Pneumatosis Intestinalis: when to worry?

Poster No.: C-1441

Congress: ECR 2013
Type: Educational Exhibit
Authors: F. Rego Costa, C. Maciel, C. Esteves, L. Melo; Porto/PT
Keywords: Gastrointestinal tract, Abdomen, CT, Conventional radiography,
Diagnostic procedure, Education, Education and training
DOI: 10.1594/ecr2013/C-1441

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Page 1 of 32
Learning objectives

Review the imaging features and clinical conditions associated with

pneumatosis intestinalis (PI) in the adult population;
Highlight key discriminatory imaging features between benign and life-
threatening causes;
Emphasize the importance of patient's overall clinical condition in the
interpretation of PI.

Background

Definition:

PI is an imaging sign (instead of a specific diagnosis/disease) that is the result of an

underlying pathologic process and can be defined as the presence of gas within the wall
of the gastrointestinal tract. All parts of the gastrointestinal tract may be affected but the
small bowel, the colon, or both are mostly involved. Extra intestinal location is rare.

Epidemiology:

PI in adults typically presents in the fifth to eighth decade. Although the incidence of PI
is low (about 0.03% in the general population), its detection appears to be increasing
mainly because of increased CT use, but also because of a real increase in incidence
(new drugs and surgical procedures are thought to be contributing).

Classification:

PI can be primary (15%) or secondary (85%) to a wide variety of underlying disorders:

Primary PI, also known as pneumatosis cystoides intestinalis, is an

asymptomatic, invariably benign idiopathic condition characterized by
multiple thin walled cysts in the bowel wall and its mesentery, containing air;
Secondary PI is traditionally divided into two categories: benign causes
and life-threatening causes. The most common life-threatening cause of PI
is intestinal ischemia.

Page 2 of 32
Table 1: Causes of secondary pneumatosis intestinalis in the adult population.
Some of the causes occur under both benign and life-threatening categories. COPD,
Chronic obstructive pulmonary disease; PEEP, Positive end-expiratory pressure; AIDS,
Acquired immunodeficiency syndrome.
References: - Porto/PT

Pathogenesis:

The pathogenesis of PI is poorly understood (the exact cause is not known), and the
range of pathologic conditions associated with its formation suggests that its development
is a multifaceted phenomenon. The current knowledge about the pathogenesis relies on
two main components/theories:

Mechanical: gas passes through the mural portion of the bowel wall. This
can occur for a number of reasons - mucosal disruption (inflammation,
necrosis, ulceration or trauma can injury the mucosa disturbing its normal
continuity); increased mucosal permeability (defective immune barrier as in
patients with immunodeficiency or treated with immunosuppressive/cytotoxic
medications); increased transabdominal pressure (bowel obstruction,
chronic obstructive pulmonary disease, trauma or vomiting can lead to direct
gas diffusion in the setting of an intact mucosal barrier). Usually, a variable

Page 3 of 32
 combination of these factors will contribute to the dissection of gas into
intramural compartments;

Bacterial: the origin of the gas. Bacteria are thought to support the presence
of intramural gas in PI mainly by two mechanisms: overgrowth and direct
invasion of the bowel wall - factors that favor the dissection of gas are
the same ones to facilitate bacterial invasion of intramural compartments;
modification of intraluminal gas content - this creates a gas gradient
between the intestinal lumen and blood (primarily through superproduction
of hydrogen) which will promote diffusion of gas across the resistive bowel
wall ("counterperfusion supersaturation" phenomenon).

Clinical features:

The signs and symptoms of PI are normally associated with the underlying disorder
rather than being a consequence of the presence of intramural gas. When PI is due to
benign causes, patients are usually asymptomatic. Some patients may have abdominal
pain, diarrhea, constipation, bloody stools or weight loss. Physical examination is rarely
abnormal unless there are peritoneal irritation signs in cases of PI due to life-threatening
causes. The pattern or extent of PI does not correlate well with the severity of symptoms
or of the underlying disease.

Laboratory data can provide important clues in determining if PI is life-threatening. The

combination of PI and a serum lactic acid level of > 2 mmol/L were associated with a
greater than 80% mortality rate.

Images for this section:

Page 4 of 32
Table 1: Causes of secondary pneumatosis intestinalis in the adult population. Some
of the causes occur under both benign and life-threatening categories. COPD, Chronic
obstructive pulmonary disease; PEEP, Positive end-expiratory pressure; AIDS, Acquired
immunodeficiency syndrome.

Page 5 of 32
Imaging findings OR Procedure details

PI can be detected on plain abdominal radiographs but CT is the most sensitive imaging
test and is usually required. Abdominal radiographs are frequently insensitive to detect
initial stages of PI or to evaluate the underlying pathology; CT is the best imaging
method for the diagnosis of PI because of its capacity for detecting even small intramural
gas collections, elucidate unclear radiographic findings and also to search for potential
causes.

Gas patterns of PI presented at CT vary considerably- linear, bubble, curvilinear or

circular low-density gas collections can be seen in the bowel wall. Sometimes there
is a combination of these patterns. Gas may be localized or diffuse throughout the
gastrointestinal tract. The circular form of PI is usually benign and most often seen
with pneumatosis cystoides intestinalis. Linear or bubble PI can be due to both benign
and life-threatening causes, and its appearance alone does not permit differentiation
between them.

Air trapped within bowel contents or between mucosal folds can mimic PI. This "pseudo-
pneumatosis" may be difficult to differentiate from true PI.

Gas distribution and "additional CT findings" are useful in the differentiation of "pseudo-
pneumatosis", benign PI and life-threatening causes of PI. The presence of the following
increases the possibility of PI due to a life-threatening cause and they should be actively
looked for:

Small extension of PI (within a specific vascular distribution)

Thickening of the bowel wall
Absent or intense mucosal enhancement
Dilated bowel due to obstruction
Hepatic portal or portomesenteric venous gas
Arterial or venous thrombus occlusion
Free intraperitoneal fluid (ascites)
Solid organ infarction (liver, spleen, kidney)
Mesenteric fat stranding

Intraperitoneal or retroperitoneal free air occupies a unique position. It can be seen with
PI due to life-threatening or benign causes (associated with the rupture of serosal and
subserosal cysts).

Page 6 of 32
Fig. 1: 75 year-old man came to the hospital with acute onset of colickly left flank pain.
Image (a) and (b): contrast-enhanced CT scan reveals intramural gas (pneumatosis)
of the caecum and ascending colon (blue arrows). Despite this pattern of localized PI,
there were no additional "worrisome" signs indicating a life-threatening cause. Image
(c) and (d): CT scan of the same patient shows pyelocalyceal ectasia with a pelvis
AP diameter of 17mm (orange arrow) which appears to be caused by an obstructive
6 mm ureteral stone (green arrow). There is also some stranding of the perinephric
fat (arrowhead). These findings are suggestive of the final diagnosis of obstructive
pyelonephritis. PI resolved with treatment of the underlying disorder.
References: - Porto/PT

Page 7 of 32
Fig. 2: 75 year-old man with chronic obstructive pulmonary disease, was hospitalized
because he had increased dyspnea and cough. Image (a) and (b): contrast-enhanced
CT scan shows bubbly parietal pneumatosis of the transverse colon (blue arrows),
without any additional CT findings. In this case, pneumatosis had a benign cause.
Patient was treated for a respiratory infection and then discharged.
References: - Porto/PT

Page 8 of 32
Fig. 3: 67 year-old woman with a history of microscopic polyangiitis. Image (a) and
(b): contrast-enhanced CT scan shows linear pneumatosis in a dilated caecum and
ascending colon (blue arrows). In this case, the presence of localized PI, bowel
dilation, diminished wall enhancement (blue arrows), mesenteric fat stranding (asterisk)
and a small amount of free intraperitoneal fluid (arrowheads) are indicators of a
possible life-threatening cause for PI. Patient underwent exploratory surgery which
confirmed the diagnosis of ischemic colitis.
References: - Porto/PT

Page 9 of 32
Fig. 4: 49 year-old woman suffering from chronic headache came to the emergency
department with severe abdominal pain after self medication with a NSAID. Image (a)
and (b): contrast-enhanced CT scan demonstrates parietal pneumatosis of the gastric
fundus (blue arrows). This finding alone could not be alarming (pneumatosis might be
a consequence of a peptic ulcer) but some additional signs point toward the severity
of the case: sudden onset of pain, thinning and diminished enhancement of the gastric
fundus wall (blue arrows), intraperitoneal free air (red arrow) adjacent to the stomach.
Altogether, these findings led to the diagnosis of gastric wall perfuration which was
confirmed surgically.
References: - Porto/PT

Page 10 of 32
Fig. 5: 82 year-old man presented with marked abdominal distension associated with
vague abdominal pain and without any signs of peritoneal irritation. Image (a): contrast-
enhanced CT scan shows caecum and ascending colon dilation with parietal thinning
and bubble-like pneumatosis (blue arrows). Image (b): CT scan of the same patient
indicates that the obstructive cause for the colon dilation was sigmoid and rectal fecal
impactation (green arrows).
References: - Porto/PT

Page 11 of 32
Fig. 6: 74 year-old man with psychiatric illness was brought to the hospital after
ingestion of nitric acid (corrosive agent). Image (a): CT scan demonstrates thinning
of the gastric and jejunal walls associated with linear pneumatosis (blue arrows).
Image (b): in one axial view, CT scan shows thinning of the gastric wall with linear
pneumatosis (blue arrow), free intraperitoneal air (red arrow) and a small amount of
hepatic portal venous gas (arrowhead). Image (c): irregularity and thickening of distal
esophagus (orange arrow) was another consequence of the ingestion of the corrosive
agent. Patient died 2 days after this CT despite surgical intervention.
References: - Porto/PT

Page 12 of 32
Fig. 7: 74 year-old man with a history of cerebral vascular disease suffered from
acute intense abdominal pain. Image (a): CT scan shows dilation of the caecum and
ascending colon, diminished wall enhancement and pronounced pneumatosis (blue
arrows). In addition, free intraperitoneal fluid (green arrow) is a contributing sign to the
picture of a life-threatening PI. Patient had ischemic colitis and was treated surgically.
References: - Porto/PT

Page 13 of 32
Fig. 8: 86 year-old woman presents with poorly localized severe abdominal pain
of acute onset, nausea and vomiting. Image (a): contrast-enhanced CT scan
demonstrates ileal dilation (periumbilical and right flank) with hypoenhancement and
bubbly parietal pneumatosis of the bowel wall (blue arrows). There is mesenteric fat
stranding (arrowhead). Image (b): CT scan of the same patient reveals hepatic portal
venous gas that was more prominent in the left lobe (red arrow). Image (c): 10mm
occlusive thrombus in the superior mesenteric artery (orange arrow) was discovered
to be the cause of the final diagnosis of mesenteric ischemia. Image (d): Abdominal
radiograph taken few minutes earlier does not clearly show pneumatosis, highlighting
its low sensitivity in the detection of this sign.
References: - Porto/PT

Page 14 of 32
Fig. 9: 85 year-old man presented with acute abdominal pain refractory to analgesics.
Image (a) and (b): contrast-enhanced CT scan shows parietal pneumatosis of a gastric
wall segment and some jejunal loops (blue arrows) together with portal venous gas
(red arrows), suggesting a worrisome cause of PI. Patient underwent surgery which
confirmed the diagnosis of gastric and jejunal ischemia.
References: - Porto/PT

Page 15 of 32
Fig. 10: 81 year-old woman with dementia was brought to the emergency department
because she was unresponsive to stimulus. Image (a), (b) and (c): contrast-enhanced
CT scan exhibits pneumatosis of numerous loops of small intestine (blue arrows)
along with portal venous gas (red arrows). Portal venous gas differs from biliary gas in
having a more peripheral location in the liver, whereas biliary air is more central. Image
(c): in this sagittal view, it is possible to spot arterial thrombosis of the thoracic aorta,
superior mesenteric and also a left atrial thrombus (orange arrows). The presence
of pneumatosis associated with these exuberant additional findings easily led to the
diagnosis of a life-threatening mesenteric ischemia. Patient died a few hours later.
References: - Porto/PT

Page 16 of 32
Fig. 11: 92 year-old man suffering from abdominal distention, obstipation and vague
abdominal discomfort when he was hospitalized. Image (a): contrast-enhanced CT
scan demonstrates localized pneumatosis of a dilated caecum (blue arrow). Image (b):
the cause for the dilation and consequent ischemic suffering of the right colon is shown
to be an obstructive tumor in the descending colon (green arrow).
References: - Porto/PT

Page 17 of 32
Fig. 12: 69 year-old woman was carried to the emergency department unconscious.
She was diagnosed with an acute myocardial infarction with cardiogenic shock
(Killip class IV). Image (a) and (b): CT scan reveals gastric dilation associated with
hypoenhancement and linear pneumatosis of the wall of the gastric fundus (blue
arrows). Despite the absence of additional CT findings pointing to a worrisome cause
of PI, in face of this clinical presentation, the most likely diagnosis is gastric ischemia
due to hypoperfusion. Patient died 1 day later.
References: - Porto/PT

Some typical CT findings of "pseudo-pneumatosis" are worth mention, because

awareness can prevent an erroneous diagnosis:

Limited to the caecum and ascending colon (because of an admixture of

liquid stool and gas);
Ending at a free gas-fluid level within the bowel lumen;
Irregularly punctuated gas column pattern.

Images for this section:

Page 18 of 32
Fig. 1: 75 year-old man came to the hospital with acute onset of colickly left flank pain.
Image (a) and (b): contrast-enhanced CT scan reveals intramural gas (pneumatosis) of
the caecum and ascending colon (blue arrows). Despite this pattern of localized PI, there
were no additional "worrisome" signs indicating a life-threatening cause. Image (c) and
(d): CT scan of the same patient shows pyelocalyceal ectasia with a pelvis AP diameter of
17mm (orange arrow) which appears to be caused by an obstructive 6 mm ureteral stone
(green arrow). There is also some stranding of the perinephric fat (arrowhead). These
findings are suggestive of the final diagnosis of obstructive pyelonephritis. PI resolved
with treatment of the underlying disorder.

Page 19 of 32
Fig. 2: 75 year-old man with chronic obstructive pulmonary disease, was hospitalized
because he had increased dyspnea and cough. Image (a) and (b): contrast-enhanced CT
scan shows bubbly parietal pneumatosis of the transverse colon (blue arrows), without
any additional CT findings. In this case, pneumatosis had a benign cause. Patient was
treated for a respiratory infection and then discharged.

Page 20 of 32
Fig. 3: 67 year-old woman with a history of microscopic polyangiitis. Image (a) and
(b): contrast-enhanced CT scan shows linear pneumatosis in a dilated caecum and
ascending colon (blue arrows). In this case, the presence of localized PI, bowel dilation,
diminished wall enhancement (blue arrows), mesenteric fat stranding (asterisk) and a
small amount of free intraperitoneal fluid (arrowheads) are indicators of a possible life-
threatening cause for PI. Patient underwent exploratory surgery which confirmed the
diagnosis of ischemic colitis.

Page 21 of 32
Fig. 4: 49 year-old woman suffering from chronic headache came to the emergency
department with severe abdominal pain after self medication with a NSAID. Image (a)
and (b): contrast-enhanced CT scan demonstrates parietal pneumatosis of the gastric
fundus (blue arrows). This finding alone could not be alarming (pneumatosis might be
a consequence of a peptic ulcer) but some additional signs point toward the severity
of the case: sudden onset of pain, thinning and diminished enhancement of the gastric
fundus wall (blue arrows), intraperitoneal free air (red arrow) adjacent to the stomach.
Altogether, these findings led to the diagnosis of gastric wall perfuration which was
confirmed surgically.

Page 22 of 32
Fig. 5: 82 year-old man presented with marked abdominal distension associated with
vague abdominal pain and without any signs of peritoneal irritation. Image (a): contrast-
enhanced CT scan shows caecum and ascending colon dilation with parietal thinning and
bubble-like pneumatosis (blue arrows). Image (b): CT scan of the same patient indicates
that the obstructive cause for the colon dilation was sigmoid and rectal fecal impactation
(green arrows).

Page 23 of 32
Fig. 6: 74 year-old man with psychiatric illness was brought to the hospital after ingestion
of nitric acid (corrosive agent). Image (a): CT scan demonstrates thinning of the gastric
and jejunal walls associated with linear pneumatosis (blue arrows). Image (b): in one axial
view, CT scan shows thinning of the gastric wall with linear pneumatosis (blue arrow),
free intraperitoneal air (red arrow) and a small amount of hepatic portal venous gas
(arrowhead). Image (c): irregularity and thickening of distal esophagus (orange arrow)
was another consequence of the ingestion of the corrosive agent. Patient died 2 days
after this CT despite surgical intervention.

Page 24 of 32
Fig. 7: 74 year-old man with a history of cerebral vascular disease suffered from acute
intense abdominal pain. Image (a): CT scan shows dilation of the caecum and ascending
colon, diminished wall enhancement and pronounced pneumatosis (blue arrows). In
addition, free intraperitoneal fluid (green arrow) is a contributing sign to the picture of a
life-threatening PI. Patient had ischemic colitis and was treated surgically.

Page 25 of 32
Fig. 8: 86 year-old woman presents with poorly localized severe abdominal pain of
acute onset, nausea and vomiting. Image (a): contrast-enhanced CT scan demonstrates
ileal dilation (periumbilical and right flank) with hypoenhancement and bubbly parietal
pneumatosis of the bowel wall (blue arrows). There is mesenteric fat stranding
(arrowhead). Image (b): CT scan of the same patient reveals hepatic portal venous gas
that was more prominent in the left lobe (red arrow). Image (c): 10mm occlusive thrombus
in the superior mesenteric artery (orange arrow) was discovered to be the cause of
the final diagnosis of mesenteric ischemia. Image (d): Abdominal radiograph taken few
minutes earlier does not clearly show pneumatosis, highlighting its low sensitivity in the
detection of this sign.

Page 26 of 32
Fig. 9: 85 year-old man presented with acute abdominal pain refractory to analgesics.
Image (a) and (b): contrast-enhanced CT scan shows parietal pneumatosis of a gastric
wall segment and some jejunal loops (blue arrows) together with portal venous gas (red
arrows), suggesting a worrisome cause of PI. Patient underwent surgery which confirmed
the diagnosis of gastric and jejunal ischemia.

Page 27 of 32
Fig. 10: 81 year-old woman with dementia was brought to the emergency department
because she was unresponsive to stimulus. Image (a), (b) and (c): contrast-enhanced
CT scan exhibits pneumatosis of numerous loops of small intestine (blue arrows) along
with portal venous gas (red arrows). Portal venous gas differs from biliary gas in having
a more peripheral location in the liver, whereas biliary air is more central. Image (c): in
this sagittal view, it is possible to spot arterial thrombosis of the thoracic aorta, superior
mesenteric and also a left atrial thrombus (orange arrows). The presence of pneumatosis
associated with these exuberant additional findings easily led to the diagnosis of a life-
threatening mesenteric ischemia. Patient died a few hours later.

Page 28 of 32
Fig. 11: 92 year-old man suffering from abdominal distention, obstipation and vague
abdominal discomfort when he was hospitalized. Image (a): contrast-enhanced CT scan
demonstrates localized pneumatosis of a dilated caecum (blue arrow). Image (b): the
cause for the dilation and consequent ischemic suffering of the right colon is shown to
be an obstructive tumor in the descending colon (green arrow).

Page 29 of 32
Fig. 12: 69 year-old woman was carried to the emergency department unconscious. She
was diagnosed with an acute myocardial infarction with cardiogenic shock (Killip class IV).
Image (a) and (b): CT scan reveals gastric dilation associated with hypoenhancement and
linear pneumatosis of the wall of the gastric fundus (blue arrows). Despite the absence
of additional CT findings pointing to a worrisome cause of PI, in face of this clinical
presentation, the most likely diagnosis is gastric ischemia due to hypoperfusion. Patient
died 1 day later.

Page 30 of 32
Conclusion

As an imaging sign, PI lacks interpretation. The importance of PI depends on the nature

and severity of the underlying condition. When detected, it must be first integrated
in the clinical context. A careful look for life-threatening signs, namely "additional
CT findings", integration with clinical symptoms, physical examination, laboratory test
results and knowledge of the patient clinical background are essential tools to a weighted
approach.

References

Morris M, Gee A, Cho S, Limbaugh K, Underwood S, Ham B, Schreiber M.

Management and outcome of pneumatosis intestinalis. Am J Surg 2008;
195:679-682.
Ho L, Paulson E, Thompson W. Pneumatosis Intestinalis in the Adult:
Benign to Life-Threatening Causes. AJR 2007; 188:1604-13.
Kernagis L, Levine M, Jacobs J. Pneumatosis intestinalis in patients with
ischemia: correlation of CT findings with viability of the bowel. AJR
2003; 180:733-736.
St Peter S, Abbas M, Kelly K. The spectrum of pneumatosis intestinalis.
Arch Surg. 2003; 138:68-75.
Wiesner W, Mortele K, Glickman J, Ji H, Ros P. Pneumatosis intestinalis
and portomesenteric venous gas in intestinal ischemia: correlation of
CT findings with severity of ischemia and clinical outcome. AJR 2001;
177:1319-1323.
Hosomi N, Yoshioka H, Kuroda C. Pneumatosis Cystoides Intestinalis:
CT findings. Abdominal Imaging 1994; 19:137-9.
Feczko P, Mezwa D, Farah M, White B. Clinical significance of
pneumatosis of the bowel wall. Radiographics 1992; 12:1069-1078.

Personal Information

Francisco Rego Costa

Email: franciscoregocosta@gmail.com

Radiology Department, Hospital de So Joo - Porto, Portugal

Oporto Medical University

Page 31 of 32
Head of Department: Prof. Dra. Isabel Ramos

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