Retroversus Implantation of a Liver Graft: A Novel Approach to the Deceased Donor with Situs Inversus Totalis

Situs inversus totalis is a rare anatomical abnormality that results in dextrocardia, mirror image of normal abdominal organs and other congenital abnormalities. Deceased donors with this condition are often declined on anatomic concerns. While there have been numerous reports of successful liver transplantation in recipients with situs inversus, review of the world's literature provided only three case reports using deceased donors with situs inversus. In this report, a novel approach to implantation of a liver graft from a donor with situs inversus is presented. To avoid possible torsion and blockage of venous outflow, a modified retroversus piggyback technique with 180 degrees ventral caudal (backwards) rotation of the liver graft along the axis of the vena cava was performed. This orientation resulted in the retro hepatic vena cava facing anteriorly and the larger anatomic liver lobe in the right upper quadrant. Excellent outcome was achieved without technical difficulty. Retroversus implantation of a liver graft from a donor with situs inversus is safe and effective and associated with favorable outcome.     

Liver transplantation in patients with situs inversus

Situs inversus has been considered an absolute contraindication to liver transplantation due to technical difficulties. Associated vascular malformation and distorted anatomy may make the procedure even more complicated or impossible. Only three cases of patients with abdominal situs inversus who underwent successful liver transplantation have been reported in the English literature. We describe two additional patients with situs inversus who suffered from biliary atresia and underwent successful liver transplantation. The preoperative evaluation and the operative procedure are presented, and technical difficulties are discussed. Since biliary atresia is associated with polysplenia syndrome, including vascular malformation and visceral malposition, we suggest that each case be extensively evaluated preoperatively to determine the size requirement for the donor liver and the feasibility of reconstruction.     

Living-donor liver transplantation for situs inversus: 2 case reports

Two cases of living-donor liver transplantation performed in patients with situs inversus are reported. The authors discuss the operative management for a situs inversus recipient to undergo liver transplantation.     

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

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 .     

Case of living donor liver transplantation in a patient with biliary atresia combined with situs inversus

Until recently, situs inversus was considered to be an absolute contraindication for liver transplantation. However, recent reports have suggested that situs inversus should not be considered a contraindication. This study presents a successful living donor liver transplantation performed in a 4-month-old male infant with biliary atresia and situs inversus. The surgical findings revealed abdominal situs inversus with polysplenia and an absent retrohepatic inferior vena cava and intestinal malrotation.     

Orthotopic liver transplantation with abdominal situs inversus and dextrocardia.

Anatomic anomalies may present technical difficulties during orthotopic liver transplantation. Abdominal situs inversus was considered a contraindication to liver transplantation. A successful liver transplant in a patient with both situs inversus and dextrocardia is described, along with a technical review.     

Situs Inversus Totalis with Malignant Lymphoma of the Stomach: Report of a Case

Situs inversus totalis is a rare congenital anomaly that often occurs concomitantly with other disorders. We report a case of situs inversus totalis with malignant lymphoma of the stomach, which was successfully treated by surgery followed by chemotherapy and irradiation. The patient was a 51-year-old woman who present with colicky pain in the left upper quadrant of her abdomen. Chest X-ray showed a right-sided heart, and ultrasonography and computed tomography (CT) of the abdomen showed a situs inversus totalis with multiple gallstones in the gallbladder. Tree-dimensional reconstructed CT of the abdomen showed no other malformations coexisting with situs inversus totalis, but a barium upper gastrointestinal series found an inverted stomach and an elevated tumor with ulceration in the center, localized in the antrum of the stomach. First, we performed a cholecystectomy, followed by a total gastrectomy with dissection of the lymph nodes and splenectomy, and Roux-en-Y reconstruction. Histopathological examination confirmed a diagnosis of malignant lymphoma of the stomach (diffuse large B-cell type) with metastasis to the regional lymph nodes. Chemotherapy using the CHOP regimen was given three times, starting 1 month postoperatively. A follow-up CT scan showed enlargement of one lymph node around the abdominal aorta and irradiation was delivered to the area of the inverted Y in the abdomen. At the time of writing, 10 months after surgery, the patient is well with no signs of recurrence and leading a normal life. Careful preoperative assessment is very important for determining the most appropriate surgical procedure in patients with situs inversus totalis associated with a malignancy. Received: April 25, 2002 / Accepted: November 19, 2002 RID="*" ID="*" Reprint requests to: S. Murakami     

Evaluation, operative management, and outcome after liver transplantation in children with biliary atresia and situs inversus.

INTRODUCTION: Biliary atresia, a common indication for liver transplantation, can be associated with situs inversus. Our experience with liver transplantation in children (n = 6) was reviewed retrospectively. PATIENTS AND METHODS: Preoperative duplex sonography, computerized tomography, and visceral angiography were obtained. Vascular anomalies identified included preduodenal portal vein (6/6), interrupted inferior vena cava (5/6), and aberrant hepatic artery (4/6). RESULTS: The liver graft was placed in a midline position. Venous continuity was achieved by donor suprahepatic inferior vena cava to recipient hepatic cloaca and direct end-to-end portal anastomosis. The donor infrahepatic inferior vena cava was oversewn. Arterial continuity was restored using either a direct branch-patch anastomosis (3/6) or a supraceliac aortic interposition graft (3/6). In retrospect, preoperative diagnostic work-up was noncontributory and outcome was not complicated by pre-existing situs inversus. CONCLUSION: Situs inversus in liver recipients requires operative technical modifications, but does not change outcome. Furthermore, extensive preoperative work-up should be avoided.     

A routine liver transplantation in a patient with situs inversus: a case report and an overview of the literature

Liver transplantation (LT) in an adult with situs inversus (SI) is extremely rare and requires precise pre-operative management. A 48-yr-old male with SI suffering from alcoholic liver cirrhosis underwent LT at our institution in March 2003. Pre-operatively, liver anatomy was determined by CT scan, three-dimensional liver reconstruction and angiography. LT was performed using the Belghiti technique with side-to-side cavo-caval anastomosis, transplanting a graft from a donor with normal anatomy. Post-operatively, the patient recovered without major complications, except an epileptic event because of a central pontine myelinolysis, and he was discharged on the 25th post-operative day. Three months after surgery, the T-drain placed intra-operatively into the donor bile duct was removed; transplant perfusion and function were stable with an actual follow-up period of 24 months. LT in patients with SI is feasible. Pre-operative imaging with three-dimensional reconstruction is a beneficial tool for operation planning in patients with rare anatomic variations.     

First orthotopic liver transplantation in patient with biliary atresia and situs inversus in spain

Biliary atresia is the most common indication for liver transplantation in the pediatric age group. The Kasai portoenterostomy has become established as the primary treatment for biliary atresia. If portoenterostomy fails, death before 2 years of age is likely without liver transplantation. The most common multiple malformation syndrome associated with biliary atresia is polysplenia syndrome, which forms a constellation of defects of body symmetry, splenic development and vascular anomalies, including situs inversus, polysplenia and others. The situs inversus was formerly considered an absolute contraindication for liver transplantation. Recently however, several case reports have been published suggesting that neither situs inversus nor this particular subset of vascular abnormalities should be considered contraindications to liver transplantation. We present one case of liver transplantation performed in patient with biliary atresia, situs inversus and polysplenia. This is the first report described in Spain for a liver transplant in a child with biliary atresia plus situs inversus.     

Hepatic Resection for Metastatic Colon Cancer in Patients with Situs Inversus Totalis Complicated by Multiple Anomalies of the Hepatobiliary System: The First Case Report

Background  Situs inversus totalis is a rare condition characterized by a mirror-image transposition of the abdominal and thoracic viscera. In order to develop safe techniques for hepatic resection, it is important to report surgical outcomes in cases complicated by situs inversus totalis and other anomalies. Case  The patient was a 64-year-old man with situs inversus totalis who had previously undergone sigmoidectomy with regional lymphadenectomy for sigmoid colon cancer at age 62. Despite postoperative adjuvant chemotherapy, tumor markers increased and multiple liver metastases were detected on abdominal ultrasonography. Enhanced computed tomography revealed not only liver metastases but also hepatobiliary anomalies associated with situs inversus totalis as follows: (1) portal vein located anterior to the common bile duct or hepatic artery, (2) proper hepatic artery arising from the superior mesenteric artery, (3) “left” (right in normal population)-sided umbilical portion of the portal vein and total ramification of intrahepatic portal branches from that point, (4) hepatic vein directly communicating to the “left” atrium. For the treatment of hepatic metastases from sigmoid colon cancer in a patient with situs inversus totalis, “left” hepatic lobectomy, partial hepatectomy, and radiofrequency ablation therapy were performed. The postoperative course was uneventful. Adjuvant chemotherapy has been continued for 2 years after the second operation and the patient is doing well without recurrence. Conclusion  Since situs inversus totalis is occasionally accompanied by multiple hepatobiliary anomalies, careful evaluation of the related anatomy using modern imaging modalities is crucial for safe hepatic resection.     

Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 years.     

ERCP and laparoscopic cholecystectomy for cholangitis in a 66-year-old male with situs inversus

A case report of the successful management of a patient with situs inversus viscerum and symptomatic choledocholithiasis and cholangitis is presented. The preoperative evaluation of the choledochus via ERCP and successful common bile duct stone extraction enabled successful laparoscopic cholecystectomy. The anatomic challenge of situs inversus viscerum mandates the selective use of intraoperative cholangiography during and upon completion of the laparoscopic cholecystectomy.     

全内脏转位的腹腔镜胆囊切除术4例报告

目的:探讨全内脏转位并胆囊结石患者施行腹腔镜胆囊切除术(laparoscop ic cholecystectomy,LC)的可行性及手术方法。方法:对1992年10月~2006年10月施行的8 256例LC中遇到的4例全内脏转位患者的临床资料进行回顾性总结。结果:4例全内脏转位患者均采用3孔法完成LC术,无中转开腹,全组患者术后均未放置腹腔引流管,恢复良好。结论:全内脏转位合并胆囊结石或胆囊息肉患者行LC是安全可行的,可与常规LC取得同样满意的临床效果。     

Accessory mitral valve tissue associated with situs inversus in an elderly patient

We report an unusual case of accessory mitral valve tissue associated with a situs inversus and missing obstruction of the left ventricular outflow tract. To our knowledge our patient is the only elderly patient with an accessory mitral valve with associated situs inversus undergoing surgical resection. The report emphasizes direct cardioscopy through the aortic annulus allowing precise excision of the abnormal tissue.     

完全性胸腹腔内脏反位合并十二指肠壶腹周围癌一例报告与文献复习

目的总结完全性胸腹腔内脏反位合并十二指肠壶腹周围癌的临床诊治方法。方法报道2006年3月收治的1例极为罕见的完全性胸腹腔内脏反位合并十二指肠壶腹周围癌行胰十二指肠切除术的病例,并复习国内外相关文献。结果本例患者病理诊断为十二指肠乳头及壶腹部腺癌Ⅰ-Ⅱ级。术后1周胆红素降至正常;但2周后出现胃-空肠吻合输出袢粘连性不完全性梗阻,经内镜下置管、鼻饲、营养支持、针灸等处理,40d后痊愈出院。经检索,全球自1936-2006年间报道的全胸腹腔内脏反位合并恶性肿瘤的患者仅15例;其中只有5例全胸腹腔内脏反位合并胰头与壶腹周围癌的报道。结论完全性胸腹腔内脏反位合并肿瘤时,若无明显禁忌证,应同样予以积极的外科治疗,术中操作应注意完全相反的解剖学结构。     

Off-pump quintuple coronary artery bypass grafting for situs inversus totalis

Dextrocardia associated with situs inversus totalis is a rare condition. A 49-year-old man with dextro-cardia and situs inversus totalis underwent off-pump quintuple coronary artery bypass grafting using bilateral internal thoracic arteries and bilateral radial arteries. The operative technique was similar to that of off-pump coronary artery bypass grafting for situs solitus. His postoperative recovery was uneventful.     

Disappearance of hepatitis B virus core deletion mutants and successful combined kidney/liver transplantation in a patient treated with lamivudine

Hepatitis B virus (HBV) core deletion variants with enhanced viral replication are associated with rapid deterioration of liver function in renal allograft recipients. Antiviral agents such as famciclovir and lamivudine offer new treatment strategies for these patients. Appearance, accumulation and persistence of HBV core deletion mutants were closely monitored in a kidney transplant recipient with liver cirrhosis before and after initiation of antiviral treatment. Under treatment with famciclovir HBV DNA concentration decreased by 50 %, HBV mutants persisted. After replacement of famciclovir by lamivudine HBV replication was reduced below the detection limit. Lamivudine was well tolerated and liver function improved. After successful combined kidney/liver transplantation the patient became HBsAg and HBV DNA (detected by PCR) negative under continuous hyperimmune globulin and lamivudine treatment. Antiviral therapy with lamivudine may be useful in treatment of progressive liver disease associated with HBV core deletion mutants in renal allograft recipients and may enable successful liver transplantation. Received: 12 June 1998 Received after revision: 16 October 1998 Accepted: 10 November 1998     

Laparoscopic appendectomy in a female patient with situs inversus: case report and literature review.

BACKGROUND: Situs inversus is an uncommon condition caused by a single autosomal recessive gene of incomplete penetration. A potential diagnostic dilemma can occur in the young female patient with a history of situs inversus who presents with pelvic pain. METHODS: A 32-year-old multiparous patient with a known history of situs inversus presented with complaints of pelvic pain. A medical history and full physical examination were indicative of possible endometriosis. RESULTS: The patient underwent an operative laparoscopy, which revealed stage II pelvic endometriosis based on the American Fertility Society Revised Classification for Endometriosis (R-AFS), with appendicular and periappendicular adhesions involving the cecum. Ablation of endometriosis and an appendectomy were performed. CONCLUSION: The authors believe the laparoscopic approach to an appendectomy is ideal in a patient with situs inversus and should be performed at the time of laparoscopy performed for another reason.     

Management of a patient with situs inversus totalis with acute cholecystitis and common bile duct stones: A case report

INTRODUCTION

The incidence of situs inversus thought to be in the range of 1:10,000–1:20,000 according to Mayo et al.

PRESENTATION OF CASE

A forty-five year-old lady presented with pain in the left hypochondrium and was diagnosed to have acute cholecystitis with choledocholithiasis in situs inversus totalis. Patient underwent endoscopic sphincterotomy and common bile duct stone extraction in view of choledocholithiasis and after common bile duct cannulation patient taken up for elective laparoscopic cholecystectomy (LC) within 24 h.

DISCUSSION

The most challenging factor for performing surgery in a patient with situs inversus is dealing with the mirror image anatomy. The first laparoscopic cholecystectomy in situs inversus was reported by Campos et al. in 1991. Since then, 50 other cases have been reported in literature making a point that laparoscopic procedure is not contraindicated.

CONCLUSION

Acute cholecystitis with choledocholithiasis is extremely rare and requires expert intervention from both the endoscopist and the laparoscopic surgeons because of the reverse anatomy and the fact that both are attuned to the constant practice of the procedures in normal anatomy.Keyword: Laparoscopic Cholecystectomy in Situs inversus totalis