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Liver transplantation in a patient with diffuse portal venous system thrombosis
Liver Transplantation in a Patient With Diffuse Portal Venous System Thrombosis M.-C. Ho, R.-H. Hu, H.-S. Lai, P.-M. Yang, M.-Y. Lai, and P.-H. Lee
P
ORTAL vein thrombosis (PVT) may occur as a consequence of end stage liver disease as a result of slowed blood flow and increased hepatic resistance.1 It was considered an absolute contraindication for orthotopic liver transplantation (OTL), because PVT usually associated with high mortality and morbidity after OTL.2 Some authors reported that OTL may be done successfully if hepatopetal flow to liver graft can be restored.3– 8 PVT is no longer an absolute contraindication for OTL in many institutes. However, OTL still considered impossible in patients with diffuse thrombosis in the portal venous system. Here, we report an experience of liver transplantation using portocaval hemitransposition in a patient with diffuse portal venous system thrombosis, and we described the hemodynamic change and complications after the operation.
CASE PRESENTATION
A 48-year-old male with liver cirrhosis of unknown cause was transferred for orthotopic liver transplantation (OLT). He did not have any past history of viral hepatitis. Neither alcoholism nor drug abuse was noted. A survey of autoimmune and metabolic liver disease all showed negative results. He worked in a factory producing plastics for many years. Nine years before OTL, the patient had an episode of esophageal varices bleeding. Gastric devascularization, esophageal transection, and splenectomy were done to stop the bleeding. After the operation, he led and uneventful life until 3 years before OTL, progressive abdominal distention was noticed. Abdominal sonography showed marked liver cirrhosis and massive ascites. Six months before OTL, recurrent gastrointestinal hemorrhage, conscious disturbance, and abdominal pain developed. Under the impression of liver cirrhosis complicated with recurrent variceal bleeding, hepatic encephalopathy, intractable ascites, and spontaneous bacterial peritonitis, he was admitted for preliver transplantation evaluation. His medical history was otherwise unremarkable. On physical examination, his conscious was drowsy. The conjunctiva was pale and the sclera was slightly icteric. He had diminished breathing sound bilaterally and slight clubbing of the fingers. The abdomen was distended with shifting dullness. The extremities are edematous. The laboratory data were hematocrit 25.4%,
platelet 420,000/L, prothrombin time 18.1 second (control 11.7), partial prothrombin time 46.1 second (control 33.8), albumin 2.2 g/dl, total bilirubin 1.5 mg/dL and creatinine 0.9 mg/dL. Chest roentgenogram showed elevation of right hemidiaphragm and bilateral pleural effusion. Abdominal sonography and computed tomography (CT) showed massive ascites, severe liver cirrhosis, and prominent collateral circulation around the gastroesophageal junction. However, the portal vein was not found. Doppler sonography also failed to show any signal of the portal flow. OTL was done using piggyback method without venousvenous bypass. A segment of inferior vena cava (IVC) and iliac vein was harvested together with the liver graft. Diffuse thrombosis in the superior mesenteric vein, splenic vein, and inferior mesenteric vein were noted during the operation. We also could not find any variceal vein with sufficient size for portal vein reconstruction. Anastomosis between the end of the donor’s portal vein, and the anterior wall of the recipient’s IVC was performed. Poor portal flow was detected by palpation and Doppler sonography during the operation. The flow improved after the IVC was occluded by a vascular clamp proximal to the anastomosis. After hepatic artery anastomosis was completed, the vascular clamp was removed due to better portal flow. The operation time was 22 hours and 30 minutes with anhepatic phase of 7 hours 10 minutes. The patient waked up 4 hours after the operation. The liver graft began to produce bile then. The INR decreased to 1.5 on the 2nd postoperative day. Doppler sonography 1 day after the operation showed patent hepatic artery and portal vein flow. Unfortunately, bleeding happened on the 7th postoperative day. Exploratory laparotomy was performed. Massive blood clot was noted at the right subphrenic area. The portal vein was patent by palpation and Doppler sonography. Acute renal failure and infection developed after the second operation. After intermittent
From the Department of Surgery, Department of Medicine (H.-S.L., P.M.Y.), National Taiwan University, Taipei, Taiwan, ROC Address reprint requests to Dr Po-Huang Lee, National Taiwan University Hospital, Department of Surgery, No. 7 Chung-Shan South Road, Taipei, Taiwan 100, ROC.
0041-1345/00/$–see front matter PII S0041-1345(00)01622-5
© 2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
2174
Transplantation Proceedings, 32, 2174–2176 (2000)
LIVER TX IN PATIENT WITH PVT
hemodialysis and strong antibiotic treatment, the patient went home smoothly. After discharge, the patient’s liver function was stable. An episode of conscious disturbance happened 2 months after the OLT. Elevated serum ammonia level was noted. The Doppler sonography showed complete occlusion of the portal vein and IVC anastomosis. The portal flow in the liver was hepatofugal. He regained his consciousness after lactulose treatment. After that, lactulose was necessary to prevent the happening of hepatic encephalopathy. However, the patient lost his consciousness again 10 months after OTL. Massive tarry stool was also noticed. Upper gastrointestinal endoscopy, colonofibroscopy and angiography failed to show any extravasation of the contrast medium. RBC scan only showed a suspected extravasation at the jejunum. The patient recovered after blood component transfusion and pharmacological treatment. DISCUSSION
The occurrence of PVT in terminal liver cirrhosis usually leads to some difficulties in OLT, since severe portal hypertension can complicate the surgical procedure.9 Sometimes the thrombosis has to be removed, or the portal vein has to be resected in order to restore a sufficient portal flow to the liver graft. A vein graft, bypassing the occluded portal vein, from different vessels of the portal vein system has been reported to establish good portal flows to the liver grafts.5– 8 One of the limiting factors of these venous jump-graft procedures is the extent of the PVT. Thrombosis involving the portal vein, splenic vein, SMV and variceal vein at the site of surgical accessible segment may make the alternative procedures impossible.10,11 The risk of OTL is so high, that it was suggested that this kind of patient should not be included as a candidate for liver transplantation, since many other better candidates are dying on the waiting list.12,13 However, the extent of venous thrombosis can not be evaluated completely before OTL using conventional noninvasive image study. In addition to this, portal vein thrombosis can develop after the patient has been on the waiting list. Once this is found during OTL, a poor outcome usually can be expected. In patients with diffuse PVT, IVC may be the only possible source of blood supply to the graft. Some reported that liver could survive and function with portal venous blood flow from IVC.14 –19 Cavoportal hemitransposition was also proposed to treat patients with diffuse portal vein thrombosis.20 However, the mortality of the reported patients was high (7/12), and the complications were not infrequent. Liver congestion, portal vein thrombosis, infection, portal hypertension, and hepatic encephalopathy may happen after OTL using this technique. Our patient experience several episodes of conscious disturbance due to hepatic encephalopathy and gastrointestinal bleeding after the OLT. They are now well controlled by pharmacological treatment. In our patient, the IVC was not occluded as that reported
2175
by others. This might cause the disappearance of the newly established portal flow. The IVC should be partially or completely ligated, so that more flow can go to the liver graft, but hyperperfusion of the liver usually causes congestion of the liver and poor liver function. The optimal portal flow should be studied and estimated to decrease the complications of this procedure. Another problem that is not well known is the hemodynamic changes of the portal venous flow. As shown in our patient, hepatopetal flow was established immediately after the operation. However, follow-up Doppler sonography showed no signal at the portocaval anastomosis, but hepatofugal portal flow was noted in the liver. The liver function remained stationary, though the hemodynamics of the portal flow changed a lot. It is possible that the collateral circulation developed after OTL, back flow from IVC, or hepatic arterial-venous shunting provide the blood flow in the portal vein. Hepatotrophic substances, nutrition necessary for the survival and growth of hepatocyte may be delivered to the liver through these ways, but this is not clearly demonstrated. In summary, portocaval hemitranspostion may be a useful technique when satisfactory hepatopetal flow to the liver graft can not be established in a patient with diffuse portal venous thrombosis. However, it is usually associated with more operation blood loss, longer operation time, and higher complication rate. The hemodynamic change of the portal venous flow is not clear. It may be the cause of many complications of this procedure. The procedure is not recommended unless all other options have been exhausted and there is an immediate risk of death. To prevent the complication of this procedure, detail evaluation of the portal venous system before OTL is important. REFERENCES 1. Okuda K, Ohnishi K, Kimura K, et al: Gastroenterology 2:279, 1985 2. Czernia KA, Badger I, Sherlock D, et al: Transplantation 2:334, 1990 3. Gonza´ EM, Garcia IG, Sanz RG, et al: Br J Surg 80:81, 1993 4. Starzl TE, Halgrimon CG, Koep KLJ, et al: Surg Gynecol Obstet 149:76, 1979 5. Shaked A, Busuttil RW: Ann Surg 214:696, 1991 6. Moreno E, Garcia I, Gomez R, et al: Br J Surg 80:81, 1993 7. Kirsch JP, Howard TK, Klintmalm GB, et al: Surgery 107:544, 1990 8. Pinna AD, Lim JW, Sugitani AD, et al: J Am College of Surg 183:527, 1996 9. Helling TS: Transplantation 40:446, 1985 10. Stieber AC, Zetti G, Todo S, et al: Ann Surg 213:199, 1991 11. Cameron JL: Surg Gynecol Obstet 176:395, 1993 12. Todo S, Reyes J, Furukawa H, et al: Ann Surg 210:649, 1989 13. Tzakis AG, Nery JR, Thompson J, et al: Transplant Proc 29:683, 1997 14. Child CG III, Barr D, Holswade GR, et al: Ann Surg 138:600, 1958 15. Silen W, Mawdsley DL, Weirich WL, et al: Arch Surg 74:964, 1957
2176 16. Starzl TE, Scanlon WA, Thornton FH, et al: Surgery 52:660, 1962 17. Riddell AG, Dacies RP, Clark AD: Lancet 2:1146, 1996 18. Starzl TE, Brown BI, Banchard H, et al: Surgery 65:504, 1969
HO, HU, LAI ET AL 19. Starzl TE, Putnam CW, Porter KA, et al: Ann Surg 178:525, 1978 20. Tzakis AG, Kirkegaard P, Pinna AD, et al: Transplantation 65:619, 1998
P
ORTAL vein thrombosis (PVT) may occur as a consequence of end stage liver disease as a result of slowed blood flow and increased hepatic resistance.1 It was considered an absolute contraindication for orthotopic liver transplantation (OTL), because PVT usually associated with high mortality and morbidity after OTL.2 Some authors reported that OTL may be done successfully if hepatopetal flow to liver graft can be restored.3– 8 PVT is no longer an absolute contraindication for OTL in many institutes. However, OTL still considered impossible in patients with diffuse thrombosis in the portal venous system. Here, we report an experience of liver transplantation using portocaval hemitransposition in a patient with diffuse portal venous system thrombosis, and we described the hemodynamic change and complications after the operation.
CASE PRESENTATION
A 48-year-old male with liver cirrhosis of unknown cause was transferred for orthotopic liver transplantation (OLT). He did not have any past history of viral hepatitis. Neither alcoholism nor drug abuse was noted. A survey of autoimmune and metabolic liver disease all showed negative results. He worked in a factory producing plastics for many years. Nine years before OTL, the patient had an episode of esophageal varices bleeding. Gastric devascularization, esophageal transection, and splenectomy were done to stop the bleeding. After the operation, he led and uneventful life until 3 years before OTL, progressive abdominal distention was noticed. Abdominal sonography showed marked liver cirrhosis and massive ascites. Six months before OTL, recurrent gastrointestinal hemorrhage, conscious disturbance, and abdominal pain developed. Under the impression of liver cirrhosis complicated with recurrent variceal bleeding, hepatic encephalopathy, intractable ascites, and spontaneous bacterial peritonitis, he was admitted for preliver transplantation evaluation. His medical history was otherwise unremarkable. On physical examination, his conscious was drowsy. The conjunctiva was pale and the sclera was slightly icteric. He had diminished breathing sound bilaterally and slight clubbing of the fingers. The abdomen was distended with shifting dullness. The extremities are edematous. The laboratory data were hematocrit 25.4%,
platelet 420,000/L, prothrombin time 18.1 second (control 11.7), partial prothrombin time 46.1 second (control 33.8), albumin 2.2 g/dl, total bilirubin 1.5 mg/dL and creatinine 0.9 mg/dL. Chest roentgenogram showed elevation of right hemidiaphragm and bilateral pleural effusion. Abdominal sonography and computed tomography (CT) showed massive ascites, severe liver cirrhosis, and prominent collateral circulation around the gastroesophageal junction. However, the portal vein was not found. Doppler sonography also failed to show any signal of the portal flow. OTL was done using piggyback method without venousvenous bypass. A segment of inferior vena cava (IVC) and iliac vein was harvested together with the liver graft. Diffuse thrombosis in the superior mesenteric vein, splenic vein, and inferior mesenteric vein were noted during the operation. We also could not find any variceal vein with sufficient size for portal vein reconstruction. Anastomosis between the end of the donor’s portal vein, and the anterior wall of the recipient’s IVC was performed. Poor portal flow was detected by palpation and Doppler sonography during the operation. The flow improved after the IVC was occluded by a vascular clamp proximal to the anastomosis. After hepatic artery anastomosis was completed, the vascular clamp was removed due to better portal flow. The operation time was 22 hours and 30 minutes with anhepatic phase of 7 hours 10 minutes. The patient waked up 4 hours after the operation. The liver graft began to produce bile then. The INR decreased to 1.5 on the 2nd postoperative day. Doppler sonography 1 day after the operation showed patent hepatic artery and portal vein flow. Unfortunately, bleeding happened on the 7th postoperative day. Exploratory laparotomy was performed. Massive blood clot was noted at the right subphrenic area. The portal vein was patent by palpation and Doppler sonography. Acute renal failure and infection developed after the second operation. After intermittent
From the Department of Surgery, Department of Medicine (H.-S.L., P.M.Y.), National Taiwan University, Taipei, Taiwan, ROC Address reprint requests to Dr Po-Huang Lee, National Taiwan University Hospital, Department of Surgery, No. 7 Chung-Shan South Road, Taipei, Taiwan 100, ROC.
0041-1345/00/$–see front matter PII S0041-1345(00)01622-5
© 2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
2174
Transplantation Proceedings, 32, 2174–2176 (2000)
LIVER TX IN PATIENT WITH PVT
hemodialysis and strong antibiotic treatment, the patient went home smoothly. After discharge, the patient’s liver function was stable. An episode of conscious disturbance happened 2 months after the OLT. Elevated serum ammonia level was noted. The Doppler sonography showed complete occlusion of the portal vein and IVC anastomosis. The portal flow in the liver was hepatofugal. He regained his consciousness after lactulose treatment. After that, lactulose was necessary to prevent the happening of hepatic encephalopathy. However, the patient lost his consciousness again 10 months after OTL. Massive tarry stool was also noticed. Upper gastrointestinal endoscopy, colonofibroscopy and angiography failed to show any extravasation of the contrast medium. RBC scan only showed a suspected extravasation at the jejunum. The patient recovered after blood component transfusion and pharmacological treatment. DISCUSSION
The occurrence of PVT in terminal liver cirrhosis usually leads to some difficulties in OLT, since severe portal hypertension can complicate the surgical procedure.9 Sometimes the thrombosis has to be removed, or the portal vein has to be resected in order to restore a sufficient portal flow to the liver graft. A vein graft, bypassing the occluded portal vein, from different vessels of the portal vein system has been reported to establish good portal flows to the liver grafts.5– 8 One of the limiting factors of these venous jump-graft procedures is the extent of the PVT. Thrombosis involving the portal vein, splenic vein, SMV and variceal vein at the site of surgical accessible segment may make the alternative procedures impossible.10,11 The risk of OTL is so high, that it was suggested that this kind of patient should not be included as a candidate for liver transplantation, since many other better candidates are dying on the waiting list.12,13 However, the extent of venous thrombosis can not be evaluated completely before OTL using conventional noninvasive image study. In addition to this, portal vein thrombosis can develop after the patient has been on the waiting list. Once this is found during OTL, a poor outcome usually can be expected. In patients with diffuse PVT, IVC may be the only possible source of blood supply to the graft. Some reported that liver could survive and function with portal venous blood flow from IVC.14 –19 Cavoportal hemitransposition was also proposed to treat patients with diffuse portal vein thrombosis.20 However, the mortality of the reported patients was high (7/12), and the complications were not infrequent. Liver congestion, portal vein thrombosis, infection, portal hypertension, and hepatic encephalopathy may happen after OTL using this technique. Our patient experience several episodes of conscious disturbance due to hepatic encephalopathy and gastrointestinal bleeding after the OLT. They are now well controlled by pharmacological treatment. In our patient, the IVC was not occluded as that reported
2175
by others. This might cause the disappearance of the newly established portal flow. The IVC should be partially or completely ligated, so that more flow can go to the liver graft, but hyperperfusion of the liver usually causes congestion of the liver and poor liver function. The optimal portal flow should be studied and estimated to decrease the complications of this procedure. Another problem that is not well known is the hemodynamic changes of the portal venous flow. As shown in our patient, hepatopetal flow was established immediately after the operation. However, follow-up Doppler sonography showed no signal at the portocaval anastomosis, but hepatofugal portal flow was noted in the liver. The liver function remained stationary, though the hemodynamics of the portal flow changed a lot. It is possible that the collateral circulation developed after OTL, back flow from IVC, or hepatic arterial-venous shunting provide the blood flow in the portal vein. Hepatotrophic substances, nutrition necessary for the survival and growth of hepatocyte may be delivered to the liver through these ways, but this is not clearly demonstrated. In summary, portocaval hemitranspostion may be a useful technique when satisfactory hepatopetal flow to the liver graft can not be established in a patient with diffuse portal venous thrombosis. However, it is usually associated with more operation blood loss, longer operation time, and higher complication rate. The hemodynamic change of the portal venous flow is not clear. It may be the cause of many complications of this procedure. The procedure is not recommended unless all other options have been exhausted and there is an immediate risk of death. To prevent the complication of this procedure, detail evaluation of the portal venous system before OTL is important. REFERENCES 1. Okuda K, Ohnishi K, Kimura K, et al: Gastroenterology 2:279, 1985 2. Czernia KA, Badger I, Sherlock D, et al: Transplantation 2:334, 1990 3. Gonza´ EM, Garcia IG, Sanz RG, et al: Br J Surg 80:81, 1993 4. Starzl TE, Halgrimon CG, Koep KLJ, et al: Surg Gynecol Obstet 149:76, 1979 5. Shaked A, Busuttil RW: Ann Surg 214:696, 1991 6. Moreno E, Garcia I, Gomez R, et al: Br J Surg 80:81, 1993 7. Kirsch JP, Howard TK, Klintmalm GB, et al: Surgery 107:544, 1990 8. Pinna AD, Lim JW, Sugitani AD, et al: J Am College of Surg 183:527, 1996 9. Helling TS: Transplantation 40:446, 1985 10. Stieber AC, Zetti G, Todo S, et al: Ann Surg 213:199, 1991 11. Cameron JL: Surg Gynecol Obstet 176:395, 1993 12. Todo S, Reyes J, Furukawa H, et al: Ann Surg 210:649, 1989 13. Tzakis AG, Nery JR, Thompson J, et al: Transplant Proc 29:683, 1997 14. Child CG III, Barr D, Holswade GR, et al: Ann Surg 138:600, 1958 15. Silen W, Mawdsley DL, Weirich WL, et al: Arch Surg 74:964, 1957
2176 16. Starzl TE, Scanlon WA, Thornton FH, et al: Surgery 52:660, 1962 17. Riddell AG, Dacies RP, Clark AD: Lancet 2:1146, 1996 18. Starzl TE, Brown BI, Banchard H, et al: Surgery 65:504, 1969
HO, HU, LAI ET AL 19. Starzl TE, Putnam CW, Porter KA, et al: Ann Surg 178:525, 1978 20. Tzakis AG, Kirkegaard P, Pinna AD, et al: Transplantation 65:619, 1998