Rare disease
Case report
1
College of Medicine, The
Pennsylvania State University,
Hershey, Pennsylvania, USA
2
Department of Surgery, Milton
S Hershey Medical Center,
Hershey, Pennsylvania, USA
Correspondence to
Dr Amanda B Cooper,
​acooper2@​pennstatehealth.​
psu.​edu
Accepted 27 March 2019
© BMJ Publishing Group
Limited 2019. No commercial
re-use. See rights and
permissions. Published by BMJ.
To cite: Renko AE,
Mirkin KA, Cooper AB. BMJ
Case Rep 2019;12:e228594.
doi:10.1136/bcr-2018-
228594
Peritoneal encapsulation: a rare cause of small
bowel obstruction
Abagayle E Renko,1 Katelin A Mirkin,2 Amanda B Cooper2
Summary
Peritoneal encapsulation syndrome (PES) is a rare
cause of small bowel obstruction (SBO) in patients
with no prior history of abdominal surgery. First
described by Cleland in 1868, PES is a congenital
condition characterised by small bowel encasement
in an accessory, but otherwise normal peritoneal
membrane.1 2 A result of abnormal rotation of the midgut
during early development, the condition causes fibrous
encapsulation of the intestines, thus preventing bowel
distention.3 While preoperative diagnosis is difficult,
several case reports have described clinical and imaging
signs that can help clinicians with not only recognising
the condition but also preparing appropriately for
perioperative discovery of anatomical variants.3 4
Background
Small bowel obstruction (SBO) is an important
cause of morbidity worldwide, accounting for up to
15% of surgical admissions for acute nontraumatic
abdominal pain.5 While adhesion formation and
hernias are commonly associated with SBOs, main-
taining a broad differential diagnosis is important
for any physician evaluating these patients. Our
patient presented at age 38 with no prior surgical
history and a 24-hour history of abdominal pain
with associated nausea, emesis and abdominal
distention. Obstruction secondary to an internal
hernia sac visualised on CT scan was suspected as
the initial aetiology. However, on exploratory lapa-
rotomy the small bowel was found to be encased in
a hernia sac, and a congenital band causing a closed
loop obstruction was visualised with an associated
anomalous artery. These findings are consistent
with peritoneal encapsulation syndrome (PES),
a rare yet clinically important cause of SBO that
deserves recognition among clinicians worldwide.
Case presentation
A 38-year-old male patient presented to the emer-
gency department of our hospital with a 24-hour
history of severe, sharp, right lower quadrant
abdominal pain with associated abdominal disten-
tion, nausea, several episodes of emesis and back
pain. This patient had no previous history of
abdominal surgery, and his medical history was
notable only for hypertension. Physical examina-
tion revealed an ill-appearing man in distress. His
pulse was 74 beats/min, temperature was 36.6°C,
blood pressure was 137/85 mm Hg. There was no
cyanosis or jaundice, and no abnormalities of the
Renko AE, et al. BMJ Case Rep 2019;12:e228594. doi:10.1136/bcr-2018-228594
chest or cardiovascular system were noted. Exam-
ination of the abdomen revealed firmness with
moderate distension and diffusely tympany to
percussion with voluntary guarding on palpation of
the left abdomen with a soft, non-distended right
abdomen. Examination was negative for rebound
tenderness. There was no hepatomegaly or spleno-
megaly noted.
Investigations
Routine laboratory workup revealed a total leuco-
cyte count of 22.05x 109/L, haemoglobin of 145 g/L
and normal serum chemistry except for an elevated
anion gap of 18 mmol/L. Serum lactate was signifi-
cantly elevated at 3.3  mmol/L. Liver function
tests, lipase and urinalysis were unremarkable. CT
scan of abdomen and pelvis (figure 1) revealed
multiple dilated loops of bowel which appeared to
be contained within a hernia sac, consistent with
an internal hernia and resulting in a closed loop
obstruction due to a congenital band.
Differential diagnosis
The differential diagnosis included an internal
hernia due to congenital adhesions, partial malrota-
tion with volvulus and a paraduodenal hernia.
Treatment
The patient was started on intravenous fluids and
antibiotics due to concern for an intra-abdominal
infection, and a nasogastric tube was placed. Given
his CT findings and accompanying lactic acidosis,
he was taken to the operating room emergently for
an exploratory laparotomy.
On opening the peritoneum, there was evidence
of a large hernia sac in the mid abdomen slightly
to the left of midline, which appeared to contain
most of the small bowel. Congenital adhesions were
noted between the omentum and left upper quad-
rant, as well as between the hernia sac and the left
peritoneal reflection. There was no evidence of a
defect in the transverse colon mesentery and the
entire colon appeared to have normal retroperito-
neal attachments. Adhesiolysis was performed, and
the hernia sac was opened. Decompressed prox-
imal small bowel with relatively dilated mid small
bowel and decompressed distal small bowel was
visualised, along with a twisted mesentery. Lysis
was performed of a congenital adhesive band from
the base of the mesentery in the left abdomen to
the right lower quadrant along the mesentery of
the terminal ileum, which relieved the closed loop
1
 Rare disease
Figure 1  Preoperative CT scan demonstrating multiple loops of
bowel encased in a hernia sac.  SMA, superior mesenteric artery; SMV
,  superior mesenteric vein.  The normal orientation of these two
structures rules out a complete malrotation.
obstruction. An aberrant artery and vein were then discovered,
initially presumed to be the right gonadal artery and vein, but
ultimately found to be an aberrant unnamed vessel. These vessels
were ligated following skeletonisation and vessel looping to
confirm bowel viability in their absence, as their position crossing
the terminal ileum posed a significant risk of subsequent reherni-
ation beneath them. The hernia sac was not fully excised, as it
was connected to the peritoneum in several places. The entire
small intestine was run proximally from the ligament of Trietz to
the ileocecal valve with no evidence of ischemia or serosal injury.
Outcome and follow-up
The patient’s postoperative recovery period was complicated
by a community-acquired pneumonia diagnosed on the second
postoperative day and treated with antibiotics. He demonstrated
return of bowel function on the third postoperative day, and was
subsequently discharged on the fourth postoperative day. Now,
4 months after surgery, he is doing well and has returned to his
normal activities without any limitations or further symptoms.
Discussion
This condition was first documented in the UK by Cleland in
1868, though it was first observed about 10 years prior.1 A
Medline search revealed that only 29 cases have been reported
since then worldwide, with only one case documented in the
USA in Chicago in 1992.6 PES should not be confounded with
abdominal cocoon syndrome, characterised by total or partial
encasement of the small bowel in a fibrocollagenous cocoon-like
sac with extensive intrinsic small bowel adhesions, or with retro-
peritoneal fibrosis, characterised by retroperitoneal tissue fibro-
sclerosis often encasing the ureters.2 7 Of note, several cases with
features more consistent with abdominal cocoon syndrome have
been reported as PES.8 Another similar yet unique condition is
sclerosing encapsulating peritonitis, in which the small bowel
is covered by a dense grey collagen membrane with a clearly
suspected underlying aetiology.9
PES is a congenital condition, thought by some to be caused
by malrotation of the midgut during development.3 Others,
however, believe its underlying aetiology is related to the
2 Renko AE, et al. BMJ Case Rep 2019;12:e228594. doi:10.1136/bcr-2018-228594
chorionic cavity entering the abdomen with the intestines during
development instead of remaining at the base of the umbilical
cord.10Patients typically present with acute gastrointestinal
symptoms such as abdominal pain, nausea and emesis; however,
several reports have described a history of episodic pain that
resolved spontaneously in the past.6 11 12 Many reports have also
described asymptomatic patients in which the condition was diag-
nosed either incidentally at laparotomy or found at autopsy.12–17
Though the condition may present as an isolated anomaly, it has
also been described with incomplete situs inversus.18
Preoperative diagnosis is difficult but possible if clinical
suspicion is high. One report discusses two clinical signs typi-
cally indicative of PES: fixed, asymmetrical distention of the
abdomen and palpating different consistencies of the abdominal
wall. While the flat area of the abdomen should be firm due to
the dense fibrous capsule, the distended area should be soft.3
Conventional radiographs are typically consistent with bowel
obstruction, as are CT images. Mitrousias et al believe preop-
erative diagnosis of this condition is aided by identification of a
‘helix sign’ on CT scan in which the loops of bowel are visualised
in a pinwheel-type pattern.4
A commonality among all patients with PES is intraopera-
tive discovery of a sac morphologically similar to peritoneum
encasing the small bowel, though some reports such as ours
also describe an obstructive band and associated aberrant artery.
In these reports, the band was divided to relieve the obstruc-
tion, and its associated vessel was observed to divide into two
branches above the terminal ileum.19–21 Histological examina-
tion of the sac typically reveals mesothelium of peritoneal origin
covering fibrovascular tissue.22
Complete sac excision, band division and aberrant artery divi-
sion is indicated to reduce risk of recurrence or further compli-
cations, even if found incidentally.20 Histological analysis of
the excised sac can be performed to confirm the diagnosis or
to exclude any concerning pathologies, if necessary. Laparotomy
has been the mainstay of treatment for this condition, though a
laparoscopic approach may be possible if the condition is prop-
erly diagnosed preoperatively.4 While overall prognosis of PES
is excellent and recurrence has yet to be described, it should not
be considered a harmless condition. Not only does the condi-
tion pose an inherent risk of strangulation and consequent bowel
necrosis requiring partial excision, but acute aortic occlusion
secondary to extrinsic compression from the bowel sac has also
been described.6 23
Learning points
►► A combination of vigilant and informed clinical and radiologic
examinations can not only aid in the detection of peritoneal
encapsulation syndrome (PES) but also reduce perioperative
and postoperative complications.
►► Awareness of radiographic signs associated with PES may
allow for preoperative detection of the condition, which can
allow for a more informed preoperative discussion about the
expected outcomes.
►► Early detection can help surgeons adequately prepare for
perioperative procedures, and help them detect anatomic
abnormalities that might lead to subsequent obstruction or
infarction.
►► Accurate preoperative diagnosis may allow surgeons to first
employ a laparoscopic approach, rather than a more invasive
laparotomy.

Contributors  AER and ABC contributed to the conception and design, while
all authors, including KAM, assisted with interpretation of data and drafting of
the article or revising it critically for important intellectual content. All authors
contributed equally to the acquisition of data or analysis, and to the final approval of
the version published. All authors also agreed to be accountable for the article and
to ensure that all questions regarding the integrity of the article are investigated and
resolved.
Funding  The authors have not received a specific grant for this research from any
funding agency in the public, commercial or not-for-profit sectors.
Competing interests  None declared.
Patient consent for publication  Obtained.
Provenance and peer review  Not commissioned; externally peer reviewed.
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Renko AE, et al. BMJ Case Rep 2019;12:e228594. doi:10.1136/bcr-2018-228594
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