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Objective: To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS)., Summary Background Data: The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined., Patients and Methods: Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis., Results: Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement., Conclusion: Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.

Background: Malignant epithelioid hemangioendothelioma is a rare vascular tumor described mostly in soft tissue, lungs, or liver. The outcome after a wide variety of therapeutic measures, ranging from extended surgical therapy to no therapy, is reported to be variable. Therefore, we reviewed our experience with resective therapy for this rare liver tumor, including orthotopic liver transplantation., Material and Methods: During a period of 5 years, seven patients with the histological diagnosis of hepatic epithelia hemangioendothelioma were seen. In five of them, the liver pathology was detected at random, and all patients were prospectively followed. The therapeutic measures and course of disease are given as case reports., Results: Three patients received formal liver resection and two received liver grafts, one partial and one whole. One further patient is scheduled for transplantation and one is undergoing alternative therapy, with the tumor remaining stable. All resected patients recovered quickly and are alive and well 2 months to 4 years later without signs of tumor recidivism., Conclusion: Anatomic liver resection, or in the case of diffuse tumor spread, orthotopic liver transplantation, show favorable long-term results for the treatment of hepatic epithelioid hemangioendothelioma. Therefore, surgical therapy is proposed as the treatment of choice for this entity.

Biliary complications such as ischemic (type) biliary lesions frequently develop following liver transplantation, requiring costly medical and endoscopic treatment. If conservative approaches fail, re-transplantation is most often an inevitable sequel. Because of an increasing donor organ shortage and unfavorable outcomes in hepatic re-transplantation, efforts to prolong graft survival become of particular interest. From a series of 1685 liver transplants, we herein report on three patients who underwent partial hepatic graft resection for (ischemic type) biliary lesions. In all cases, left hepatectomy (Couinaud's segments II, III and IV) was performed without Pringle maneuver or mobilization of the right liver. All patients fully recovered postoperatively, but biliary leakage required surgical revision twice in one patient. At last follow-up, two patients presented alive and well. The other patient with persistent hepatic artery thrombosis (HAT), however, demonstrated progression of disease in the right liver remnant and required re-transplantation 13 months after hepatic graft resection. Including our own patients, review of the literature identified 24 adult patients who underwent hepatic graft resection. In conclusion, partial graft hepatectomy can be considered a safe and beneficial procedure in selected liver transplant recipients with anatomical limited biliary injury, thereby, preserving scarce donor organs.

The results of end-to-end cavocavostomy during adult liver transplantation were analyzed with special regard to caval complications. In a series of 1000 liver transplants, we observed 17 patients who suffered from postoperative caval obstruction (6 patients) or caval stenosis (11 patients), for an incidence of 1.7%. Surgical therapy was performed in 10 patients (58.8%), and 5 patients required retransplantation (29.4%). Four patients died during the later postoperative course. Two fatalities were related to caval complications, resulting in a mortality rate of 11.8%. Our results indicate that end-to-end cavocavostomy is a safe technique for cavocaval anastomosis. For only a few exceptions, such as pediatric transplantation, reduced size livers, or size mismatch between donor and recipient, should alternative techniques such as end-to-side or side-to-side cavocavostomy be performed.

Rationale and Objectives: The authors correlate computed tomography (CT) findings in biphasic spiral technique with histopathology in patients with hepatocellular carcinoma (HCC) who had undergone liver resection (LR) or orthotopic liver transplantation (OLT)., Methods: Preoperative biphasic spiral CT findings in 33 consecutive patients (23 men, 10 women, aged 43-74 years; LR group: n = 17; OLT group; n = 16) with liver cirrhosis and HCC were reviewed retrospectively by consensus of two radiologists and correlated with pathology from liver specimens., Results: Of the 16 patients in the OLT group with 1 to 5 confirmed HCC lesions (total lesions: 29; mean lesion diameter: 2 cm; range: 0.6-5.0 cm), CT before OLT depicted 22 lesions in 15 patients (sensitivity for lesions with a diameter of 0.5-1.0 cm, 20%; for lesions 1.1-2.0 cm, 82%; and for lesions 2.1-3.0 cm and > 3.0 cm, 86% and 100%, respectively). Among the 17 patients in the LR group (total lesions: 21; mean lesion diameter: 5.4 cm; range: 1.0-11.0 cm), CT detected 18 lesions. Lesion-by-lesion sensitivity, as correlated with pathology, was calculated at 76% and 86% in the OLT and LR groups, respectively (overall sensitivity, 80%). The diameter of CT detected lesions, compared with liver specimens, corresponded in 90% of lesions (maximum deviation, 15%). Characteristic CT findings of HCC included unenhanced hypoattenuating focal liver lesions (32 lesions), with hyperattenuation (38 lesions) in the arterial phase of contrast material administration., Conclusions: Biphasic spiral CT for preoperative HCC detection correlated with pathology in 80%, thus proving this technique to represent a sensitive imaging modality for pretherapeutic evaluation of HCC.

Abstract: In the therapy of hilar cholangiocarcinoma, the most favorable survival rates over the long-term are achieved by a surgical concept involving a no-touch-technique, en-bloc-resection and wide tumor-free margins. Currently, these goals can be best achieved by our strategy to combine extended right hepatic resections and principle portal vein resection. In spite of extending resectability to patients with locally advanced tumors, formally curative resections could be performed in 80% of the patients. The 5-year survival rate in these patients is 61%. Liver transplantation had been abandoned by most centers in the 1980s due to poor overall results. Recently, a neoadjuvant strategy involving radiochemotherapy has been reported to result in excellent survival figures at least in a subset of patients suffering from cholangiocellular carcinoma arising in a primary sclerosing cholangitis (PSC). This protocol has been mainly proposed by the Mayo Clinic group and reached 5-year survival rates of 80% in those patients in whom it had been applicable. A substantial drop out rate from this neoadjuvant regimen due to tumor progression or treatment related complications is still a problem. [Copyright &y& Elsevier]