Acta chir belg, 2004, 104, 742-744

Pneumatosis Cystoides Intestinalis : Report of Two Cases

M. Turan*, M. Sen*, R. Eglmez**
Departments of General Surgery* and Pathology**, Cumhuriyet University, Faculty of Medicine, Sivas, Turkey.

Key words. Pneumatosis cystoides intestinalis ; small intestine ; pneumatosis intestinalis.

Abstract. We present two cases of Pneumatosis Cystoides Intestinalis (PCI) involving the small intestine : emergency
operations was mandatory. At surgery, the affected ileal segments were resected. After surgery, clinical courses were
uneventful. PCI is a sign, not a disease, and therefore its significance is directly related to the patients overall clinical
status. Treatment is not indicated in asymptomatic patients, whereas immediate surgical intervention is necessary in
patients with abdominal catastrophes.

Introduction

Pneumatosis cystoides intestinalis is an uncommon con-

dition characterized by the presence of multiple gas-
filled cysts in the submucosa or subserosa of the gas-
trointestinal tract (1). The diagnosis may be difficult,
and the condition is frequently overlooked. Even when
correctly identified, its clinical relevance is often misin-
terpreted. It must be emphasized that PCI is a sign, not a
disease, and therefore its significance is directly related
to the patients overall clinical status. We present two
cases of PCI involving the small intestine ; they needed
emergency operations due to acute abdomen.

Case 1
A 39-year old male was admitted to the emergency room
(ER), for nausea and generalized abdominal bloating
associated with colicky abdominal pain and diarrhoea
for two days. He had no history of abdominal surgery,
pulmonary emphysema, connective tissue disease, gas-
trointestinal peptic ulcer, or long-term trichloro-ethylene
exposure. On physical examination, abdomen was dis-
tended, there was diffuse abdominal pain on palpation,
with rebound tenderness and muscular rigidity. Upright
abdominal radiographs demonstrated free air below the
diaphragm. Laboratory investigations revealed a haemo-
globin of 13.9 g/dl, white blood cell count of 11,200 per
ml. No abnormal findings were seen in laboratory bio-
chemical tests. An emergency laparotomy was per-
formed with diagnosis of acute abdomen. There were
multiple gas bubbles in the submucosa of the eighty cen-
timeters ileal segment (Fig. 1). Some of the gas bubbles
were burst. The bowel felt spongy and cryptic on palpa-
tion. Although there was patchy serosal discoloration,
possibly caused by ischaemic change, no adhesion or
strangulation of the small intestine was found. Large
Fig. 1
There were multiple air bubbles in submucosa of eighty centi-
meters ileal segment in case 1 (arrows). There was also patchy
serosal discoloration, possibly caused by ischaemic changes
(open arrow).
bowel and other abdominal organs were normal. There
was 500 cc. free peritoneal fluid in abdominal cavity.
Fluid analysis showed a low albumin gradient but was
otherwise normal including negative amylase, cytology
and cultures for microorganisms and tuberculosis.
Intraoperatively, an affected 80 cm ileal segment was
resected and end-to-end primary one layer (with
3/0 polydeoxanone) intestinal anastomosis was per-
formed.
Histopathological examination of the resected speci-
men showed multiple cysts in submucosa of the intes-
tine, and giant cells lining the cysts wall. Pneumatosis
infiltrated partly into the muscularis propria. There was
no changes due to either submucosal microabscesses,
Pneumatosis Cystoides Intestinalis 743

necrosis, phlegmonous enteritis, or thrombi in any large colitis in whom it has a fulminant course (3) ; b) in
blood vessels, or bacterial infiltration. Following adults with obstructive pulmonary disease, in whom it
surgery, the clinical course was uneventful. The patient has a benign course (4) ; c) in adults and children who
was discharged on postoperative day 7 without any have a wide variety of underlying disorders determining
problem. At his control visit (postoperative day 20), his prognosis ; and d) as an incidental finding in endoscop-
physical examination, laboratory and X-ray findings ic mucosal biopsies taken for a wide variety of indica-
were normal. tions (1).
PCI may be asymptomatic, or at the other extreme, it
Case 2 may be associated with life-threatening septic illness, as
in adults with intestinal infarction or in premature
A 44-year old male was admitted to ER for nausea, vom-
infants with necrotizing enterocolitis who present with
iting and generalized abdominal bloating associated
abdominal distention and septic shock. The following
with colicky abdominal pain. He had peptic ulcer for
symptoms are reported in decreasing order of frequency
5 years, but no history of abdominal surgery, pulmonary
in patients with PCI : diarrhoea, bloody stools, abdomi-
emphysema, connective tissue disease, or long-term
nal pain, abdominal distention, constipation, weight
trichloro-ethylene exposure. On physical examination,
loss, and tenesmus from rectal involvement (1). The
he felt abdominal pain on palpation, with rebound ten-
plain abdominal X-ray is the most common way to iden-
derness especially in right subcostal region. Laboratory
tify pneumatosis. Sonography and computed tomogra-
investigations revealed a haemoglobin of 12.8 g/dl,
phy are additional methods to detect PCI and determine
white blood cell count of 14,200 per ml ; BUN 40 mg/dl
its cause (1).
and creatinine of 2.5 mg/dl. Serum K+ , Na+ and Cl -
The mechanism of intramural gas production remains
levels were decreased to 2.3 mEq/L, 122 mEq/L and
unclear. Various theories have been proposed to explain
89 mEq/L respectively. Upright abdominal radiographs
the aetiology of PCI, an uncommon condition character-
demonstrated air-fluid levels. An emergency laparotomy
ized by multiple gas cysts in the intestinal wall. A
was performed with diagnosis of acute abdomen.
mechanical theory postulates that these gas collec-
Multiple gas bubbles were found in submucosa of the
tions originate from ruptured bullae in pulmonary
seventy centimeters ileal segment. The bowel felt
emphysema (4) or from breaches in the gut mucosa
spongy and cryptic on palpation and there was patchy
caused by intestinal obstruction, colonoscopy, or bowel
serosal discoloration, possibly caused by ischaemic
surgery (5). This theory is supported by the occurrence
change. His stomach was also distended due to pyloric
of PCI after endoscopy ; the cause is insufflated air pen-
ulcer. Intraoperatively, the affected 70 cm ileal segment
etrating the mucosa (6), probably through microbreaks
was resected and end-to-end primary one layer (with 3/0
induced by the trauma of the procedure. Some reports
polydeoxanone) intestinal anastomosis was performed.
suggest that gas-forming bacteria enter the submucosa
Since the patient had also pyloric stenosis, bilateral trun-
through mucosal rents and produce gas within the
cal vagotomy + antrectomy + gastroenterostomy +
intestinal wall (7). Mucosal abnormalities noted in PCI
Braun anastomosis were performed. After surgery, the
are believed to be the sites of entry for gas-producing
clinical course was uneventful. The patient was dis-
bacteria (7). This bacterial theory is supported by the
charged on postoperative day 8 without any problem. At
observation that resolution of the cysts in some cases
his control visit (postoperative day 20), his physical
may be achieved by metronidazole treatment (8). The
examination, laboratory and X-ray findings were nor-
evidence against the bacterial theory is that cultures of
mal.
the cysts are consistently sterile and that cyst rupture
Histopathological examination of the specimen
does not produce acute bacterial peritonitis. A recent
showed multiple cysts in submucosa of the intestine, and
theory proposes that the cysts are maintained by unusu-
giant cells lining the cysts wall. Pneumatosis infiltrated
al high partial pressures of hydrogen in the lumen of the
partly into the muscularis propria. In this case, there
bowel (2). A high H2 tension is thought to reduce the par-
were found no changes due to either submucosal micro-
tial pressure of nitrogen in the tissue below that in
abscesses, necrosis, phlegmonous enteritis, or thrombi
venous blood, thus preventing effective gas absorption.
in any large blood vessels, or bacterial infiltration, too.
Appropriate management of the patients with PCI
depends on the overall clinical picture. Treatment is not
Discussion indicated in asymptomatic patients, whereas immediate
surgical intervention is necessary in patients with
Pneumatosis cystoides intestinalis may involve any part abdominal catastrophes. Sometimes, it is impossible to
of the gastrointestinal tract from the oesophagus to the attribute the signs and symptoms to either the underly-
rectum (2). PCI occurs in several clinical settings : a) in ing disease or PCI itself. Thus, management should be
premature infants with underlying necrotizing entero- approached on an individual basis. The reported cases
744
needed surgical interventions and post surgery clinical
courses were uneventful.
These cases illustrate the need for increased aware-
ness of this rare, but on patient basis, rapidly progressive
and fatal condition.
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M. Turan
Gokcebostan Mah. 2. Sok. Hurriyet Apt. Daire No :2
TUR-58070 Sivas, Turkey
Tel. : + 90-346-225043
Fax : + 90- 346 2262162
E-mail : mturan_1999@yahoo.com