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Under FK 506-based immunosuppression, the entire cadaver small bowel except for a few proximal and distal centimeters was translated to 17 randomly matched patients, of whom two had antigraft cytotoxic antibodies (positive cross-match). Eight patients received the intestine only, eight had intestine in continuity with the liver, and one received a full multivisceral graft that included the liver, stomach, and pancreas. One liver-intestine recipient died after an intestinal anastomotic leak, sepsis, and graft- versus-host disease. The other 16 patients are alive after 1 to 23 months, in one case after chronic rejection, graft removal, and retransplantation. Twelve of the patients have been liberated from total parenteral nutrition, including all whose transplantation was 2 months or longer ago. The grafts have supported good nutrition, and in children, have allowed growth and weight gain. Management of these patients has been difficult and often complicated, but the end result has been satisfactory in most cases, justifying further clinical trials. The convalescence of the eight patients receiving intestine only has been faster and more trouble free than after liver-intestine or multivisceral transplantation, with no greater difficulty in the control of rejection.

The current role of liver transplantation in treating malignant tumors of the liver is uncertain, except for select histologic types. Pooled data on the results of liver transplantation in 12 children with hepatoblastoma is presented here. One half of the children are alive 24 to 70 (44 ± 19) months after transplantation with no evidence of recurrence. Three patients (25%) died of tumor recurrence and three (25%) died of other causes. Unifocal and intrahepatic tumors were associated with better prognosis compared to the multifocal tumors and tumors with extrahepatic spread (p = 0.04 and 0.13). Microscopically vascular invasion and the predominance of embryonal and/or anaplastic epithelium were associated with a poor prognosis compared to the tumors with no vascular invasion and with predominantly fetal epithelium (p = 0.08 and 0.1). It is concluded that continued efforts to treat unresectable hepatoblastomas by liver transplantation is justified and the role of adjuvant chemotherapy in improving the results needs to be better defined.

The linear growth of 29 patients was evaluated from two to 4⅓ years after liver transplantation. All patients received cyclosporine and low-dose prednisone. Eight patients displayed acceleration of linear growth velocity and were above the fifth percentile at the end of the evaluation period. Four patients grew normally prior to transplantation and continued to grow normally after the surgical procedure. Only four patients dropped from higher levels to below the fifth percentile. Thirteen patients were less than the fifth percentile before and after surgery; ten of these 13 patients have attained normal or accelerated growth velocity. Good linear growth has been achieved in more than three fourths of patients who underwent liver transplantation. © 1987, American Medical Association. All rights reserved.

Confirmation of patency of the portal vein by either ultrasound or angiography is a routine part of the evaluation of patients being considered for hepatic transplantation. Complete thrombosis of the portal vein usually has been viewed as precluding successful orthotopic hepatic replacement. In addition, some pediatric patients present with extremely small portal veins which, although patent, have proved to be thick walled and sclerotic. Our recent experience has shown that, in both of these situations, successful and complete revascularization of hepatic allografts is quite feasible by using a vein graft to ensure adequate portal venous flow.

During the last 5 years, liver transplantation has become a service as opposed to an experimental operation. The most important factor in making this possible has been the introduction of cyclosporine-steroid therapy. At the same time, liver transplantation has been made more practical by improvements in diagnosing and managing other causes of postoperative hepatic dysfunction. Tissue typing and matching have played no role in improving the results of liver transplantation. With the demonstration that performed antibody states are irrelevant, even avoidance of positive cross-matches caused by cytotoxic antibodies and observance of ABO blood group barriers have become unnecessary if the recipient's needs are great. With the exceptions of malignancy and cirrhosis, the nature of the underlying hepatic disease has not profoundly influenced the results. Retransplantation has played an important role in improving survival, although the costs of retransplantation have been extremely high.

A venous bypass technique (BP) that does not require the use of systemic anticoagulation is used routinely at our institution in all adult patients during the anhepatic phase of liver transplantation (LT). Complete cardiopulmonary profiles were obtained in a subset of 28 consecutive cases. During the anhepatic phase while on bypass, mean arterial pressure, central venous pressure, and pulmonary arterial wedge pressure were maintained at prehepatectomy levels. Oxygen consumption fell secondary to a decrease in temperature and the removal of the liver. Consequently, cardiac index fell without an increase in arterial-venous O2 content difference, reflecting adequate tissue oxygenation. Compared with 63 patients in a previous series given LT without bypass (NBP), the 57 total BP patients experienced better postoperative renal function (p < 0.001), required less blood use during surgery (p < 0.01), and had better survival 30 days after LT. The equivalency of 90-day survival in these groups results from the lack of effect of BP on the long-term survival of patients considered at high risk for metabolic reasons. BP patients at high risk for technical considerations, however, survived LT whereas NBP patients did not. BP offers other advantages important in establishing LT as a service-oriented procedure.

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 reserved

Venous bypass restores normal hemodynamic physiology during the critical anhepatic phase of orthotopic transplantation of the liver. Its routine use in adults undergoing transplantation in Pittsburgh has resulted in lower operative blood losses, a lower frequency of postoperative renal failure, and a greater probability of survival for all but the highest risk patients. Because it allows for a longer anhepatic phase, the surgeon has the option of tailoring the native hepatectomy to the needs of the individual case, even to the point, in difficult cases, of obtaining most of the hemostasis after removal of the native liver, but before sewing in the donor organ. Selective use of bypass in children may offer similar advantages.

Fifty-four patients underwent total hepatectomy and liver replacement in the presence of a primary liver malignancy. in 13 recipients in whom the hepatic tumors were incidental to some other endstage liver disease, recurrence was not seen and 12 of the 13 patients are alive after 4 months to 15 1/2 years. In contrast, tumors recurred in 3 of every 4 patients who received liver replacement primarily because of hepatic malignancies that could not be resected by conventional techniques of subtotal hepatectomy and who lived for at least 2 months after transplantation. The most encouraging results were in patients with the fibrolamellar hepatocellular carcinomas that grow slowly and metastasize late, but even with this lesion, the recurrence rate was 57%. In future trials, additional effective anticancer therapy will be needed to improve the results of liver transplantation for primary liver malignancy, but what an improved strategy should be has not yet been defined.

One hundred seventy orthotopic liver transplants were performed under conventional immunosuppression with azathioprine and steroids with 1- and 5-year survivals of 32.9% and 20.0%, respectively. Since the introduction of cyclosporine-prednisone therapy in March 1980, 313 primary orthotopic liver transplants have been performed. Actuarial survivals at 1 and 5 years have improved to 69.7% and 62.8%, respectively. Biliary atresia is now the most common indication for liver replacement. In adults, primary biliary cirrhosis and sclerosing cholangitis have become more common indications for transplantation, and alcoholic cirrhosis and primary liver malignancy as indications have declined. Early enthusiasm for liver transplantation in patients with hepatic cancer has been tempered by the finding that recurrence is both common and rapid. An increasing number of patients with inborn errors of metabolism originating in the liver are receiving transplants, including patients with Wilson's disease, tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage disease, familial hypercholesterolemia, and hemochromatosis. Survival in this group of patients has been excellent (74.4% at 1 and 5 years). A hemophiliac who received a transplant for postnecrotic cirrhosis has survived and may have been cured of his hemophilia. About 20% of patients require retransplantation for rejection, technical failure, or primary graft failure. Only 4 of the patients receiving retransplants under conventional immunosuppression survived beyond 6 months, and all died within 14 months of retransplantation. Sixty-eight patients have received retransplants under cyclosporine-prednisone. Thirty-one patients are surviving, all for at least 1 year. Six of the 12 patients requiring a third transplant are alive 2 to 3 years after the primary operation. An aggressive approach to retransplantation in the patient with a failed graft is justified.

The massive surgical assault associated with hepatic transplantation makes a high frequency of complications almost inevitable. In this review of 225 patient records, selected at random from cases of liver transplantation in Pittsburgh over a 2 1/2 year period ending in January 1985, 87.2% of patients experienced at least one significant complication that threatened their survival or that of the graft and that often prolonged their hospitalization. Familiarity with the complications may facilitate earlier recognition, with consequently early and more effective management in future cases.

Fourteen patients with fibrolamellar hepatoma were treated with radical excision. In eight, a subtotal hepatic resection was performed from 16 months to more than 16 years ago. None of the patients have died and recurrences have been seen in only one patient. Six other patients had total hepatectomy and hepatic replacement. Two of these six patients have died of metastases and a third is living with recurrent tumor. This experience has justified the continuing use of quite aggressive extirpative procedures for the treatment of fibrolamellar hepatoma.

Hepatic artery thrombosis is a dreadful complication of orthotopic liver transplantation. It should be suspected in cases of fulminant liver failure, delayed bile leak, or intermittent sepsis of unknown cause after liver transplantation. Accurate diagnosis is assisted by ultrasound and computerized tomography scans, but usually requires arteriography. Prompt retransplantation is required in most of the cases. © 1985 by The Williams & Wilkins Co.

A group of 118 adults who underwent primary, orthotopic transplantation of the liver over a 4-year period served as the subjects of a detailed examination of their ability to survive the first 6 months as a function of their preoperative condition. As a result, a scoring system was developed empirically in an attempt to separate very high-risk from relatively low-risk patients. The scoring method is based on the high degree of correlation between survival probability and various patient characteristics. It allows for additional scoring to account for the dramatic effect of operative blood loss on the eventual outcome. The curve that best describes the relationship between patient scores and survival probability is sigmoidal in shape. Many patients will have scores located on the curve between the inflection points. They represent a group whose relative risk is difficult to estimate but for whom operative blood loss or the occurrence of surgical complications may prove particularly telling.

Between March 3, 1981, and June 1, 1984, 216 children were evaluated for orthotopic liver transplantation. Of the 216 patients, 117 (55%) had recelved at least one liver transplant by June 1, 1985. Fifty-five (25%) died before transplantation. The 117 patients who received transplants were grouped according to severity of disease and degree of general decompensation at the time of transplantation. The severity of a patient's medical condition with the possible exception of deep hepatic coma, did not predict outcome following orthotopic liver transplantation. Seventy variables were assessed at the time of the evaluation. Twenty-three of the 70 variables were found to have prognostic significance with regard to death from progressive liver disease before transplantation. These 23 variables were Incorporated into a multivariate model to provide a means of determining the relative risk of death among pediatric patients with end-stage liver disease. This information may allow more informed selection of candidates awaiting liver transplantation. © 1987 The C. V. Mosby Company.

Over 45 months, 119 angiographic examinations were performed in 95 patients prior to liver transplantation, and 53 examinations in 44 patients after transplantation. Transplantation feasibility was influenced by patency of the portal vein and inferior vena cava. Selective arterial portography, wedged hepatic venography, and transhepatic portography were used to assess the portal vein if sonography or computed tomography was inconclusive. Major indications for angiography after transplantation included early liver failure, sepsis, unexplained elevation of liver enzyme levels, and delayed bile leakage, all of which may be due to hepatic artery thrombosis. Other indications included gastrointestinal tract bleeding, hemobilia, and evaluation of portal vein patency in patients with chronic rejection who were being considered for retransplantation. Normal radiographic features of hepatic artery and portal vein reconstruction are demonstrated. Complications diagnosed using results of angiography included hepatic artery or portal vein stenoses and thromboses and pancreaticoduodenal aneurysms. Intrahepatic arterial narrowing, attenuation, slow flow, and poor filling were seen in five patients with rejection.

A simplified technique for conversion of a complex hepatic arterial supply into a common channel is described. This technique permits single vessel anastomosis in the recipient of a liver transplant.

A girl aged 6 years 9 months with severe heart disease secondary to homozygous familial hypercholesterolaemia underwent orthotopic cardiac transplantation and her liver was replaced with the liver of the same donor. In the first 10 weeks after transplantation serum cholesterol fell to 270 mg/dl from preoperative concentrations of more than 1000 mg/dl. © 1984.

Sixty-three primary and 21 retransplant cadaver kidney allografts were placed in 77 patients over a one-year period with three- to six-month follow-up. Eight primary grafts (12.7%) and six retransplants (28.6%) were lost to rejection. Patient mortality was 3.9%. There were no grafts lost and no deaths due to opportunistic infections. Renal function at 6 months after transplantation was similar in all primary transplant recipients regardless of risk factors, including advanced age, diabetes, or the need for postoperative dialysis. © 1985, National Kidney Foundation, Inc.. All rights reserved.

A technique of veno-venous bypass without heparin has been developed for use during the anhepatic phase of transplantation of the liver. With this method, the ability to compress the temporarily obstructed vena caval and portal venous systems has made hepatic transplantation an easier procedure.

Numerous causes can lead to hepatic dysfunction following orthotopic liver transplantation. The most common cause is rejection, which is usually nonpreventable. The clinical presentation, time of onset, and even treatment are variable. Other causes, such as perioperative ischemic injury, vascular thrombosis, and complications of bile duct reconstruction may be preventable with good surgical technique. Infections can also be minimized by careful adjustment of immunotherapy, avoidance overimmunosuppression, and the judicious use of antibiotics. Hepatic dysfunction following orthotopic liver transplantation requires rapid assessment and proper treatment in order to prevent serious and possibly fatal complications.

Since the introduction of cyclosporine-prednisone for primary immunosuppression, retransplantation has become a feasible option for patients whose primary grafts are failing, which may result from primary graft nonfunction, intractable rejection, or consequent to technical complications. Although survival of patients requiring second grafts is less good than in those whose initial graft functions well, 2-year survival rates of 49% have been achieved in retransplanted patients, a record that mandates serious consideration of this approach when the primary graft begins to fail. In general, the retransplant procedure is technically easier, with less blood loss, than is the initial operation. When the reoperation is done electively, it should be done before serious clinical deterioration compromises the chances for success.

Left hepatic trisegmentectomy was successfully performed upon four patients in whom the true left lobe of the liver and all, or part, of the anterior segment of the right lobe of the liver were removed in continuity. Three of the patients had carcinoma of the liver, and the fourth patient had a hemangioma and arteriovenous malformation. This procedure, which has not been described before, should allow subtotal hepatic resection to be performed upon some patients who have lesions that have been classified as inoperable, in the past.

Pancreaticoduodenal transplantation was performed with cyclosporine and steroid immunosuppressive therapy in 10 mongrel dogs that had pancreaticoduodenectomy. There was an average animal survival of 50.6 days and an average duration of normoglycemia of 41.8 days. We suggest that pancreaticoduodenal transplantation, which was abandoned as a clinical procedure 10 years ago, be reconsidered for clinical application. © 1984.

Ninety-seven cadaveric renal transplants were performed upon 96 patients during 1981. The one year patient mortality was 2.1 per cent. Seventy of the recipients were undergoing trasplantation for the first time. Of these patients, 38 were treated with cyclosporine and steroids with a one year graft survival rate of 89.5 per cent. The other 32 primary recipients were treated with azathioprine and steroids with a one year graft survival rate of 50 per cent. The difference between the cyclosporine-steroid versus conventional therapy groups was significant. Cyclosporine and steroids were also used to treat 26 patients who underwent retransplantation with 27 cadaveric grafts. The one year graft survival time was 77.8 per cent; most of the graft losses were in presensitized patients. The results with retransplantation were twice as good as in historical control groups.