Clinical Imaging 65 (2020) 1–4

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Clinical Imaging
journal homepage: www.elsevier.com/locate/clinimag

Body Imaging

Emphysematous cystitis with extraperitoneal gas: new insights into T

pathogenesis via novel CT findings☆
⁎,1
Paul R. Wojack , Inessa A. Goldman
Montefiore Medical Center Department of Radiology, 111 E 210th St, Bronx, NY 10467, USA

A R T I C LE I N FO A B S T R A C T

Keywords: Emphysematous cystitis is a relatively rare disease entity characterized by intramural and/or intraluminal
Emphysematous bladder gas best depicted by cross-sectional imaging. Its disease mechanism is not well understood. A case of a
Cystitis diabetic patient with emphysematous cystitis is presented and is notable for the rare finding of extraperitoneal
Pathogenesis gas in the pelvis based on a review of 114 case reports. Herein we propose a distension-based mechanism with
Disease mechanism
intramural bacterial seeding as the pathogenesis of emphysematous cystitis based on the patient's imaging and
the disease's established associations with diabetes and E. coli. The ability to recognize extraperitoneal pelvic gas
as a feature of emphysematous cystitis allowed prompt diagnosis. This facilitated early commencement of
successful treatment in a diabetic patient in whom the diagnosis was not suspected clinically.

1. Introduction
Emphysematous cystitis is a relatively rare disease entity char-
acterized by urinary tract infection associated with intramural and/or
intraluminal bladder gas. The exact incidence and prevalence of this
condition are not well-described in the current literature. The most
common presenting complaint is abdominal pain, found in 80% of
patients [9]. Other common symptoms include pneumaturia (70%),
dysuria (50%), urinary frequency (50%), and urinary urgency (50%)
[9]. Diabetes mellitus is the most common predisposing risk factor,
present in up to 50% of patients with emphysematous cystitis [8].
Advanced age and female sex are also significant risk factors with a 2:1
female-to-male ratio and an average age of 66 years at diagnosis [8,25].
E. coli and K. pneumoniae are the most frequently implicated causative
organisms, accounting for up to 80% of cases, though infections with
Enterococcus, Candida, and polymicrobial infections have also been re-
ported [9]. The presence of intramural gas is pathognomonic for em-
physematous cystitis and presence of intraluminal gas, in the absence of
recent history of bladder instrumentation, is highly suggestive of the
condition. The diagnosis is typically made via CT imaging, but can also
be established by plain radiographs or ultrasound [24]. Presented here
is a case of emphysematous cystitis with an unusual CT finding of ex-
traperitoneal gas extending from the bladder wall into the prevesical
and perivesical spaces without evidence of bladder rupture. To the
authors' knowledge, this is the first documented case of such a finding
and may shed light onto the underlying disease mechanism of emphy-
sematous cystitis, which has hitherto been poorly described.
2. Case
A 61-year-old female presented to the emergency department due to
two days of worsening left lower abdominal pain and multiple episodes
of non-bloody, non-bilious vomiting. Her past medical history was
significant for type 2 diabetes mellitus, multiple hospitalizations for
gastroparesis, stage III chronic kidney disease, congestive heart failure,
and peripheral arterial disease. The patient described her pain as 9/10
in severity. Initial physical examination revealed generalized abdom-
inal tenderness without rebound or guarding. The exam was otherwise
unremarkable; the patient was afebrile with stable vital signs. Pertinent
laboratory results on presentation included blood glucose of 328 mg/
dL, a urinalysis with 6–10 white blood cells, but negative leukocyte
esterase and negative nitrates. Urine culture ultimately revealed
100,000 CFU of E. coli and 50,000 of Enterococcus, but these results only
became available two days after the admission. Blood cultures re-
mained negative throughout the patient's hospitalization. CT imaging of
the abdomen and pelvis without contrast was obtained in the
Emergency Department, revealing a distended, urine-filled bladder with
gas partially outlining the bladder wall and extending into the

☆
This research did not receive any specific grant from funding agencies in the public, commercial, or not for-profit sectors.
⁎
Corresponding author.
E-mail address: pwojack@mail.einstein.yu.edu (P.R. Wojack).
1
Present Affiliation: Geisinger Bloomsburg Hospital, 549 Fair St, Bloomsburg, PA, 17815, USA.

https://doi.org/10.1016/j.clinimag.2020.04.008
Received 12 November 2019; Received in revised form 23 March 2020; Accepted 7 April 2020
0899-7071/ © 2020 Elsevier Inc. All rights reserved.
P.R. Wojack and I.A. Goldman Clinical Imaging 65 (2020) 1–4

Fig. 1. Axial, coronal and sagittal CT images of the bladder (lung windows) demonstrate air outlining the bladder wall and also extending posteriorly into the right
sacrosciatic notch (black arrow, 1A), inferiorly into the perivesical space (1B, black arrow) and into the anterior space of Retzius (1B and 1C, star).

Fig. 2. Emphysematous cystitis in a different patient as shown on a transverse gray-scale ultrasound image of the bladder (left) and a corresponding non-contrast
axial CT image of the pelvis (right). Intramural gas is represented by the white asterisks. On ultrasound, air appears as bright, hyperechoic foci scattered along the
bladder wall. On CT scan both intramural gas (white asterisk) and intraluminal layering nondependent gas are noted.

extraperitoneal soft tissues (Fig. 1). Despite the highly unusual ap-
pearance of extraperitoneal gas, the diagnosis of emphysematous cy-
stitis was made on the basis of gas outlining the bladder contour, in-
dicative of intramural air, which is virtually pathognomonic for the
disease.
Initial management of the patient consisted of placement of a Foley
catheter. Following placement, the patient reported significant im-
provement in her abdominal pain. The patient was admitted for in-
travenous antibiotic therapy based on the diagnosis of emphysematous
cystitis; she received treatment for two days with IV ceftriaxone and
was then transitioned to oral cefdinir. At this point, she reported
complete resolution of her symptoms and was discharged.
3. Discussion
Emphysematous cystitis has been suggested as a cause of gas within
multiple locations throughout the body, including the spinal canal,
heart, and vena cava [13,16,19,26]. However, extraperitoneal gas in
the pelvis, specifically extending into the sacrosciatic notch and ante-
rior space of Retzius, has not been previously described as a feature of
emphysematous cystitis. Because extraperitoneal gas is not a known
finding associated with emphysematous cystitis, in our case the diag-
nosis was not immediately confirmed and other possible causes of ex-
traperitoneal air had to be considered including necrotizing fasciitis,
bladder rupture, colovesical or vesicovaginal fistulae, or rectal per-
foration. While necrotizing fasciitis was initially a concern, the presence
of gas paralleling the bladder wall was indicative of intramural air in
the bladder, a finding virtually pathognomonic of emphysematous cy-
stitis, which eliminated other diagnostic considerations in its favor.
Additionally, as the extraperitoneal air appeared to emanate from the
bladder wall, it was reasonable to assume that the bladder was the
source of the above gas. Furthermore, lack of free fluid in the pelvis, as
would be expected in case of a bladder rupture, and absence of peri-
rectal inflammation, inevitable in case of rectal perforation, corrobo-
rated the diagnosis of emphysematous cystitis. Colovesical and vesi-
covaginal fistulae were thought unlikely due to lack of intraluminal gas
in the bladder. Finally, absence of recent bladder or rectal in-
strumentation further supported the diagnosis and effectively ruled out
other causes of extraperitoneal gas.
In our case the diagnosis of emphysematous cystitis was readily
made on the CT scan and the patient quickly improved once appro-
priate therapy was begun. No further imaging was obtained for this
patient neither at the time of the diagnosis in the Emergency
Department, nor during the patient's hospital admission. While this
inadvertently limited our ability to demonstrate imaging findings of this
unusual case of emphysematous cystitis on other modalities, which may
be considered a weakness of this particular report, familiarity with its
typical imaging appearance is important, especially on ultrasound. In a
patient with dysuria or pelvic pain, ultrasound of the bladder may be
the first or only imaging exam ordered and prompt recognition of the
findings may be life-saving. Sonography of a bladder with intramural
gas typically demonstrates highly echogenic reverberation artifact due
to the presence of said gas (Fig. 2), often with “dirty” shadowing seen in
the bladder lumen [8]. Occasionally, highly echogenic foci of gas can be
seen to move dependently with the patient's position; this phenomenon
has been referred to as the “champagne” or “effervescent” sign pre-
viously in reference to emphysematous cholecystitis, but can be seen
with emphysematous cystitis as well [7,23].

2
P.R. Wojack and I.A. Goldman
Fig. 3. Axial CT image (lung windows) demonstrates pneumatosis intestinalis
with extraluminal air extending into the mesentery. Note intramural gas within
the dependent wall of the jejunum (thin arrow) and extensive extraluminal gas
dissecting into the mesentery (thick arrow).
Beyond the rare feature of gas dissecting into extraperitoneal tis-
sues, this patient's imaging findings are significant as they shed light on
the likely pathophysiology of emphysematous cystitis which has thus
far been poorly defined in the literature [5]. The presence of extra-
peritoneal air strongly suggests disruption of the bladder serosa.
However, the absence of extraperitoneal urine indicates that the
bladder mucosa is intact. This suggests that emphysematous cystitis
may share similar pathophysiologic pathways with pneumatosis in-
testinalis and emphysematous cholecystitis.
Pneumatosis intestinalis is characterized on imaging by air in the
intestinal walls, as seen below in Fig. 3. While multiple theories exist
regarding the mechanism behind this entity, one of the leading theories
posits that distension of the bowel leads to microscopic tears in the
mucosa [6,21,22]. Distension of the bowel and/or mucosal injury can
occur through multiple mechanisms including intestinal obstruction,
endoscopic instrumentation and trauma, or via autoimmune disease of
the bowel. Subsequently, it has been hypothesized that air can enter the
bowel wall directly, or that gas-producing organisms can enter and
produce intramural gas [11,22]. In some cases this intramural gas can
form sub-serosal cysts, which can then rupture, leading to in-
traperitoneal free air without full-thickness bowel perforation [11].
Another important disease entity which shares common features
with emphysematous cystitis is emphysematous cholecystitis. This
condition is also more prevalent in diabetic patients (at least 50% of
affected patients have diabetes) and shares E. coli as a common cau-
sative organism with emphysematous cystitis [2,7,12]. Considerably
more literature exists regarding the etiology of emphysematous chole-
cystitis as opposed to emphysematous cystitis, with prior authors sug-
gesting that emphysematous cholecystitis develops due to intramural
anaerobic bacterial seeding of the gallbladder wall in the setting of
gallbladder wall ischemia [3,24]. Interestingly, strong association exists
between acalculous and emphysematous cholecystitis: the proportion of
acalculous cholecystitis is three-fold in emphysematous cholecystitis
compared to that in acute cholecystitis [15]. This supports distention of
the gallbladder with resultant stasis and mucosal injury, providing
pathway for intramural bacterial seeding, as one of the common pa-
thogenesis mechanisms. As with emphysematous cystitis, emphysema-
tous cholecystitis often presents with intramural and/or intraluminal
gas on plain film and associated reverberation artifact on ultrasound
[7]. An example of CT and sonographic findings in emphysematous
cystitis is illustrated in Fig. 4.
Reflecting on the pathogenesis of both pneumatosis intestinalis and
emphysematous cholecystitis, we propose that a similar distension
3
Clinical Imaging 65 (2020) 1–4
mechanism with bacterial seeding occurs with emphysematous cystitis;
distension of the bladder creates transient microscopic tears in the
bladder mucosa. Small amounts of bacteria-laden urine enter the
bladder wall and seed the intramural space, after which there is sub-
sequent healing of the mucosa. The bacteria then undergo anaerobic
respiration, resulting in gas production which, with subsequent dis-
tention, leads to serosal disruption. This allows for the radiographic
appearance seen above in Fig. 1, wherein gas extends from the bladder
wall into the extraperitoneal tissues without evidence of extraperitoneal
fluid to suggest a full-thickness rupture. Additionally, one should note
the similarities between Fig. 1 and Fig. 3; just as pneumatosis in-
testinalis is sometimes associated with air dissecting into the mesentery
or even abdominal free air, this case demonstrates dissection of air into
the tissues adjacent to the bladder [22]. Similarly, one case has been
described of gas dissecting into the retroperitoneal tissues of a patient
with emphysematous cholecystitis [4]. Given the radiological simila-
rities between these three disease entities and the similar causative
bacteria between emphysematous cystitis and cholecystitis, it is rea-
sonable to conclude that a similar disease mechanism likely exists be-
tween these entities.
This hypothesis is consistent with the above patient's imaging
findings and with the disease's association with diabetes mellitus. First,
it has been suggested that high tissue glucose concentrations and poor
tissue perfusion favor the development of emphysematous cystitis [10].
This patient, who has type 2 diabetes mellitus, likely has high tissue
glucose concentrations and probably has poor tissue oxygen con-
centrations due to cardiovascular disease. A high-glucose, low-oxygen
environment would be ideal conditions for facultative anaerobes to
undergo anaerobic respiration. E. coli, which is the most commonly
identified organism in emphysematous cystitis (present in approxi-
mately 60% of cases), is capable of mixed-acid fermentation in anae-
robic conditions, producing carbon dioxide as a byproduct [1,14].
Klebsiella pneumoniae, which is capable of similar fermentation, is im-
plicated in approximately 10–20% of cases [1]. Lactulose and tissue
proteins may also serve as substrate for these microbes to undergo
anaerobic fermentation [5]. This is supported by previous studies which
have demonstrated higher urinary concentrations of lactulose in dia-
betic patients [17]. In addition to providing substrate for gas-forming
microorganisms, diabetes mellitus may also contribute to underlying
bladder distension via some degree of neurogenic dysfunction, which
simultaneously serves as a mechanism for creating the mucosal tears
described above and predisposes the patient to urinary tract infections
due to urinary stasis [18,20]. In this patient, for instance, it is reason-
able to assume that she may have a neurogenic bladder given the
bladder distension seen on imaging, especially since she has already
shown evidence of diabetic neuropathy in the form of gastroparesis.
Finally, it is important to acknowledge the critical role that prompt,
appropriate imaging played in this patient's care. Emphysematous cy-
stitis was not considered in the patient's differential diagnosis prior to
obtaining CT imaging and a diagnosis of emphysematous cystitis via
imaging directly led to the patient's admission to the hospital and ap-
propriate administration of intravenous antibiotics. Therefore, in ad-
dition to providing key insights into the pathogenesis of emphysema-
tous cystitis, this case highlights the important role that timely CT
imaging plays in this condition which may not be readily recognized on
clinical grounds alone, and which has been shown to carry a mortality
rate of 12% or greater [1].
4. Conclusion
Emphysematous cystitis is an uncommon condition whose patho-
genesis has remained poorly understood. Based on the above patient's
imaging findings, it can be surmised that disruption of the bladder
serosa occurred without simultaneous disruption of the bladder mu-
cosa. This suggests that emphysematous cystitis has a distension-based
mechanism similar to pneumatosis intestinalis and emphysematous
P.R. Wojack and I.A. Goldman
Fig. 4. Contrast enhanced sagittal CT image (left) and gray-scale longitudinal ultrasound image (right) in a patient with emphysematous cholecystitis. On CT
imaging, a small, low density focus in the fundus of the gallbladder represents intraluminal gas (white arrow). Sonographically, this appears as an echogenic focus
with the associated reverberation artifact commonly seen in emphysematous infections.
cholecystitis, but with seeding of the bladder wall with bacteria-laden
urine. This hypothesis is consistent with the disease's association with
diabetes mellitus and E coli. This case also highlights the importance of
prompt CT imaging in the management of this frequently insidious
condition.
Declaration of competing interest
None.
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