AJR 2003; 180:213-214
© American Roentgen Ray Society
Localized Intestinal Lymphangiectasia: CT Findings
Dal Mo Yang1 and
Dong Hae Jung2
1 Department of Radiology, Gachon Medical College, Gil Medical Center, 1198,
Guwol-Dong, Namdong-Gu, Inchon, 405-760, South Korea.
2 Department of Pathology, Gachon Medical College, Gil Medical Center, Inchon,
405-760, South Korea.
Received April 1, 2002;
accepted after revision June 3, 2002.
Address correspondence to D. M. Yang.
Introduction
Intestinal lymphangiectasia is a rare disease characterized by severe
edema, thickening of the small-bowel wall, protein-losing enteropathy,
ascites, and pleural effusion
[1]. Findings at pathology
include a dilatation of the lymphatics in the mucosa and submucosa of the
small bowel with resultant bowel wall thickening due to edema and congestion
[2,
3].
Intestinal lymphangiectasia may be generalized or localized, depending on
the site of blockage of mesenteric lymphatic drainage
[4]. Most reported cases of
intestinal lymphangiectasia are of a generalized form
[2,3,4,5,6,7],
and CT has shown diffuse thickening of the small-bowel wall—a result of
engorgement of the villi that contain the dilated lymphatics. However, to our
knowledge, CT findings of localized intestinal lymphangiectasia have not
previously been reported. We describe the CT findings in a case of localized
intestinal lymphangiectasia.
Case Report
A 37-year-old man was transferred to our emergency department with blunt
trauma resulting from an automobile crash. The patient stated that he was well
before the collision. He complained of abdominal pain; however, his vital
signs were stable. Physical examination revealed a tenderness in the left
lower quadrant of the abdomen. All laboratory test results were normal.
We administered 120 mL of iopromide (Ultravist; Schering, Ansung, Korea) IV
and then performed helical CT (Somatom Plus 4; Siemens, Erlangen, Germany) of
the abdomen. The CT images showed a moderate amount of retroperitoneal
hematoma adjacent to the thickened descending colon. In addition, we saw a
focal circumferential thickening (
10 mm) of the small-bowel wall with low
attenuation (<30 H) (Fig.
1A). No evidence of ascites or pleural effusion was found.
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Fig. 1A. —37-year-old man with localized intestinal lymphangiectasia.
Contrast-enhanced CT scan shows circumferential thickening of wall of jejunum
(arrow) with low attenuation. Moderate amount of hematoma can be seen
in left anterior pararenal space.
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On the patient's seventh day in the hospital, we again performed abdominal
helical CT after IV administration of the contrast medium. Images at this time
showed that the amount of retroperitoneal hematoma had decreased, although the
wall thickening of the descending colon and small bowel was unchanged
(Fig. 1B).
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Fig. 1B. —37-year-old man with localized intestinal lymphangiectasia.
Contrast-enhanced CT scan obtained 7 days after admission shows hematoma of
left retroperitoneum is decreased, but wall thickening of jejunum
(arrow) is not changed.
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On his 11th day in the hospital, the patient developed hematochezia. An
exploratory laparotomy was performed under a diagnosis of rupture of the
intramural hematoma of the bowel. During surgery, a short segment of
descending colon was found to be necrotic, and a short segment of jejunum also
looked necrotic. A segmental resection of the descending colon and jejunum was
performed. Gross pathologic examination revealed a thickened and necrotic
colon wall and an ulcer of 1.3 cm. A short segment of small-bowel wall was
thickened and yellow (Fig. 1C).
Microscopic examination revealed dilated lymphatics in the mucosa and
submucosa of the jejunum and marked engorgement of the plicae circulares
(Fig. 1D).
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Fig. 1C. —37-year-old man with localized intestinal lymphangiectasia.
Photograph of gross specimen of portion of jejunum shows segmental wall
thickening with prominent plicae circulares. Mucosa is yellow due to large
amount of lymphatic fluid in dilated lymph vessels.
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Fig. 1D. —37-year-old man with localized intestinal lymphangiectasia.
Photomicrograph of mucosal and submucosal tissue from wall of jejunum shows
severe lymphatic dilatation in submucosa with marked engorgement of plicae
circulares. Lymphatic dilatation is also visible in villi. (H and E,
x10)
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Discussion
Intestinal lymphangiectasia can be primary, resulting from a congenital
lymphatic blockage, or secondary, resulting from inflammatory or neoplastic
involvement of the lymphatic system
[4]. The pathogenesis of
localized intestinal lymphangiectasia is unclear. However, it may develop when
the lymphatic blockage involves a limited segment of the bowel
[4]. Although traumatic
disruption of the lymphatic system and the subsequent development of
intestinal lymphangiectasia may be possible, the cause of the localized
intestinal lymphangiectasia in our patient is not clear.
In primary and secondary intestinal lymphangiectasia, CT findings have been
described as diffuse nodular thickening of the small bowel without adenopathy
or hepatosplenomegaly [2] and
as linear hypodense streaking densities in the small bowel caused by dilated
lymphatic channels [3]. A halo
sign that consists of a middle zone of lower attenuation of lymph in the
submucosa surrounded by an outer ring of higher attenuation representing the
muscularis propria and serosa may be present in the small-bowel wall
[5].
Our case showed some differences from previous reports of CT findings in
intestinal lymphangiectasia. First, intestinal lymphangiectasia is typically a
diffuse process with ascites and pleural effusion
[1,2,3,4,5,6,7].
In contrast, our patient had localized involvement of the small bowel and no
ascites or pleural effusion. Second, in previously reported CT findings in
lymphangiectasia, the zone of low attenuation in the wall of the small bowel
has been thin [2,
5,6,7].
In our patient, however, the zone of low attenuation in the small-bowel wall
was thicker than has been reported. This CT finding is well correlated with
the pathologic findings in our patient: the hypodense zone, which is caused by
dilated lymphatics and the lymphatic dilatation in the mucosa and submucosa of
the small bowel, is more severe and broad than described in earlier reports
[2,
3,
5].
The usual differential diagnosis in a thickened bowel wall that shows
homogeneously low attenuation on contrast-enhanced CT includes ischemia,
infarction, inflammation, and lymphoma
[8]. We think that localized
intestinal lymphangiectasia should be included in the differential diagnosis
of a thickened bowel wall with homogeneously low attenuation on CT.
References
- Vardy PA, Lebenthal E, Shwachman H. Intestinal lymphangiectasia: a
reappraisal. Pediatrics
1975;55:842
-851[Abstract/Free Full Text]
- Fakhri A, Fishman EK, Jones B, Kuhajda F, Siegelman SS. Primary
intestinal lymphangiectasia: clinical and CT findings. J Comput
Assist Tomogr 1985;9:767
-770[Medline]
- Puri AS, Aggarwal R, Gupta RK, et al. Intestinal lymphangiectasia:
evaluation by CT and scintigraphy. Gastrointest Radiol
1992;17:119
-121[Medline]
- Fox U, Lucani G. Disorders of the intestinal mesenteric lymphatic
system. Lymphology
1993;26:61
-66[Medline]
- Stevens RL, Jones B, Fishman EK. The CT halo sign: a new finding in
intestinal lymphangiectasia. J Comput Assist Tomogr
1997;21:1005
-1007[Medline]
- Horton KM, Corl FM, Fishman EK. CT of nonneoplastic diseases of the
small bowel: spectrum of disease. J Comput Assist
Tomogr 1999;23:417
-428[Medline]
- Horton KM, Fishman EK. Uncommon inflammatory diseases of the small
bowel: CT findings. AJR
1998;170:385
-388[Free Full Text]
- Macari M, Balthazar EJ. CT of bowel wall thickening: significance
and pitfalls of interpretation. AJR
2001;176:1105
-1116[Free Full Text]
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Introduction
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