Intestinal pneumatosis: differential

diagnosis

Giulia Lassandro, Stefano Giusto

Picchi, Federica Romano, Giacomo
Sica, Roberta Lieto, Giorgio Bocchini,
Salvatore Guarino, et al.
Abdominal Radiology

ISSN 2366-004X

Abdom Radiol
DOI 10.1007/s00261-020-02639-8

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https://doi.org/10.1007/s00261-020-02639-8

SPECIAL SECTION: INTESTINAL ISCHEMIA

Intestinal pneumatosis: differential diagnosis

Giulia Lassandro1 · Stefano Giusto Picchi1 · Federica Romano2 · Giacomo Sica2 · Roberta Lieto2 · Giorgio Bocchini2 ·
Salvatore Guarino2 · Francesco Lassandro2 

Received: 17 March 2020 / Revised: 21 June 2020 / Accepted: 4 July 2020

© Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract
Intestinal pneumatosis (IP) is an infrequent radiological sign defined as pathological gas infiltration into the bowel wall. It
may be associated to different underlying clinical conditions—inflammatory bowel diseases, malignancies, chemotherapy,
infections, immune deficiency status, trauma, intestinal ischemia, and necrosis—that are often related to emergency state
and require a prompt diagnosis. All the imaging techniques, especially abdominal radiography and Computed Tomography,
could detect the presence of IP and discern the forms related to emergency conditions. The differential diagnosis is essential
to start an immediate clinical or surgical management and treatment. The aim of this article is to review the radiological
features of IP in different illnesses, with particular attention to differential diagnosis.

Keywords  Pneumatosis · Intestinal ischemia · Computed tomography · Emergency radiology · Necrotizing enterocolitis ·
Pneumatosis cystoides intestinalis

Abbreviations to porto-mesenteric vein gas (PMVG) or pneumoperitoneum

IP Intestinal pneumatosis (PNP) [1, 3].
NEC Necrotizing enterocolitis With the advent of more advanced radiological techniques
PMVG Porto-mesenteric vein gas such as computed tomography (CT), IP has been more fre-
PNP Pneumoperitoneum quently observed in many other clinical conditions, includ-
CT Computed tomography ing inflammatory bowel disease, connective tissue disease,
PCI Pneumatosis cystoides intestinalis surgery, immune deficiency or chemotherapy, with a wide
variety of possible outcomes [4, 5], therefore the prognostic
value of IP has been revised.
Introduction

Intestinal pneumatosis (IP) is a rare condition that affects Classification and physiopathology

0.03% of the population [1]. It is defined as pathological
gas infiltration into the bowel wall. IP has been known for a IP can be classified into primary (15% of cases) and second-
long time: it was first described by Du Vernoy in 1730 [2]. ary forms (85%). The two forms are clearly different entities
Conventional radiology has a high sensitivity in recog- with their own clinical significance and disparate therapeutic
nizing air and so it has proved useful in the diagnosis of approaches [6] (Fig. 1).
IP. Radiological detection of IP has been related to intes- The primary form consists of Pneumatosis Cystoides
tinal infarction in adults and to necrotizing enterocolitis Intestinalis (PCI), an idiopathic gas infiltration of the bowel
(NEC) in infants. In any case, the IP discovery has long wall that resembles a bunch of grapes (Fig. 2).
been considered a medical emergency, especially if related Secondary forms of IP are more frequent and associated
to a wide range of pathological patterns, more often related
to acute intestinal ischemia. Thus, it is a radiological sign
* Francesco Lassandro
[email protected]
associated with a variety of pathological conditions and is
not a nosographic entity itself.
1
Department of Advanced Biomedical Sciences, Federico II The etiology of IP is unclear, in 1998 Pear introduced
University, Naples, Italy a classification of the major pathogenic mechanisms
2
Department of Radiology, Monaldi Hospital, Naples, Italy

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Fig. 1  Classification of IP in
Primary and Secondary forms

Fig. 2  This is the case of a 62 yo female with unexpected pneuma- demonstrate multiple gas-filled cysts in the submucosa of the bowel
tosis cystoides intestinalis at a CT examination who underwent for walls (a, b, arrows) and free peritoneal air (c)
pelvic cancer follow-up. Sagittal (a) and axial (b, c) reconstructions

causally involved in intramural gas formation, which Over time, in addition to Pear’s classification, three
included (i) bowel necrosis, (ii) mucosal disruption, (iii) pathogenetic hypotheses have been accepted:
increased mucosal permeability and (iv) pulmonary dis-
eases [7]. It is probable that different mechanisms may (i) mechanical hypothesis—increase of intraluminal
work in different conditions to produce IP. pressure resulting in mechanical damage to the bowel

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wall and leading to gas migration from the gastroin-

testinal lumen to the intestinal wall [8];
(ii) bacterial hypothesis—aerogenic bacteria that pen-
etrate the bowel mucosal barrier and ferment in the
intestinal wall [9];
(iii) pulmonary hypothesis—in chronic lung diseases,
such as chronic obstructive pulmonary disease
(COPD) and asthma, increased thoracic pressure
causes alveolar rupture and gas diffusion towards the
gastrointestinal serosa by following perivascular or
perilymphatic routes [9, 10].
Fig. 4  Iatrogenic IP after endoscopic positioning of clips in a bleed-
IP is a relevant radiological sign due to its strict correla- ing gastric ulceration. Gas was reabsorbed spontaneously and the
tion to intestinal infarct. Bowel necrosis could cause intesti- patient recovered without any other medical problem
nal wall gas infiltration, eventually spreading in mesenteric
and portal vessels (Fig. 3a–e) [11]. bowel obstruction (Fig. 5) or in the pyloric obstruction
Mucosal disruption has been associated with iatrogenic of the newborn may result in gastrointestinal passage of
lesions (i.e., nasogastric tube or endoscopic maneuvers) air [9].
(Fig. 4) [12] and to blunt abdominal trauma, where the In addition, mucosal damage and increased mucosal
sudden compression of intragastric gas can dissect the permeability may be the reason why IP is observed in
mucosa passing into the gastric wall and mesenteric veins conditions like cancer (Fig. 6), inflammatory diseases and
[13]. Similarly, an increased intraluminal pressure in intestinal infections [14].

Fig. 3  IP in patients with intestinal infarcts. a Small amount of gas in different patterns of IP, from bubble-like (white arrows) to circum-
small bowel loop (arrows). b A predominant bubble-like pattern of IP ferential (black arrows), presence of mesenteric fluid and fat strand-
(arrows) in a not too advanced phase. c Coronal reconstruction. An ing. e IP (black arrows) associated with mesenteric gas (white arrow).
advanced phase with a circumferential pattern of IP (black arrows), Association of IP and PMVG is more frequently observed in transmu-
gas in superior mesenteric vein and in portal branches (white arrows), ral bowel necrosis
and presence of peritoneal fluid close to the liver. d Coexistence of

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Fig. 6  IP in large bowel cancer, localized in the ascending colon

permeability may be associated with subsequent aerogenic

bacterial penetration and this is thought to be the reason that
IP can be observed in immunosuppressed patients without
other evident bowel involvement [9].
The pulmonary hypothesis has been advocated in the
genesis of IP, since in several cases it has been reported the
association of IP with COPD [17].

Imaging techniques

Abdominal radiography and CT are the main imaging tech-

niques used to diagnose IP.
A proper X-ray abdomen examination should always
include, in addition to upright projection or latero-lateral
projection in not standing patients, a supine antero-posterior
projection to analyze air distribution and better recognize IP.
Gas in portal branches is visible on X-ray like linear trans-
parencies peripherally located on the liver shadow.
CT has been shown to be more sensitive than radiography
in IP [18] and PMGV detection [19].
CT scan can be performed without or with intravenous
contrast agent injection. Anyway, IP in benign and asympto-
Fig. 5  a IP detected in the cecum and ascending colon on CT scan matic forms may be an ancillary finding in CT examination
in a patient with intestinal obstruction due to cancer in the sigmoid not performed to examine the intestinal wall.
colon. b The same case in coronal reconstruction. c Another case of When a CT examination is focused on bowel wall analy-
IP of the cecum in distal large bowel obstruction
sis, especially in emergency conditions, contrast injection is
mandatory. Acquisitions should be obtained in arterial and
The bacterial etiology hypothesis has been supported venous phases and the evaluation should be completed with
by analyses of the gas collected in the intramural cysts in multiplanar reconstructions [20, 21]. Neutral intraluminal
PCI, which is characterized by high levels of hydrogen and bowel contrast is not recommended in emergency condi-
nitrogen [15]. Intestinal cells do not produce these gasses tions; however, its use could be considered according to
so a bacterial origin has been hypothesized. This is also specific clinical indications.
in accordance with persistence of cysts in PCI that would In order to visualize IP, it is relevant to evaluate CT with
be difficult to explain and also with a report of a therapeu- appropriate window settings, typically a window level of
tic effect of antibacterial drugs [16]. Increased mucosal − 25 Hounsfield units (HU) and window width of 500 HU

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Fig. 7  Radiological signs to
search in the presence of sec-
ondary forms of IP

allows a clear distinction between fat and air with still a fair likely that these conflicting reports are a consequence of the
distinction of solid parenchyma and bowel wall. low number of observed cases.
CT angiography and a careful study of the whole abdo- PCI is generally a benign pathological condition but can
men in the venous phase are critical in putting a radiologi- also present with sub-occlusion [31] and require surgery.
cal diagnosis. IP is an important sign arising the suspect of PCI may be associated with PNP which can spontaneously
intestinal ischemia, but the final judgment requires a system- reabsorb [32].
atic search of associated signs, through a careful evaluation In the majority of cases, there are no symptoms associ-
of intestinal lumen and wall, arterial and venous mesenteric ated to PCI; however, when present clinical manifestations
vessels, mesentery, peritoneum and abdominal solid organs, of PCI are non-specific: it may present with abdominal pain
taking in account the morphologic and the dynamic aspects, (59%), diarrhea (53%), nausea and vomiting (14%), mucus in
in particular searching for enlarged loops, air or fluid filled stool (12%) and hematochezia (12%) [29]. These symptoms
loops, air-fluid levels, thickness or thinning of the intesti- are often a consequence of PCI’s complications, including
nal wall, PMVG, vascular thromboembolism, thickening of peritoneal irritation due to PNP, volvulus, intestinal obstruc-
mesenteric folds, mesenteric edema or fat stranding, perito- tion or ischemia, that occur in 3% of cases and could require
neal fluid, pneumoperitoneum (PNP), bowel wall enhance- urgent surgical intervention [33]. Cases have been described
ment features, prominence of intestinal folds, solid organs in which PCI is associated to intussusception; in these cases,
enhancement features (Fig. 7). PCI can be considered as a cause of obstruction [34], or
Ultrasonography (US) was also used in detecting IP [22]; instead as the effect of the obstruction [35].
however, US detection of IP is not easy for the disturbing In PCI, abdominal X-ray usually shows multiple gas
effects of intraluminal air and this technique is mainly used, pockets or small round lucencies in the bowel wall. Con-
but nor exclusively, in newborns. trast-enhanced CT may better reveal intramural gas, often
Occasionally IP may be detected on MRI, anyway MRI disposed as a bunch of grapes, and the regular and non-
has little indication in the study of IP and related conditions inflamed mucosa that surround the air cysts. This intramural
[23, 24] (Fig. 8). gas could spread in the portal venous system or it could be
associated to a small volume of PNP (Fig. 2).
A feature of PCI is the mismatch between the impres-
Primary form of IP: PCI sive radiological signs in front of minimal or absent clinical
signs.
The primary form of IP is represented by PCI, an idiopathic
gas infiltration of the bowel wall disposed as a bunch of
grapes.
Secondary forms
PCI, according to Jamart [25], primarily affects the small
intestine, but it has also been described in the large intes-
Secondary forms of IP are more frequent than PCI (85% vs
tine and some authors report a higher frequency in this part
15%) and may occur in a wide range of pathological con-
of the intestine [26, 27]. Recently, Ling [28] and Wu [29]
ditions, as inflammatory bowel diseases, malignancies and
reported that in a Chinese ethnic cohort colonic localization
chemotherapy, infections, immune deficiency status, trauma
was more frequent, with a colon/small bowel ratio of 1.3:1.
or medical maneuvers, but it is a sign mainly related to intes-
There is a high variability in the different reports in the male/
tinal ischemia and necrosis [36].
female distribution: Horiuki et al. [30] report a higher fre-
In secondary forms, IP is a radiological sign and not an
quency in women, while Wu et al. [29] report that PCI is
illness in itself [36], then it does not have a proper clinical
more frequent in males (male/female ratio of 2.4:1). It is

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infarct [38]. Although laboratory tests are useful, diagnosis

more often requires a CT confirmation [39].
IP is a frequent radiological sign of acute intestinal
ischemia and its early recognition is of high value because
permits the immediate surgical treatment and the appropriate
medical supportive therapy, with consequent prevention of
necrosis [37, 40, 41].
IP may be detected with X-rays, which can also clarify its
pattern and distribution, presence of PMVG and other asso-
ciated findings that can direct radiologists’ and clinicians’
attention towards a specific diagnosis. Nowadays, X-ray is
used in newborns [42] (Fig. 9a–d), while in adults it has
been overtaken by CT scans [10, 43].
On CT, IP usually appears as a linear and/or bubbly pat-
tern of intramural gas associated to additional findings as
thin bowel wall, absent or reduced mucosal enhancement,
dilated bowel, ascites and arterial occlusion [19]. IP is some-
times associated with PMVG, that is differentiated from
pneumobilia in X-rays by its characteristic tubular branching
lucencies that extend to the periphery of the liver, whereas
Fig. 8  IP on axial T2 MRI scan (arrow). This was an occasional find- biliary air is more central [1].
ing in a patient with PCI Furthermore, IP in intestinal infarction has been con-
sidered an indicator of poor prognosis related to advanced
necrosis [44], notably if it is associated to PMVG. These
expression and its symptoms depend on the underlying data have been studied by Wiesner et Al., who analyzed 23
disease. IP is usually associated to late phase of intesti- patients and concluded that bubble-like IP may be related to
nal ischemia and infarction is usually seen in late phase, partial mural bowel ischemia, while a band-like pattern of
requiring a very prompt diagnosis to start an immediate and IP in association with PMVG was associated to transmural
aggressive treatment. Conversely, IP induced by the men- bowel infarction. In their experience, IP and PMVG were
tioned non-ischemic causes commonly shows a less aggres- associated to a mortality rate of 44% and 56%, respectively,
sive clinical course and often requires conservative manage- whereas when IP and PMVG were simultaneously present,
ment rather than surgery [37]. the mortality rate increased up to 72% [45].
In the secondary IP, the radiological appearances are Greenstein et al. retrospectively studied IP in 40 patients
more varied. It is better detected and evaluated with CT over 10 years. They found an overall surgical rate of 35%
scans, which is the gold standard technique to detect IP and with hospital mortality rate of 20%. The authors showed how
should be used in adults in any case in which either X-rays combining radiological findings with clinical and labora-
or US raise the suspicion and in any patient with abdominal tory results led to better management and better prognostic
acute symptoms. evaluation of IP [46].
In our previous experience, 52% of IP in adults was
related to intestinal infarction with a mortality rate of 30.4%,
Intestinal ischemia
rising to 50% when associated with PMVG [47].
In strong agreement with our data, Treyaud et al. reported
Acute mesenteric ischemia is a life-threatening condition
bowel ischemia in 53.7% of IP detected on CT and in 69.6%
caused by decreased blood flow to the bowel [20]. Its clini-
when PMVG was also present. In this study, the only other
cal findings are non-specific, as abdominal pain, often more
radiological sign related to ischemia was the reduction of
severe than that expected on the basis of physical examina-
intestinal bowel wall enhancement. These authors also
tion, nausea, vomiting, and diarrhea [20], therefore clini-
emphasize the importance of clinical and laboratory data
cal diagnosis may be difficult. In case of acute ischemia,
[48].
abnormal laboratory tests include leukocytosis, hemocon-
centration, metabolic acidosis, and elevated levels of lactate,
NEC
LDH, D-dimer, amylase and liver enzymes [21]. Notably,
an increased lactate level is often a late marker of intestinal
NEC is a pediatric disease that mostly affects the premature
infants’ intestine. The intestinal wall is invaded by bacteria,

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which cause local infection and inflammation, resulting in a detection of sub-serosal gas could be more difficult because
bowel wall destruction. of the presence of concurrent intraluminal air [51].
In newborns IP may be a good indicator of NEC, usually IP, thick bowel wall (> 2.5 mm), PMVG ,and reduced
starting from distal ileus and right colon, spreading then to peristalsis are independent diagnostic factors associated with
the entire intestine and eventually evolving to bowel necrosis NEC [52]. Cuna et al. in a meta-analysis found that US has
(Fig. 9a–d). low sensitivity but high specificity for diagnosis of NEC
In contrast, IP in infants is not pathognomonic for NEC, [53]. Direct US recognition of IP has been made in other
but when associated to abdominal distension, hematochezia, emergencies in infants, James and Itazaki reported cases of
paralytic ileus, NEC becomes a probable diagnosis. In non- intussusception [54, 55].
neonatal pediatric patients, Kurbegov found that all of their CT is rarely performed in children due to its invasiveness,
patients with IP associated with PMVG had necrotic bowel, but it could be appropriate in complicated cases to detect IP
all of them required surgery or died before intervention [49]. and related PMVG (Fig. 10).
Bowel US may be a useful adjunct to abdominal X-ray
in the evaluation of infants with clinical suspicion of NEC, Other
avoiding radiation exposure in this radio-sensitive popu-
lation such as children [50]. In IP, US shows hyperechoic Other recognizable cases of IP include infection [36], recent
dots and lines in the edematous thickened bowel wall, while abdominal traumas [13], iatrogenic gastrointestinal lesions

Fig. 9  a–d Gastrointestinal
pneumatosis in newborns with
NEC (arrows)

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A relatively frequent clinical situation in which IP may

occur is bowel obstruction (Fig. 5) [46]. In this case patients’
clinical presentation may be acute and the recognition of a
cause of obstruction or a transition point should guide the
radiologist. In some cases, intestinal necrosis and obstruc-
tion can be present simultaneously, this happens in the case
of a twisted loop or of an internal or external hernia [42, 43].

Differential diagnosis

The distinction between PCI and the secondary form of IP

is usually straightforward, since the morphological findings
are quite different in the two forms.
On plain films it is very difficult to confidently detect IP,
especially when there are enlarged bowel loops, PNP and/
Fig. 10  PMVG in a newborn. Hepatic gas is located peripherally, or retropneumoperitoneum. Possible pitfalls are superim-
unlike pneumobilia position of loops that may create false images (Fig. 11) or
retropneumoperitoneum that may mimic IP (Fig. 12).
It is easier to detect PMVG, because of the linear trans-
(Fig. 4) [12], inflammatory bowel diseases (IBD), cancer parencies on the liver shadow. In this case the radiologist
(Fig. 6) and immunosuppression [14]. has to take care to distinguish PMVG from pneumobilia and
In these cases, IP is a sign associated to the typical radio- to recognize accompanying radiological signs, since portal
logical findings of the underlying disease (Table 1). air embolization may occur after umbilical catheterization.
In IBDs, fever, weight loss, and recurrent sub-occlusive US plays a role in the diagnosis of PMVG, because of the
symptoms are frequent clinical findings, related to radiologi- good detection of gas in portal branches. It is important to
cal stenotic pattern with high contrast-enhancement of the not confuse PMVG with pneumobilia—a distinction based
ileal wall. on a correct recognition of portal vessels, ultimately using
IP may be associated to bowel cancer, where obstruction, color doppler analysis. PMVG usually spreads peripherally
rectal bleeding, fatigue and weight loss are typical symptoms in the liver, whereas pneumobilia has more typically a more
and CT scan permits direct visualization of cancer and its central distribution toward hepatic hilum (Fig. 13) [1, 12].
complications. As said before, CT is the gold standard technique in
Moreover, abdominal traumas or iatrogenic gastrointes- detection of both primary and secondary forms of IP. PCI
tinal lesions may present with IP, often associated to active has a specific radiologic pattern defined by multiple clusters
bleeding, mucosal disruption, fractures and/or other trauma’s of rounded cystic gas collections in the bowel wall, involving
complications. either the small or large bowel [47, 56].

Table 1  Imaging features in IP differential diagnosis

Pathology Clinical findings Imaging features

PCI Absent or non-specific Multiple clusters of rounded cystic gas collections in the
bowel wall
Ischemia/infarction Severe abdominal pain disproportionate to physical exam, More frequent linear pattern of IP, presence of PMVG, thin
leukocytosis, metabolic acidosis, elevated levels of lactate bowel wall with lack of enhancement, possible vascular
obstruction and mesentery fat stranding
Obstruction Abdominal distension, vomiting, inability to pass gas or Bowel overdistension up to a transition point with air-fluid
stool levels
Bowel cancer Symptoms of obstruction, rectal bleeding, fatigue, weight Direct visualization of cancer, intestinal obstruction
loss
IBD Fever, weight loss, recurrent sub-occlusive symptoms Stenosis with high enhancement of the ileal wall
NEC Abdominal distension, hematochezia, paralytic ileus Bowel overdistension, air-fluid levels, thick bowel wall

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Fig. 11  X-rays in a newborn. Overimposition of bowel loops mimics

a gastric pneumatosis (arrows). Previous and subsequent X-rays were
normal, and there were no symptoms suggesting acute abdominal dis-
ease

Fig. 13  a Portal gas in mesenteric infarction: hyperechoic lines in

portal branches are found up to the edge of the liver (arrows). b Pneu-
mobilia: hyperechoic lines (black arrows) adjacent to fluid-filled por-
tal vessels (white arrows)

The cystic presentation is more often associated to a

benign cause [31], this pattern may also be misinterpreted
in both senses: bowel content (Fig.  14a) and intestinal
folds (Fig. 14b) can trap bubbles of air mimicking IP [55],
so that false negative and false positive are possible. Usu-
Fig. 12  Presence of pneumoretroperitoneum mimicking parietal
pneumatosis of the ascending colon on X-ray scan. Gas localization
ally trapped bubbles are located in the anterior part of the
and distance from the bowel permit the distinction from IP

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Fig. 14  a Bubbles surrounding semisolid intestinal content in cecum. aerial levels in the small bowel, is compatible with the diagnosis of
In this case, gas bubbles are trapped in feces and not within the bowel intestinal infarction, but the exclusive anterior location of gas is not
wall. The patient did not have any abdominal acute complaint. b Bub- that of IP. c Another case of gas confined anteriorly in small bowel
bles entrapped in intestinal folds in the anterior part of the bowel. lumen simulating IP
The radiological context, characterized by enlarged loops with hydro-

loop (Fig. 14c), whereas localization of IP is completely abdominal emergencies should prompt a careful search for
independent of position [57, 58]. associated findings and point clinicians to the possible pres-
In secondary forms of IP, the different morphologic pat- ence of bowel necrosis.
terns of gas distribution are described as linear, circumfer-
ential, and bubble-like (Figs. 3, 5, 6) [36].
Linear pattern of IP has been more frequently related Compliance with ethical standards 
to intestinal infarction in adults, with a rate of 75.5% [31].
Moreover, linear pattern is also associated to NEC in new- Conflict of interest  We declare we have not conflicts of interest.
borns and infants.
On the other hand, bubble-like gas distribution, more
often associated to a benign cause [31], maybe also related References
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