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Ho et al.

Abdominal Imaging • Review


Pneumatosis Intestinalis in
the Adult

Pneumatosis Intestinalis in the Adult:


Benign to Life-Threatening Causes
Lisa M. Ho1 OBJECTIVE. The frequency of detection of pneumatosis intestinalis (PI) appears to be in-
Erik K. Paulson creasing. This increase may be the result of increased CT use. New medications and surgical pro-
William M. Thompson cedures have been reported to be associated with an increase in the incidence of PI. The purpose of
this review is to provide an update on the imaging features and clinical conditions associated with PI.
Ho LM, Paulson EK, Thompson WM CONCLUSION. This article illustrates the imaging findings of PI due to benign and life-
threatening causes, with emphasis placed on describing newly associated conditions and also
the imaging appearance on CT.

neumatosis intestinalis (PI) is de- In this article, we divided PI into two cat-

P fined as the presence of gas in the


bowel wall [1–4]. This imaging
finding is associated with numer-
egories: benign causes and life-threatening
causes (Appendix 1). It is important to un-
derstand that PI is a sign not a disease, and
ous conditions, ranging from benign to life it must be interpreted relative to the pa-
threatening [1–5]. The overall incidence of tient’s overall clinical condition. Therefore,
PI in the general population has been re- clinical symptoms and laboratory data pro-
ported to be 0.03% based on an autopsy se- vide the most important clues in determin-
ries [4]. Although PI can be seen on abdom- ing whether PI is due to benign or life-
inal radiographs, CT is the most sensitive threatening causes.
imaging test for identification of PI [6]. The
CT detection of PI appears to be increasing, Pathogenesis
likely as a consequence of increased use of Although the cause of PI appears to be
CT technology [7]. Increased imaging detec- multifactorial, the exact cause is not known.
tion of PI could also be due to an increased Two main theories have been proposed in
incidence of PI. Relatively new surgical pro- the medical literature. A mechanical theory
cedures and medications associated with PI hypothesizes that gas dissects into the bowel
may be contributing to an increase in inci- wall from either the intestinal lumen or the
dence of PI. The aim of this article is to de- lungs via the mediastinum [1] due to some
scribe the imaging appearance and clinical mechanism causing increased pressure (i.e.,
findings of PI in the adult population. bowel obstruction or emphysema). A bacte-
rial theory proposes that gas-forming bacilli
Classification System enter the submucosa through mucosal rents
Keywords: colon, CT, gastrointestinal radiology, ischemia,
small bowel
In 1754, Duvernoy wrote the first report of or increased mucosal permeability and pro-
PI, which appeared in the French literature duce gas within the bowel wall [1].
DOI:10.2214/AJR.06.1309 [8]. Since then, numerous case reports and re- Studies have shown that gas collections in
views have appeared in the world literature. In the bowel wall can have a hydrogen content
Received October 5, 2006; accepted after revision
1998, Pear [5] undertook the most recent of up to 50%. Hydrogen is a product of bac-
December 29, 2006.
comprehensive review in the U.S. radiology terial metabolism and is not produced by hu-
1All authors:
Department of Radiology, Duke University literature. His classification scheme was man cells [1]. The major argument against
Medical Center, Box 3808 DUMC, Durham, NC 27710. based on the current evidence and theories re- the bacterial theory is that long-standing
Address correspondence to L. M. Ho (lisa.ho@duke.edu). garding the cause and clinical significance of pneumoperitoneum can occur with PI and
AJR 2007; 188:1604–1613
PI. In his review, PI was classified pathogen- rarely is it associated with peritonitis [8]. A
ically into four categories: bowel necrosis, combination of both theories is also plausi-
0361–803X/07/1886–1604
mucosal disruption, increased mucosal per- ble. Bacterial overgrowth in the gastrointes-
© American Roentgen Ray Society meability, and pulmonary disease. tinal tract from a variety of causes can lead

1604 AJR:188, June 2007


Pneumatosis Intestinalis in the Adult

Fig. 1—Examples of
linear and bubbly
pneumatosis intestinalis
(PI).
A, Abdominal CT image in
54-year-old woman
shows extraluminal gas
tracking along small
bowel mesentery (black
arrowhead) and linear PI
(arrows) in this case of PI
associated with
jejunostomy tube (white
arrowhead).
B, Abdominal CT image in
56-year-old man shows
bubbly PI (arrows) and
free air (arrowheads) in
this case of PI in patient
on chemotherapy for
colon cancer.

A B

Fig. 2—69-year-old
woman with guaiac-
positive stool—benign
cause of pneumatosis
intestinalis (PI).
A, Scout radiograph from
air-contrast barium
enema shows cystic PI
(arrow) consistent with
pneumatosis cystoides
intestinalis.
B, Spot film images from
air-contrast barium
enema show polypoid
filling defects (arrows)
due to gas in bowel wall
(arrowheads) from
pneumatosis cystoides
intestinalis.
A B

to excessive hydrogen gas production, discomfort, which is usually related to the vated serum lactic acid of > 2 mmol/L [3].
bowel distention, and subsequently, dissec- underlying associated medical condition. A recent study found that the combination
tion of intraluminal hydrogen gas into the Physical examination is rarely abnormal un- of PI and a serum lactic acid level of > 2
bowel wall. less there are peritoneal signs from intesti- mmol/L was associated with a greater than
nal perforation in cases of PI due to life- 80% mortality rate [9].
Clinical Features threatening causes. Laboratory values in the
In cases of PI due to benign causes, espe- presence of intestinal ischemia may reveal Imaging Methods and Findings
cially PI associated with pulmonary disease, acidosis with a blood pH of < 7.3, a hyper- Abdominal radiography and CT are the
the patients are usually asymptomatic [1–4]. amylasemia of > 200 IU/L, a serum bicar- most frequently used techniques for diagno-
Some patients may have mild abdominal bonate level of < 20 mmol/L, and an ele- sis of PI. CT has been shown to be more sen-

AJR:188, June 2007 1605


Ho et al.

Fig. 3—69-year-old man wall in the coronal, sagittal, and axial planes
on chemotherapy for may allow a more confident diagnosis of PI
head–neck cancer with
mild abdominal pain— and portal venous gas.
benign cause of On both radiographs and CT, PI usually
pneumatosis intestinalis appears as a low-density linear or bubbly
(PI).
A, Abdominal CT image
pattern of gas in the bowel wall (Fig. 1). It
using soft-tissue window can be a combination of both linear and bub-
setting shows PI of bly bowel-wall gas. There also may be cir-
cecum and ascending cular collections of gas in the bowel wall
colon (arrows).
B, Abdominal CT image (Fig. 2). Occasionally, bowel contents
shows PI of cecum and mixed with air or air trapped between mu-
ascending colon cosal folds can mimic PI. Viewing CT im-
(arrows) is much better
seen using lung window ages with lung windows may accentuate the
setting. Patient improved detection of PI, especially in the colon [11]
without any special (Fig. 3). Because CT is more sensitive than
therapy.
radiography in detecting PI, CT can be used
to clarify ambiguous radiographic findings
and also to search for potential causes [11].
The circular form of PI is usually benign
and most often seen with pneumatosis cys-
toides intestinalis (PCI). Linear or bubble-
like PI can be due to both benign and life-
threatening causes, and its radiographic or
A
CT appearance alone does not allow differ-
entiation between them. In PI due to benign
causes, the bowel wall is usually normal.
The presence of additional findings such as
bowel wall thickening, absent or intense
mucosal enhancement, dilated bowel, arte-
rial or venous occlusion, ascites, and he-
patic portal or portomesenteric venous gas
increases the possibility of PI due to a life-
threatening cause [15, 17] (Fig. 4). PI that is
confined to a portion of the small or large
bowel within a specific vascular distribution
also increases the likelihood that ischemia is
the cause of PI. Intraperitoneal or retroperi-
toneal free air can be seen with PI due to
life-threatening or benign causes [6, 18–20].
The association of spontaneous pneumo-
peritoneum with PI has been attributed to
the rupture of serosal and subserosal cysts in
the bowel wall [8].
Portal venous gas is differentiated from
biliary gas by its characteristic tubular
branching lucencies that extend to the pe-
riphery of the liver, whereas biliary air is
B
more central (Fig. 5). The use of coronal re-
formatted images with MDCT may improve
detection of portomesenteric gas owing to
the oblique vertical orientation of the mesen-
sitive than radiography at detecting PI further improve the detection of PI and he- teric vessels [15, 21].
[10–14]. CT has also been shown to be more patic portal and portomesenteric venous Several reports in the literature have ad-
sensitive than radiography at detection of gas; 16- and 64-MDCT scanners are capable dressed the capability of CT of distinguish-
hepatic portal and portomesenteric venous of generating isotropic data sets that allow ing early and nontransmural mesenteric is-
gas [12, 15, 16], the presence of which in- multiplanar reformations with a spatial res- chemia from full-thickness and irreversible
creases the possibility of PI due to life- olution similar to or even greater than the transmural infarction [17, 22, 23]. Both Ker-
threatening causes. Advances in CT may axial plane. The ability to study the bowel nagis et al. [23] and Weisner et al. [22] found

1606 AJR:188, June 2007


Pneumatosis Intestinalis in the Adult

A
Fig. 4—79-year-old woman after recent surgery for gastric cancer. Patient developed
abdominal pain and blood pH, 7.24; lactic acid, 8.1 mmol/L; and plasma bicarbonate
(HCO3), 18 mmol/L—life-threatening cause of pneumatosis intestinalis (PI).
A, Supine digital abdominal radiograph shows free air (arrows), small-bowel
distention, and small-bowel PI (arrowheads).
B and C, Abdominal CT images show free air (long arrows) and small-bowel PI (short
arrows, C) but also hepatic portal venous gas (arrowheads, B) not seen on
radiograph. At surgery, diffuse ischemia of small bowel was found. Patient died 1
week later.

that linear PI was seen more frequently than Although the discovery of hepatic portal with hepatic portal and portomesenteric
bubbly PI in patients with transmural bowel or portomesenteric venous gas helps to dis- venous gas [15, 21, 22, 24–27].
infarction. Furthermore, both research stud- tinguish between benign and life-threatening Sonography can also be used to detect PI
ies found that the detection of PI in associa- causes of PI, it may also occur with or with- [28, 29]. This technique is more commonly
tion with portomesenteric venous gas corre- out PI as a result of nonischemic conditions. applied to the pediatric patient in whom
lated strongly with transmural bowel Mesenteric abscess formation, portomesen- avoidance of ionizing radiation is preferred
infarction, whereas PI without evidence of teric thrombophlebitis, sepsis, abdominal [30]. PI seen on sonography has been de-
portomesenteric venous gas was frequently trauma, severe enteritis, cholangitis, chronic scribed as linear or focal echogenic areas
seen in cases of nontransmural intestinal is- cholecystitis, pancreatitis, inflammatory within the bowel wall [31]. It can also ap-
chemia. The overall survival rate was higher bowel disease, and diverticulitis and after pear as a continuous echogenic ring in the
in patients with nontransmural intestinal is- gastrointestinal surgery or liver transplanta- bowel wall [32].
chemia compared with those patients with tion are some of the various nonischemic Rarely, PI can also be seen on MRI.
transmural intestinal infarction. clinical conditions that have been associated Rabushka and Kuhlman [33] described two

AJR:188, June 2007 1607


Ho et al.

A B
Fig. 5—Comparison of hepatic portal venous gas and biliary gas in two different patients.
A, 23-year-old woman after heart transplant admitted for mild rejection but no abdominal symptoms and normal laboratory results. Abdominal CT image shows hepatic portal
venous gas in periphery of liver (arrows).
B, 60-year-old man after Whipple procedure for pancreatic cancer. Abdominal CT image shows gas in bile ducts in central part of liver (arrowheads).

cases of PI seen with MR. They found cir-


cumferential collections of air adherent to or
within the bowel wall that became more ap-
parent on gradient-echo images due to bloom-
ing artifact associated with magnetic field in-
homogeneities at air–tissue interfaces.

Benign Causes of PI
Appendix 1 lists benign causes of PI in
the adult. The number of benign conditions
associated with PI appears to be increasing.
This observation may be the effect of in-
creased use of cross-sectional imaging. In
most cases, the natural history of PI due to
benign causes is not known because there is
often no imaging follow-up. Spontaneous
resolution and recurrent episodes have been
described in the literature [4, 8, 34]. PCI is
Fig. 6—51-year-old man one subset of PI that is invariably benign.
after lung transplant for PCI is characterized by circular collections
cystic fibrosis. Patient of gas in the bowel wall and its mesentery
had free air on routine
chest radiograph and no [11, 34] (Fig. 2). It almost always occurs in
abdominal symptoms the colon. On barium enema studies, it can
and normal laboratory mimic polyps when viewed en face
results—benign cause of
(Fig. 2B), but in profile the gas cysts can be
pneumatosis intestinalis
(PI). clearly identified within the colon wall
A–C, Digital abdominal (Fig. 2B).
radiograph (A) and Pulmonary causes of PI are usually benign
abdominal CT images (B
and C) show free air and range from congenital to acquired. Cystic
(arrows, A and B) and fibrosis, asthma, and chronic obstructive pul-
diffuse linear PI of colon monary disease have a well-known associa-
(arrowheads). Patient
was observed and
tion with PI [35, 36]. PI has been reported in
discharged. patients who have undergone organ transplan-
(Fig. 6 continues on next tation [37, 38]. We have encountered a num-
page) ber of cases (Fig. 6) after lung transplantation
A [18, 39–41]. Although steroid therapy is one

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Pneumatosis Intestinalis in the Adult

B C
Fig. 6 (continued)—51-year-old man after lung transplant for cystic fibrosis. Patient had free air on routine chest radiograph and no abdominal symptoms and normal
laboratory results—benign cause of pneumatosis intestinalis (PI).
A–C, Digital abdominal radiograph (A) and abdominal CT images (B and C) show free air (arrows, A and B) and diffuse linear PI of colon (arrowheads). Patient was observed
and discharged.

A B
Fig. 7—27-year-old woman with history of scleroderma who presented with abdominal distention. Physical examination and laboratory results were normal—benign cause
of pneumatosis intestinalis (PI).
A, Supine abdominal radiograph shows PI (arrows) of small bowel.
B, Upright abdominal radiograph shows pneumoperitoneum (arrows).

AJR:188, June 2007 1609


Ho et al.

A B
Fig. 8—Patients with sudden onset of abdominal pain—life-threatening cause of
pneumatosis intestinalis (PI).
A, Supine abdominal radiograph in 60-year-old man shows PI of small bowel
(arrows).
B, Superior mesenteric arteriogram of same patient as A shows acute thrombosis
(arrows) resulting in small-bowel ischemia and infarction. Patient died.
C, Abdominal CT in 65-year-old woman shows acute thrombus (arrows) in superior
mesenteric artery.

possible cause of PI in the post–lung trans- is a common opportunistic infection in lung tive therapy that includes bowel rest and em-
plantation period, cytomegalovirus (CMV) transplant recipients, which can manifest as piric antiviral medication.
colitis has also been implicated as a cause of gastrointestinal disease [18]. In our experi- Systemic diseases and intestinal disor-
PI in the lung transplant patient. CMV colitis ence, these patients respond well to conserva- ders make up a large number of causes of PI.

1610 AJR:188, June 2007


Pneumatosis Intestinalis in the Adult

Fig. 9—19-year-old man with toxic megacolon due to Crohn’s disease—life-


threatening cause of pneumatosis intestinalis (PI). Emergency colectomy was
performed.
A and B, Supine (A) and upright (B) abdominal radiographs show diffuse PI of colon
(arrows).

A B

These include collagen vascular disease mucosal structural integrity and allow Conclusion
such as scleroderma [42, 43] (Fig. 7) and in- dissection of intraluminal air into the intesti- There are many benign and life-threatening
flammatory bowel disease [19, 44, 45]. John nal wall. PI has been associated with medica- causes of PI. The imaging appearance of both
et al. [45] reported that CT evidence of PI in tions that cause bowel distention or diarrhea. may look very similar. Therefore, correlation
patients with Crohn’s disease usually corre- The development of PI in cancer patients has with clinical history, physical examination,
lated with a higher severity of disease. How- also been attributed to several chemothera- and laboratory test results is the best indicator
ever, the presence of PI in these patients did peutic agents [4, 51]. Sorbitol, lactulose, and of whether PI is due to a benign or life-threat-
not dictate a specific course of treatment, voglibose have also been reported to cause PI. ening cause. PCI is one subset of PI that is al-
and therapy was based on the overall clini- In most of these cases, PI resolved with dis- most always benign. In cases of PI associated
cal picture. continuation of the medication [4, 52–55]. with suspected bowel ischemia, the additional
The association of PI with AIDS was fre- detection of hepatic portal or portomesenteric
quently reported in the early days of the HIV Life-Threatening Causes of PI venous gas increases the likelihood of trans-
epidemic [20, 46, 47]. However, this finding Mesenteric ischemia (Fig. 4) is the most mural bowel infarction.
appears to have become less common, pre- common life-threatening cause of PI (Appen-
sumably due to the effectiveness of new med- dix 1). Occasionally, thromboembolization is
ications and treatments for patients with HIV. proven as a cause of the ischemia [6] (Fig. 8), References
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APPENDIX 1. Causes of Pneumatosis Intestinalis in the Adult: Benign and Life-Threatening Causes and Associations

A. Benign causes Medications


• Corticosteroids
Pulmonary
• Chemotherapeutic agents
• Asthma
• Lactulose
• Bronchitis
• Sorbitol
• Emphysema
• Voglibose
• Pulmonary fibrosis
• Positive end-expiratory pressure (PEEP) Organ transplantation
• Cystic fibrosis • Bone marrow
• Kidney
Systemic disease
• Liver
• Scleroderma
• Cardiac
• Systemic lupus
• Lung
• AIDS
• Graft versus host
Intestinal causes
Primary pneumatosis
• Pyloric stenosis
• Idiopathic (up to 15% of cases and usually involves the colon)
• Intestinal pseudoobstruction
• Pneumatosis cystoides intestinalis
• Enteritis
• Peptic ulcers
B. Life-threatening causes
• Bowel obstruction
• Adynamic ileus Intestinal ischemia
• Inflammatory bowel disease
Mesenteric vascular disease
• Ulcerative colitis
• Crohn’s disease Intestinal obstruction (especially strangulation)
• Leukemia
• Perforated jejunal diverticulum Enteritis
• Whipple’s disease Colitis
• Intestinal parasites
• Collagen vascular disease (especially scleroderma) Ingestion of corrosive agents
• Diverticulitis Toxic megacolon
Iatrogenic Trauma
• Barium enema
• Jejunoileal bypass Organ transplantation (especially bone marrow transplants)
• Jejunostomy tubes Collagen vascular disease
• Postsurgical anastomosis
• Endoscopy

Note—A number of causes and associations occur under both benign and life-threatening categories.

AJR:188, June 2007 1613

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