Meyer, Thomas

Schröpfer, Engelbert

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Engelbert Schröpfer

Thomas Meyer

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Surgical aspects of pneumatosis cystoides intestinalis: two case reports

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Case report
Open Access
Surgical aspects of pneumatosis cystoides intestinalis:
two case reports
Engelbert Schröpfer* and Thomas Meyer
Address: Department of General, Visceral, Vascular and Transplant-Surgery, Julius-Maximilians-University of Würzburg, Würzburg, Germany
Email: ES* - Schroepfer_e@chirurgie.uni-wuerzburg.de; TM - Meyer_T@chirurgie.uni-wuerzburg.de
*Corresponding author
Received: 9 March 2009 Accepted: 17 July 2009 Published: 12 August 2009
Cases Journal 2009, 2:6452 doi: 10.4076/1757-1626-2-6452
This article is available from: http://casesjournal.com/casesjournal/article/view/6452
© 2009 Schröpfer and Meyer; licensee Cases Network Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
Introduction: Pneumatosis cystoides intestinalis is a rare disease usually caused by an underlying
condition. It is defined as air filled cysts within the wall of the gastrointestinal tract. The purpose of
this paper is the development of an algorithm for the surgical therapy of PCI based one two case
reports.
Case presentations: A 17-year-old girl with Down syndrome and leucopenia due to chemotherapy
for acute lymphatic leukemia was admitted with acute septic conditions and PCI. Explorative
laparotomy revealed acute ischemia of the right colon and resection of the affected intestine was
performed. After a short interval in the intensive care unit the patient was referred to the pediatric
department. The second patient, a 79 year old man, with urothelial carcinoma and carcinoma of the
prostate presented a distended abdomen. CT-scan revealed PCI and adhesive strangulation of
intestines. Therefore only adhesiolysis was performed and PCI was treated conservatively.
Conclusion: Only patients with increased inflammatory parameters in laboratory findings or signs of
sepsis, peritonitis or bowel perforation in combination with PCI should receive an explorative
laparotomy.
Introduction
Pneumatosis cystoides intestinalis (PCI) is defined as air
filled cysts within the wall of the gastrointestinal tract.
Duvernoi first documented PCI in his manuscript “Aer
intestinorum tam sub extima quam intima tunica inclus-
sus”, published 1730 [1]. In 1876, Bang described the
histomorphologic changes caused by PCI [2] and in 1899
Hahn described PCI living patients [3]. The first
preoperative diagnosis of PCI through radiologic findings
was made by Baumann-Schenker 1939 [4].
The incidence of PCI is unknown; the frequency is
reported to be highest in the sixth decade. Primary PCI is
extremely rare, in most cases PCI is due to an underlying
disease or condition (traumatic and mechanical, inflam-
matory and autoimmune diseases, drug induced, immuno-
suppression, transplantation or neoplasm) [5]. In a review
of 213 pathologic specimens, Koss noted 1952 that 85%
of PCI are attributed to a secondary disease process [6]. All
parts of the gastrointestinal tract may be affected; the small
bowel is involved in 42%, the colon in 36% or both in
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22% of the cases [7]. The purpose of this paper is the
development of an algorithm for the surgical therapy of
PCI based one two case reports.
Case presentations
Case report 1
The first case reports on a 17 year old white European girl,
with Down syndrome, steroid induced diabetes and
relapsed acute lymphatic leukemia (ALL) since four
months. She was admitted to our surgical department
from the pediatric intensive ward for further treatment of
progressive sepsis and free abdominal air. Because
of chemotherapy for ALL, the patient was in pancytopenia,
with white blood cell count at 100 per µl [5.000 - 10.000
per µl], platelets at 28.000/µl [150.000 - 450.000 per µl]
and Hb at 7.6 g/dl [12-16 g/dl]. Due to a CRP concen-
tration of 18.98 mg/dl [0-0.5 mg/dl], an antibiotic
treatment with Ceftazidim, Gentamycin and Teicoplanin
had already been started on the pediatric intensive care
ward. Blood culture test showed presence of beta lactam
resistant Escherichia coli, therefore Meropenem was
added. Abdominal sonography revealed free abdominal
air near the portal vein.
Cardiopulmonary resuscitation was performed on the
pediatric intensive care ward due to acute circulatory
failure. After resuscitation abdominal computer tomogra-
phy (CT) was carried out and multiple air filled cysts
within the cecal wall could be seen (Figure 1A and B). The
sonographic finding of free air near the portal vein was
confirmed in CT. The decision for immediate laparotomy
under emergency conditions was made based on the
radiologic findings.
During surgery acute ischemia of the right hemicolon and
the transverse colon could be seen (Figure 2). Resection of
the right ischemic hemicolon was performed and because
of the aplasia and sepsis, we decided on closure of the
colon-stump and ileostomy. Fresh frozen plasma, red
blood cell concentrates and platelet concentrates were
given postoperative and antibiotic treatment was changed
to Meropenem, Vancomycin, Ciprofloxacin, Metronidazol
and Fluconazol. Patient was isolated and treatment with
granulocyte-colony stimulating factor (GCSF) was started.
On recovery from the septis, catecholamine treatment was
reduced and after extubation patient was referred to the
pediatric department for continuing chemotherapy.
Figure 1A and B. Abdominal and thoracic computer tomography of patient 1 coronal view: Multiple air filled cysts in the
intestine wall along the right hemicolon can be seen (red arrows). Diagnosis Pneumatosis cystoides intestinalis was made.
The sonographic findings (free abdominal air near the portal vein) were not confirmed in CT.
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Pathological findings of the resected tissue revealed an
acute hemorrhagic infarction of the colon with necrosis of
the mucosa and ectasia of the submucosal and subserosal
vessels, without proof of thromboses. No bacteria and no
malignancy were found.
Case report 2
A 79 year old, white European male patient suffering from
an urothelial carcinoma of the bladder and carcinoma of
the prostate was treated in the department of urology.
Radical cysto-prostatectomy (RO) was performed in
January 2008. Two days after the operation, an acute
ischemia of the right leg occurred and angiography revealed
a complete obstruction of the right common iliac artery.
A femoro-fermoral cross-over bypass was performed by the
department of vascular surgery. Additionally, due to a
beginning compartment syndrome of the right leg,
fasciotomy was performed. After several debridements of
the fasciotomy-wound and vacuum-assisted closure of the
wound, mesh skin grafting was carried out.
One month after cysto-prostatectomy the patient showed
signs of an acute abdomen with progressive abdominal
pain and guarding. His white blood cell count was
14.400 per μl and the CRP concentration was
4.75 mg/dl [0-0.5 mg/dl]. Lactate measurement
was 1.6 mmol/l [0.7 - 2.1 mmol/l]. Because plain
radiography showed signs of large bowel obstruction
(Figure 3), CT was carried out. Abdominal CT revealed
large bowel obstruction and air filled cysts in the whole
colonic wall. (Figure 4A and B). The suspected diagnosis of
adhesive bowel obstruction was made. Due to the
radiologic findings and clinical signs of an acute abdomen
explorative laparotomy and adhesiolysis were performed.
Two months after admittance the patient was discharged
into rehabilitation.
Discussion
PCI is a rare disease usually caused by an underlying
condition. The incidence is unknown due to the asympto-
matic course of the disease in most cases. Primary PCI is
extremely rare, Shawn et al. divided the underlying
conditions for secondary PCI into six groups, traumatic
and mechanical, inflammatory and autoimmune, infec-
tious, pulmonary, drug induced and other [8]. The most
common symptoms of PCI are diarrhea or constipation,
bloody stools, abdominal pain, flatus or weight loss. It has
been suggested that these symptoms are caused by
mechanical effects of the cysts, but in many cases even
large cysts remain asymptomatic; thus showing no
correlation between clinical manifestation and the
amount of intramural gas [9]. Approximately 3% of
afflicted patients experience a complication, such as
pneumoperitoneum, volvolus, intestinal obstruction
intussusception, hemorrhage or perforation.
The pathogenesis of PCI has been discussed for a long time
and multiple theories exist. Three different possibilities for
the source of gas within the intestine wall have been
considered: intraluminal gas, pulmonary gas and gas
produced by bacteria. Two basic mechanical features are
responsible for intrusion of intraluminal gas into the
bowel wall: mucosal injury, the most important and
prevalent feature, and increased intralumenal pressure; or
Figure 3. Plain abdominal radiography of patient 2:
Distension of the large bowel and gas-fluid-levels as signs for
a large bowel obstruction.
Figure 2. Photography during operation of patient 1: View of
the colon from the ceacum to the descending colon with livid
discoloration of the right hemicolon and the transverse colon
as a sign of acute ischemia.
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both. Increased intralumenal pressure may be produced by
bowel obstruction, ileus, or iatrogenically by upper or
lower GI endoscopy. Damages in the mucosa may result
from an inflammatory process, a defect in the gut immune
barrier and steroid or cytotoxic medical therapy.
The possibility of pulmonary gas as a source for PCI is
based on the theory of air migration along vessels within
the mediastinum, retroperitoneum and mesentery after
alveolar rupture in pulmonary diseases. The absence of
interstitial emphysema within the lung or the mesentery in
many of the cases has led to contrasting opinions
regarding this theory [10].
The disappearance of PCI during antimicrobial treatment
in some cases has led to the theory that gas producing
bacteria is reasonable for PCI [9]. Direct invasion of the
wall and alteration of the intraluminal gas content enables
bacteria to form intramural gas. Mucosal damage and
immune deficiency leads to bacterial invasion of intra-
mural compartments. However, there is a lack of evidence
for bacteria within the cysts themselves [7]. Direct gas
diffusion across the mucosa is caused by a gradient
between intraluminal and serum partial pressures. Bacteria
produce intraluminal hydrogen tension higher than
nitrogen tension in blood leading to hydrogen diffusion
towards the submucosal vessels, where it is followed by
nitrogen, oxygen and carbon dioxide from circulation
[11].
Plain radiography, barium enema, ultrasound, abdominal
CT and endoscopy can be helpful in diagnosing PCI, CT
being the best method with the highest sensitivity.
Figure 5. Algorithm of surgical management of PCI.
Figure 4A and B. Abdominal computer tomography of patient 2 coronal view: Multiple air filled cysts in the intestine wall along
the right hemicolon and a massive dilatation of the whole colon can be seen. Diagnosis pneumatosis cystoides intestinalis
(red arrows) and ileus of the colon was made. The suspected level of obstruction is indicated with blue arrows.
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According to Jamart, PCI can be detected in approximately
two thirds of patients with plain radiographic findings
[12]. Many authors describe the value of ultrasound for
diagnosing PCI and portal venous air [13]. Barium enema
has been used but the findings can be confused with
polyposis as they have similar appearances [14]. During
endoscopy macroscopic elevations of the mucosa can be
seen and some authors recommend puncture to confirm
the diagnosis [7]. But removing of a polyp that in fact was
a cyst has caused perforation of the bowel in some cases
[15]. Computed tomography can distinguish PCI from
intraluminal air or submucosal fat and provides survey of
the abdomen for diagnosis of associated pathological
conditions.
Patients with a radiographic diagnosis of PCI should have
a thorough history and physical examination and only
along with the knowledge of the underlying disease the
decision for an explorative laparotomy can be made. PCI
can be managed conservatively in most cases [5,8]. The
major problem for surgeons is identifing the patients
requiring surgical intervention. Laparotomy should not
be performed based on radiographic findings alone.
Good results with inspired oxygen have been reported in
PCI with underlying diseases that do not require surgery
[16]. Pneumoperitoneum is not necessarily caused by
bowel perforation but may represent a ruptured cyst [17].
Gas in the portal system is associated with a high
mortality rate of 37% and often occurs with ischemic
bowel [18].
In case of a radiologic diagnosis of PCI in a patient, we
recommend a standardized procedure (Figure 5). With
laboratory findings of elevated CRP or white blood cell
count, as well as signs of sepsis, bowel perforation or free
gas near the portal vein immediate surgery is indicated. In
patients with normal or slightly increased inflammatory
parameters in blood samples and no signs of sepsis, bowel
perforation or free gas, we recommend modest therapy
comprising of i.v. antibiotics and, optionally, endoscopy
for further clarification as well as reevaluation after a
definite time interval (12-24h). Conservative management
is preferable, but in the case of increasing CRP or white
blood cell surgery should be performed.
Our first case demonstrates that PCI in radiographic
findings in combination with sepsis or signs of infection is
always an indication for explorative laparotomy. PCI was
likely caused by bacterial invasion of the intestine wall
because of immune deficiency. The positive testing for
Escherichia coli in blood culture confirmed this suspicion.
The second patient underwent a laparotomy because of
ileus. An operation wouldn’t have been necessary based on
the PCI alone, which was in our opinion a consequence of
the increased intralumenal pressure.
Conclusion
PCI as a radiographic finding does not routinely require
laparotomy. A thorough history and physical examination
are obligatory since PCI is mostly a consequence of an
underlying disease. PCI can be managed conservatively in
most cases. A lapartomy without clear indication might
even worsen the general condition of the patient. Patients
with increased inflammatory parameters in laboratory
findings or signs of sepsis, peritonitis or bowel perforation
in combination with PCI should receive an explorative
laparotomy.
Abbreviations
ALL, acute lymphatic leukemia; CRP, C-reactive protein;
CT, Computed tomography; GCSF, granulocyte-colony
stimulating factor; PCI, Pneumatosis cystoides intestinalis.
Consent
Written informed consent was obtained from the parents
of patient 1 and from patient 2 for publication of the case
reports. A copy of the written consents are available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors’ contributions
ES analyzed the data and drafted the manuscript and was
the main coordinator. TM and CG participated with
editorial support.
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Surgical aspects of pneumatosis cystoides intestinalis: two case reports

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