Living related donor liver transplantation for primary sclerosing cholangitis with hepatocellular carcinoma and Crohn's disease: a case report.

INTRODUCTION: Primary sclerosing cholangitis (PSC) is a premalignant condition associated with a risk of developing cholangiocarcinoma in 10% to 20% of patients. However, the prevalence of hepatocellular carcinoma (HCC) in patients with PSC is estimated to be only 2%. In addition, PSC often occurs in association with inflammatory bowel disease. Ulcerative colitis occurs in 60% of PSC patients and Crohn's disease occurs in about 10%. We diagnosed a patient as having PSC with HCC and concomitant Crohn's disease prior to living related donor liver transplantation (LRDLT). PATIENTS AND METHODS: A 63-year-old woman was diagnosed as having PSC with solitary HCC. Preoperative liver condition was Child-Pugh grade C. Colonoscopic findings showed an active longitudinal ulcer in the terminal ileum and clinically diagnosed Crohn's disease. The medical treatment was 5'-aminosalicylates. She received a left lobe graft from her daughter. The immunosuppressants were tacrolimus and azathioprine, which was changed to predonisolone because of leukopenia and moderate acute cellular rejection, after which the postoperative course was uneventful. Colonoscopic findings revealed disappearance of the longitudinal ulcer in the terminal ileum at 15 months after LRDLT. There was no evidence of PSC or HCC recurrence. CONCLUSIONS: Generally, PSC with HCC is rare with a poor prognosis; however, LRDLT may be a treatments option although the patient with PSC had a combined HCC. Moreover, aminosalicylates together with the immunosuppressants may be effective for the clinical management of concomitant Crohn's disease.

Inferior mesenteric venous left renal vein shunting for decompression of excessive portal hypertension in adult living related liver transplantation.

In the present study, we investigated the effect of decompression of excessive portal hypertension by inferior mesenteric venous (IMV) left renal vein shunting in 7 cirrhotic patients with esophago-gastric varices and 2 patients who underwent adult living related donor liver transplantation (ALRDLT). The portal pressure remarkably decreased after shunting in all patients with esophago-gastric varices (388 +/- 42 mm H2O vs. 247 +/- 57 mm H2O; P < .05). It also decreased after a shunt operation in patients who had undergone liver transplantation. We report that the excessive shear stress by portal hypertension after small-for-size LRDLT induces a liver injury and the decompression of portal hypertension by splenic arterial ligation or splenectomy prevents postoperative liver injury following massive hepatectomy and small-for-size LRDLT. Our present studies suggested that IMV left renal vein shunting might prevent postoperative liver injury by partial decompression of excessive portal hypertension following small-for-size LRDLT.

Splenic arterial ligation prevents liver injury after a major hepatectomy by a reduction of surplus portal hypertension in hepatocellular carcinoma patients with cirrhosis.

BACKGROUND/AIMS: In this study, we investigated whether a reduction of surplus portal hypertension after a major hepatectomy by SPL (splenic arterial ligation) prevents a liver injury in cirrhotic patients with hepatocellular carcinoma. METHODOLOGY: Six hepatocellular carcinoma patients (SPL group) with liver cirrhosis (67 +/- 10 years old, ICGR15: 21.0 +/- 9.8%, T.Bil: 1.1 +/- 1.2 mg/dL) underwent major hepatectomy with splenic arterial ligation in order to reduce excessive portal hypertension after hepatectomy from 1998 to 2000, July. The patients (n = 15, 60 +/- 9 years old, ICGR15: 11.5 +/- 5.9%, T.Bil: 0.66 +/- 0.15 mg/dL) who underwent liver resection above subsegmentectomy in the same period (control group) served as the control for SPL group. RESULTS: In the SPL group, the portal pressures before hepatectomy were 26 +/- 7 cm H2O and those after hepatectomy were 29 +/- 6 cm H2O. The portal pressure after splenic arterial ligation decreased to 24.5 +/- 6.3 cm H2O. The splenic tissue blood flows before SPL were 16.8 +/- 5.6 mL/min/100 g, while those after SPL were 7.2 +/- 2.2 mL/min/100 g. The portal pressures before hepatectomy were 17 +/- 2 cm H2O and those after hepatectomy were 19 +/- 2 cm H2O in the six control patients. At the peak levels of liver function after surgery, T.Bil was 2.6 +/- 1.5 mg/dL, GOT was 165 +/- 59 IU/L, and GPT was 107 +/- 49 IU/L. All patients could discharge without complications except for one case with bile leakage in SPL. At the peak levels of liver function in control group, T.Bil was 3.7 +/- 1.9 mg/dL, GOT was 404 +/- 227 IU/L, and GPT was 322 +/- 171 IU/L. At the peak levels of liver function after surgery, T.Bil was 3.4 +/- 1.3 mg/dL, GOT was 398 +/- 289 IU/L, and GPT was 319 +/- 220 IU/L. Conversely, there were 11 episodes of complications (11/15), including two cases of hospital death resulting from liver failure in patients who underwent right lobectomy, in the control patients. CONCLUSIONS: The decompression of surplus portal hypertension by SPL might be effective in the prevention of post hepatectomized liver injury and the improvement of postoperative mortality and morbidity.

[Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism in three patients with systemic lupus erythematosus and antiphospholipid syndrome].

Pulmonary hypertension (PH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Several potential mechanisms have been postulated for narrowing of vessels as a result of pulmonary vasculitis and pulmonary thromboembolism caused by antiphospholipid antibodies. Pulmonary thromboendarterectomy for chronic pulmonary thromboembolism is performed to alleviate pulmonary hypertension. We report three rare cases of SLE with antiphospholipid syndrome in patients who presented with PH secondary to chronic pulmonary thromboembolism. Pulmonary thromboendarterectomy was performed, and all patients remained well without deterioration of PH after surgery. Pulmonary thromboendarterectomy should be considered as an effective method of treatment for this disease.

Liver transplantation surgical techniques for extensive retroperitoneal tumor with major blood vessel involvement: a case report.

A case of a 71-year-old man with a huge retroperitoneal tumor situated behind the liver, which strongly compressed the liver inferior vena cava (IVC), and gastrointestinal tract is described. With the techniques of whole liver extraction and autologous orthotopic liver transplantation, we successfully removed the tumor. We have the surgical techniques, essential elements, and indications for this procedure.

Isolated dissection of the superior mesenteric artery after living donor liver transplantation: a case report.

Isolated dissection of the superior mesenteric artery (SMA) not associated with aortic dissection is rare, particularly after living donor liver transplantation (LDLT). We experienced a case of isolated dissection of the SMA after LDLT performed in a 56-year-old man diagnosed with hepatitis B virus-related cirrhosis and hepatocellular carcinoma within the Milan criteria. He had no past history of hypertension or diabetes mellitus. At 6 days after LDLT, the patient underwent an emergency portal vein thrombectomy with ligation of a huge left gastric vein shunt. Thereafter anticoagulant and antiplatelet therapy were initiated. At 12 days after LDLT, a contrast-enhanced computer assisted tomography (CT) scan revealed the presence of a thrombus in a false lumen and a thin flap enlarged in the SMA. Because he presented neither abdominal pain nor biochemical data suggesting mesenteric ischemia, he was treated with antihypertensive agents in addition to anticoagulant and antiplatelet therapy. The thrombus in the false lumen was reduced and the intimal flap in the SMA disappeared according to the results of a CT scan 4 months after LDLT. He has remained free of symptoms for 4 years. The strategy to treat isolated SMA dissection is not well established. Urgent surgery is indicated for acute symptomatic forms with a suspicion of mesenteric ischemia; conservative treatment is indicated for patients with minimal, resolving, or no pain, but requires close follow-up.

The new method of time-lag ligation for portosystemic shunt using coronary artery bypass graft occluder for adult living donor liver transplantation.

We performed a living donor liver transplantation (LDLT) for a 57-year-old man who had end-stage liver failure with portal hypertension and an inferior mesenteric vein-left testicular vein (IMV-LTV) shunt. At operation, we did not clamp the shunt but encircled it with a coronary artery bypass graft (CABG) occluder (Sumitomo Bakelite K.K., Japan), which was passed outside the body through the abdominal wall to time-lag ligation (TLL). On postoperative day (POD) 5, we observed decreased portal flow. We performed TLL of the shunt using the CABG occluder without re-laparotomy. The portal flow increased, while the portal vein pressure increased slightly. In LDLT, portosystemic shunt has been reported to be a cause of portal thrombus formation or graft liver atrophy due to decreased PV flow in the mid postoperative period. However, perioperative ligation of a portosystemic shunt may prevent regeneration of the grafted liver because of excessive portal hypertension. Therefore the technique of time-lag ligation of a portosystemic shunt using a CABG occluder may be a minimally invasive, useful method to achieve physiological liver graft regeneration.