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A rare case of segmental small bowel pneumatosis intestinalis: a case report. 
Introduction
Pneumatosis intestinalis (PI) is a rare condition that was first described by Du Vernoy in
1730 from cadaveric dissections.1 It remains a difficult condition to successfully manage and
presents a challenge to surgeons in both an acute and outpatient setting.1 The overall
incidence of PI in the general population has been reported to be 0.03%.2 It is thought that the
increased availability and use of Computed Tomography (CT) has resulted in greater
detection rates with an incidence of 0.37% being reported CT studies.3,4 PI has numerous
aetiological causes resulting in a spectrum of clinical presentations ranging from subacute
bowel obstruction to no overt symptoms. In early case reports, PI was considered as either
primary or secondary in origin, with a quoted 85% of cases secondary to necrotic, non-
necrotic gastrointestinal or pulmonary pathology.5 We describe a rare case of segmental PI of
the small bowel presenting with acute abdominal pain.
Case Report
A 74 year old Caucasian male was referred to the Gastroenterology outpatient with a very
gradual onset of abdominal distension and pain over a period of one year associated with
intermittent absolute constipation and occasional vomiting. His symptoms were aggravated
by meals and by posture particularly when driving for long periods of time. He described a
significant loss of weight thought to be approximately thirteen kilos over a year. His medical
background included atrial fibrillation, polymyalgia rheumatica, anaemia and hypertension.
Surgical history included an open bilateral inguinal hernia repair as well as recurrent
umbilical hernia repairs. Upon examination, he was found to be 6 feet 4 inches tall with a
weight of 92 kgs (BMI 25). He was also noted to have signs suggestive of marfan’s syndrome
including pectus carinatum, ectopia lentis and a high arched palate. The abdomen was
distended but soft and non-tender with normal bowel sounds. Routine blood tests revealed a
normocytic anaemia with a haemoglobin of 11.3 g/dl and a mean corpuscular volume of 82.
Liver, kidney and bone profiles were unremarkable. Endoscopic investigations were
undertaken, an OGD demonstrated Barrett’s and a subsequent colonoscopy was found to be
normal. An initial plain chest radiograph demonstrated bilateral free sub diaphragmatic air
(figure 1). A Computed Tomography (CT) abdomen/pelvis demonstrated extensive
pneumatosis intestinalis involving his small bowel with free intraperitoneal air reported as
possibly secondary to a malrotation of his proximal small bowel (figure 2).
Due to a lack of improvement in his symptoms he was referred for a surgical opinion.
Following a surgical review it was elected to perform an elective exploratory laparotomy.
Intra-operatively, he was found to have extensive and bulky pneumatosis intestinalis
extending from the duodenal-jejenal flexure to the terminal ileum with only a few very short
segments of normal bowel found (figure 3). There was no evidence of a malrotation, bands
or strictures. The small bowel mesentery was noted to be especially long along its entire
length. The colon was entirely normal as well as the rest of the intra-peritoneal organs. Small
bowel resection was not undertaken in view of the absence of any significant length of
normal bowel. The post- operative recovery was uneventful.
Discussion
As our case demonstrates, in the majority of cases the provisional diagnosis of PI is made
based on the classic radiological findings on CT demonstrating extra-luminal gas in the
bowel wall. The pathogenesis remains uncertain, but it is believed to be as a result of multiple
contributing factors.6 It is thought that it can occur as a result of two different processes. It
can either occur due to intra-luminal gas traversing microbreaks in the bowel mucosa
secondary to inflammation or ischaemia. Alternatively, it may be as a result of direct gas
diffusion across an intact mucosal membrane, such as in instances of increased abdominal
pressure.7 As previously mentioned secondary PI is associated with many different
aetiological factors although not an exhaustive list, known associations include mechanical
causes such as volvulus, autoimmune disease such as Crohns disease, infective agents such as
HIV, connective tissue disorders such as scleroderma, respiratory aetiologies for example
COPD, drug toxicities, graft versus host disease, transplantation and gut ischaemia.1 Our case
is of interest as the cause of his PI was secondary to small bowel volvulus secondary to a long
and hypermobile small bowel mesentery. We believe that the patient’s small bowel
mesenteric length could be related to the patient’s suspected Marfan’s syndrome. It is well
recognised that PI can be secondary to connective tissue disorders, however there are no
reports of an association with Marfan’s syndrome.1 Interestingly, our hypothesis is supported
by the fact that bowel obstruction in Marfan’s syndrome has been reported secondary to long
caecal and sigmoid mesenteries.8 The location of PI varies and may be anywhere from the
stomach to the rectum. The location of the disease can act as a good guide to the aetiology
with proximal disease usually being secondary to pyloric stenosis, gastric malignancy or
ulcers whereas distal disease may be as a result of diverticulitis or mesenteric ischaemia.9
Most reports of PI describe continuous portions of diseased bowel secondary to an intramural
disease process or a mechanical obstruction. Interestingly, segmental disease as was seen in
our case is rarely found. The initial priority in the management of PI is to clarify whether the
underlying pathology is life threatening or a benign cause. As was seen in our case, it is
common for imaging such as plain radiographs or CTs to demonstrate features suggestive of
free intraperitoneal gas (figure 1). This is thought to be as a result of the perforation of
individual gas filled cysts.10 However, it is important to consider the possibility of a
perforated viscus and it is thus crucial to correlate radiographic signs with clinical findings.
Involvement of a Radiologist at an early stage is advisable as certain radiographic features
such as crescentic or linear gas collections can be indicative of a bowel infarction.11 In the
case of a suspected life threatening condition such as bowel ischaemia, surgical intervention
in the form of an emergency exploratory laparotomy is mandatory. In view of this, features of
low-flow vascular states such as sepsis, congestive heart failure, use of ionotropic agents,
and other causes of hypotension in the acute setting should lead to a high index of suspicion
for ischaemic bowel and a low threshold for surgery.9 Conservative management involving
nasogastric bowel decompression maybe appropriate if a serious underlying cause of the PI
has been excluded.12 However, with high associated mortality rates of between 22-50% non-
surgical management should be used with caution.
Conclusion
This case demonstrates that both surgical and gastroenterology expertise are required to
successfully manage this challenging condition. In addition, our case highlights that although
pre-operative imaging has an important role in PI, clinical judgement is paramount in tackling
this rare condition.
http://www.sciencedirect.com/science/article/pii/S2210261211000599
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