A 17‐yr‐oId female received a liver transplant for type I glycogen storage disease. A year later, when she experienced variceal gastrointestinal hemorrhage, an angiogram revealed thrombosis of the portal vein with hepatopetal collateral channels. A distal splenorenal shunt was performed because of failure of sclerotherapy to control subsequent bleeding episodes and the fact that the liver function was normal. This patient continues to have normal hepatic function with a patent splenorenal shunt 4 yr after the shunting procedure. This case illustrates the feasibility of a distal splenorenal shunt to alleviate portal hypertension in cases of thrombosis of the portal vein following hepatic transplantation if the liver function is normal. Copyright © 1989, Wiley Blackwell. All rights reserve

Esquivel, CO

Malatack, J

Marino, IR

Scantlebury, VP

Shaw, BW

Starzl, TE

Zajko, AB

PubMed

Malateck, J

Name not available

Distal splenorenal shunt for portal vein thrombosis after liver transplantation

D-Scholarship@Pitt

0002-9270/89/8401-0067 THE AMERICAN JOURNAL OF GASTROENTEROLOGY Copyright © 1989 by Am. con. of Gastroenterology ~;D? Q(P4, Vol. 84, No. I, 1989 Printed in U.S.A. Distal Splenorenal Shunt for Portal Vein Thrombosis after Liver Transplantation Ignazio R. Marino, M.D., Carlos O. Esquivel, M.D., Ph.D., Albert B. Zajko, M.D., Jeffrey Malatack, M.D., Velma P. Scantlebury, M.D., Byers W. Shaw, M.D.,"" and Thomas E. Starzl, M.D., Ph.D. Departments of Surgery, Pediatrics, and Radiology, University Health Center of Pittsburgh, University of Pittsburgh, and the Veterans Administration Medical Center, Pittsburgh, Pennsylvania A 17-yr-old female received a liver transplant for type I glycogen storage disease. A year later, when she experienced variceal gastrointestinal hemorrhage, an angiogram revealed thrombosis of the portal vein with hepatopetal collateral channels. A distal splenorenal shunt was performed because of failure of sclerotherapy to control subsequent bleeding episodes and the fact that the liver function was normal. This patient contin-ues to have normal hepatic function with a patent sple-norenal shunt 4 yr after the shunting procedure. This case illustrates the feasibility of a distal splenorenal shunt to alleviate portal hypertension in cases of throm-bosis of the portal vein following hepatic transplantation if the liver function is normal. INTRODUCTION Survival rates and rehabilitation after orthotopic liver transplantation (OLT) have greatly improved during the so-called cyclosporine era (1). Nevertheless, tech-nical problems are still significant (2) and reoperation is often required. Thrombosis of the hepatic artery and problems associated with biliary tract reconstruction represent the most important technical complications, with an incidence of7% and 13.2%, respectively (3,4). Thrombosis of the portal vein after OLT (reported incidence, 1.8%) has been observed almost exclusively in children (5). The diagnosis usually results from the development of clinical signs of portal hypertension, such as variceal hemorrhage, splenomegaly, and hyper-splenism. The treatment is aimed at relieving the pre-senting symptoms or correcting the underlying cause. In our experience, the therapy for posttransplantation portal vein thrombosis depends on the clinical presen-tation, residual portal flow, and liver function. Conse-quently, the therapeutic choices are sclerotherapy or retransplantation. We report herein a very unusual case of late portal thrombosis following OLT in a child. As a sequel to * Department of Surgery, University of Nebraska Medical Center, Omaha, Nebraska. Received Mar. 1, 1988; accepted July 6, 1988. 67 this, the patient developed portal-portal hepatopetal collaterals sufficient to maintain good liver perfusion but unable to decompress the portal hypertension. She was successfully treated with a splenorenal shunt. CASE REPORT A 17-yr-old white female with end-stage liver disease secondary to type 1 glycogen storage disease was re-ferred in January 1982 to our institution for OLT evaluation. Nine years before the referral, she under-went end-to-side portacaval shunt to improve her growth and the metabolic abnormalities associated with glycogen storage disease (6). This treatment was effec-tive in improving some aspects of her disease, such as lipid and uric acid metabolism, acidosis, and growth retardation. However, surgery did not relieve the fre-quency of hypoglycemia, and appeared to have wors-ened the clinical effect of the hypoglycemic episodes (7). Frequent daytime feeds and nighttime nasogastric feedings were necessary after the portacaval shunt to avoid symptomatic hypoglycemia. During this period, the child was followed with serial ultrasound examinations of the liver which began to reveal intrahepatic masses. A biopsy of one of these lesions proved to be an adenoma. In September 1981, the patient experienced the onset of severe abdominal pain and liver swelling which was thought to be related to a hemorrhage into the largest of the adenomas which led to biliary obstruction and hepatic failure. The child had an alkaline phosphatase of 3000 IU /L, ammonia of 315 j.Lg/L, and increased liver enzyme levels. Two months later, her condition deteriorated further, and she became jaundiced. The patient improved on total parenteral nutrition alone, and she returned to her base-line state of health. The decision was made to make the patient a liver transplant candidate, based on the fact that she had hepatic ade-nomas with the potential risk of developing carcino-matous changes or liver failure or both. Liver transplantation was performed using the tech-nique previously described (8, 9). Immunosuppression 68 MARINO et al. consisted of cyclosporine and prednisone ( I). Her initial postoperative course was complicated by a bile leak which was treated with drainage and sealed sponta-neously. Nevertheless, liver function remained excellent and her subsequent clinical course was uneventful. One year after OLT, the patient was evaluated for gastrointestinal tract bleeding. Endoscopy showed esophageal and gastric varices. Angiography demon-strated portal vein thrombosis and hepatopetal collat-eral formation (Fig. I). Liver enzyme levels were nor-mal. Treatment consisted of blood transfusions and endoscopic sclerosis of the varices. Despite this, she had several episodes of significant upper gastrointestinal tract bleeding over the next 3 months. Liver biopsy was normal, as were liver enzyme levels. Because of repeated episodes of gastrointestinal tract bleeding and the subsequent need for multiple trans-fusions, a distal splenorenal shunt for control of portal hypertension was performed in June 1983. The anas-tomosis measured approximately 13 mm in diameter. The splenorenal shunt decompressed the varices and resolved the gastrointestinal tract bleeding. Follow-up angiography performed in May 1984 (Fig. 2), ultrasound performed in December 1986, and, more recently, a nuclear magnetic resonance test demon-strated a widely patent splenorenal shunt. Over the past 4 yr, the patient has not had any significant gastroin-testinal tract bleeding. The most recent liver enzyme levels, performed in March 1987, were normaL FIG. I . Hepatopetal venous collaterals after portal vein thrombo-sis following liver transplantation. Occlusion of the extrahepatic portal vein (long slraighl arrow) at tbe junction of the splenic and superior mesenteric veins demonstrated on subtraction venous phase film of selective superior mesenteric arteriogram. There is reconsti-tution of the intrahepatic portal vessels (shari siraighl arrow) via multiple tortuous and dilated collateral vessels (curved arrows) to the right of the superior mesenteric vein and in the hilar region . There is portal hypertension as evidenced by retrograde flow in the splenic vein and marked gastroesophageal varices. Vol. 84, No.1, 1989 FIG. 2. A widely patent splenorenal shunt (arrow) is demonstrated on splenic venogram performed I yr after surgery. This study was performed by passing a catheter retrogradely through the shunt into the splenic vein via the left renal vein. (Courtesy of Philip Stanley, M.D., Children's Hospital of Los Angeles, CA). DISCUSSION Thrombosis of the portal vein after OLT has been reported by Lerut et al. (5) in seven of 393 hepatic grafts. The contributing factors in five of the seven patients were size mismatch, recipient portal vein thrombosis, and recipient portal vein hypoplasia. In the patient reported herein, the pre-existing end-to-side portacaval shunt may have predisposed the portal vein to thrombose. Thrombosis of the portal anastomosis is usually fol-lowed by the development of collateral circulation, as first described by Hahn et al. (10). Such collaterals develop secondary to the high pressure in the extrahe-patic portal venous system and, essentially, are of two types: portal-portal or hepatopetal, and portal-systemic or hepatofugal (I 1). Although some authors do not believe that hepatopetal collaterais that develop after portal vein thrombosis could be of any importance in maintaining good hepatic perfusion (12, 13), others have demonstrated that such collateral circulation is January 1989 SPLENORENAL SHUNT IN LIVER TRANSPLANT A nON 69 important in reestablishing portal flow to the liver (11, 14). In nontransplant patients with extrahepatic portal vein obstruction, the normal sinusoidal pressure is con-sidered to be fundamental in favoring a periportal collateral pathway around the obstructed vein to the liver parenchyma (15). The development of a hepato-petal collateral circulation results from the presence of many small collateral veins around the extrahepatic portal vein and within the hepatoduodenal and hepa-tocolic ligaments. These small vessels enlarge because of a high pressure gradient between the proximal throm-bosed extrahepatic portal vein, and the intrahepatic portal vein and sinusoidal system. The development of he pat ope tal collaterals after por-tal vein thrombosis is clearly understandable in a native liver, but is more difficult to explain in a liver allograft. Thus, because all potential portal collaterals are divided during harvesting of the liver, the only connection between the splanchnic circulation and the allograft liver is via the portal anastomosis. Apparently, collat-erals develop between the recipient extrahepatic portal segment and the allograft liver hilum, thus becoming the anatomical pathway for a possible hepatopetal col-lateral circulation. In our case, the blood flow through these new collaterals was sufficient to perfuse the allo-graft liver with the necessary hepatotrophic portal fac-tors for a normal hepatic physiology, but not sufficient to maintain normal portal pressure. Consequently, por-tal hypertension developed and led to esophageal var-ices and severe gastrointestinal tract bleeding. In this setting, the therapeutic choices are sclerotherapy, re-transplantation, or portal diversion surgery. Sclerotherapy is a good method to use to control acute variceal bleeding, but it is not always effective in permanent control of gastroesophageal bleeding and in the treatment of gastric varices. In the latter condition, the persistent hypertension in the gastric veins repre-sents a life-threatening situation, since bleeding from erosive gastritis is a leading cause of death in this condition (16). As demonstrated in our patient, re-peated sclerotherapy was ineffective in achieving defin-itive control of bleeding. Retransplantation is a therapeutic option practiced routinely at our institution, rescuing those patients whose primary grafts are failing for primary nonfunc-tion, intractable rejection, or technical complications (17). In our case, retransplantation was not performed because graft function remained excellent and its his-tologic architecture was nornlal. The final therapeutic option, portasystemic shunting, would hopefully eliminate the recurrent bleeding epi-sodes. The use of a selective shunting procedure might allow control of bleeding while preserving the necessary high pressure in the intestinal venous system. To main-tain collateral portal perfusion with hepatotrophic por-tal factors (16, 18), the distal splenorenal shunt was physiologically the most ideal shunt for the purpose just outlined. Further, the alternative mesocaval shunt was not selected because potential complications with this particular portosystemic diversion could lead to thrombosis of the superior mesenteric vein and subse-quent loss of the hepatic allograft. In the distal spleno-renal shunt, the surgical dissection is away from the hepatic hilum, which minimizes the risk of jeopardizing a future second transplant if one is needed (19). The long-term patency of the shunt without any recurrence of bleeding, and the excellent quality of liver function 5 yr posttransplantation and 4 years after portal shunt-ing, clearly demonstrate the reliability of this therapeu-tic choice. ACKNOWLEDGMENTS Supported by research grants from the Veterans Ad-ministration and by Project Grant AM 29961 from the National Institutes of Health, Bethesda, Maryland. Reprint requests: Carlos O. Esquivel, M.D., Pacific Presbyterian Medical Center, P.O. Box 7999, San Francisco, CA 94120. REFERENCES I. Esquivel CO, Marino IR, Iwatsuki S, et al. Long-term results of hepatic transplantation during the cyclosporine era: The Pitts-burgh experience. In: Touraine JL, Traeger J, Betuel H, et aI., eds. Transplantation and clinical immunology. XIX. The long-term transplant patient. Amsterdam: Elsevier, 1987: 185-96. 2. Gordon RD, Makowka L, Bronsther OL, et aI. Complications of liver transplantation. In: Toledo-Pereyra LH, ed. Complications of organ transplantation. New York: Marcel Dekker, 1987:329-54. 3. Tzakis AG, Gordon RD, Shaw B Jr, et al. Clinical presentation of hepatic artery thrombosis after liver transplantation in the cyclosporine era. Transplantation 1985;4:667-71. 4. Lerut J, Gordon RD, Iwatsuki S, et aI. Biliary tract complications in human orthotopic liver transplantation. Transplantation 1987;43:47-50. 5. Lerut J, Tzakis AG, Bron K, et aI. Complications of venous reconstruction in human orthotopic liver transplantation. Ann Surg 1983;205:409-14. 6. Starzl TE, Putnam CW, Porter KA, et aI. Portal diversion for the treatment of glycogen storage disease in humans. Ann Surg 1973; 178:525-39. 7. Greene HLT, Slonim AE, Burr 1M: Type I glycogen storage disease: A metabolic basis for advances in treatment. Pediatrics 1979;26:63-92. 8. Starzl TE, Iwatsuki S, Van Thiel DH, et al. Evolution of liver transplantation. Hepatology 1982;2:614-36. 9. Malatack JJ, Finegold ON, Iwatsuki S, et al. Liver transplantation for type I glycogen storage disease. Lancet 1983;1073-5. 10. Hahn M, Massen 0, Necki M, et al. Die Eck'sche Fistel zwischen der unteren Hohlvene und der pfortader und ihre Foigen fuer den Organismus. Arch Exp Pathol PharrnakoI1983;32:162-21O. II. Zajko AB, Bron KM: Hepatopeta! collaterals after portal vein thrombosis following liver transplantation. Cardiovasc Intervent Radiol 1986;9:46-8. 12. Voorhees AB, Chaitman E, Schneider S, et al. Portal-systemic encephalopathy in the noncirrhotic patient. Effect of portal-70 MARINO et al. systemic shunting. Arch Surg 1973;107:659-63. 13. Graver SE, Schwartz SI. Extrahepatic portal hypertension: A retrospective analysis. Ann Surg 1979; 189:566-74. 14. Warren DW, Millikan WJ Jr, Smith RB III, et al. Noncirrhotic portal vein thrombosis. Physiology before and after shunts. Ann Surg 1980;192:341-9. 15. Henderson JM, Warren WD. Surgical complications of cirrhosis and portal hypertension. In: Sabiston DC, ed. Textbooks of surgery. Philadelphia: W. B. Saunders, 1986:1095-116. 16. Warren WD. Control of variceal bleeding: Reassessment of ra-Vol. 84, No.1, 1989 tionale. Am J Surg 1983;145:8-16. 17. Shaw BW, Gordon RD, Iwatsuki S, et al. Retransplantation of the liver. Semin Liver Dis 1985;5:394-401. 18. StaTZI TE. Judd lecture: Portal hepatotrophic factors: A century of controversy. In: Najarian JS, Delaney JP, eds. Surgery of the liver, pancreas, and biliary tract and pancreas. New York: Inter-continental Medical Book Corporation, 1975:525-38. 19. Esquivel CO, Klintmalm JG, Iwatsuki S, et al. Liver transplan-tation in patients with patent splenorenal shunts. Surgery 1987; 100:705-15. 

Distal Splenorenal Shunt for Portal Vein Thrombosis after Liver Transplantation

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Distal Splenorenal Shunt for Portal Vein Thrombosis after Liver Transplantation

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