AJR 2005; 185:89-91
© American Roentgen Ray Society
Rescue TIPS in Acute Budd-Chiari Syndrome
Hani Abujudeh,
Daniel Contractor,
Andrew Delatorre and
Baburao Koneru
Department of Radiology, C320, UMDNJ-New Jersey Medical School, 150
Bergen Street, Newark, NJ 07103.
Received November 20, 2003;
accepted after revision April 20, 2004.
Address correspondence to H. Abujudeh
(judehmd{at}yahoo.com).
Introduction
Budd-Chiari syndrome (BCS) is caused by obstruction of the hepatic venous
outflow tract resulting from obstruction of the hepatic vein, the inferior
vena cava (IVC), or both. Most cases are due to thrombotic occlusion of the
hepatic veins resulting from a hypercoagulable state
[1]. BCS can manifest in an
acute or chronic form; medical, surgical, and interventional treatments are
available. A transjugular intrahepatic portosystemic shunt (TIPS) has been
described to treat BCS. Most published reports discuss TIPS for treatment of
the chronic form
[2-9].
We present a rescue TIPS procedure in a patient who presented with acute
BCS.
Case Report
A 32-year-old woman was admitted to a community hospital with an acute
onset of abdominal distention, pain, and lethargy. Her medical history was
significant for Factor V Liden mutation, which was diagnosed after the patient
experienced postpartum deep venous thrombosis 6 years earlier. She was managed
with warfarin sodium (Coumadin, Bristol-Myers Squibb) for 4 years, but
Coumadin was discontinued 2 years before this admission. Her medical history
was otherwise unremarkable. The patient took no medications and was allergic
to penicillin. Family history was significant for a twin sister afflicted with
Factor V Liden mutation. Socially, the patient was a professor of psychology
at a local university. She did not smoke and had a glass of wine occasionally
with meals.
A review of systems was unremarkable. On physical examination, her blood
pressure was 105/60 mm Hg, heart rate was 78 bpm, and temperature was
99.2°F. She was well nourished. Examination of the head and neck revealed
mildly icteric sclera and a supple neck without adenopathy or thyroid
enlargement. The lungs were clear and equal to auscultation. The heart had a
regular rhythm without murmurs. The abdomen was protuberant without organ
enlargement. Neurologically, the affect was markedly blunted with response to
simple commands only. No focal motor or sensory deficits were seen.
Laboratory studies revealed AST 6,005 U/L, ALT 5,800 U/L, total bilirubin
3.1 mg/dL, international normalized ratio (INR) 5.8 sec, creatinine 2.6 mg/dL,
hemoglobin 10.7 mol/L/UL, and platelets 234 T/UL. Sonogram of the liver was
unremarkable except for poorly visualized hepatic veins. Three units of fresh
frozen plasma were administered without improvement in the INR. The patient
was transferred to a liver transplant center. On arrival, AST and ALT were
4,800 U/L and 3,700 U/L, respectively. The INR was 5.5 sec. A repeat sonogram
of the liver confirmed a lack of hepatic venous flow and a diagnosis of acute
BCS was made. No ascites was seen. Although the patient was coagulopathic
secondary to liver failure, in view of her hypercoagulable state, an infusion
of fresh frozen plasma was initiated to increase the proportionate quantity of
functional nonmutated Factor V. On the third hospital day, AST and ALT were
4,421 U/L and 3,021 U/L, respectively. Total bilirubin was 2.8 mg/dL and
creatinine was 2.1 mg/dL. Abdominal distention increased with the onset of
lower extremity edema. In view of the acuity of the signs and symptoms, a
decision was made to proceed to emergency TIPS.
Technique
Under general anesthesia, the right internal jugular vein was
sonographically accessed with a 5-French coaxial micropuncture introducer set
(Cook) and upgraded to a 10-French sheath. A wire was advanced to the inferior
vena cava (IVC) and venographies were performed. Attempts to access the
hepatic vein revealed only a hepatic stump and microvessels
(Fig. 1A). The Rosch-Uchida
system (Cook) was advanced and the tip placed at the hepatic stump. The liver
was punctured and CO2 portal venography performed
(Fig. 1B). Using the hepatic
vein remnant as a starting point and knowing the location of the portal vein,
a Colapinto's needle (Cook) was used to access the portal vein uneventfully.
An Amplatz wire (Medi-tech Boston Scientific) was introduced into the portal
vein. Portal venography was performed that showed hepatofugal flow
(Fig. 1C) The hepatic
parenchyma was dilated with a 6-mm diameter balloon and two 10 x 60 mm
Wallstents (Schneider Stent, Inc.) were placed, followed by stent dilatation
with a 10-mm balloon. Poststent placement portal venography was performed that
showed prompt flow from the portal vein through the TIPS
(Fig. 1D).
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Fig. 1B —32-year-old woman admitted to a community hospital with acute
onset of abdominal distention, pain, and lethargy. Intraparenchymal injection
of CO2. Portal vein is clearly visualized; multiple vein
collaterals are also seen with no hepatic vein.
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Fig. 1C —32-year-old woman admitted to a community hospital with acute
onset of abdominal distention, pain, and lethargy. Venogram of portal vein
shows hepatofugal flow and visualization of varices.
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Fig. 1D —32-year-old woman admitted to a community hospital with acute
onset of abdominal distention, pain, and lethargy. Portography performed after
TIPS placement shows rapid flow of contrast medium through stent and into
inferior vena cava and right atrium.
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After TIPS, the patient's respiratory status deteriorated. She required
several days of mechanical ventilator support. On 60% O2, she
maintained oxygen saturation of 90-93%. On day 2 post-TIPS placement, the
patient was briskly diuresed. She was subsequently extubated on day 3. Her
transaminases, creatinine, and INR improved during this time. On day 5,
sonogram surveillance of the liver revealed a patent shunt. Little clinical
evidence of encephalopathy was seen. The patient was discharged from the
hospital on day 9; on discharge, her AST and ALT were 41 U/L and 61 U/L,
respectively. The total bilirubin was 2.8 mg/dL. A follow-up at 2 months
revealed a patent shunt, and AST and ALT were 30 U/L and 33 U/L, respectively.
The total bilirubin was 1.7 mg/dL.
Discussion
TIPS is a percutaneous minimally invasive procedure in which a side-to-side
portacaval shunt is created. TIPS was first performed in Germany in 1988 and
later in the United States in 1990
[4] to treat portal
hypertension and its complications. In the last decade, TIPS was rapidly
applied to the treatment of actively bleeding varices, control of refractory
cirrhotic ascites, hepatic chylothorax, and hepatorenal failure and
hepatopulmonary syndrome [1].
Once it was shown that the TIPS could be placed with relative ease, the
procedure was extended to more challenging cases such as BCS, portal venous
thrombosis, and polycystic liver disease. TIPS was also performed from the
left internal jugular vein and even in the absence of a right superior vena
cava
[1-3,
5,
6].
The treatment of BCS can be medical, surgical, or interventional. In the
setting of chronic BCS, the medical management involves anticoagulation and
diuretics. Medical treatment alone is contemplated if liver biopsy shows
hepatic congestion only and no hepatocellular necrosis. Once hepatocellular
necrosis is suggested, surgical options such as a portosystemic shunt should
be considered [1,
9]. Surgical decompression may
reverse the hepatic congestion and halt the progression of the disease. Other
surgical options include liver transplantation, particularly if there is
significant fibrosis [9].
BCS can have a high mortality rate; in a series of 1,975 patients with BCS,
the mortality rate was 90% within 3.5 years of symptom onset
[8]. Although the mortality
rate has significantly improved, mostly due to improved anticoagulation and
other novel procedures, acute BCS remains a condition with a poor prognosis in
the absence of liver transplantation
[7]. In some cases of BCS, the
cause of venous outflow obstruction is a focal stenosis or a web in the
hepatic vein or IVC that can be treated with balloon angioplasty, stent
placement, or both. In other cases, there is only a nubbin of the hepatic
vein. Creating a TIPS in BCS, particularly when all three hepatic veins are
occluded, can be technically demanding. The difficulties encountered are
mainly due to the absence of the occlusion of the hepatic vein and the
stiffness of the liver parenchyma. The needle often does not penetrate the
liver and bounces back to the right atrium.
Several methodologies have been used to perform TIPS in BCS, including
direct IVC puncture. In this method, the posterior wall of the IVC provides
support for the puncturing set. In addition, the operator modifies the angle
on the set from 15° to 35° to make the curve more acute, since the
distance between the IVC and the portal vein is shorter. Correct targeting of
the portal vein can be accomplished by positioning a radioopaque marker on the
skin. This can be accomplished by sonogram guidance
[6]. In other cases, the
remaining hepatic vein stump is used as the take-off point for the puncture
[5]. In some cases,
CO2 portal venography was successfully performed by holding a
9-French sheath firmly against the hepatic vein wall
[10]. In our case,
CO2 portal venography was performed by injecting CO2
within the liver parenchyma. Identification of the portal vein with
CO2 made subsequent access significantly easier. We used the
hepatic vein stump as our starting point for liver puncturing to access the
portal vein.
In conclusion, patients with acute BCS can be rescued by performing TIPS,
giving patients the time needed until transplantation. Technically, TIPS in
BCS is a challenging procedure. The puncture site can be performed from the
remnant hepatic stump if available or directly from the IVC, and
CO2 portal venography can be performed from an intraparenchymal
location safely.
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Introduction
Case Report
