Orthotopic, but Reversed Implantation of the Liver Allograft in Situs Inversus Totalis A Simple New Approach to a Difficult Problem

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1 American Journal of Transplantation 2009; 9: Wiley Periodicals Inc. Case Report C 2009 The Authors Journal compilation C 2009 The American Society of Transplantation and the American Society of Transplant Surgeons doi: /j x Orthotopic, but Reversed Implantation of the Liver Allograft in Situs Inversus Totalis A Simple New Approach to a Difficult Problem S. C. Rayhill a,,d.scott a,s.orloff a,j.-l.horn b, J. Schwartz c, A. Zaman c, A. Sasaki c, W. S. Naugler c, M. Chang c, J. Gaumond a, Y. Wu d and J. Ham a a Division of Liver and Pancreas Transplantation, Department of Surgery, b Department of Anesthesiology, c Division of Hepatology, Department of Medicine, OHSU, Portland, OR and d Division of Transplantation, Department of Surgery, University of Arkansas, Little Rock, AR Corresponding author: Stephen Corrigan Rayhill, rayhills@ohsu.edu Situs inversus totalis is a rare congenital anomaly in which the heart and abdominal organs are oriented in a mirror image of normal. It provides a unique challenge as there is no established technique for liver transplantation in these patients. Employing two major alterations from our standard technique, a liver was transplanted in the left subphrenic space of a patient with situs inversus totalis. First, the liver was flipped 180 from right to left (facing backward). Second, a reversed cavaplasty (anterior, not posterior, donor suprahepatic caval incision) was performed. Otherwise, it was standard, with end-to-end anastomoses of the portal vein, hepatic artery and bile duct. Three years after the entirely uneventful transplant, the recipient continues to enjoy the benefits of a normally functioning liver. The described technique prevented torsion, kinking and tension on the anastomosed structures by allowing the liver to sit naturally in an anatomical position in the left hepatic fossa. As it required no special measurements or maneuvers, the technique was easy to execute and required no donor liver size restrictions. This novel technique, with a reversed cavaplasty and a 180 right-to-left flip of the liver into a left-sided hepatic fossa, may be ideal for situs inversus totalis. Key words: Cavaplasty, liver transplantation, situs inversus Received 10 November 2008, revised 26 February 2009 and accepted for publication 20 March 2009 Introduction Patientswithsitus inversus totalis present unique technical challenges for liver transplantation. In this rare congenital anomaly, the thoracic and abdominal organs are oriented in a mirror image of the normal anatomical location. Thus, along with dextrocardia, the liver, portal structures, and the vena cava are located on the left side of the patient. The organs themselves lie in reversed left-to-right orientation with the right lobe equivalent of the liver (segments 5 8) located laterally on the left side in an empty left subphrenic space (the stomach and spleen are on the right). The problem for liver transplantation is that, due to the rarity of situs inversus (1 in 4000 to births), donor livers are almost always right sided (1). As in the steric biological constraints of an opposite handed enantiomer, the normal right-sided donor liver does not fit naturally into the left side of the abdomen of a patient with situs inversus. Most cases of transplantation for situs inversus have been performed in children. This is because biliary atresia, a cause of end-stage liver disease in children, is commonly associated with situs inversus (it occurs in 10 20% of children with situs inversus). Otherwise, patients with situs inversus are not at an increased risk for the development of liver disease (2). Thus, liver transplantation in adults with situs inversus is rare. Although situs inversus was considered to be an absolute contraindication to liver transplantation, several surgical solutions have been proposed and successfully performed (1 9). However, due to the infrequent occurrence of this problem and the complexity of the situation, there remains no accepted approach to liver transplantation in patients with situs inversus, especially in adult patients. Ideally, a liver allograft placed in a patient with situs inversus totalis would be situated such that the large right lobe of the donor liver would rest in the capacious left subphrenic space of the recipient (i.e. in their hepatic fossa). In this arrangement, the convex posterior surface of the right lobe of the donor liver would lie in the correspondingly concave left subphrenic space. Also ideally, such placement would maintain the lateral orientation of the common bile duct, albeit on the left, so that there would be no crossing of the 1602

2 Liver Recipient with Situs Inversus Totalis portal structures. Herein, we describe a novel approach that achieves both of these ideals. Materials and Methods Recipient and donor characteristics A 53-year-old woman with situs inversus totalis and end-stage liver disease due to primary biliary cirrhosis presented for evaluation for liver transplantation in Besides situs inversus totalis, the only other unusual anatomical finding was an accessory spleen. Her past medical history was remarkable only for having had a laparoscopic cholecystectomy. At 5 feet 5 inches and 135 lbs (BMI 22 kg/m 2 ), she was a fit, but profoundly jaundiced woman. A CT scan confirmed complete situs inversus with dextrocardia and the absence of hepatocellular cancer. Her vascular structures were otherwise normal (there can often be vena cava abnormalities with situs inversus), with standard hepatic arterial anatomy (4). The donor was a 5 feet 1 inch 112 lb 49-year-old woman who had suffered head trauma. The operation In October 2005, with a MELD score of 23, the patient underwent an uneventful liver transplant. In addition to a middle hepatic artery, the donor liver had both a replaced (or accessory) right hepatic artery and a replaced (or accessory) left hepatic artery. To prepare the donor liver for a single arterial anastomosis, an oval Carrel patch on the replaced right hepatic artery was anastomosed onto an obliquely cut donor splenic artery. The vena cava was prepared for cavaplasty (10). A standard bilateral subcostal incision with an upper midline extension was performed. An abdominal survey confirmed the presence of complete situs inversus with dextrocardia (Figure 1). The liver was extremely cirrhotic and shrunken, with the deeply bilious appearance typical of primary biliary cirrhosis. Massive portal collaterals could be seen throughout the abdomen and on the inner abdominal wall where innumerable small collateral vessels produced an unusual purple hue. There were several large, bile stained lymph nodes. Figure 1: The left-sided native liver in situs inversus totalis. Note the stomach crossing from the right subphrenic space. in the recipient vena cava was begun at the inferior edge of the right-sided left and middle hepatic vein trunk equivalent (chosen because it was situated more rightward, facilitating the anastomoses and providing a more natural lie), although a typical incision incorporating all three veins could have been used. The allograft was brought into the field, flipped from right to left, and beginning with the back wall, a broad-based, tension-free, 5 cm long cavaplasty anastomosis was constructed with ease (Figures 2B and C). After shortening the donor portal vein to an appropriate length, a standard, tension-free portal vein anastomosis was performed (Figure 3). The cross After preparing the porta hepatis, veno-venous bypass was instituted (both percutaneous peripheral and portal) to allow for unencumbered time for the evaluation of an ideal approach for implantation. The vena cava was left intact. The left-sided right hepatic vein equivalent was ligated and divided with a roticulating vascular stapler (2.0 mm), and the hepatectomy was performed leaving the left and middle hepatic vein trunk equivalent open (Figure 2A). To test various possible positions for implantation, the donor liver was temporarily brought into the field. Several approaches were considered, including a 90 left lateral rotation of the liver (2,9), a left-shifted, orthotopic position (1,3,5 8) and the present new technique, with the liver flipped 180 from right to left (facing backward). With the new orientation, the right lobe of the donor liver fell naturally into the empty left subphrenic space of the recipient (her hepatic fossa) and the bile duct, hepatic artery and portal vein, although emerging more anteriorly than usual, lay correctly in the sagittal plane (i.e. both bile ducts were lateral) with ample length for tension-free anastomoses. As it appeared to yield a simple and safe approach, quite similar to that of our standard technique, this orientation was chosen. The cavaplasty was modified to accommodate the backward orientation of the liver by making the incision in the anterior (instead of posterior) wall of the upper cuff of the donor vena cava (10). The maximal length incision (approaching the junction with the liver) was made. A corresponding incision Figure 2: (A) The liver has been removed. Note an intact vena cava with an open right-sided left and middle hepatic vein trunk equivalent and a wide-open left subphrenic space (the left hepatic fossa). The portal bypass cannula is visible in the portal vein (PV). (B) The cavaplasty with an anterior incision in the upper cuff of the donor vena cava. The back wall is being anastomosed from the inside. (C) The completed, tension-free, reversed cavaplasty. American Journal of Transplantation 2009; 9:

3 Rayhill et al. Figure 3: The portal vein (PV) anastomosis (in progress). Note the lack of tension on the vein. The recipient hepatic artery (HA) trunk is also visible. T-tube or a stent. With excellent hemostasis, strong Doppler signals and a soft, healthy appearing liver, the abdomen was closed. The liver off-ice to portal flow time (warm ischemic or anastomotic time) was 34 min and the total operative time was 5 h and 29 min. Blood loss was minimal. Figure 2: Continued. clamps were removed, terminating an uneventful anhepatic phase. With minimal bleeding, venovenous bypass was promptly removed. Despite facing somewhat more anteriorly than usual, the hepatic artery and bile duct were easily reapproximated without tension (Figure 4). A continuous end-to-end biliary anastomosis was performed without employing a Figure 4: The completed hepatic artery (HA), portal vein (PV) and bile duct (BD) anastomoses. Note that all three structures sit naturally, without crossing. Above is the ligated lower inferior vena cava (IVC) American Journal of Transplantation 2009; 9:

4 Liver Recipient with Situs Inversus Totalis To date, few cases of liver transplantation in patients with situs inversus have been reported in the literature and most were in children. Despite the small number of cases reported, numerous different techniques have been described. Thus, there remains no established surgical approach to liver transplantation in patients with this rare anomaly. Figure 5: A diagram of the orthotopic, but reversed, liver transplant. Results Preoperatively, the patient had child s C cirrhosis with a MELD score of 23. The transplant and postoperative recovery were entirely uneventful. With consistently normal hemodynamics and urine output, no pharmacological support was required. The maximum AST and ALT were 303 and 152 IU/I, and the bilirubin dropped from 16 to 2.5 mg/dl by postoperative day 2. She was discharged home in excellent condition on postoperative day 7. Three years following transplantation, having suffered no complications, rejection, or recurrent disease, she continues to do well, enjoying the benefits of a normally functioning liver. Her laboratory values remain normal. Discussion This report describes an excellent outcome using a simple new technique for liver transplantation in patients with situs inversus totalis (Figure 5). With this technique, the normal donor liver is simply flipped 180 from right to left (facing backward), but is otherwise transplanted in the usual fashion. The only other modification from our standard transplantation technique (10) was to make an anterior (instead of posterior) incision in the upper donor vena cava, creating a reversed cavaplasty. As it required no special maneuvers and imposed no donor liver size restrictions, this technique was easy to execute. The first report on liver transplantation in patients with situs inversus described an orthotopic placement of a normal donor liver in a 6-year old with situs inversus without dextrocardia (3). Subsequently, Wente et al. described a similar approach in an adult with situs inversus totalis (1). The donor liver was transplanted in the usual orientation and fashion, but shifted to the left to accommodate the left-sided location of the recipient s portal structures. In addition to a side-to-side caval anastomosis, the portal structures, including the bile duct, were anastomosed end-to-end (although, of necessity because of the anomalous anatomy, crossing one another). Despite theoretical concerns, there were no problems due to the large right lobe overlying the spine. Tucker et al. also transplanted a patient using this orientation for the donor liver (4). In this case, a standard piggyback technique was used. However, due to a 40 rotation of the liver caused by impingement from the recipient s stomach and spleen, the liver had to be stabilized by plication of the left diaphragm and placement of a Sengstaken Blakemore tube (filled with 400 cc of saline) in the left subphrenic space. Watson, Barone, Heimbach (using a living donor right lobe), and Braun have also reported the successful use of this left-shifted approach, all employing a choledochojejeunostomy (5 8). A quite different approach was described by Klintmalm et al., who positioned the liver in the left-upper quadrant and rotated it 90 clockwise to the left (9). The lower donor vena cava was anastomosed perpendicularly to the lateral wall of the recipient inferior vena cava (the superior cava was over-sewn). In this orientation, the donor right lobe was located (somewhat awkwardly) in the left subphrenic space. The bile duct (reconstructed over a T-tube) crossed anterior to the portal vein and the hepatic artery was swung underneath it. Hoyos et al. also successfully employed a similar technique (2). The theoretical risk associated with this technique is kinking of the portal structures and torsion of the venous outflow if the liver were to swing out of place; however, no such problem has been reported. A technique similar to the present technique has recently been described for the transplantation of a liver from a donor with situs inversus totalis into an anatomically normal recipient. As with the present technique, the liver was flipped 180 (from left to right) and inplanted backward (11). The present technique for liver transplantation in a patient with situs inversus and dextrocardia (situs inversus totalis) may be the ideal technique, as it results in an orthotopically placed liver transplant sited naturally in an anatomical position in the appropriate, but opposite-sided, hepatic fossa. Not only are the portal structures not crossed, but also as the liver sits easily in the hepatic fossa and has a large cavaplasty anchoring it, there is minimal risk of rotation with consequent kinking of structures. Unlike the American Journal of Transplantation 2009; 9:

5 Rayhill et al. non-flipped approach for orthotopic placement, it does not lie over the spine and is not pushed laterally by the rightsided stomach and spleen. With the present technique, despite dextrocardia, the donor and recipient vena cavas meld naturally into one another. As the portal structures are handled in a fashion identical to a standard right-sided liver transplant, there is no need for routine vascular grafting or use of a roux en Y choledochojejeunostomy. Thus, there is little risk of torsion, kinking or tension on the anastomosed structures with this new technique. In summary, the present report describes a simple new technique for liver transplantation in recipients with situs inversus totalis. The principle innovations involve flipping the liver from right to left for backward implantation and the use of an anterior incision in the donor vena cava for the cavaplasty. As this technique requires no special measurements or maneuvers and is easy to execute, we propose that other liver transplant centers may want to consider adopting this approach to liver transplantation in recipients with situs inversus. Acknowledgments We would like to thank our dedicated transplant team: coordinators Kami Faulkner, Jay Anne Nussbaum, Jill Salisbury, Kelly Sparks, Judy Storfjell, Tom Swanson and Sharlene Winters; office team members Bonnie Brown, Jennifer Galos, Katie Herzog and Sarah Prinslow; and administrative team members led by Mike Seely, Tim Stevens and Michelle Bechtholdt. In addition, we would like to thank Ginny McDonald and Katie Wasinger and their team for their invaluable help in the operating room. We would like to thank Maren Maggio for her great work with the manuscript. References 1. Wente MN, Thorn M, Radeleff B et al. A routine liver transplantation in a patient with situs inversus: A case reportand an overview of the literature. Clin Transplant 2006; 20: Hoyos S, Guzman C, Correa G, Restrepo JC, Franco H, Cardenas A. Orthotopic liver transplantation in an adult with situs inversus: An easy way to fit the liver. Ann Hepatol 2006; 5: Raynor SC, Wood RP, Spanta AD, Shaw BW Jr. Liver transplantation in a patient with situs inversus. Transplantation 1988; 45: Tucker O, Prachalias A, Kane P, Rela M. Graft positioning at liver transplantation in situs inversus. Liver Transpl 2006; 12: Watson CJ, Rasmussen A, Jamieson NV et al. Liver transplantation in patients with situs inversus. Br J Surg 1995; 82: Barone GW, Henry ML, Elkhammas EA, Tesi RJ, Ferguson RM. Orthotopic liver transplantation with abdominal situs inversus and dextrocardia. Am Surg 1992; 58: Heimbach JK, Menon KV, Ishitani MB et al. Living donor liver transplantation using a right lobe graft in an adult with situs inversus. Liver Transpl 2005; 11: Braun F, Rodeck B, Lorf T et al. Situs inversus of donor or recipient in liver transplantation. Transpl Int 1998; 11: Klintmalm GB, Bell M, Husberg BS et al. Liver transplant in complete situs inversus: A case report. Surgery 1993; 114: Wu YM, Voigt M, Rayhill S et al. Suprahepatic venacavaplasty (cavaplasty) with retrohepatic cava extension in liver transplantation: Experience with first 115 cases. Transplantation 2001; 72: Pomposelli JJ, DaCosta MA, McPartland K, Jenkins RL. Retroversus implantation of a liver graft: A novel approach to the deceased donor with situs inversus totalis. Am J Transplant 2007; 7: American Journal of Transplantation 2009; 9: