The Korea n J ourna l of Inte rnal Me dic ine

Vol. 15, No. 1, J anua ry, 2000

Ile o c e c a l U lc e r w it h a C e c o c e c a l Fis t u la in
Be h c e t ' s D is e a s e
* +
Hy u n Ky u Ch a n g , M .D. , J e e S o o Kim , M . D. , Ha in g s u b R. Ch u n g , M .D.
De p a rt m e nt o f Int e rn a l M e d ic in e , De p a rt m e nt o f Ge n e ra l S u rg e ry *,
De p a rt m e nt o f Diag n o s t ic Pat h o lo g y + ,
A s a n -Fo u n d a t io n Ka ng n u n g Ho s p it a l, Ka n g n u ng , Ko re a

W e d e s c rib e a c a s e o f Be h c e t 's d is e a s e ( B D) w h ic h s h o w e d t h e ile o c e c a l

u lc e r a n d c e c o c e c a l f is t u la . T h is 3 8 -y e a r-o ld m a n
b e c a u s e o f c o lic ky p a in in t h e rig ht lo w e r a b d o m e n
f is t u la f o rm at io n in B D. In t h o s e , s o m e a re re la t e d
w it h c e c o c e c a l f is t u la , p o s s ib ly a s s o c ia t e d w it h
re p o rt e d in t h e lit e rat u re .
h a d a p p e n d e c t o m y s ix y e a rs a g o
(R LA ) . T h e re a re s o m e re p o rt s o n
t o s u rg e ry a n d o t h e rs a re n o t . B D
a p a s t o p e ra t io n , h a s n o t b e e n

Ke y W o rd s : Be h c e t 's d is e a s e , Ce c o c e c a l f is t u la , Ile o c e c a l u lc e r, A p p e n d e ct o m y

inflammation in the appendix. He was referred to the

INT RO DUCT IO N university medical center and diagnosed as having an
intestinal BD. He had been treated with low- dose
Though gastrointestinal symptoms are relatively
prednisolone and sulfasalazine. After surgery, intermittent,
common in BD, ulcerative change of the intestine is not
severe abdominal pain occurred while he had been on
that frequent. The most common sites for intestinal BD
medication on and off.
are the terminal ileum and the cecum. There are reports
On examination, he had a regular pulse of 120
of fistula formation in BD. These include rectovaginal
beats/min, blood pressure of 70/40 mm Hg and body
fistula 1 ) , ves icovaginal fistula 2 ) , aortoatrial fistula 3 ) and
temperature at 36.5. His conjunctiva was mildly pale.
postoperative complication such as aortoenteric fistula 4 )
The lungs were clear to auscultation. Though his
and enterocutaneus fistula 5 ) . Our patient, who presented
abdomen was soft, the abdominal examination revealed
with massive, bloody diarrhea and severe pain in RLA,
tenderness and rebound tenderness in RLA. He had a
had a large, deep cavitating ileocecal ulcer along with a
hematocrit of 33.5%, a white blood cell count of 11.8
cecocecal fistula around the previous appendectomy s ite.
109/mm3 and a platelet count of 185 109/mm3 . The
antinuclear antibody, rheumatoid factor and antineutrophil
CA S E cytoplasmic antibody were negative. Plain abdomen
s howed no specific findings except for the focal ileus in
A 38- year- old man presented with massive, bloody
RLA.
diarrhea and colicky pain in RLA. He had been having
Even with enough saline and transfusion of packed
recurrent oral ulcer, genital ulcer and erythema
red blood cells, he had persisting hypotension and severe
nodosum- like lesion for the past ten years. Six years
abdominal pain. Emergency ileocecal segmental resection
ago, he had an appendectomy done at a primary clinic.
was performed. Pathologic examination revealed large
At that time, the multiple ulcers were noted in the
cavitating ulcers (figure 1), lymphocyte aggregates and
ileocecal region and histology showed minimal
lymphocytic vasculitis (figure 2) in the ileocecal region.
There was cecocecal fistula (figures 3 & 4), presumably
Address reprint requests to : Hy un Ky u Chang, Asan around the previous appendectomy site.
Kangnung Hospital, Department of Internal Medicine, 4 15 He was started on oral prednisolone 10 mg/day,
Bangdongri, Sachunmy un, Kangnung- City, Kang wondo, s ulfasalazine 2 g/day and cyclophosphamide 100 mg/day
Korea, 2 10- 711
The Korea n J ourna l of Inte rnal Me dic ine
Vol. 15, No. 1, J anua ry, 2000

Fig ure 1. Histopathologic exa mination of the ulce r showing

necroinfla mmation on the right side of the photograph (H&E
sta in, origina l magnification40).

Fig ure 3 . Gross photograph of this ileocecectomy

specime n, revea ling cecoceca l fistula de monstrated by
the probe . Ileoceca l va lve is destroyed with deep
ulceration. The probe is pass ing throuch the cavitating
ulcer in the most proxima l cecum on the left uppe r to
more dista l non- ulce rated portion of the cecum on the
right portion of the photograph.

Fig ure 2 . Ma rked perivascula r lymphocytic infiltration noted,

a nd some in the wa ll of this a rte riole as we ll (H&E sta in,
origina l magnification 100).

in the s ixth postoperative day. His postoperative course

was uneventful.

DIS C US S IO N
BD is a multisystemic disorder characterized by oral
ulcer, genital ulcer, uveitis and s kin lesions , most likely
occurring with the underlying vasculitis. There is other
organ involvement of joints, heart and lungs , as well as
neurologic and gastrointestinal involvement. The etiology
remains unclear. Genetic and environmental factors
probably have a role in the pathogenesis. There are no
specific diagnostic or laboratory tests for BD. Diagnos is
depends upon the proper history and clinical
manifestations. Our case fulfilled the diagnostic criteria of
Fig ure 4 . Photomicrograph showing fistulous track pa rtly
lined by the colonic epithe lia l tissue (H&E sta in, origina l
magnification40).
the Intestinal Study Group for Behcet's disease 6 ) .
Many patients complained of gastrointestinal symptoms
s uch as nausea, vomiting and abdominal pain, but the
ulcerative changes in the intestine were found in 1% or
less of all patients with BD7 ) . The commonest sites for the
ulcerative changes of intestinal BD were terminal ileum in
44%, followed by the ileocecal region in 34% and the
The Korea n J ourna l of Inte rnal Me dic ine
Vol. 15, No. 1, J anua ry, 2000
cecum in 12%5 ) . The clinical manifestations of intestinal
BD are s imilar to inflammatory bowel disease, especially
Crohn's disease. But lymphoid aggregates, submucosal
fibros is, no granuloma 8 ) , and deep penetrating, easily
perforating ulcer9 ) in Behcet's colitis help to distinguish it
from Crohn's disease. Histology in our case revealed
lymphoid aggregates, no granuloma and deep penetrating
ulcers in the ileocecal region. About 22% of the patients
with intestinal BD developed symptoms mimicking
appendicitis during the clinical course 1 0 ) . Because the
most common sites of intestinal BD are the terminal
ileum and the ileocecal region, some cases can be
diagnosed as an appendicitis. Our case had a past
history of erroneous diagnosis with an appendectomy.
The opening of the cecocecal fistula was around the s ite
of the appendectomy. The exact mechanism of fistula
formation in BD is unclear. We could s peculate that
vasculitis and ulcer necros is, along with pathergy reaction
by surgical trauma in our case, might have contributed to
the fistula formation.
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