. Most perforations were diagnosed between day 3 and day 5
following the caesarian section.

Acute colonic pseudo-obstruction is also well-documented after kidney transplantation, and possible
contributing factors include obesity, cumulative dose of prednisone received, and mycophenolate
mofetil.

Acute colonic pseudo-obstruction usually involves the cecum and right hemicolon, although
occasionally colonic dilation extends to the rectum. Acute colonic pseudo-obstruction appears to be
more common in men and in patients over the age of 60 . In surgical patients, symptoms usually
present at an average of five days postoperatively.

In patients with acute colonic pseudo-obstruction, increasing colonic diameter accelerates the rise in
tension on the colonic wall, increasing the risk of colonic ischemia and perforation. The risk of
colonic perforation increases when cecal diameter exceeds 10 to 12 cm and when the distention has
been present for greater than six days. The duration of dilation is probably more important than the
absolute diameter of the colon.

The main clinical feature in patients with acute colonic pseudo-obstruction is abdominal distension.
Abdominal distension usually occurs gradually over three to seven days but may develop rapidly
within 24 to 48 hours. Approximately 80 percent of patients have associated abdominal pain.
Nausea and vomiting may be seen in up to 60 percent of patients. Constipation and, paradoxically,
diarrhea have also been reported in approximately 50 and 40 percent of patients, respectively

On physical examination, the abdomen is tympanitic, but bowel sounds are present. Approximately
65 percent of patients with a viable colon have mild abdominal tenderness on physical examination.
However, the presence of fever, marked abdominal tenderness, and the presence of peritoneal signs
(eg, guarding, rigidity, rebound tenderness) are suggestive of colonic ischemia or perforation or their
impending development.

The diagnosis of acute intestinal pseudo-obstruction should be suspected in patients with

abdominal distension or pain and a physical examination that reveals a distended and tympanitic
abdomen. The diagnosis of acute intestinal pseudo-obstruction is established by abdominal imaging.
Colonoscopy should not be used to make the diagnosis of acute intestinal pseudo-obstruction, as
insufflation of air may increase the colonic dilatation.

perform a complete blood count, electrolytes, and serum lactate levels, serum thyroid stimulating
hormone. serum aminotransferases, alkaline phosphatase, bilirubin, amylase, and lipase levels, In
patients with diarrhea, we also perform stool cultures and stool evaluation for Clostridioides
(formerly Clostridium) difficile toxin. . If present, leukocytosis is usually due to the patient's
underlying disease or impending perforation and is not associated with an uncomplicated pseudo-
obstruction. Metabolic abnormalities, especially hypokalemia, hypocalcemia, and hypomagnesemia,
are common, occurring in more than 50 percent of patients

perform an abdominal computed tomography (CT) scan to establish the diagnosis of acute intestinal
pseudo-obstruction and to rule out other causes of intestinal obstruction. In the absence of access
to a CT scan, a contrast enema using a water-soluble contrast can be used to establish the diagnosis,
provided that there is no evidence of peritonitis on physical examination.

●Abdominal CT scan – proximal colonic dilatation, often with an intermediate transitional zone at or
adjacent to the splenic flexure and a characteristically absent structural cause of colonic obstruction.

●Abdominal radiographs – Plain and upright abdominal radiographs show a dilated colon, often
from the cecum to the splenic flexure and occasionally to the rectum; haustral markings are normal
the role of abdominal radiographs is limited to monitoring the colonic diameter once the diagnosis
of acute colonic pseudo-obstruction has been established by abdominal CT or contrast enema.

DIFFERENTIAL DIAGNOSIS

Mechanical obstruction — Patients with mechanical obstruction frequently complain of crampy

abdominal pain; however, lack of pain, especially in the older adult or postoperative patient
receiving opiates, does not exclude that diagnosis. A "cut-off sign" (lack of gas in the distal colon or
rectum) or small bowel air-fluid levels on abdominal radiographs can also be seen in patients with
acute colonic pseudo-obstruction. However, a mechanical obstruction can be differentiated from
acute colonic pseudo-obstruction by visualization of an obstructing lesion on abdominal CT or
contrast enema. (

Toxic megacolon — Unlike patients with acute intestinal pseudo-obstruction, patients with toxic
megacolon have evidence of significant systemic toxicity with fever, tachycardia, altered sensorium,
and abdominal pain. Patients with toxic megacolon often have a history of severe bloody diarrhea or
other signs or symptoms of chronic inflammatory bowel disease. On abdominal upright and plain
films, there is evidence of colonic distension, but the normal colonic haustral pattern is either absent
or severely disturbed with "thumbprinting" due to the presence of submucosal edema, or thickening
of the colonic wall

MANAGEMENT — The goal of management is to decompress the colon in order to minimize the risk
of colonic perforation and ischemia, which are associated with a high mortality . Approach to
management
●Given the risk of colonic ischemia and perforation, patients with acute colonic pseudo-obstruction
should be carefully monitored with serial physical examinations and plain abdominal radiographs
every 12 to 24 hours to evaluate the colonic diameter. In addition, we perform laboratory tests
every 12 to 24 hours, including a complete blood count and electrolytes.

●Initial management of acute colonic pseudo-obstruction is usually conservative in patients without

significant abdominal pain, extreme (>12 cm) colonic distension, or signs of peritonitis and those
who have one or more potential factors that are reversible

In patients with cecal diameter >12 cm and in patients who have failed 24 to 48 hours of
conservative therapy, we use pharmacologic therapy with neostigmine. Resolution of acute colonic
pseudo-obstruction was significantly higher with only one dose of neostigmine

In patients who fail or who have contraindications to neostigmine, we perform colonoscopic

decompression. In patients whose acute colonic pseudo-obstruction may be precipitated by opiates,
we administer subcutaneous methylnaltrexone, prior to percutaneous or surgical decompression .
Because of the possibility of recurrence after the colonoscopic decompression, some authors
recommend use of a multiperforated rectal tube and oral or colonic administration of polyethylene
glycol laxative

Percutaneous colostomy should be reserved for patients who fail endoscopic decompression and are
not surgical candidates. We reserve surgical decompression for patients who fail endoscopic and
pharmacologic therapy or have evidence of perforation or peritonitis [21].

Supportive care — Supportive care with removal of precipitants is the first step in the management
of patients with acute colonic pseudo-obstruction. Supportive care can be continued for 24 to 48
hours in the absence of significant pain, extreme (>12 cm) colonic distension, or signs of peritonitis.
Supportive care includes the following:●Treatment of the underlying disease (eg, infection,
congestive heart failure).●Discontinuation of medications that can decrease colonic motility (eg,
opiates, calcium channel blockers, medications with anticholinergic side effects) and avoidance of
laxatives ●Patients should be given nothing by mouth. Intravenous fluids should be administered to
maintain normovolemia and electrolyte abnormalities corrected. ●Decompression of the
gastrointestinal tract by placement of a nasogastric tube attached to intermittent suction and a
rectal tube attached to gravity drainage. While gentle tap water enemas can be administered, their
use should be limited given the risk of perforation ●Patients should be encouraged to ambulate, if
possible. Patients should be placed in a prone position with the hips elevated on a pillow or the knee
chest position with the hips held high. These positions should be alternated with right and left lateral
decubitus positions each hour.

Neostigmine — Neostigmine, an acetylcholinesterase inhibitor, is indicated in patients with acute

colonic pseudo-obstruction and cecal diameter >12 cm or in patients who fail 24 to 48 hours of
conservative therapy. Neostigmine (2 mg) should be delivered by slow intravenous injection over
five minutes, with continuous monitoring of vital signs and electrocardiograph for 30 minutes and
continuous clinical assessment for 15 to 30 minutes . Patients should be kept supine on a bedpan,
and atropine should be available at the bedside to treat bradycardia associated with neostigmine. In
patients with successful colonic decompression, we administer polyethylene glycol electrolyte
balanced solution to decrease the risk of recurrence. Patients with a partial response or recurrence
after initial resolution should be treated with repeat administration of neostigmine [29]. It is the
author's practice not to repeat dosing within 24 hours.
In the author's experience, lower doses (1.5 mg) may also be effective and may possibly decrease
abdominal cramping, nausea, and vomiting. Some side effects (particularly bradycardia and
bronchoconstriction) might be reduced by coadministration of glycopyrrolate, an anticholinergic
agent that has limited activity on the muscarinic receptors of the colon.

●Adverse effects and contraindications – Adverse effects of neostigmine include bradycardia,

hypotension, asystole, seizures, restlessness, tremor, bronchoconstriction, nausea, vomiting,
salivation, diarrhea, sweating, and abdominal cramps. Relative contraindications to the use of
neostigmine include recent myocardial infarction, acidosis, asthma, bradycardia, peptic ulcer
disease, and therapy with beta-blockers. The use of neostigmine in pregnancy, although reported,
has not been well studied ●Efficacy – Approximately 88 to 94 percent of patients have
decompression of colonic distension with neostigmine. In patients treated with neostigmine, the
median time to response was four minutes (range 3 to 30 minutes)

Nonsurgical methods of colonic decompression in patients with acute colonic pseudo-obstruction

consist of colonoscopic decompression with or without placement of a decompression tube and
percutaneous decompression.

Colonoscopic decompression — Colonoscopic decompression is a technically difficult procedure and

has a perforation rate of approximately 3 percent [34]. It is contraindicated in patients with colonic
perforation or peritonitis. An oral bowel preparation should not be administered prior to
colonoscopy, due to the risk of aspiration in the presence of pseudo-obstruction. We do not
administer enemas prior to the colonoscopy, as there is little stool passed following their
administration because of the dilatation and lack of propulsion in the colon and the risk of colonic
perforation. We place a decompression tube with the aid of a guidewire at the time of colonoscopy
as this may reduce the need for repeated colonoscopic decompression. The guidewire is passed
through the channel of the colonoscope after reaching the distal transverse colon. Air is suctioned
from the colon and the wire left in place as the colonoscope is gently removed. The decompression
tube (customized with several extra side holes, if necessary) is then passed over the guidewire and
left in the transverse colon. To minimize air inflation, the whole colon should not be examined and
the guidewire should not be delivered into the cecum. The decompression tube should be placed to
gravity drainage and flushed every four to six hours. In case series, colonoscopic decompression
alone is definitive therapy in less than 50 percent of patients, but, with concurrent placement of a
decompression tube, success rates of 88 percent have been reported.

Percutaneous decompression — While percutaneous cecostomy may be effective for treatment of

acute colonic pseudo-obstruction, it is invasive and can be complicated by local infection and
bleeding [37-40]. In addition, percutaneous decompression has not been directly compared with
colonoscopic decompression or surgery. Percutaneous decompression can be accomplished by
endoscopically guided insertion of a plastic tube into the left colon or cecum, allowing
decompression and irrigation. Alternatively, tube placement in the right colon requires a combined
endoscopic and radiologic approach (fluoroscopic guidance) [12].

Surgery — In the absence of a colonic perforation, a surgically placed cecostomy tube or a segmental
or subtotal resection with primary anastomosis can be performed to decompress the colon [41]. In
the patients with a colonic perforation, a total colectomy, ileostomy, and Hartmann procedure are
performed in order to retain the option of future ileorectal anastomosis. The Hartmann procedure
involves resection of the diseased colon, an end-colostomy, and creation of a rectal stump; this is
followed by colostomy closure three months later.
Other — In case reports, patients with acute intestinal pseudo-obstruction have been treated with
erythromycin (250 mg IV every eight hours for three days or orally 250 mg four times daily for 10
days), but the responses to treatment have been inconsistent, with only gradual improvement over
12 to 24 hours of therapy . In one case report of acute colonic pseudo-obstruction in the setting of
opioid use that was refractory to neostigmine, administration of subcutaneous methylnaltrexone
resulted in successful decompression

Colonic ischemia and perforation are the two main complications of acute intestinal pseudo-
obstruction, which develop in approximately 3 to 15 percent of patients. The mortality rate in acute
intestinal pseudo-obstruction in the absence of complications is approximately 15 percent with early
appropriate management as compared with 36 to 44 percent in patients with a perforated or
ischemic bowel