An attempt to unravel features of Pneumatosis
cystoides intestinalis
Introduction:
Pneumatosis cystoides intestinalis(PCI) is a rare disease characterized by
presence of multiple gas filled cysts in subserosal or submucosal wall of large
intestine or small intestine(1). PCI are most commonly due to an underlying
disease or can be idiopathic. Understanding of etiology and pathogenesis is
necessary in each individual case for appropriate management.
Materials and methods:
It was a retrospective study.All enteral resected specimens received in
department of pathology PESIMSR, kuppam from January 2008 to September
2011 were studied. Clinical details and histopathological slides were retrieved
from archives in the department of pathology.
Aim of study:
To study frequency, clinical, and morphological characteristics of histologically
diagnosed PCI.
Results:
Enteral resections were 38 in number during this period. Most common
indication for resection was perforation. Most of enteral resections were seen in
>40 year age group(table1) and in males(M-24:F-14). Small bowel resections
were common among all enteral resections(table2). Three cases of PCI were
seen and all were in small bowel resections .
Case 1: 60year male presented with lower abdomen pain and vomiting.
Ultrasound showed bowel wall thickening in ileocaecal region and free fluid in
paracolic gutter. 3 segments of small intestine was received in our department
which showed mucosal ulceration and multiple cystic spaces in the wall(fig1,2).
Microscopically these cystic spaces had no lining epithelium which was
surrounded by foreign body giant cells(fig3,4).
Case 2: 55year male presented with pain abdomen and fever. Segment of small
intestine was received in our department which showed edematous mucosa.
Microscopically these cystic spaces had no lining epithelium which was
surrounded by foreign body giant cells.
Case 3: 16year male presented with sudden pain abdomen. Small intestine
perforation was found intraoperatively. Grossly perforation was confirmed.
Microscopically these cystic spaces had no lining epithelium which did not
show foreign body giant cells(fig5,6).
Discussion: Only 3 cases of PCI with described microscopic features were
found in literature. Saber A et al (2) will be designated as case 4, Sakurai Y et
al(1) will be designated as case 5, Mutha S et al(3) will be designated as case 6.
Age of presentation in literature(case 4,5,6)was 45 to 60years and our case 1&2
had similar age group but case 3 was younger age group (16 years) . All the
cases in the literature (case 4,5,6) and our cases(case 1,2,3) had common
presenting symptom as pain abdomen. Abdominal distension was seen in 2 of
compared cases(case 5&6), none of our cases showed distention of
abdomen(table3). Grossly our cases showed mucosal ulceration , edema and
perforation. In addition case1 showed multiple cystic space in wall. All cases of
literature had multiple cysts grossly(table4). Microscopy showed cystic space in
all the cases. All cases had giant cell reaction except case 3 and 4(table5).
Pathogenesis can be by bacterial, mechanical or pulmonary for PCI . (fig7).In
case 4 and 5 pathogenesis was bacterial , mechanical or pulmonary factor but in
cases 1 and 2 the cause/pathogenesis was mechanical factor .In Case 3
pathogenesis was contradicted weather perforation lead to PCI or PCI was
cause of perforation(table6). Complications of PCI include intestinal
obstruction, pneumoperitoneum , intussusception , volvulus , haemorrhage and
intestinal perforation(2) . No therapy is required for asymptomatic cases .(2) For
primary PCI Metronidazole and hyperbaric oxygen are used(5). For Secondary
PCI with or without complication surgery is indicated . (2) In all our cases the
therapy was surgery because they presented with intestinal obstruction.
Conclusion: Pathogenesis is unclear and many .(2) Therapy of PCI can be
conservative or surgical depending on the etiology or any complication (5).Once
the disease is set in , perforation can lead to PCI or vice versa and it is difficult
to know which came first. (2) It is an under recognized feature often mistaken
for artifact.
Awareness of this rare entity and high index of suspicion with knowledge of
etiopathogenesis can avoid major bowel surgery
References
1. Sakurai Y ,Hikichi M , Isogaki J, Furuta S, Sunagawa R, Inaba K et al.
Pneumatosis cystoides intestinalis associated with massive free air
mimicking perforated diffuse peritonitis . World J Gastroenterol . 2008 ;
14 : 6733 – 56.
2. Saber A. Pneumatosis intestinalis with complete remission : a case report
.Cases journal .2009 ; 2 : 7079 – 82.
3. Mutha S, Kumbhalkar DT, Bobhak SK. Pneumatosis cystoides
intestinalis – a case report . Indian J Pathol Microbiol. 1999; 43 :157 -8.
4. Hughes DTD, Gordon KCD , Swann JC, Bolt GL. Pneumatosis cystoides
intestinalis. Gut. 1966; 7 : 553 – 7.
5. Braumann C, Menenkes C, Jacobi CA. Pneumatosis intestinalis – a
pitfall for surgeon? . Scandinavian Journal of surgery . 2005 ; 94 : 47 –
50.
Table 1.- total no of enteral resections-38
Age group(years)
0-20
21-40
41-60
>60
No of cases
7
9
14
8
Table-2
Resections
Small intestine
Large intestine
both
No. of cases
27
7
4
Table-3
Our cases of PCI
Clinical features
Case 1
• Lower abdomen pain
• Vomiting
Case 2
• Pain abdomen
• Fever
Case 3

Case 4 .Saber A
 Pain upper abdomen
Acute abdomen
Case 5 .Sakurai Y
et al
• Pain abdomen
• Abdominal distension
Case 6 .Mutha S et
al
• Generalized pain abdomen
• Abdominal distension
Table-4
cases of PCI
Case 1
Case 2
Case 3
Compared cases
4 . Saber A
5 . Sakurai Y et al
6 . Mutha S et al
Table-5
Gross
3 segments of small intestine with
ulceration
C/S : Multiple cystic spaces
Segment of small intestine with ed
mucosa
Segment of small intestine with an
perforation
Gross
Serosal intestinal air cysts involving
ileum
Multiple gas filled subserosal vesic
bowel wall & the mesentry of smal
Multiple transparent thin walled cy
sizes
cases of
PCI
Microscopy
Case 1.
• Cystic spaces
• Giant cells
• Subserosa
Case 2.
• Cystic spaces
• No lining
epithelium
• Giant cell
reaction.
• Submucosal
& subserosa
Case 3.
• Cystic spaces
• No giant cell
reaction
Compared M Microscopy
cases
oscopy
4 . Saber A
• Cysts in
submucosa
with varying
sizes &
shapes.
• No giant cell
reaction
5 . Sakurai Y
et al
6 . Mutha S
et al
• Cystic spaces
in sub
mucosa and
sub serosa
• Multiple cysts
of varying
sizes
predominantl
y in
subserosa &
submucosa
• Mucosa is
normal
• Giant cell
reaction.
Table-6
cases
findings
Case 1
-No pulmonary causes
-Intraoperative finding
showed entero vesical
fistula
-Microscopy showed no
underlying cause ,Only
chronic inflammatory
process with PCI was
noted was noted
-Volvulus intestinal
obstruction
-No much clinical detail
-Intraoperative finding
showed ileal perforation
Case 2
Case 3
pathogenesis
Mechanical factor
Mechanical
factor
Perforation or
PCI which one
came first?
Fig-1
Multiple cystic spaces in cut section of small intestine
Fig-2
Multiple cystic spaces in wall of small intestine
Fig-3 cystic spaces with no lining epithelia.H&E,400X
Fig-4 Cystic spaces with surrounding giant cell reaction.H&E,100X
Fig-5 cystic space with no giant cell reaction.H&E,400X
Fig-6 cystic space with no
giant cell
reaction.H&E,100X
Fig-7
Pathogenesis (4 , 5)
Mucosal Injury
cough bout
Loss of integrity of
Severe
intestinal mucosa
Bacterial invasion into
rupture
intramural compartment
Alveolar
Gas enters through these
spaces
mediastinum
BACTERIAL INFECTION
Air into
Permeates submucosa
Air tracks
through
diaphragm
and retro - peritoneal tissue
MECHANICAL FACTOR
Emerge along
intestinal arteries in subserosal planes
PULMONARY FACTOR