Annals of Vascular Surgery
Volume 24, Issue 7 , Pages 953.e1-953.e5, October 2010

Mesojugular Shunt for Budd-Chiari Syndrome: A Case Report With Long-Term Follow-Up

Department of Vascular Surgery, Xuanwu Hospital, Capital Medical University, Beijing, P.R. China

published online 05 July 2010.

Article Outline

Background

We report a case of a mesojugular shunt for Budd-Chiari syndrome (BCS) with long-term follow-up.

Methods

A 32-year-old woman presented with late stage BCS. She was treated with a mesojugular shunt to relieve her portal hypertension. In a 17-year follow-up, she had a pregnancy, a postpartum period, and was diagnosed with systemic lupus eythematosus. She has resumed work as an operating room nurse and is having a normal life. Her graft remains patent.

Conclusion

A mesojugular shunt has less surgical trauma and satisfactory long-term patency than other shunts. It can be used to treat severe patients with BCS who are not candidates for intervention.

 

Budd-Chiari syndrome (BCS) is a clinical condition caused by hepatic venous outflow obstruction located 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 obstruction.1, 2 The clinical symptoms and histopathologic features of this syndrome were 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 endoluminal 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 myeloproliferative diseases, a Factor V Leiden mutation, antiphospholipid syndrome, Behcet's disease, paroxysmal nocturnal hemoglobinuria, oral contraceptive use, and pregnancy.1, 4, 5, 6 The basic therapeutic strategies consist of medical management, 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 delivery. She was diagnosed with systemic lupus eythematosus (SLE) and ileotyphus during the follow-up. Her graft has remained patent as of the last visit.

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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. She was an operating 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 heart and lung disease. In January 1991, the patient was admitted to another hospital because of jaundice and ascites. 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. Palpation of the spleen was not 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 μmol/L; and direct bilirubin, 47.88 μmol/L. She had normal 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 blood were negative. A chest X-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 from 51-37 cm of a water column. After surgery, she was treated with warfarin (2.5 mg/d) until she achieved pregnancy. She resumed her work as an operating room nurse (including night-shift), even though the third author (the surgeon who 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 clinic, we found out that she was pregnant and spontaneously 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 confirmed 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 hospitalization, 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: protein 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 showed 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 contrast media in the cross-section was clearly seen (Fig. 2).

  • View full-size image.
  • 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.

  • View full-size image.
  • Fig. 2 

    Computed tomography angiography follow-up 17 years after surgery. A, B Show the patent graft in a longitudinal view; C, D Show the graft in different cross-sections.

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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 national estimate was scanty. The prognosis of untreated patients with symptomatic BCS is poor. About 90% of the patients die in 3 years.4 Medical 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 treatment.1, 12 In brief, the lesions are divided into three types: (1) web or short-segment stenosis or occlusion in the suprahepatic IVC, (2) long-segment stenosis or occlusion in the IVC involving the suprahepatic portion of the IVC, and (3) involvement 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 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 mesenteric 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 shunting, requires a much longer length of prosthesis, and has a greater chance for inducing grafting thrombosis.3 In this case, the prosthetic length was 28 cm in length, and after surgery this patient had a pregnancy, 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 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 suggest using it to treat severe patients with BCS without the possibility for intervention.

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References 

  1. Wang ZG, Jones RS. Budd-Chiari syndrome. Curr Probl Surg. 1996;33:83–211
  2. Janssen HL, Garcia-Pagan JC, Elias E, et al. Budd-Chiari syndrome: a review by an expert panel. J Hepatol. 2003;38:364–371
  3. Chung RT, Iafrate AJ, Amrein PC, et al. Case records of the Massachusetts General Hospital. Case 15-2006. A 46-year-old woman with sudden onset of abdominal distention. N Engl J Med. 2006;354:2166–2175
  4. Valla DC. Primary Budd-Chiari syndrome. J Hepatol. 2009;50:195–203
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  6. Valla DC. Budd-Chiari syndrome and veno-occlusive disease/sinusoidal obstruction syndrome. Gut. 2008;57:1469–1478
  7. Wang ZG, Zhang FJ, Meng QY, et al. Evolution of management for Budd-Chiari syndrome: a team's view from 2564 patients. ANZ J Surg. 2005;75:55–63
  8. Rajani R, Melin T, Björnsson E, et al. Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival—an 18-year experience. Liver Int. 2009;29:253–259
  9. Okuda H, Yamagata H, Obata H, et al. Epidemiological and clinical features of Budd-Chiari syndrome in Japan. J Hepatol. 1995;22:1–9
  10. Ulrich F, Pratschke J, Neumann U, et al. Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome. Liver Transpl. 2008;14:144–150
  11. Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004;350:578–585
  12. Wang ZG, Zhang FJ, Li XQ, et al. Management of Budd-Chiari syndrome: what is the best approach?. J Gastroen Hepatol. 2004;19:S212–S218
  13. Wang ZG, Zhang H, Pu LQ, et al. Can endothelial seeding enhance patency and inhibit neointimal hyperplasia? Experimental studies and clinical trial of endothelial seeded venous prostheses. Int Angiol. 2000;19:259–269
  14. Ochs A, Sellinger M, Haag K, et al. Transjugular intrahepatic portosystemic stent-shunt (TIPS) in the treatment of Budd-Chiari syndrome. J Hepatol. 1993;18:217–225
  15. Rossle M, Olschewski M, Siegerstetter V, et al. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 2004;135:394–403

PII: S0890-5096(10)00148-2

doi:10.1016/j.avsg.2010.02.050

Annals of Vascular Surgery
Volume 24, Issue 7 , Pages 953.e1-953.e5, October 2010

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