An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. End-to-end anastomosis of the recipient suprarenal vena cava to the graft portal vein (a left lateral section from the patient’s mother) was performed. An end-to-side portocaval shunt with the recipient portal vein was constructed to mitigate portal hypertension. The early postoperative course was relatively uneventful. However, persistent hepatitis caused by infection with Cytomegalovirus and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, a living-donor retransplantation (a left lateral section from the patient’s grandmother) was performed. One month after retransplantation, severe acute rejection that eventually required OKT3 treatment developed. The patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed. Cavoportal hemitransposition should be included in the armamentarium of the transplant surgeon for the management of extensive portal system thrombosis and portal vein hypoplasia. An additional shunt may be useful in mitigating portal hypertension.

Key words : Liver, Transplantation, Retransplantation, Living-donor transplantation, Cavoportal hemitransposition

Portal vein thrombosis and portal vein hypoplasia in liver transplant recipients can be usually managed by various techniques such as eversion thrombectomy or the use of jump grafts for inflow from the superior mesenteric vein (SMV) or a suitable collateral vein [1, 2]. In some patients, however, none of those options is viable, and a rare procedure such as cavoportal hemitransposition (CPHT) is required.

Cavoportal transposition is the transection of the infrahepatic vena cava and the portal vein and switched reanastomosis of the vessels. In that procedure, an anastomosis is created between the lower stump of the portal vein and the upper stump of the infrahepatic vena cava and also between the lower stump of the vena cava and the upper stump of the portal vein. Originally devised by Child and colleagues [3] for use in experimental investigations, cavoportal transposition was used by Starzl and colleagues [4] to treat glycogen storage disease, and treatment in the first 2 patients was successful. Unfortunately, the third patient so treated died of overperfusion of the liver and resultant hepatic failure [4]. More recently, Tzakis and colleagues reported the use of a modified procedure for transplantation in patients with extensive portal vein thrombosis: the use of the vena cava to provide portal inflow to the graft (hence the term “hemitransposition”) [5]. The decision to use that technique as a salvage procedure is based on unexpected operative findings or retransplantation necessitated by portal thrombosis; it is also a primary option in selected patients. Generally favorable results have been achieved with the use of CPHT [6-19] (Tables 1 and 2).

We report the case of a female infant who underwent living-related liver transplantation with CPHT and successful retransplantation 9 months later for persistent hepatitis caused by Cytomegalovirus (CMV) infection and chronic rejection. The pertinent literature on CPHT is also reviewed. Patients with renoportal anastomosis (transection of the caval end of the renal vein and anastomosis to the graft portal vein) were excluded from this study.

Case Report

An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. She had no varices. A left lateral section graft (240 g; graft weight/body weight, 2.6%) was obtained from the patient’s mother. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. Because there was no suitable collateral vein for anastomosis, the decision to perform CPHT was made. The graft hepatic vein was anastomosed to the common orifice of the recipient middle and left hepatic veins. The suprarenal vena cava was clamped and transected. The inferior end was used to provide inflow to the portal vein of the graft. The superior end was sutured. An end-to-side portocaval shunt with the hypoplastic recipient portal vein was constructed to mitigate portal hypertension. The graft hepatic artery was anastomosed to the right hepatic artery. Biliary reconstruction was performed with a Roux loop. The early postoperative course was relatively uneventful. The patient demonstrated excellent renal function and no edema of the lower trunk. The results of computed tomographic angiography 6 weeks after the operation showed a patent cavoportal anastomosis and a patent portocaval shunt. Similar findings were obtained from an examination performed 6 months after transplantation. The portal vein enlarged over time (Figures 1 and 2). However, persistent hepatitis caused by CMV infection and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, living-donor retransplantation (a left lateral section from the patient’s grandmother; 234 g; graft weight/body weight, 2.2%) was performed. Inflow to the graft portal vein was provided by transecting the prior anastomosis between the recipient vena cava and the graft portal vein and anastomosing the vena cava to the new graft portal. The recipient portal vein had enlarged to approximately 1 cm in diameter, and the patent portocaval shunt prevented splanchnic congestion. No prominent retroperitoneal collateral vessels were observed. One month after retransplantation, severe acute rejection developed and was eventually treated with OKT3 therapy. This patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed.

Discussion

A review of the literature revealed that CPHT is an effective method for circumventing extensive portal system thrombosis and portal vein hypoplasia in patients who have undergone liver transplantation. CPHT may be performed in either of 2 ways: 1) by partial or total ligation of the suprarenal vena cava and anastomosis of the graft portal vein to the lower part of the vena cava directly or with vein graft interposition or 2) by transection of the retrohepatic vena cava and direct anastomosis of the lower part to the graft portal vein [5].

The major drawback of either procedure is that complications (primarily variceal bleeding and ascites) caused by unrelieved portal hypertension usually develop (Tables 1 and 2). Spontaneous shunts or surgical shunts constructed in prior operations or during transplantation may exert a protective effect against those complications. Anastomosis of the hypoplastic portal vein to the inferior vena cava (as performed in the patient described in this report) and even makeshift shunts with retroperitoneal collateral vessels appear to be useful [11, 13]. Pinna and colleagues proposed prophylactic splenectomy and gastric devascularization to prevent variceal bleeding [9]. The patient in our report had no varices but did have a hypoplastic portal vein; the collateral vessels carried enough blood to the caval system. She experienced no variceal bleeding. Usually, the medical control of ascites that develops after CPHT has been effective [9].

However, forcing all inferior vena caval flow through the liver may result in the small-for-size syndrome with associated hyperperfusion. The third patient who underwent cavoportal transposition for glycogen storage disease died from that complication [4]. Primary nonfunction (PNF) was encountered only in the initial 4-center report by Tzakis and colleagues [5] in 2 of the 9 patients studied (1 demonstrated PNF and the other exhibited primary dysfunction [PDF]) but in no subsequent patient until the 2006 report by Lipshutz and colleagues, which documented PNF in 3 of 7 study subjects [19]. In the report by Tzakis and colleagues [5], both patients with PNF underwent salvage retransplantation, 1 for thrombosis of the vein graft that was anastomosed to the SMV, and the other because the hypoplastic portal vein did not sustain the graft. The first of those 2 patients died of PNF and sepsis on the second postoperative day, and the other (who had PDF) died 4 weeks after surgery. Both of those recipients were severely ill, but graft overperfusion was not observed by the authors. Although Lipshutz and colleagues [19] ascribed the problem specifically to overperfusion (3 elective cases), the association is not readily apparent because the graft-to-recipient body weight ratios were 4, 5.1, and 6%, respectively. Two of the grafts in their study were in situ split left lateral section grafts, and 1 was a whole organ graft. Although the corresponding right trisection grafts functioned in the patients in that study, disparate function of split liver grafts has been reported; ie, 1 of the split grafts may demonstrate excellent function and the other may be lost as a result of primary nonfunction [20]. The unexplained PNF of grafts after CPHT remains to be elucidated.

Another hemodynamic implication of CPHT is that caval blood must pass through a sieve-like barrier (the liver) instead of the retrohepatic vena cava, which provides almost no resistance to flow. Complications such as renal dysfunction, lower-body edema, and an increased tendency for the development of deep venous thrombosis (DVT) have been attributed to this factor [5, 8, 9]. Although renal failure has been reported to require treatment with hemodialysis [10, 12], medical measures are usually adequate for the management of transient renal dysfunction and fluid retention [9]. Prophylactic anticoagulation against DVT has been recommended [8]. Our standard protocol for pediatric living-donor liver transplantation includes heparin perfusion followed by low-molecular-weight heparin and dipyridamole as prophylaxis against hepatic artery thrombosis. This regimen may be effective against DVT as well. DVT in a patient with cavoportal hemitransposition may lead to unusual complications such as saddle embolus of the portal vein from the lower extremity (ie, hepatic instead of pulmonary embolism) [6, 15].

In summary, CPHT is a very effective procedure when operative findings (extensive portal vein thrombosis and portal vein hypoplasia) or postoperative complications (extensive portal thrombosis) render other more conventional techniques useless. The complications of unrelieved portal hypertension are less severe or nonexistent in patients who have undergone previous procedures against variceal bleeding, those with healthy collateral vessels (no varices), and those who have undergone a simultaneous shunt or devascularization procedure. In other patients, endoscopic procedures appear to achieve adequate control of bleeding. Because the published experience with CPHT is still limited and the long-term consequences of undergoing that procedure are unknown, further data are required to establish the use of that procedure in recipients of a primary, elective, cadaveric, or living-donor [21] liver transplant.

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