- Email: [email protected]
Management of Budd-Chiari syndrome by hepatic vein stenting after extended right hepatectomy
Management of Budd-Chiari Syndrome by Hepatic Vein Stenting After Extended Right Hepatectomy By M. Benesch, C. Urban, H. Deutschmann, K.A. Hausegger, and M. Ho¨llwarth Graz, Austria
Massive splenomegaly and ascites production with hypoproteinemia and hypocoagulability was observed in a 15-yearold boy after extended right hepatectomy for hepatocellular carcinoma (HCC). Angiography disclosed a kinking of the left hepatic vein immediately before the junction with the inferior vena cava. Ascites disappeared completely, and laboratory values normalized after placement of a 3-cm long balloon expandable stent. The current case shows that Budd-Chiari
syndrome caused by hepatic outflow obstruction after major hepatic surgery can be managed effectively by percutaneous stent placement. J Pediatr Surg 37:1640-1642. Copyright 2002, Elsevier Science (USA). All rights reserved. INDEX WORDS: Hepatocellular carcinoma, extended right hepatectomy, Budd-Chiari syndrome, stent placement.
P
RIMARY MALIGNANT liver tumors are rare in children and account for approximately 0.5 to 2% of all malignancies in this age group.1-4 Whereas hepatoblastoma occurs mostly in the first 3 years of life, the median age of children with hepatocellular carcinoma (HCC) at diagnosis is 10 years.2-4 We present the case of a 15-yearold boy who underwent extended right hepatectomy for HCC. Because of a kinking of the remaining left hepatic vein, Budd-Chiari syndrome developed postoperatively (BCS), and was managed successfully by stent insertion. CASE REPORT The patient presented with a one-week history of painless abdominal enlargement. Physical examination found a palpable tumor in the upper right quadrant of the abdomen but was otherwise normal. Abdominal sonography and magnetic resonance imaging (MRI) showed a 21 ⫻ 17 ⫻ 15.5-cm tumor in the right lobe of the liver (Fig 1). The retrohepatic inferior vena cava was invaded by the tumor and completely occluded above the right adrenal vein. The entire blood from the lower part of the body was drained by the vertebral veins. Initial laboratory investigations found discrete elevation of liver enzymes (ALT, 31 U/L; AST, 32 U/L), anemia (hemoglobin, 114 g/L), and a serum alpha-fetoprotein (AFP) level of greater than 880 U/mL (normal, 0 to 7 U/mL). Histology of tumor tissue obtained on open liver biopsy was consistent with the diagnosis of HCC. Tumor resection was performed via extended transverse laparotomy. Extended right hepatectomy (trisegmentectomy) including resection of the retrohepatic vena cava and segment I was necessary to remove the tumor completely. The retrohepatic vena cava was resected above the right
From the Division of Pediatric Hematology and Oncology, Departments of Pediatrics, Radiology, and Pediatric Surgery, University of Graz, Austria. Address reprint requests to Christian Urban, MD, Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Graz, Auenbruggerplatz 30, A-8036 Graz, Austria. Copyright 2002, Elsevier Science (USA). All rights reserved. 0022-3468/02/3711-0028$35.00/0 doi:10.1053/jpsu.2002.36203 1640
Fig 1. Coronal magnetic resonance imaging of the abdomen shows a large, well-circumscribed mass in the right lobe of the liver.
adrenal vein and below the confluence of the left hepatic vein into the inferior vena cava. The remaining segments II and III were fixed to the diaphragm to achieve uninhibited blood flow from the left liver vein into the vena cava. Postoperatively, the patient had massive splenomegaly and ascites. Up to 10 L of peritoneal fluid were removed daily by the 2 abdominal drains. Despite maximal diuretic therapy, daily ascites production could only be reduced to 4 to 5 L. Laboratory evaluation at that time identified hypoproteinemia (46 g/L; normal, 64 to 82 g/L), low serum cholinesterase (364 U/L; normal, 2,800 to 8,000 U/L), and hypocoagulability (quick value, 40%; normal, 70% to 120%; fibrinogen, 1.50 g/L; normal, 1.8 to 4.0 g/L; activated partial thromboplastin time [aPTT], 65 seconds; normal, 25.0 to 36.0 seconds). High doses of albumin and clotting factors had to be replaced daily. A relaparotomy was performed on the 13th postoperative day but excluded any significant fluid loss by major abdominal lymphatic vessels. Thrombosis of the portal or remaining left hepatic vein was excluded by color-coded Doppler sonography. However, angiography after placement of an angiography catheter into the left hepatic vein showed a kinking of the left hepatic vein immediately before the junction with the inferior vena cava (Fig 2). Intravascular pressure measurement showed a translesional gradient of 10 mm Hg. Over a teflon-coated guidewire (Bentson, William Cook Company, Denmark) a
Journal of Pediatric Surgery, Vol 37, No 11 (November), 2002: pp 1640-1642
BUDD-CHIARI SYNDROME
1641
grounds, pulsed Doppler sonography, inferior vena cavography/functional hepatography, and liver biopsy.5 A number of different clinicopathologic conditions causing occlusion of the main hepatic veins or inferior vena cava above the hepatic veins may lead to BCS.6-15 “Classical” BCS occurs because of primary hepatic vein thrombosis on the basis of a hypercoagulable state, which may be found in factor V Leiden mutations, antiphospholipid antibody syndrome, protein C deficiency, paroxysmal nocturnal hemoglobinuria, vasculitis syndromes such as Behcet disease, or malignant hematologic disorders.7-11 However, various other obliterative processes affecting the hepatic veins or inferior vena cava (eg, membranous obstruction, diaphragmatic hernia, blunt hepatic trauma) also may cause BCS.12-14 BCS also has been reported as a complication after major hepatic surgery or orthotopic liver transplantation.15-17 In our patient with HCC the lack of distant metastases and the fact that the tumor involved the right lobe of the liver only allowed the surgeon to perform a complete removal of the tumor. As a complication, short length kinking of the remaining left hepatic vein resulted in the development of BCS, which did not respond to conservative medical treatment. Stent implantation, which has been shown to be a feasible and safe therapeutic option for BCS in the last decade15-19 was, therefore, regarded Fig 2. Angiography shows a kinking of the left hepatic vein short before the junction with the inferior vena cava (arrow).
3-cm long balloon expandable stent (Palmaz, Johnson & Johnson Interventional Systems, Warren, NJ) was inserted. The stent was dilated to a diameter of 13 mm. After stent placement, dilatation of the stent with a 14-mm ⫻ 4-cm angioplasty balloon was performed to smooth out wrinkles or folds. Control angiography showed unlimited flow through the left hepatic vein into the inferior vena cava with no translesional pressure gradient (Fig 3). No complications were observed during and after the procedure. Subsequently, ascites disappeared completely within 2 days, and laboratory values returned to normal. After postoperative recovery, the patient received 7 courses of chemotherapy over a 7-month period consisting of ifosfamide (total dose [TD], 11.5 g), cisplatin (TD, 305 mg), doxorubicin (TD, 260 mg), carboplatin (TD, 1720 mg), and etoposide (TD, 860 mg). As shown by follow-up abdominal and chest computed tomography scans the patient is free of local recurrence or distant metastases 3 years after diagnosis. Serum AFP levels normalized postoperatively and color-coded sonography continuously shows a patent venous lumen at the stent site.
DISCUSSION
BCS is caused by hepatic vein or inferior vena cava obstruction above the hepatic veins. It is a rare condition, particularly in children. Clinically, this syndrome is characterized by abdominal pain and distension, jaundice, gastrointestinal bleeding, hepatosplenomegaly, portal hypertension with ascites production, and sometimes liver failure.5 Diagnosis of BCS is based on clinical
Fig 3. Angiography after stent placement shows unlimited flow in the left hepatic vein.
1642
BENESCH ET AL
the best chance to restore hepatic vein outflow, thus leading to disappearance of ascites and normalization of pathologic laboratory values. In addition, stent insertion allowed to administer adjuvant chemotherapy immedi-
ately after recovery to maintain the remission achieved by tumor resection. The current case illustrates that childhood BCS occuring after major hepatic surgery can be managed effectively by percutaneous stent placement.
REFERENCES 1. Stocker JT: An approach to handling pediatric liver tumors. Am J Clin Pathol 109:67-72, 1998 (suppl 1) 2. Exelby PR, Filler RM, Grosfeld JL: Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey-1974. J Pediatr Surg 10:329-337, 1975 3. Lack EE, Neave C, Vawter GF: Hepatocellular carcinoma. Review of 32 cases in childhood and adolescence. Cancer 52:1510-1515, 1983 4. Chen JC, Chen CC, Chen WJ, et al: Hepatocellular carcinoma in children: Clinical review and comparison with adult cases. J Pediatr Surg 33:1350-1354, 1998 5. Singh V, Sinha SK, Nain CK, et al: Budd-Chiari syndrome: Our experience of 71 patients. J Gastroenterol Hepatol 15:550-554, 2000 6. Okuda K, Kage M, Shrestha SM: Proposal of a new nomenclature for Budd-Chiari syndrome: Hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 28:11911198, 1998 7. Mahmoud AE, Elias E, Beauchamp N, et al: Prevalence of the factor V Leiden mutation in hepatic and portal vein thrombosis. Gut 40:798-800, 1997 8. Bayraktar Y, Balkanci F, Bayraktar M, et al: Budd-Chiari syndrome: A common complication of Behcet’s disease. Am J Gastroenterol 92:858-862, 1997 9. Sugano S, Suzuki T, Makino H, et al: Budd-Chiari syndrome attributed to protein C deficiency. Am J Gastroenterol 91:777-779, 1996 10. Wyatt HA, Mowat AP, Layton M: Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome. Arch Dis Child 72:241-242, 1995
11. Saca LF, Szer IS, Henar E, et al: Budd-Chiari syndrome associated with antiphospholipid antibodies in a child: Report of a case and review of the literature. J Rheumatol 21:545-548, 1994 12. Odell JA, Rode H, Millar AJ, et al: Surgical repair in children with the Budd-Chiari syndrome. J Thorac Cardiovasc Surg 110:916923, 1995 13. Yonekura T, Kubota A, Hoki M, et al: Intermittent obstruction of the inferior vena cava by congenital anteromedial diaphragmatic hernia: An extremely rare case of Budd-Chiari syndrome in an infant. Surgery 124:109-111, 1998 14. Markert DJ, Shanmuganathan K, Mirvis SE, et al: Budd-Chiari syndrome resulting from intrahepatic IVC compression secondary to blunt hepatic trauma. Clin Radiol 52:384-387, 1997 15. Bilbao JI, Pueyo JC, Longo JM, et al: Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc Intervent Radiol 20:112-119, 1997 16. Mazariegos GV, Garrido V, Jaskowski-Phillips S, et al: Management of hepatic venous obstruction after split-liver transplantation. Pediatr Transplant 4:322-327, 2000 17. Sze DY, Semba CP, Razavi MK, et al: Endovascular treatment of hepatic venous outflow obstruction after piggyback technique liver transplantation. Transplantation 68:446-449, 1999 18. Witte AM, Kool LJ, Veenendaal R, et al: Hepatic vein stenting for Budd-Chiari syndrome. Am J Gastroenterol 92:498-501, 1997 19. Weernink EE, Huisman AB, van Baarlen J, et al: Treatment of the Budd-Chiari syndrome by insertion of a wall-stent in the hepatic vein after percutaneous transluminal angioplasty: The necessity of follow-up. Eur J Gastroenterol Hepatol 8:85-88, 1996
Massive splenomegaly and ascites production with hypoproteinemia and hypocoagulability was observed in a 15-yearold boy after extended right hepatectomy for hepatocellular carcinoma (HCC). Angiography disclosed a kinking of the left hepatic vein immediately before the junction with the inferior vena cava. Ascites disappeared completely, and laboratory values normalized after placement of a 3-cm long balloon expandable stent. The current case shows that Budd-Chiari
syndrome caused by hepatic outflow obstruction after major hepatic surgery can be managed effectively by percutaneous stent placement. J Pediatr Surg 37:1640-1642. Copyright 2002, Elsevier Science (USA). All rights reserved. INDEX WORDS: Hepatocellular carcinoma, extended right hepatectomy, Budd-Chiari syndrome, stent placement.
P
RIMARY MALIGNANT liver tumors are rare in children and account for approximately 0.5 to 2% of all malignancies in this age group.1-4 Whereas hepatoblastoma occurs mostly in the first 3 years of life, the median age of children with hepatocellular carcinoma (HCC) at diagnosis is 10 years.2-4 We present the case of a 15-yearold boy who underwent extended right hepatectomy for HCC. Because of a kinking of the remaining left hepatic vein, Budd-Chiari syndrome developed postoperatively (BCS), and was managed successfully by stent insertion. CASE REPORT The patient presented with a one-week history of painless abdominal enlargement. Physical examination found a palpable tumor in the upper right quadrant of the abdomen but was otherwise normal. Abdominal sonography and magnetic resonance imaging (MRI) showed a 21 ⫻ 17 ⫻ 15.5-cm tumor in the right lobe of the liver (Fig 1). The retrohepatic inferior vena cava was invaded by the tumor and completely occluded above the right adrenal vein. The entire blood from the lower part of the body was drained by the vertebral veins. Initial laboratory investigations found discrete elevation of liver enzymes (ALT, 31 U/L; AST, 32 U/L), anemia (hemoglobin, 114 g/L), and a serum alpha-fetoprotein (AFP) level of greater than 880 U/mL (normal, 0 to 7 U/mL). Histology of tumor tissue obtained on open liver biopsy was consistent with the diagnosis of HCC. Tumor resection was performed via extended transverse laparotomy. Extended right hepatectomy (trisegmentectomy) including resection of the retrohepatic vena cava and segment I was necessary to remove the tumor completely. The retrohepatic vena cava was resected above the right
From the Division of Pediatric Hematology and Oncology, Departments of Pediatrics, Radiology, and Pediatric Surgery, University of Graz, Austria. Address reprint requests to Christian Urban, MD, Division of Pediatric Hematology and Oncology, Department of Pediatrics, University of Graz, Auenbruggerplatz 30, A-8036 Graz, Austria. Copyright 2002, Elsevier Science (USA). All rights reserved. 0022-3468/02/3711-0028$35.00/0 doi:10.1053/jpsu.2002.36203 1640
Fig 1. Coronal magnetic resonance imaging of the abdomen shows a large, well-circumscribed mass in the right lobe of the liver.
adrenal vein and below the confluence of the left hepatic vein into the inferior vena cava. The remaining segments II and III were fixed to the diaphragm to achieve uninhibited blood flow from the left liver vein into the vena cava. Postoperatively, the patient had massive splenomegaly and ascites. Up to 10 L of peritoneal fluid were removed daily by the 2 abdominal drains. Despite maximal diuretic therapy, daily ascites production could only be reduced to 4 to 5 L. Laboratory evaluation at that time identified hypoproteinemia (46 g/L; normal, 64 to 82 g/L), low serum cholinesterase (364 U/L; normal, 2,800 to 8,000 U/L), and hypocoagulability (quick value, 40%; normal, 70% to 120%; fibrinogen, 1.50 g/L; normal, 1.8 to 4.0 g/L; activated partial thromboplastin time [aPTT], 65 seconds; normal, 25.0 to 36.0 seconds). High doses of albumin and clotting factors had to be replaced daily. A relaparotomy was performed on the 13th postoperative day but excluded any significant fluid loss by major abdominal lymphatic vessels. Thrombosis of the portal or remaining left hepatic vein was excluded by color-coded Doppler sonography. However, angiography after placement of an angiography catheter into the left hepatic vein showed a kinking of the left hepatic vein immediately before the junction with the inferior vena cava (Fig 2). Intravascular pressure measurement showed a translesional gradient of 10 mm Hg. Over a teflon-coated guidewire (Bentson, William Cook Company, Denmark) a
Journal of Pediatric Surgery, Vol 37, No 11 (November), 2002: pp 1640-1642
BUDD-CHIARI SYNDROME
1641
grounds, pulsed Doppler sonography, inferior vena cavography/functional hepatography, and liver biopsy.5 A number of different clinicopathologic conditions causing occlusion of the main hepatic veins or inferior vena cava above the hepatic veins may lead to BCS.6-15 “Classical” BCS occurs because of primary hepatic vein thrombosis on the basis of a hypercoagulable state, which may be found in factor V Leiden mutations, antiphospholipid antibody syndrome, protein C deficiency, paroxysmal nocturnal hemoglobinuria, vasculitis syndromes such as Behcet disease, or malignant hematologic disorders.7-11 However, various other obliterative processes affecting the hepatic veins or inferior vena cava (eg, membranous obstruction, diaphragmatic hernia, blunt hepatic trauma) also may cause BCS.12-14 BCS also has been reported as a complication after major hepatic surgery or orthotopic liver transplantation.15-17 In our patient with HCC the lack of distant metastases and the fact that the tumor involved the right lobe of the liver only allowed the surgeon to perform a complete removal of the tumor. As a complication, short length kinking of the remaining left hepatic vein resulted in the development of BCS, which did not respond to conservative medical treatment. Stent implantation, which has been shown to be a feasible and safe therapeutic option for BCS in the last decade15-19 was, therefore, regarded Fig 2. Angiography shows a kinking of the left hepatic vein short before the junction with the inferior vena cava (arrow).
3-cm long balloon expandable stent (Palmaz, Johnson & Johnson Interventional Systems, Warren, NJ) was inserted. The stent was dilated to a diameter of 13 mm. After stent placement, dilatation of the stent with a 14-mm ⫻ 4-cm angioplasty balloon was performed to smooth out wrinkles or folds. Control angiography showed unlimited flow through the left hepatic vein into the inferior vena cava with no translesional pressure gradient (Fig 3). No complications were observed during and after the procedure. Subsequently, ascites disappeared completely within 2 days, and laboratory values returned to normal. After postoperative recovery, the patient received 7 courses of chemotherapy over a 7-month period consisting of ifosfamide (total dose [TD], 11.5 g), cisplatin (TD, 305 mg), doxorubicin (TD, 260 mg), carboplatin (TD, 1720 mg), and etoposide (TD, 860 mg). As shown by follow-up abdominal and chest computed tomography scans the patient is free of local recurrence or distant metastases 3 years after diagnosis. Serum AFP levels normalized postoperatively and color-coded sonography continuously shows a patent venous lumen at the stent site.
DISCUSSION
BCS is caused by hepatic vein or inferior vena cava obstruction above the hepatic veins. It is a rare condition, particularly in children. Clinically, this syndrome is characterized by abdominal pain and distension, jaundice, gastrointestinal bleeding, hepatosplenomegaly, portal hypertension with ascites production, and sometimes liver failure.5 Diagnosis of BCS is based on clinical
Fig 3. Angiography after stent placement shows unlimited flow in the left hepatic vein.
1642
BENESCH ET AL
the best chance to restore hepatic vein outflow, thus leading to disappearance of ascites and normalization of pathologic laboratory values. In addition, stent insertion allowed to administer adjuvant chemotherapy immedi-
ately after recovery to maintain the remission achieved by tumor resection. The current case illustrates that childhood BCS occuring after major hepatic surgery can be managed effectively by percutaneous stent placement.
REFERENCES 1. Stocker JT: An approach to handling pediatric liver tumors. Am J Clin Pathol 109:67-72, 1998 (suppl 1) 2. Exelby PR, Filler RM, Grosfeld JL: Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey-1974. J Pediatr Surg 10:329-337, 1975 3. Lack EE, Neave C, Vawter GF: Hepatocellular carcinoma. Review of 32 cases in childhood and adolescence. Cancer 52:1510-1515, 1983 4. Chen JC, Chen CC, Chen WJ, et al: Hepatocellular carcinoma in children: Clinical review and comparison with adult cases. J Pediatr Surg 33:1350-1354, 1998 5. Singh V, Sinha SK, Nain CK, et al: Budd-Chiari syndrome: Our experience of 71 patients. J Gastroenterol Hepatol 15:550-554, 2000 6. Okuda K, Kage M, Shrestha SM: Proposal of a new nomenclature for Budd-Chiari syndrome: Hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 28:11911198, 1998 7. Mahmoud AE, Elias E, Beauchamp N, et al: Prevalence of the factor V Leiden mutation in hepatic and portal vein thrombosis. Gut 40:798-800, 1997 8. Bayraktar Y, Balkanci F, Bayraktar M, et al: Budd-Chiari syndrome: A common complication of Behcet’s disease. Am J Gastroenterol 92:858-862, 1997 9. Sugano S, Suzuki T, Makino H, et al: Budd-Chiari syndrome attributed to protein C deficiency. Am J Gastroenterol 91:777-779, 1996 10. Wyatt HA, Mowat AP, Layton M: Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome. Arch Dis Child 72:241-242, 1995
11. Saca LF, Szer IS, Henar E, et al: Budd-Chiari syndrome associated with antiphospholipid antibodies in a child: Report of a case and review of the literature. J Rheumatol 21:545-548, 1994 12. Odell JA, Rode H, Millar AJ, et al: Surgical repair in children with the Budd-Chiari syndrome. J Thorac Cardiovasc Surg 110:916923, 1995 13. Yonekura T, Kubota A, Hoki M, et al: Intermittent obstruction of the inferior vena cava by congenital anteromedial diaphragmatic hernia: An extremely rare case of Budd-Chiari syndrome in an infant. Surgery 124:109-111, 1998 14. Markert DJ, Shanmuganathan K, Mirvis SE, et al: Budd-Chiari syndrome resulting from intrahepatic IVC compression secondary to blunt hepatic trauma. Clin Radiol 52:384-387, 1997 15. Bilbao JI, Pueyo JC, Longo JM, et al: Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc Intervent Radiol 20:112-119, 1997 16. Mazariegos GV, Garrido V, Jaskowski-Phillips S, et al: Management of hepatic venous obstruction after split-liver transplantation. Pediatr Transplant 4:322-327, 2000 17. Sze DY, Semba CP, Razavi MK, et al: Endovascular treatment of hepatic venous outflow obstruction after piggyback technique liver transplantation. Transplantation 68:446-449, 1999 18. Witte AM, Kool LJ, Veenendaal R, et al: Hepatic vein stenting for Budd-Chiari syndrome. Am J Gastroenterol 92:498-501, 1997 19. Weernink EE, Huisman AB, van Baarlen J, et al: Treatment of the Budd-Chiari syndrome by insertion of a wall-stent in the hepatic vein after percutaneous transluminal angioplasty: The necessity of follow-up. Eur J Gastroenterol Hepatol 8:85-88, 1996