Vol 43 # 3 September 2011 - Kma.org.kw

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Splenic Vein Thrombosis: A Rare Complication of Celiac Disease
or a manifestation of an autoimmune phenomenon.
Our patient had normal homocysteine levels despite
a low red cell folate.
Some authors suggested that factors such as
dehydration and hyperviscosity due to high levels of
circulating antibodies may predispose to thrombosis.
In our case, the patient was not dehydrated, and
although plasma viscosity was not checked, his ESR
was low, voting against hyperviscosity.
Hypoalbuminemia is an important factor which
(in our opinion) is often overlooked. Low circulating
albumin causes extravasation of intravascular fluid,
which in turn decreases plasma volume, and may
predispose to thrombosis. Indeed, our patient had
clear manifestation of fluid redistribution with
ascites and lower limb edema. This, however, does
not explain why patients with occult CD can still
have thrombotic events.
Another interesting phenomenon is the homology
between different forms of transglutaminase and
coagulation factor XIII [16] . Some patients with CD
have circulating IgA antibodies against Factor
XIII. Although this has not been shown to lead
to a hypercoagulable state, it nonetheless signals
a connection between CD and the coagulation
cascade.
The CT angiography in our patient excluded
the possibilities of malignancy, pancreatic and
renal diseases. The negative thrombophilia screen
excluded the possibility of hereditary and acquired
thrombophilic disorder, leaving CD as the sole
cause.
Old methods of diagnosis of ISVT include
spleno-portography. More recently late-phase celiac
angiography and endoscopic ultrasonography
have emerged as the investigations of choice [17,18] .
Computed tomography, magnetic resonance, and
ultrasound imaging are also used [19] .
The natural history of ISVT is not welldocumented.
Older studies have suggested that
ISVT results in a high likelihood of gastric variceal
bleeding necessitating splenectomy. Advances in
cross-sectional imaging have led to the identification
of SVT in patients with minimal symptoms [19] .
Heider et al demonstrated that gastric variceal
bleeding occurs in only 4% of patients, suggesting
that splenectomy should not be done routinely [20] .
Thus, splenectomy was deferred in our patient.
The risk of major variceal hemorrhage is
increased with warfarin therapy, and therefore
some authors advise against it [21] . On the other
hand, Ikeda et al found that patients with total SVT
are at greater risk for thrombus propagation, and
therefore, they are candidates for anticoagulation
therapy [22] . Other authors recommend acute and
chronic anticoagulation for SVT particularly when an
underlying hypercoagulable condition is present [23] .
September 2011
Our patient had active disease due to noncompliance
with gluten-free diet, resulting in a
hypercoagulable state [24] . The aim of anticoagulation
was to prevent further thrombus propagation and
to aid recanalization until the disease goes into
remission [25] . We were encouraged by the absence of
endoscopic features of high variceal bleed risk [26] and
the normal coagulation profile.
There is no clear evidence-based guidance for
the duration and degree of anticoagulation in such
cases. Our patient was anticoagulated for a total
of six months to achieve an INR of 2 - 3. We are
following him up closely for any signs of bleeding,
and we intend to do a follow up endoscopy and CT
angiography to assess his disease regression.
Splenectomy is the treatment of choice, should he
bleed [27] . Sclerotherapy and gastric variceal banding
have also been done successfully [28] . Splenic arterial
embolization is not well studied and is associated
with splenic abscess formation. It is performed in
patients with high operative risk and those with
diffuse metastatic disease [29] .
CONCLUSION
Splenic vein thrombosis is a very rare complication
of active and occult CD. The pathophysiology
of thrombosis in CD is not clearly defined. Leftsided
portal hypertension, splenomegaly with
hypersplenism and gastric varices can occur, and
rarely, can have fatal consequences. Splenectomy is
the treatment of choice in the event of gastric variceal
bleeding. The role of anticoagulation in such cases
is not well-defined and need to be tailored to each
case.
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