Cas cliniques

DOI of or

Departmencal University,

Correspondgery, XuanwuStreet, Xuanwyahoo.cn

Ann Vasc SurDOI: 10.1016/� Annals of V�Edit�e par ELS

Shunt m�esent�erico-jugulaire pour syndromede Budd-Chiari: Un cas avec suivi �along terme

Hong Yong Duan, Xin Wu, Zhong Gao Wang, Jian Zhang, Jian Xin Li, Yong Quan Gu,

Heng Xi Yu, Bing Chen, P�ekin, R.P. de Chine

Objectif : Nous rapportons un cas de shunt m�esent�erico-jugulaire pour syndrome de Budd-Chiari (BCS) avec suivi �a long terme.M�ethodes : Une femme de 32 ans s’est pr�esent�ee �a un stade tardif de BCS. Elle a �et�e trait�eeavec un shunt m�esent�erico-jugulaire pour soulager son hypertension portale. Avec un suivi de17 ans, elle a eu une grossesse, une p�eriode puerp�erale, et a �et�e diagnostiqu�ee avec lupus�eryth�emateux syst�emique diss�emin�e. Elle a repris le travail en tant qu’infirmi�ere de salled’op�eration et a une vie normale. Son pontage reste perm�eable.Conclusion : Un shunt m�esent�erico-jugulaire est moins agressif chirurgicalement avec uneperm�eabilit�e �a long terme meilleure que d’autres shunts. Il peut etre employ�e pour traiter lespatients graves avec BCS qui ne sont pas candidats �a une intervention.

Budd-Chiari syndrome (BCS) is a clinical condition

caused by hepatic venous outflow obstruction loca-

ted anywhere from the hepatic veins to the supra-

hepatic inferior vena cava (IVC), and even below

the right atrium, regardless of the cause of the obs-

truction.1,2 The clinical symptoms and histopa-

thologic features of this syndromewere described by

Budd in 1845 and Chiari in 1899.3 With respect to

etiology, BCS can be classified as primary and

secondary types. The former is caused by endolu-

minal venous lesions, such as thromboses and webs,

while the latter is caused by external invasion or

compression, such as malignant tumors or large

cysts.2 The risk factors for primary BCS include

iginal article: 10.1016/j.avsg.2010.02.050.

t of Vascular Surgery, Xuanwu Hospital, Capital Medi-P�ekin, R.P. de Chine.

ence : Zhong Gao Wang, Department of Vascular Sur-Hospital, Capital Medical University, No.45 Changchunu District, Beijing, P.R. China, E-mail: zhonggaowang@

g 2010; 24: 953.e1-953.e5j.acvfr.2011.02.022ascular Surgery Inc.EVIER MASSON SAS

myeloproliferative diseases, a Factor V Leiden

mutation, antiphospholipid syndrome, Behcet’s

disease, paroxysmal nocturnal hemoglobinuria, oral

contraceptive use, and pregnancy.1,4-6 The basic

therapeutic strategies consist of medical mana-

gement, interventional techniques, transjugular

intrahepatic portosystemic shunts (TIPS), various

surgical shunts, and liver transplantation.

We report a case of a 32-year-old woman with

BCS. A mesojugular shunt was placed to relieve

her severe portal hypertension. Over a 17-year

follow-up, she had a pregnancy and normal deli-

very. She was diagnosed with systemic lupus

eythematosus (SLE) and ileotyphus during the

follow-up. Her graft has remained patent as of the

last visit.

CASE REPORTS

A 32-year-old woman was admitted to our hospital on

February 24, 1992 because of abdominal distention, short

breath, lassitude, and jaundice. Shewas anoperating room

nurse with an 8-year history of menoxenia and acyesis

after marriage. She denied any tobacco and alcohol or

hepatotoxic drug consumption. She had no history of

1036.e15

1036.e16 Cas cliniques Annales de chirurgie vasculaire

heart and lung disease. In January 1991, the patient was

admitted to another hospital because of jaundice and asci-

tes. An inferior cavography revealed total occlusion of the

IVC involving the supra-hepatic portion. She was then

diagnosed with BCS. She had no effective treatment until

admission to our hospital. On admission, she was afebrile,

the heart rate was 119/min, the respiratory rate was 28/

min, and the blood pressure was 120/96 mm Hg. Chest

percussion revealed a flat note on the right lower chest.

Superficial varices on the thoracoabdominal wall with

upward blood flow were noted. The liver was 5 cm below

the right costal margin and the shifting dullness was clear.

Palpationof the spleenwasnot apparent because of ascites.

The abdominal girth was 91 cm. She had moderate edema

and varicose veins in both lower limbs.

The blood cell count was as follows: total leukocyte,

5 � 109/L; erythrocytes, 3.83 � 1012/L; platelets, 118 �109/L; and hemoglobin, 117 g/L. The liver function tests

revealed the following: a normal glutamate pyruvate

transaminase; serum albumin, 26 g/L; total bilirubin, 68.4

mmol/L; and direct bilirubin, 47.88 mmol/L. She had nor-

mal renal function and was euglycemic. The coagulation

function tests revealed a prothrombin time of 20.5 sec and

a prothrombin activity of 36.4%. The hepatitis B surface

antigen and fecal occult bloodwerenegative.A chestX-ray

revealed a right pleural effusion. An abdominal Doppler

ultrasound showed an enlarged liver and spleen, a

moderate amount of ascites, and long-segment IVC

thrombosis. An upper gastroenterography revealed a

grade III varicose vein of the esophagus. According to the

history and results, a diagnosis of BCS was established and

her liver function was Child-Pugh stage C or Wang’s IV

according to the severity of the BCS (the most severe

category).1 Considering her severe disease and risk, she

was treated with a mesojugular shunt through a tunnel

posterior to the sternum. The graft used was of 14 mm

inner diameter and 28 cm long externally supported by

expanded polytetrafluoroethylene (Gore Tex, Newark,

DE) prosthesis, as previously described.7 Approximately

4,800 mL of flavescens ascites was drained when the

abdomen was opened. After establishment of blood flow

through the mesojugular shunt, her portal blood pressure

decreased from51-37 cmof awater column.After surgery,

she was treated with warfarin (2.5 mg/d) until she achie-

ved pregnancy. She resumed her work as an operating

room nurse (including night-shift), even though the third

author (the surgeonwho performed the procedure) firmly

suggested that ‘‘no nightshifts in the OR.’’ Thus, she

resumed her normal life until the time of this writing.

In addition, during her visit to a local hospital or our cli-

nic, we found out that she was pregnant and sponta-

neously delivered a healthy baby girl at term in the

second year postoperatively. In the 5th postoperative

year, she was healthy and her graft was patent, as confir-

med by Doppler ultrasound. The ultrasound showed

that the maximal and minimal blood flow rates were

0.46 m/sec and 0.16 m/sec, respectively.

In October 2004, the patient was admitted because of

macrohematuria and fever. The diagnoses of SLE and

ileotyphus were made based on the 1997 ARA criteria

and hemoculture, respectively. During the hospitaliza-

tion, a Doppler ultrasound showed that the portal flow

was completely retrograde and the graft was patent. A

bone marrow biopsy was performed and no abnormality

was found. Other laboratory studies were as follows: pro-

tein C and S, normal; antithrombin III, 72% (normal

range: 77-127%); positive anticardiolipin antibody, and

elevated erythrocyte sedimentation rate, 81 mm/hr. The

liver function tests were normal. She was given aspirin

(75 mg/d) at the time of discharge.

In May 2009, she was called for a follow-up. She had a

well-proportioned build with slightly dilated veins on the

abdominal wall (Fig. 1) and slight varicose veins on both

lower extremities. The newest Doppler ultrasound sho-

wed that the graft was patent with a maximal blood flow

rate of 0.99 m/sec. Computed tomography angiography

showed that the graft was patent and the contrastmedia in

the cross-section was clearly seen (Fig. 2).

DISCUSSION

BCS is a rare disease. According to Rajani et al.8 the

incidence and prevalence of BCS in Western

populations were 0.8 per million per year and 1.4

per million, respectively. In Japan, the incidence

and prevalence were estimated to be 0.2 per million

per year and 2.4 per million in 1989, respectively.9

In Shandong province of China, the prevalence was

about 64 per million in 1988;1 however, the natio-

nal estimate was scanty. The prognosis of untreated

patients with symptomatic BCS is poor. About 90%

of the patients die in 3 years.4Medical therapy alone

had a higher mortality compared with surgical

shunts,10 which can reverse the hepatic congestion

and prevent the cirrhosis.1,11 We have established

classification for BCS as described previously on the

basis of site and extent of occlusive lesions so as to

conveniently select the means for surgical treat-

ment.1,12 In brief, the lesions are divided into three

types: (1) web or short-segment stenosis or occlu-

sion in the suprahepatic IVC, (2) long-segment

stenosis or occlusion in the IVC involving the

suprahepatic portion of the IVC, and (3) involve-

ment of the hepatic veins alone. Furthermore, the

first two types can be divided into sub-types

according to the status (i.e., whether or not the

hepatic vein is involved). The third type can also be

divided into two sub-types according to the extent

of the hepatic vein involved. The lesion of our

patient belongs to the second type (long-segmental

occlusion of the IVC). Surgical shunts play an

essential role for patients with this type. However,

there is no possibility for placing a cavoatrial or

mesoatrial shunt for this patient because both

Fig. 1. Photo: A The patient has a somewhat slender

configuration and well-proportioned body build 17 years

after surgery; B Slightly dilated veins appear on the

abdomen, since the IVC hypertension was not effectively

relieved by the mesojugular shunt.

Vol. 24, No. 7, 2010 Cas cliniques 1036.e17

require a thoracotomy. In consideration of the

patient’s poor preoperative condition, a retrosternal

mesojugular shunt was placed through an upper

abdominal incision to isolate the superior mesen-

teric vein (SMV), and a small cervical incision to

isolate the right internal jugular vein. The graft was

planted into a tunnel posterior to the sternum. The

abdominal side of the prosthesis was used for a

prosthesis-SMV anastomosis, and the cervical part

was used for a prosthesis-internal jugular venous

anastomosis. Both of them were in an end-to-side

fashion. Those maneuvers are relative easy and

safe for a skilled vascular surgeon to complete. This

patient had a rather excellent outcome 17 years

after the mesojugular shunt, indicating that this

approach reached our expected purpose, i.e., not

only to properly relieve her portal hypertension but

also to avoid significant surgical trauma. Compared

with other types of shunts, the mesojugular shunt

has the particular advantage of treating severely ill

patients with BCS.7,12 Theoretically, the long-term

patency of the mesojugular shunt should be the

poorest since it has the longest distance for shunt-

ing, requires amuch longer length of prosthesis, and

has a greater chance for inducing grafting throm-

bosis.3 In this case, the prosthetic length was 28 cm

in length, and after surgery this patient had a pre-

gnancy, a postpartum period and SLE, all of which

are hypercoagulable states and could theoretically

easily cause graft failure due to thrombosis. The

graft is still patent as of this writing. Our prosthetic

IVC study indicated that for an endothelialization

prosthesis, the 100 day patency was 100%.13 Up to

17 years later, the adequacy of endothelialization of

the graft is not clear, however, it can be presumed

that there is proper endothelialization on the inner

surface of this particular graft according to our series

on venous grafts,13 and no remarkable neointima is

formed according to the cross-sections of the graft

(Figs. 2C, D). We therefore can expect a much

longer patency in this still young and healthy

patient. Thus, it is of paramount importance to have

a close and successive observation of this case. A

20-, 25-, or 30-year patency could be identified for

such a long venous prosthesis. Clinically, in our

experience, 5- and 10-year patencies of cavoatrial

shunts are 79.8 and 57.1%, respectively.7 The

patency duration of this patient’s graft is now much

longer than those mentioned. We had another male

patient with BCS of the second type with severe

status. He underwent the same operation as the

female patient. His graft was patent until he died of

hepatic cancer in the 12th year after the shunt

operation. We attribute this satisfactory result to the

pump-like action, i.e., the graft between the heart

and the sternum is rhythmically compressed by the

heart. The action provides additional compression

and relaxation, which propels the blood flow to run

from the SMV in the abdomen to the internal

jugular vein. That is to say, an extra-mechanism for

propelling the blood flow is formed, in addition to a

pressure gradient tapering from the abdominal side

Fig. 2. Computed tomography angiography follow-up 17 years after surgery. A, B Show the patent graft in a longi-

tudinal view; C, D Show the graft in different cross-sections.

1036.e18 Cas cliniques Annales de chirurgie vasculaire

of the mesojugular shunt to the jugular venous side,

which drives the blood from the portal vein to the

systemic vein. Thus, both mechanisms together

create a rather excellent effect as it does in this case.

Although surgical shunts have been increasingly

substituted by TIPS after 1993,14 surgical shunts are

still a good choice for patients who cannot be treated

with TIPS in experienced hospitals. This case report

is a good example of this. Surgical shunts can also be

used for correcting TIPS failure.15

In conclusion, the mesojugular shunt could

have a satisfactory long-term patency, and thus we

Vol. 24, No. 7, 2010 Cas cliniques 1036.e19

suggest using it to treat severe patients with BCS

without the possibility for intervention.

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