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If the ratio liver graft weight/recipient body weight ratio is below 0.8%, there is a high risk of primary non function of the graft in almost 50% of cases. We report the first elective Auxiliary Partial Orthotopic Liver Transplantation using a small cadaveric left graft for cirrhosis. The graft weight/recipient body weight rate was 0.6%. This technique avoided the small-for-size syndrome in the early postoperative period. The native right liver was resected two months later. With a follow-up of 5 years, the patient is in good condition. This original technique could be a solution to increase the number of transplantations using small grafts, especially left grafts, provided by splits or living donors.

Unlabelled: Nine centers have reported 15 cases of liver transplantation for Rendu-Osler-Weber's disease with liver involvement. Six cases are reported to analyse the specific technical and hemodynamical aspects., Patients and Methods: Five women and 1 man were transplanted for Rendu-Osler-Weber's disease. The clinical presentation was biliary disease in 3 cases, portal hypertension in 2 cases, cardiac failure in 1 case. Systemic hemodynamics were assessed at the beginning and at the end of the transplantation procedure., Results: The procedure lasted from 11 to 15 hours (median=13 hours and 15 minutes). Blood transfusion during the procedure varied from 16 to 88 blood units (median=59 blood units). Six patients had hyperkinetic syndrome at the beginning of the procedure. At the end of transplantation, mean arterial pressure significantly increased (from 66 +/- 2 to 72 +/- 6 mmHg, p<0.05), whereas cardiac output (from 9.2 +/- 3.0 à 5.7 +/- 0.5 L/mn, p<0.05) significantly decreased. Two patients died at D2 and D11 and 4 are alive 3 to 7.5 years (median=4 years 9 months) after transplantation with a normal liver function and without any cardiac symptoms., Conclusion: Liver transplantation for Rendu-Osler-Weber's disease is a difficult procedure. When successful, liver transplantation is curative of both the liver disease and the hyperkinetic state.

Most cases of infections described after steroid treatment for severe acute alcoholic hepatitis are of bacterial origin. However, the rate of bacterial infections in these patients is not higher than in those who are not treated by steroids. The opportunistic infections are even more rare. We report two cases of patients with cirrhosis and human immunodeficiency virus, treated for alcoholic hepatitis with steroids and who subsequently developed severe pneumopathy due to Pneumocystis carinii. One patient had a concommitant cytomegalovirus infection and both of them died. Pneumocystis carinii infections usually occur in patients a decreased immune cellular response. Steroid treatments and also alcohol may be responsible for these opportunistic infections. Alcohol may have an immunosuppressive effect by decreasing recruitment of CD4 and CD8 lymphocytes to the lungs. In conclusion, Pneumocystis carinii pneumonia is a potential complication of steroid treatments for acute alcoholic hepatitis and should be suspected in case of unexplained pulmonary infection.

Objective: Liver transplantation was contraindicated in patients with diffuse thrombosis of the portal vein until the recent use of graft portal vein reperfusion with the caval flow or one of its tributaries. Long term results of these procedures are reported here., Patients and Methods: Eight patients with diffuse portal vein thrombosis were transplanted by portal reperfusion via latero-terminal anastomosis between the native caval vein and the graft portal vein (2 patients) or termino-terminal between the native left renal vein and the graft portal vein (6 patients)., Results: Three patients died 3, 3 and 6 months following transplantation from intracerebral hemorrhage, cardiac arrest, and chronic rejection respectively. Three patients had complicated portal hypertension. Five patients were alive at home with a median follow-up of 9 months (2 to 37 months) with normal liver and kinase functions., Conclusion: Portal reperfusion with the caval vein flow allows transplantation of patients with diffuse poral vein thrombosis. According to our experience and to the analysis of the literature, reno-portal anastomosis is preferable to cavo-portal reconstruction.

Aim: Liver-graft shortages justify the development of adult living-related liver transplantation. The preliminary experience with this technique at Paul-Brousse Hospital is reported. PATIENTS ET METHODES: From January to July 2000, 7 adult to adult living-related liver transplantations were performed. Donors were 5 females and 2 males aged 20 to 53 years old (median: 41). A right liver graft was harvested in all cases. Recipients were 5 males and 2 females aged from 17 to 58 years old (median: 50) transplanted for viral cirrhosis (4 cases including 2 with hepatocellular carcinoma), subfulminant hepatitis (1 case), hepatocellular carcinoma on a healthy liver (1 case), and epithelioid hemangioendothelioma (1 case). Follow-up ranged from 41 to 157 days (median: 117 days)., Results: One donor had a biliary fistula that healed spontaneously. One donor had asterixis for 24 hours. The 7 donors are alive at home without any late complications. One recipient was retransplanted for hepatic artery thrombosis and 2 recipients had a biliary fistula that healed spontaneously. The 7 recipients are alive at home with normal liver function., Conclusion: Our experience and other reports suggest that adult to adult living-related liver transplantation is feasible with rare mortality and low morbidity in donors. Results in recipients are comparable to those obtained with cadaveric grafts. For a given patient the possibility of living related donation might extend the indications for transplantation without penalizing patients waiting for a cadaveric graft.

Background: We report hare the first adult case of combined liver-small bowel transplantation performed in France., Case Report: A double liver + small bowel graft was transplanted in a 21-year-old patient hospitalized for 4 years for a short bowel syndrome requiring total parenteral nutrition. The patient also had severe hepatic fibrosis. The immediate post-operative period was uneventful. Two and one-half years after the double graft, the patient is on strictly oral nutrition, no longer has a stomy and lives a normal life in his home., Discussion: The advent of tacrolimus has led to long-term success of bowel grafts, developed earlier in children and now possible in adults. Combined liver-small bowel transplantation is formally indicated in patients with cirrhogenic liver disease associated with ineversible small bowel failure.

Objective: We report the digestive, hepatic, intestinal and nutritional history of the first combined liver-small bowel transplantation performed in France in an adult recipient. Currently, the clinical course has been favorable at 30 months., Methods: In May 1997, a 21-year-old man underwent a double liver-small bowel transplantation for irreversible chronic bowel failure with severe chronic liver disease subsequent to post-surgical short-bowel syndrome. After 28 laparotomies, there were 15 cm of small bowel left for the gastric anastomosis. The patient had a terminal jejunostomy and a left colostomy, excluding a short segment of the transverse colon and the rectum. Total parenteral nutrition including lipids and been initiated in December 1992 (3500 kcal/d) and had led to severe complications. Intestinal absorption before and after the graft were studied with the balance method (input-output) and used bomb calorimetry and measurements of the steatorhhea and creatorrhea. Functional enterocyte mass was assessed from serum citrullin., Results: The postoperative period after the liver-bowel graft (220 cm small bowel with terminal ileostomy and jejunostomy) was uneventful. The immunosuppression protocol included tacrolimus, corticosteroids and azathioprin. One mild episode of acute rejection occurred at day 26 and was controlled with a corticosteroid bolus. No episode of liver rejection occurred. Moderate renal failure regressed partially after reestablishing the fluid-electrolyte balance and adapting tacrolimus dosage. Total parenteral nutrition which had sustained the patient for 4 and a half years was definitely discontinued three months after transplantation. Oral nutrition was initially associated with enteral nutrition (from day 20 to day 90) and became exclusive three months after the transplantation. Intestinal absorption coefficients measured before tranplantation, at 3 months (2200 kcal/d, ileostomy flow 1600 g/d), and at 18 months (2400 kcal/d, ileostomy flow 1300 g/d) post transplantation were, respectively, 22%, 90% and 88% for overall calorie absorption, 25%, 65% and 73% for fat absorption, and 47%, 83% and 67% for nitrogen absorption. At 18 months post-graft, there was a spectacular improvement in the patient's neurological status and his liver function was normal. Endoscopy, radiography, histology, and immunohistochemistry explorations were normal. Ileo-rectal anastomosis was re-established at 23 months post-transplantation. At 30 months the patient is living in his home and on 100% oral nutrition., Conclusion: Clinical, nutritional and functional outcome at 30 months in this first French case of liver-small bowel transplantation in an adult recipient has been excellent.

Objectives: After hepatico-jejunostomy, endoscopic exploration of the biliary tract is not possible, and percutaneous transjejunal catheterization seems to be an attractive option., Patients: This is a 10 year prospective evaluation of 55 percutaneous transjejunal biliary interventions in 53 patients., Results: Thirty nine patients had biliary lithiasis, 10 had suspected recurrent biliary cancer, 5 biliary stenosis, and 1 angiocholitic intrahepatic abscess. Initial success was obtained in all patients and 155 procedures were performed. Interventions included strictures, dilatation, stone extraction, stent insertion and tumor biopsy. The complication rate was 15% (mainly benign biliary sepsis) with no deaths and no surgical reoperations. Thirty two of the 39 patients with biliary lithiasis had successful extraction. Eight of the 10 patients with cancer had an endoscopic biopsy and the 2 others underwent drainage. The 5 patients with benign strictures underwent dilatation and stenting. The intrahepatic abscess was treated completely by drainage., Conclusions: The feasibility of this technique, the low morbidity and the lack of mortality has been demonstrated. This technique is well accepted by patients and may be an alternative to open surgery which is known to be very difficult and risky in patients who have had one or several prior operations.

Liver disease is a rare complication of pregnancy which can be serious for both the mother and the infant. In particular, HELLP syndrome (hemolysis, elevated liver enzymes and a low platelet count) is a life threatening complication of severe preeclampsia. Pregnancies complicated by the HELLP syndrome are usually associated with increased maternal and perinatal morbidity and mortality including disseminated intravascular coagulopathy, pulmonary and cerebral oedema, acute renal failure, rupture of the liver hematoma and a variety of hemorrhagic complications. The HELLP syndrome occurs in 4 to 12% of patients with severe preeclampsia and prompt delivery is the only treatment. We report two cases of HELLP syndrome which developed in women during delivery and without any predictive factors during pregnancy.

Objectives: Patients who continue to bleed despite standard treatment including sclerotherapy have a poor prognosis with a mortality up to 90%. TIPS has been used as salvage therapy for ruptured oesophageal varices refractory to all conventional treatments., Methods: During a period of 3 years, 65 cases of variceal rupture in cirrhotic patients were treated at our center and a salvage TIPS was performed in 15 patients (23%) for active uncontrolled hemorrhage despite standard medical and endoscopic treatment (Child A, 2; B, 1; C, 12). The procedure was technically successful in all cases and hemorrhage was controlled in 11/15 cases (73%)., Results: Three patients died of persistent bleeding and liver failure; one case of moderate and persistant hemorrhage was controlled by transfusions until bleeding ceased. This patient was transplanted 3 months after TIPS and is alive 3.5 years later. Two patients had early recurrence of hemorrhage due to TIPS thrombosis. These 2 cases of thrombosis were deobstructed but both patients died of liver failure despite bleeding control. Overall, 7 patients died within 60 days of TIPS by hemorrhage and/or liver failure. One patient died of liver failure 7 months after TIPS following surgery for aortic aneurysm. None of the 8 survivors after 60 days had bleeding recurrence or encephalopathy. Actuarial survival was 42.7 +/- 14% at 1 and 2 years., Conclusion: TIPS is currently the alternative of choice for persistant bleeding refractory to standard management. However despite control of hemorrhage, operative mortality remains high due to the underlying severe cirrhosis.

Objectives: The aim of this work was to evaluate outcome in a continuous homogeneous series of more than 1000 liver transplantations in order to determine risk groups., Methods: Between November 1984 and February 1995, 1052 isolated orthotopic liver transplantations were performed in 922 patients (530 males, 392 females; mean age 41.7 years; age range 10 months - 78 years) at the Paul-Brousse Hospital liver transplantation unit. Immunosuppression was based on cyclosporin in all patients with FK506 in the most recent cases., Results: Actuarial survival at 1,5 and 8 years for the 922 patients was 80.9, 71.7, and 69.1%. Certain factors affecting the intrinsic risk of transplantation were identified and could be used to calculate supplementary risk due to one or more other risk factors. In adults under 55 years in UNOS stage 1 or 2 (not hospitalized at call in) transplanted after 1990 for non-recurrent (absence of cancer, non-viral disease) chronic liver disease, the risk of death at 1 year was 6.5% and 4.4% between the first and second year. For patients transplanted for acute liver failure and for patients transplanted for chronic liver disease in UNOS stade 3 or 4 (hospitalized or in an intensive care unit at call in), there was a supplementary risk of death at 1 year of 20.3%, 13.3% and 31.6% respectively. There was no supplementary risk of death in these three groups after 1 year. In patients over 55 years, there was a 4.4% supplementary risk during the first year after transplantation and a 2% increase between the first and second year. In patients transplanted for cancer, the supplementary risk was 9.7% during the first year, 11.6% between the first and second year and 2.1% between the third and fifth year., Conclusions: On the basis of these results, it was possible to develop a method for assessing liver transplantation outcome in different units. The proposed criteria is the 1 year survival of patients with the basic risk alone, without any supplementary risk, as well as the retransplantation index (mean number of grafts used per patient). For the Paul-Brousse unit, this criteria is 93.5% (survival at 1 year) for a retransplantation index of 1.1.

Despite the availability of whole blood cyclosporine assays, the different response of individual patients to its administration following transplantation continue to pose clinical problems, particularly in respect of toxicity. The aim of this study was to know if the inter-individual variations in the hepatic concentration of cytochrome P-450 3A, that metabolizes cyclosporine into several metabolites with very limited immunosuppressive activity, could be associated with cyclosporine toxicity. 59 consecutive liver transplant recipients were studied. Immunosuppression was with cyclosporine, azathioprine and methylprednisolone. The relative concentration of P-450 3A was assessed by immunoblot analysis using a specific monoclonal antibody on liver graft biopsy. Twelve patients experienced toxic neurological and renal complications. Six of these patients had cyclosporine levels in the therapeutic range. There was an excellent correlation between the occurrence of complications and cyclosporine whole blood levels (P < 10(-4), the first day post-op after a standard dose of cyclosporine (1 mg/kg). Cytochrome P-450 3A hepatic content assessed in a groups of 34 patients exhibited a 10-fold variation (m = 94 +/- 47 AU (Arbitrary Units)/mg). Eight of these patients who developed cyclosporine toxicity had a lower graft P-450 3A levels (m = 52 +/- 19 AU/mg, P = 3.10(-4), cf. patients with no toxicity). This highlights the importance of the first dose of cyclosporine and indicates that cytochrome P-450 3A can provide information which should allow individualized immunosuppression with cyclosporine maintaining therapeutic levels but avoiding toxicity in susceptible individuals.

The purpose of this study was to compare the results of two therapeutic methods used in an emergency to control oesophageal variceal bleeding resistant to medical treatment in cirrhotic patients: portal diversion (116 patients) or transhepatic embolization of the varices (84 patients). Portal diversion proved more effective in stopping the haemorrhage than embolization but the overall mortality rate was about the same with the two procedures. An analysis of the results taking into account the patient's condition failed to show any difference in mortality between the two methods. Indeed, the very high mortality rate in the most severely ill patients may oblige to question their usefulness in such cases. In the least severe patients, we prefer portal diversion which is quicker and can be performed at any time. In patients of the intermediate group transhepatic embolization seems to be less hazardous than portal diversion.

The relationship between liver trophicity and the early (24 h) or late (14 days) modifications of the hepatic blood flow were studied in the rat after several types of portosystemic shunts (portacaval shunt, mesocaval shunt, portacaval transposition and arterialization of the portal vein after portacaval shunt). Twenty-four hrs after portacaval shunt, the total hepatic blood flow was decreased to 60 p. 100 of the initial value and remained so during the following days. Within a week, there was a liver atrophy with a decrease of the LW/BW to 60 p. 100 of the preoperative value. Fourteen days after operation, the hepatic blood flow per gram of liver was similar to that observed preoperatively. Whatever the type of shunt, there was a significant relationship between total hepatic blood flow and liver weight, 14 days after operation. Thus, after portal diversion, the quality of the blood perfusing the liver has little incidence upon the maintenance of liver trophicity. These results suggest that hemodynamic factors are more important than hormonal (pancreatic) factors in the constitution of liver atrophy after end-to-side portacaval shunt.

We report the case of a twenty-one-year old woman using oral contraceptives who underwent partial hepatectomy for fibrolamellar carcinoma. Anti-HBs and anti-HBc antibodies were present in the serum and chronic hepatitis was associated with the carcinoma. Study of the tumor with DNA hybridization techniques showed the presence of integrated DNA sequences of the hepatitis B virus in the tumor cells. This observation underlines the possible interactions of different factors in hepatic carcinogenesis, and suggests that estrogens may possibly act as a cocarcinogenic factor associated with viral infection.

Intra-operative echography provides precise information on anatomy of the liver and accurately locates hepatic tumours, even small ones, thereby improving the operative strategy. It is most useful in biliary surgery for detection and treatment of intrahepatic lithiasis. By displaying undetected hepatic metastases, echography of the liver is of considerable value in surgery of common tumours of the digestive tract.

A medical audit was conducted in a Paris hospital on patients who, in 1975, underwent cholecystectomy for uncomplicated gallstones. The audit comprised the duration of pre- and post-operative hospitalisation, the Social Security grading of medical acts, biochemical and radiological examinations and the quality of medical care, as assessed by morbidity and mortality rates. The results were brought to the knowledge of all members of the surgical team concerned. Similar studies conducted two and five years later showed that self-restriction on the part of the physicians resulted in improved costs through shorter stays in hospital (especially before surgery) and less extensive and numerous examinations, the incidence of complications remaining the same.

Fifty-two patients operated for acute necrotising pancreatitis are reported. All severely-ill patients, operated early in order to perform a necrosectomy, died subsequently. Patients without severe illness were operated either for a complication of their pancreatic necrosis or electively for biliary lithiasis. The postoperative mortality was 29 p. 100 and 0 p. 100 respectively. Pancreatic necrosectomies were associated with a high morbidity whether performed for complications (64 p. 100) or during elective biliary surgery (33 p. 100). From this study, it appears that there is no indication for early necrosectomy in the severe forms of acute necrotising pancreatitis. However, pancreatic abscess remains a formal indication for drainage. It seems also justified to perform elective surgery without necrosectomy for biliary lithiasis complicated by acute necrotising pancreatitis.

Fulminant hepatitis was observed in a 44-year-old patient with cirrhosis, 38 days after the beginning of a treatment by disulfiram. Hepatitis was associated with fever and hypereosinophilia. Liver transplantation was performed with success. We reviewed 15 previously published cases of disulfiram-induced hepatitis. They occurred from 10 to 180 days after the beginning of the treatment by disulfiram, aminotransferases were increased whereas alkaline phosphatases were not markedly changed; there was either focal or widespread necrosis. Fulminant hepatitis was observed mainly in patients with alcoholic chronic liver disease or in patients who continued to ingest disulfiram while jaundice was already present. An immunoallergic mechanism is thought to be responsible for disulfiram-induced hepatitis.