AJR 2005; 184:1584-1586
© American Roentgen Ray Society
Phlebosclerotic Colitis: Imaging Findings of a Rare Entity
V. Markos1,2,
S. Kelly1,
W. C. Yee1,
J. E. Davis3,
R. E. Cheifetz4 and
A. Alsheikh3
1 Department of Radiology, Vancouver General Hospital, Vancouver, BC V5Z 1M9,
Canada.
2 Department of Radiology, Gloucester Royal Hospital, Great Western Rd.,
Gloucestershire GL1 3NN, England.
3 Department of Pathology, Vancouver General Hospital, Vancouver, BC V5Z 1M9,
Canada.
4 Department of Surgery, Vancouver General Hospital, Vancouver, BC V5Z 1M9,
Canada.
Received May 27, 2004;
accepted after revision August 24, 2004.
Address correspondence to V. Markos
(vmarkos{at}hotmail.com).
Introduction
Phlebosclerotic colitis affects the colon with venous drainage into the
superior mesenteric vein. This can cause intestinal obstruction due to venous
engorgement of the ileocecal valve secondary to sclerosis of the draining
veins. Although descriptions of this condition have existed in the Japanese
literature [1] since 1989, Yao
et al. [2] first coined the
term "phlebosclerotic colitis" in 2000. On review of the
literature, we believe this entity has never been reported previously in North
America.
Case Report
A 53-year-old man, who is Taiwanese by birth but a resident in Canada since
1987, presented to the emergency department in November 2003. He had been
feeling well until 4 days previously when he developed abdominal distention,
discomfort, and constipation. He had vomited twice the previous day and once
on the day of presentation. He stated that on a visit to Taiwan 4 years ago,
he had been diagnosed with ischemic colitis of the right colon. This
apparently resolved with no specific treatment or surgery, and he remained
asymptomatic. He indicated that he had tested positive for hepatitis B but had
experienced no complications to date. The patient gave no other significant
medical history; he had no allergies and was taking no medications. On
examination, he appeared well. His abdomen was mildly distended, and
tenderness was elicited in the right lower quadrant. There were no signs of
peritonitis.
Laboratory tests included the following: white blood cell count, hemoglobin
B, platelet count, lactate, liver function test, international normalized
ratio, partial thromboplastin time, electrolytes, and creatinine, all of which
were normal. His amylase level was elevated at 206 IU/L (normal range,
35–90 IU/L) and, among other possibilities, was thought to be due to
early pancreatitis or intestinal obstruction.
On radiography, multiple tortuous threadlike calcifications were seen in
the region of the right colon, and there were dilated loops of small bowel
(Fig. 1A). The colonic gas
pattern was entirely normal. The findings were diagnostic for small-bowel
obstruction.
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Fig. 1A. —53-year-old man with phlebosclerotic colitis. On abdominal
radiograph, multiple tortuous threadlike calcifications are seen throughout
ascending colon and in proximal transverse colon. Calcification can be seen
along mesenteric veins draining ileocecal region (arrow). Note
dilatation of small-bowel loops and no gaseous distention of colon, diagnostic
for small-bowel obstruction.
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On CT (Figs. 1B and
1C), the right and proximal
transverse colon were thick-walled with numerous serpiginous venous
calcifications within the bowel wall and adjacent mesentery. Mural thickening
was more marked at the mesenteric attachment with increased adjacent hazy
density suggesting mesenteric vascular congestion. The ileocecal valve and a
short segment of adjacent terminal ileum were also thickened. The resulting
obstruction led to small-bowel dilatation proximally. The left colon was
unremarkable, and although the serum amylase level was elevated, the pancreas
appeared normal.
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Fig. 1B. —53-year-old man with phlebosclerotic colitis. Axial CT images
confirm presence of numerous serpiginous calcifications (arrowheads,
B and C) within right colon and in adjacent mesentery. Although
CT was performed after IV contrast administration, density of calcification in
mesenteric veins is obvious. Mural thickening (arrow, B) is
especially marked at mesenteric attachment in ascending colon with increased
adjacent hazy density suggesting mesenteric vascular congestion. Obstruction
of small bowel distally due to swelling of ileocecal valve from venous
congestion results in dilatation of small-bowel loops.
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Fig. 1C. —53-year-old man with phlebosclerotic colitis. Axial CT images
confirm presence of numerous serpiginous calcifications (arrowheads,
B and C) within right colon and in adjacent mesentery. Although
CT was performed after IV contrast administration, density of calcification in
mesenteric veins is obvious. Mural thickening (arrow, B) is
especially marked at mesenteric attachment in ascending colon with increased
adjacent hazy density suggesting mesenteric vascular congestion. Obstruction
of small bowel distally due to swelling of ileocecal valve from venous
congestion results in dilatation of small-bowel loops.
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On angiography (Fig. 1D),
the marginal arteries were irregular and the vasa recta of the right colon was
tortuous. The venous runoff was not obtained because the primary aim at that
time was to exclude arterial disease.
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Fig. 1D. —53-year-old man with phlebosclerotic colitis. Selective
superior mesenteric digital subtraction angiogram obtained in arterial phase
shows tortuosity of vasa recta (arrowheads) and marginal arteries
(arrow) of right colon. This finding was subtle.
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At laparotomy, the right colon and terminal ileum appeared congested,
chronically thickened, and fibrotic. Obstruction from a swollen ileocecal
valve resulted in dilatation of the proximal small bowel. A right
hemicolectomy was performed along with resection of 10 cm of distal ileum.
Microscopic examination (Fig.
1F) of the cecum and ascending colon revealed thickened veins in
the submucosa from sclerosis and hyalinization. Some of the hyalinized
sclerotic veins had extensive transmural calcification. Calcified veins
extended through the bowel wall into the surrounding fat. There was atrophy of
the mucosa and mucosal hemorrhage with extensive fibrosis in the submucosa.
Tortuous veins were present in the submucosa, muscularis propria, and serosa.
The appendix and terminal ileum also showed extensive mucosal hemorrhage with
dilated submucosal and subserosal veins. The pathologist concluded that
findings were from phlebosclerotic colitis.
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Fig. 1F. —53-year-old man with phlebosclerotic colitis. Photomicrograph
shows extensive submucosal fibrosis with thickened sclerosed veins
(arrowheads), some of which show extensive calcification
(arrows). (H and E, x10)
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Discussion
Ischemic bowel disease is most often related to arterial thromboembolic
disease. Venous abnormalities leading to ischemia have only rarely been
described [3]. Phlebosclerotic
colitis is a term coined to describe a condition where there is ischemia of
the large bowel caused by sclerosis and calcification of the mesenteric vein
wall.
In the literature, there have been many reoports, mainly of individual
cases, with a few authors having more extensive experience with this disorder.
As most of these reports are written in Japanese, we obtained our information
from the few articles written in English. The most recent review of the
literature was by Iwashita et al.
[4], who described the findings
in seven of their own patients and reviewed 14 other cases. According to the
literature, ours appears to be the first case reported in North America and
the first individual with no known Japanese decent. Over the last 17 years
since moving to Canada from Taiwan, our patient has visited relatives in
Taiwan but has never been to Japan. He presented with intestinal obstruction;
hence, a stool sample could not be sent for parasitic organisms before the
emergency right
hemicolectomy.
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Fig. 1E. —53-year-old man with phlebosclerotic colitis. Radiograph of
right hemicolectomy specimen shows threadlike calcifications within thickened
cecum and ascending colon. Arrowhead marks the terminal ileum and arrow
indicates appendix.
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Yao et al. [2] described
three cases in which the radiologic features were identical to the case
presented here—that is, vascular calcifications in the region of the
right hemicolon on abdominal radiography and colonic wall thickening with
adjacent mesenteric venous calcifications on CT. They proposed that the entity
be called "phlebosclerotic colitis" to differentiate it from
typical ischemic colitis.
It has been postulated that phlebosclerosis in the tributaries of the
superior mesenteric vein results in disturbance of the normal venous return
from the right colon with secondary ischemic colitis. The pathogenesis of
phlebosclerosis is unknown. In none of the cases described so far, nor in our
case, was there evidence of portal hypertension. Calcification has been
previously described in the portal, splenic, and superior mesenteric veins
[5,
6] in patients with cirrhosis
and portal hypertension. Extensive involvement of tributaries at the bowel
wall has, to our knowledge, never been reported with portal hypertension.
Phlebosclerosis is hence thought to be separate from calcification seen in the
portal vein secondary to thrombosis due to portal hypertension.
Patients may present with recurrent diarrhea, chronic severe lower
abdominal pain, nausea, vomiting, and tarry stool with tests positive for
fecal occult blood. Complications include intestinal obstruction from a
swollen ileocecal valve [2]. A
single case has been reported of a coexisting polyp with carcinoma in the
adenoma [6].
On barium enema [2,
7] various features have been
described, which include thickening of the colonic wall; thumb printing;
disappearance of haustral folds; and luminal irregularity, luminal narrowing
and rigidity more marked in the cecum and ascending colon. Also, swelling of
the ileocecal valve may be seen.
On angiography, dilatation of the veins along the vasa recta has been
described in the venous phase.
The etiology of phlebosclerosis remains unclear, but the radiographic and
pathologic findings are distinct and indeed may be pathognomonic.
References
- Iwashita A. Monthly meeting of the Research Society for Early
Gastric Cancer [in Japanese]. (abstr) Stomach
Intestine 1989;18:422
- Yao T, Iwashita A, Hoashi T, et al. Phlebosclerotic colitis: value
of radiography in diagnosis—report of three cases.
Radiology2000; 214:188
–192[Abstract/Free Full Text]
- William LF Jr. Mesenteric ischemia. Surg Clin North
Am 1988;68:331
–353[Medline]
- Iwashita A, Yao T, Schlemper RJ, et al. Mesenteric phlebosclerosis:
a new disease entity causing ischemic colitis. Dis Col
Rectum 2003;46:209
–220[Medline]
- Verma V, Cronin DC 2nd, Dachman AH. Portal and mesenteric venous
calcification in patients with advanced cirrhosis. AJR2001; 176:489
–492[Abstract/Free Full Text]
- Ayuso C, Luburich P, Vilana R, Bru C, Bruix J. Calcifications in
the portal venous system: comparison of plain films, sonography, and CT.
AJR 1992;159:321
–323[Abstract/Free Full Text]
- Oshitani N, Matsumura Y, Kono M, et al. Asymptomatic chronic
intestinal ischemia caused by idiopathic phlebosclerosis of mesenteric vein.
Dig Dis Sci2002; 47:2711
–2714[Medline]
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Introduction
Case Report
