DOI:10.2214/AJR.07.2109
AJR 2007; 189:786-790
© American Roentgen Ray Society
Intramural Fat in the Duodenum and Proximal Small Intestine in Patients with Celiac Disease
Francis J. Scholz1,
Spencer C. Behr and
Christopher D. Scheirey
1 All authors: Department of Diagnostic Radiology, Lahey Clinic, 41 Mall Rd.,
Burlington, MA 01805.
Received February 22, 2007;
accepted after revision May 20, 2007.
Address correspondence to F. J. Scholz
(francis.j.scholz{at}lahey.org).
Abstract
OBJECTIVE. The purpose of this article is to describe and illustrate
intramural fat in the duodenum and jejunum, to our knowledge a previously
undescribed finding in celiac disease.
CONCLUSION. Celiac disease is known to produce inflammation of the
duodenum and jejunum. We propose that postinflammatory intramural fat
deposition occurs in a distribution likely unique for celiac disease. CT scans
of the chest and abdomen obtained for many indications include these portions
of the bowel. Celiac disease is now recognized as a common disease, and the
recognition of intramural fat in the duodenum and jejunum on CT may allow
earlier diagnosis.
Keywords: abdominal imaging • celiac disease • duodenitis • duodenum • gastrointestinal imaging • intramural fat • jejunitis • jejunum • sprue
Introduction
Celiac disease is a common disease that is often difficult to diagnose. CT
scans are currently obtained for a variety of indications. We wished to
determine if there were any unique findings on CT to allow the earlier
diagnosis of celiac disease.
Materials and Methods
Methods
Retrospective review of charts, images, and laboratory results of patients
with proven celiac disease was approved by the hospital institutional review
board. Subjects were obtained from a list of patients proven by our laboratory
to have tissue transglutaminase antibody titers diagnostic of celiac disease.
Following this list, we used the PACS to review all abdominal and pelvic CT
scans.
One hundred thirty-six CT scans of the chest or abdomen obtained in 82
patients with celiac disease proven by tissue transglutaminase or biopsy were
reviewed by a gastrointestinal radiologist with 33 years of experience. After
the initial review revealed striking intramural fat in the duodenum and
jejunum in some patients, all scans were systematically reviewed for this
finding. Negative attenuation (in Hounsfield units) was required in the wall
of the bowel to be considered intramural fat.
Results
Prominent intramural fat in the duodenum and jejunum was noted in 11
patients. A 12th patient was discovered during the study, and subsequent chart
review established the diagnosis of celiac disease. In axial cross section of
bowel, fat created a circle of low density
(Fig. 1A). In longitudinal
cross section it created longitudinal stripes of fat
(Fig. 2C). In some patients the
fat was noted within the folds of the bowel
(Fig. 1C).
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Fig. 1A —81-year-old woman (patient 1) who underwent virtual
colonoscopy screening for colorectal cancer. Review of her chart showed much
earlier proven diagnosis of celiac disease that had not been noted in chart
for decades. Medical history also included psoriatic arthritis treated with
methotrexate. and CT scans show thick layer of intramural duodenal fat.
Penetration of common bile duct (arrow) through wall to mucosa is
seen (A). Coronal reconstruction also defines penetration of duct
(arrow) across layer of intramural fat (B).
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Fig. 2C —65-year-old woman (patient 2) with known celiac disease and
iron deficiency anemia. Her medical history includes seasonal allergies,
gastroesophageal reflux disease, and hyperlipidemia. CT scans of thorax 4
years after A and B show layer of intramural fat in duodenum
(straight arrow) and jejunum (curved arrows).
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Fig. 1C —81-year-old woman (patient 1) who underwent virtual
colonoscopy screening for colorectal cancer. Review of her chart showed much
earlier proven diagnosis of celiac disease that had not been noted in chart
for decades. Medical history also included psoriatic arthritis treated with
methotrexate. Axial slice distal to A shows fat in duodenal folds
creating linear array (arrows) of perpendicularly oriented deposits
of fat.
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To our knowledge, intramural fat in the duodenum and jejunum has not been
previously described. We present four illustrative examples of this
pattern.
Examples
Patient 1, an 81-year-old woman, was screened for colorectal cancer with
virtual colonoscopy which showed a thick layer of intramural duodenal fat. CT
showed the penetration of the common bile duct through the wall to the mucosa
(Fig. 1A,
1B,
1C,
1D). Review of the patient's
chart showed a much earlier proven diagnosis of celiac disease, which had not
been noted in her chart for decades. One year before her CT, an upper
gastrointestinal series was performed for pain, and a scarred duodenum with
"diverticula" but no ulceration was noted. Her medical history
included psoriatic arthritis treated with methotrexate.
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Fig. 1B —81-year-old woman (patient 1) who underwent virtual
colonoscopy screening for colorectal cancer. Review of her chart showed much
earlier proven diagnosis of celiac disease that had not been noted in chart
for decades. Medical history also included psoriatic arthritis treated with
methotrexate. and CT scans show thick layer of intramural duodenal fat.
Penetration of common bile duct (arrow) through wall to mucosa is
seen (A). Coronal reconstruction also defines penetration of duct
(arrow) across layer of intramural fat (B).
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Fig. 1D —81-year-old woman (patient 1) who underwent virtual
colonoscopy screening for colorectal cancer. Review of her chart showed much
earlier proven diagnosis of celiac disease that had not been noted in chart
for decades. Medical history also included psoriatic arthritis treated with
methotrexate. Upper gastrointestinal series performed for pain 1 year before
A and B shows a scarred duodenum with diverticula
(arrows) but no ulceration.
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Patient 2, a 65-year-old woman with known celiac disease, presented to her
primary care physician with iron deficiency anemia. An upper gastrointestinal
barium study showed a fold-free duodenum with a "bubbly" bulb
(Fig. 2A,
2B,
2C). The appearance of the
jejunum was featureless, with weblike strictures indicating active celiac
disease that was subsequently successfully treated with strict gluten
restriction. Four years later a cough and an abnormal chest radiograph
prompted CT of her thorax, which showed a layer of submucosal fat in the
duodenum and jejunum. Her medical history included seasonal allergies,
gastroesophageal reflux disease, and hyperlipidemia.
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Fig. 2A —65-year-old woman (patient 2) with known celiac disease and
iron deficiency anemia. Her medical history includes seasonal allergies,
gastroesophageal reflux disease, and hyperlipidemia. and Upper
gastrointestinal barium studies show fold-free duodenum with
"bubbly" bulb (arrow, A) and featureless
appearance of jejunum (B), where weblike strictures indicate active
celiac disease.
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Fig. 2B —65-year-old woman (patient 2) with known celiac disease and
iron deficiency anemia. Her medical history includes seasonal allergies,
gastroesophageal reflux disease, and hyperlipidemia. and Upper
gastrointestinal barium studies show fold-free duodenum with
"bubbly" bulb (arrow, A) and featureless
appearance of jejunum (B), where weblike strictures indicate active
celiac disease.
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Fig. 3A —78-year-old woman (patient 3) with known adult-onset celiac
disease and right-sided abdominal pain. Her medical history includes asthma
occasionally treated with prednisone, recurrent sinusitis, and
gastroesophageal reflux disease. and Upper gastrointestinal barium studies
show celiac disease with "bubbly" nodularity in bulb and in
fold-free duodenal sweep (arrows). Malabsorption pattern was also
seen.
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Fig. 3B —78-year-old woman (patient 3) with known adult-onset celiac
disease and right-sided abdominal pain. Her medical history includes asthma
occasionally treated with prednisone, recurrent sinusitis, and
gastroesophageal reflux disease. and Upper gastrointestinal barium studies
show celiac disease with "bubbly" nodularity in bulb and in
fold-free duodenal sweep (arrows). Malabsorption pattern was also
seen.
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Fig. 3C —78-year-old woman (patient 3) with known adult-onset celiac
disease and right-sided abdominal pain. Her medical history includes asthma
occasionally treated with prednisone, recurrent sinusitis, and
gastroesophageal reflux disease. Three years later, after patient reported
progressive shortness of breath, CT scans show fine line of intramural fat
density (arrows) in jejunum in addition to intramural duodenal
fat.
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Patient 3, a 78-year-old woman with known adult-onset celiac disease,
presented to her primary care physician with right-sided abdominal pain. An
upper gastrointestinal barium study was performed in June 1999, at which time
celiac disease with a "bubbly" nodularity in the bulb and in the
fold-free duodenal sweep (Fig.
3A,
3B,
3C) was noted in addition to a
malabsorption pattern. In March 2002, she presented to a pulmonologist because
of progressive shortness of breath; a CT scan showed a fine line of intramural
fat density in the jejunum and duodenum. Her medical history included asthma
that was occasionally treated with prednisone, recurrent sinusitis, and
gastroesophageal reflux disease.
Patient 4, a 37-year-old man with no significant medical history, presented
to his primary care physician with right lower quadrant pain. Abdominopelvic
CT was ordered to evaluate for appendicitis, but none was seen. The patient's
pain persisted, prompting upper endoscopy and colonoscopy 11 months later.
Celiac disease was diagnosed at biopsies of the small intestine. The patient's
subsequent antiendomysial antibody test was strongly positive, confirming
celiac disease. Review of the original CT scan showed intramural fat in the
duodenum and jejunum (Fig.
4).
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Fig. 4 —37-year-old man (patient 4) with no significant medical
history and right lower quadrant pain. Abdominopelvic CT scans show intramural
fat in duodenum (straight arrows) and jejunum (curved
arrows). Small-bowel biopsies led to diagnosis of celiac disease.
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Discussion
Celiac disease is the result of gluten ingestion stimulating an autoimmune
destruction of small-bowel mucosal epithelium in patients with a genetic
predisposition. It is now recognized as a common disease, with a prevalence of
about 1% in the Western hemisphere
[1]. In certain subgroups, its
prevalence is markedly higher. Three to six percent of patients with type 1
diabetes and 10–15% of symptomatic patients with iron deficiency anemia
have celiac disease [1].
Disease activity varies from patient to patient, but it is thought to be a
lifelong process that may become symptomatic at any time.
Destruction of the protective villous lining of the duodenum and jejunum in
celiac disease predisposes to inflammation from gastric, biliary, and
pancreatic secretions. Once denuded, the duodenal bulb and descending
duodenum, exposed to gastric acid, are most susceptible. Abnormalities noted
in the duodenum on endoscopy
[2] and upper gastrointestinal
series
[3–5]
are believed to be sensitive nonlaboratory abnormalities indicating celiac
disease. The tissue transglutaminase antibody test is the most sensitive and
widely used test to detect the disease in symptomatic patients.
As discussed by Tomei et al.
[6] in a review of 28 patients,
certain findings are suggestive of celiac disease on CT. These include loss of
jejunal folds with an increased number of ileal folds, increased fluid in the
small bowel, intestinal dilatation, vascular engorgement, transient
asymptomatic intussusception, duodenal inflammation and thickening, and
lymphadenopathy. Four of 28 patients were noted to have isolated thickening of
the duodenum. One patient was noted to have low density in the wall of the
duodenum that was not further characterized. The most specific finding noted
to suggest celiac disease was reversal of the jejunoileal fold pattern.
Intramural fat deposition, thought to be a response to prior bowel
inflammation, has been described in the terminal and distal ileum in patients
with Crohn's disease [7]. It
has also been noted in the colon in patients with both Crohn's disease and
ulcerative colitis
[8–10].
Intramural fat has been also been described in the terminal ileum and colon in
asymptomatic patients with no known prior disease process
[11]. Although it is
considered a chronic response to inflammation, intramural fat has been shown
to occur as rapidly as 12 days after a patient has undergone chemotherapy for
lymphoma or leukemia [12]. A
single report of intramural fat has been described in a patient with radiation
enteritis [13].
To our knowledge, neither fat deposition in the duodenum and jejunum nor
its cause due to celiac disease has previously been reported. With a
prevalence of 1% or more, celiac disease is common and statistically can be
expected to be seen in about 1% or more of all CT scans. Celiac disease may be
difficult to diagnose. Although antibody tests are highly sensitive and
specific, early symptoms are nonspecific and vague. Nondiarrheal
presentations, including irritable bowel, anemia, guaiac-positive stools,
osteoporosis, renal stone disease, and malignancy, now exceed presentations
with diarrhea [14]. The
severity of celiac disease may wax and wane over time, further delaying its
diagnosis. Our fourth example is a patient who had an 11-month interval after
the CT examination before antibody testing finally confirmed the diagnosis.
During this interval, the patient had additional fruitless doctor visits and
tests.
Other inflammatory diseases of the proximal small bowel might produce
intramural fat. Crohn's disease does occur in the duodenum and jejunum and
might produce intramural fat, as has been described in the distal small bowel
and colon. None of our patients had evidence of Crohn's disease, but all had
proven celiac disease. Systematic examination of the CT scans of patients with
duodenal Crohn's disease may reveal intramural fat. In our experience, we have
never noted duodenal fat in the CT scans of patients with proximal small-bowel
Crohn's disease, nonsteroidal antiinflammatory drug-induced enteritis, or
chronic pancreatitis.
Because of the large number of CT scans that visualize the upper abdomen,
including routine CT of the abdomen and pelvis, CT of the chest, virtual
colonoscopy, and CT for renal stone disease, we believe intramural fat should
be a useful and perhaps specific diagnostic finding in celiac disease. In our
retrospective review, 11 (13.4%) of 82 patients had intramural fat. The 12th
case was prospectively diagnosed during the course of our study.
The lack of sensitivity of this finding is probably due to the extreme
variability in the severity of disease expression, which is related to both
genetic predisposition and gluten dietary load. However, this finding may be
the first (patient 4) or the last (patient 1) indication of celiac disease.
Although other clinical, laboratory, and radiographic findings may become
normal with either a decrease in or complete cessation of gluten ingestion,
intramural fat may be a long-lasting residual of the disease or may persist in
patients with subclinical disease activity. Patient 1 had no abdominal
symptoms when the striking intramural fat was discovered during virtual
colonoscopy. The disease was no longer considered a disease by the patient,
but rather a dietary condition. Her physician had actually stopped listing
celiac disease in chart notes.
Celiac disease is a significant and greatly underdiagnosed disease process
according to the 2004 National Institutes of Health (NIH) consensus panel
[15]. Although we have not
established the sensitivity of proximal small-bowel fat deposition, when
intramural fat is found in the duodenum and proximal small bowel on CT, celiac
disease must be considered. Further observation and study are required to
determine whether intramural fat in the duodenal and jejunum may occur in
other disease processes. We believe from our study and review of the
literature that it may be the most specific and unique CT finding of celiac
disease.
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Abstract
Introduction
