Dyspnea due to an uncommon vascular tumor: leiomyosarcoma of the infrahepatic vena cava inferior.

BACKGROUND: Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignancy that originated from the smooth muscle tissue of the vascular wall. Diagnoses, as well as, treatment of the disease are still challenging and to date, a radical surgical resection of the tumor is the only curative approach. CASE REPORT: We report on the case of a 49-year old male patient who presented with suddenly experienced dyspnea. Besides bilateral pulmonary arterial embolism, a lesion close to the head of the pancreas was found using CT scan, infiltrating the infrahepatic IVC. Percutaneous ultrasound-guided biopsy revealed a low-grade LMS. Intraoperatively, a tumor of the IVC was observed without infiltration of surrounding organs or distant metastases. Consequently, the tumor was removed successfully, by en-bloc resection including prosthetic graft placement of the IVC. Histological workup revealed a completely resected (R0) moderately differentiated LMS of the IVC. CONCLUSION: LMS of the infrahepatic IVC is an uncommon tumor, which may present with dyspnea as its first clinical sign. Patients benefit from radical tumor resection. However, due to the poor prognosis of vascular LMS, a careful follow-up is mandatory.

Hepatic resection in liver transplant recipients: single center experience and review of the literature.

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.

Clinical implications of hepatic preservation injury after adult liver transplantation.

Several advances in organ preservation have allowed for improved results after liver transplantation; however, little information is available regarding the clinical impact of preservation injury on the postoperative course. The medical records of 889 liver transplants were retrospectively reviewed. Preservation injury was classified according to postoperative aspartate aminotransferase values as minor (<1000 U/L), moderate (1000-5000 U/L), or severe (>5000 U/L). The following criteria were analyzed and compared according to the extent of preservation injury: patient and graft survival, retransplantation rate, duration of hospitalization and postoperative ventilation, as well as incidence of rejection, infection, and hemodialysis. The majority of patients received a liver with minor preservation injury (75.9%), whereas 22.7% and 1.3% of grafts showed moderate or severe injury. Graft survival was significantly lower in patients with severe preservation injury, when compared to minor or moderate injury. The relative risk for initial nonfunction was 39.36-fold increased (95% confidence interval (ci): 10.3-150.2), as it was increased for duration of postoperative ventilation (6.92-fold; 95%ci: 2.1-22.3) and hemodialysis (6.13-fold; 95%ci: 1.9-19.3). Since the incidence of retransplantation was significantly increased (50%), patient survival remained comparable between all groups. Severe preservation injury had a tremendous impact on the postoperative clinical course, requiring the maximum medical effort to achieve adequate patient survival.

Primary permanent arterialization of the portal vein in liver transplantation.

Permanent total arterialization of the portal vein in liver transplantation has been described as a method of providing portal inflow after insufficient thrombectomy due to chronic occlusion of the portal-vein system. A specific problem is the restriction of the arterial inflow and its long-term adaptation after transplantation. We describe here the surgical techniques and clinical course of three patients who underwent portal-vein arterialization for liver transplantation. Two patients had an uneventful course. In one patient, a flow reduction by means of coil embolization of one arterial inflow branch was performed; thereafter, the patient recuperated well. Analysing the microcirculation of an arterialized graft in comparison with liver grafts with normal non-arterialized portal-vein inflow, we observed an increase in inter-sinusoidal distance and a decrease in sinusoidal red blood cell velocity. From a technical point of view, we recommend permanent portal-vein arterialization by an iliac artery graft interposition from the subdiaphragmatic aorta. The inflow to the portal vein can easily be reduced by the banding of the arterial graft interposition.

Diagnosis and treatment of arterial steal syndromes in liver transplant recipients.

Arterial steal syndromes (ASSs) after orthotopic liver transplantation (OLT) are characterized by arterial hypoperfusion of the graft caused by shifting of blood flow into the splenic or gastroduodenal artery. Despite their potentially devastating consequences, such as ischemic biliary tract destruction or graft failure, ASSs have received little attention to date. We report the incidence, diagnosis, and treatment of ASS among 1,250 consecutive OLTs. ASS was observed in 69 patients (5.9%). All these patients presented with elevated liver enzyme levels, impaired graft function, or cholestasis. Treatment consisted of splenectomy (n = 18), coil embolization of the splenic or gastroduodenal artery (n = 29), or reduction in splenic artery blood flow through administration of an artificial stenosis (banding; n = 9). Mild symptoms of ASS did not warrant treatment in 13 patients. However, 3 of these patients developed ischemic biliary destruction requiring re-OLT. Serious complications also were observed after splenectomy and coil embolization, requiring re-OLT in 18%. Conversely, no complications were observed after banding the splenic artery. Prophylactic banding of the splenic artery was performed in 97 patients and was associated with a complication rate of 4%. In summary, the incidence of ASS is similar to that of other vascular complications. Untreated ASSs may lead to serious complications in more than 30% of patients. Of a variety of treatment options, banding the splenic artery was associated with the lowest complication rate. Banding also may be performed prophylactically in selected patients to prevent the development of ASS after OLT.

Hepatic artery thrombosis after adult liver transplantation.

The incidence, clinical presentation, therapeutic options, and outcome of hepatic artery thrombosis (HAT) were analyzed in a series of 1,192 consecutive adult orthotopic liver transplantations (OLTs). HAT after OLT was observed in 30 cases, resulting in an incidence of 2.5%. The incidence of HAT increased 5.76-fold when the donor hepatic artery was reconstructed with an interposition graft to the supraceliac aorta (P <.05). Early HAT (within the first 30 days after OLT) occurred in 14 of these patients (46.7%), whereas in 16 patients (53.3%), HAT occurred beyond 30 days post-OLT. Clinical presentation of HAT ranged from an increase in serum transaminase levels with or without cholestasis to liver abscess and biliary complications, including cholangitis, bile duct stenosis or necrosis, to liver dysfunction and failure. Impairment of graft function was observed in patients with early HAT, whereas biliary tract destruction was seen more often in patients with late HAT. In only 1 patient was HAT clinically asymptomatic. Therapy consisted of recombinant plasminogen lysis with hepaticojejunostomy, liver abscess drainage, endoscopy or surveillance, and surgical thrombectomy. In 14 of 30 patients (46.7%), the occurrence of HAT required re-OLT. Nine patients with HAT died during follow-up; however, only 4 of these deaths were related to HAT, resulting in a mortality rate of 13.3%. Our results indicate that HAT is a rare but serious complication after OLT, requiring re-OLT in almost 50% of patients. In particular, conservative treatment modalities may significantly prolong graft survival, thus postponing re-OLT.

[Orthotopic liver transplantation in case of TIPS stent dislocation]. / Orthotope Lebertransplantation bei disloziertem TIPS-Stent.

Transjugular intrahepatic portosystemic shunts (TIPS) are indicated in patients with liver cirrhosis and portal hypertension for treatment of variceal bleeding or refractory ascites. Additionally implantation of stents may lead to stent dislocation or thrombosis in up to 20 % of cases. Detailed information about stent dislocation and its impact on subsequent orthotopic liver transplantation (OLT) is rare regarding the literature. We report on a patient suffering from ethyltoxic liver cirrhosis in which OLT was technically complicated by a thrombosed TIPS stent, dislocated in the portal vein. This stent was implanted prior to OLT due to refractory ascites and partial portal vein thrombosis. We conclude that TIPS stent insertion, especially in liver transplant candidates, should only be performed by radiologists in centers with expertise and experience.

Results of end-to-end cavocavostomy during adult liver transplantation.

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.