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Ultrasonography. Update on liver technique.

Sonography is very useful in the evaluation of the patient with severe liver disease before and after the placement of a TIPS, and before and after organ transplantation. Efforts to use ultrasound to evaluate for primary and metastatic lesions to the liver have been reviewed. With its Doppler and color flow capabilities, ultrasound remains an important modality for hepatic imaging, especially in the evaluation of portal vein patency and hepatic artery thrombosis.

Hepatic Veno-Occlusive Disease↗

Ultrasonography of the liver. An update on new applications.

Sonography is highly useful in evaluating the patient with severe liver disease before and after the placement of a TIPS and before and after organ transplantation. Efforts to use ultrasound imaging to evaluate for primary and metastatic lesions to the liver have been reviewed. With its Doppler and color flow capabilities, ultrasound imaging will remain an important modality for hepatic imaging, especially in the evaluation of portal vein patency and hepatic artery thrombosis.

Humans↗

Budd-Chiari syndrome complicating pre-eclampsia: diagnosis by magnetic resonance imaging.

A 25-year-old primigravida with third-trimester pre-eclampsia developed severe right upper quadrant pain, marked serum aminotransferase elevation, and disseminated intravascular coagulation after cesarean delivery. Computed tomography of the liver showed a right hepatic abnormality that on magnetic resonance imaging had the appearance of hemorrhage; partial thrombosis of the right hepatic vein extending to the inferior vena cava was also seen. Anticoagulation was begun and the patient completely recovered; follow-up imaging 8 months later was normal. This case suggests that nonfatal forms of Budd-Chiari syndrome may complicate pre-eclampsia; predisposition to partial venous outflow obstruction in the pre-eclamptic patient may be pathogenetically related to disseminated intravascular coagulation. This entity may be clinically confused with, or misdiagnosed as, spontaneous hepatic hemorrhage, with or without capsular rupture. In such circumstances, magnetic resonance may be diagnostically useful.

Adult↗

Portosystemic shunting in children during the era of endoscopic therapy: improved postoperative growth parameters.

BACKGROUND: Surgical portosystemic shunting has been performed less frequently in recent years. In this retrospective study, recent outcomes of portosystemic shunting in children are described, to evaluate its role in the era of endoscopic therapy. METHODS: Retrospective chart review of children who underwent surgical portosystemic shunt procedures between October 1994 and October 1997. RESULTS: Twelve children (age range, 1-16 years) underwent shunting procedures. The causes of portal hypertension were extrahepatic portal vein thrombosis (n = 6), congenital hepatic fibrosis (n = 2), hepatic cirrhosis (n = 2), and other (n = 2). None of the patients were immediate candidates for liver transplantation. Types of shunt included: distal splenorenal (n = 10), portocaval (n = 1), and other (n = 1). Median follow-up was 35 months (range, 24-48 months). All patients are currently alive and well with patent shunts. The mean hospital stay was 8 days. Three patients required readmission for further interventions because of shunt stenosis in two and small bowel obstruction in the other. Mild portosystemic encephalopathy was seen in one child with pre-existing neurobehavioral disturbance. Excluding a patient who underwent placement of a portosystemic shunt for a complication of liver transplantation, mean weight-for-age z score in nine prepubertal patients improved from -1.16 SD to +0.15 SD (P = 0.023), and mean height-for-age z score from -1.23 SD to 0.00 SD (P = 0.048) by 2 years after surgery. CONCLUSIONS: Surgical portosystemic shunting is a safe and effective method for the management of portal hypertension in childhood. Patients show significant improvements in growth parameters after the procedure. Surgical portosystemic shunting should be actively considered in selected children with portal hypertension.

Adolescent↗

The role of imaging in patients with ascites.

Imaging plays a significant role in patients with ascites for assessing the size and the causes of ascitic fluid, for assisting sampling or draining of ascitic fluid, for selecting candidates for TIPS placement, and for surveilling and detecting complications after TIPS placement. Sonography and CT are primary imaging tools, and MR imaging is used in selected cases such as demonstration of peritoneal or ascitic fluid enhancement, particularly in patients with compromised renal function. Interventional procedures combined with CT and sonography play a major role in the assessment of patients considered for TIPS placement because of refractory esophageal bleeding or ascites. Before the TIPS procedure, these procedures are used to exclude contraindications such as polycystic liver disease, hypervascular hepatic tumors, and portal vein thrombosis. After the TIPS procedure, sonography combined with interventional procedures is used to detect immediate complications such as hemorrhage or intractable shunt-induced encephalopathy due to excessive portosystemic diversion and to detect and treat mid- to long-term complications such as shunt stenosis and occlusion through a routine surveillance program.

Ascites↗

Prognosis following transcatheter arterial embolization for 121 patients with unresectable hepatocellular carcinoma with or without a history of treatment.

AIM: To retrospectively evaluate the prognosis of patients with hepatocellular carcinoma (HCC) with or without a history of therapy for HCC following transcatheter arterial embolization (TAE). METHODS: One hundred and twenty-one patients with HCC treated with TAE from 1992 to 2004 in our hospital were enrolled in this study. Eighty-four patients had a history of treatment for HCC, while 37 did not. At the time of entry, patients with extra-hepatic metastasis, portal vein tumor thrombosis, or Child-Pugh class C were excluded. TAE was repeated when recurrence of HCC was diagnosed by elevated tumor markers, or ultrasonography or dynamic computed tomography findings. RESULTS: Tumor size was larger and the number of tumors was fewer in patients without past treatment (P<0.01). However, there were no differences in tumor node metastasis (TNM) stage or survival rate between the 2 groups. A bilobular tumor and high level of alpha-fetoprotein (AFP) (>100 ng/mL) were factors related to a poor prognosis in patients with a history of HCC. CONCLUSION: The prognosis following TAE is similar between HCC patients with and without past treatment. Early diagnosis of HCC or recurrent HCC and obtaining good local control against HCC before entry to a repeated TAE course can improve prognosis.

Aged↗

[Expanding the grafts pool for pediatric liver transplantation with segmental grafts from living donor and cadaveric reduced size or split grafts].

AIM: To compare short and long-term results of pediatric liver transplantation (LT), utilizing segmental grafts from living donors (LD) vs. cadaveric (CAD) reduced size or split grafts. METHODS: A retrospective analysis of a single center experience (1993-2000), comparing the surgical outcome, the graft function and the survival rates between these groups. RESULTS: Of 195 LTs in pediatric recipients (age < 18), 48 (25%) were with LD grafts and 47 (24%) with CAD grafts (reduced size, n = 27, or split, n = 20). The mean age and weight of the LD recipients were 1.8 +/- 3 yrs and 9.0 +/- 8.1 kg vs. 3.5 +/- 4 yrs. and 15.2 +/- 14.5 kg in the CAD group. The distribution of etiologies was comparable (EHBA, 54% vs. 49%; inborn errors in metabolism, 12.5% vs. 12%; acute idiopathic hepatic failure, 12.5% vs. 14.2%). The severity of pretransplant disease and the fraction of acute hepatic failure cases were also comparable, although less LD grafts were used for urgent re-transplantation due to primary non-function or vascular complications (1 case in the LD group vs. 6 in the CAD group). The median warm ischemia time was similar (43 min; range, 28-87 min vs. 45 min; range 12-82 min), but the median cold ischemia time was significantly different (60 min; range 43-298 in LD vs. 637 min; range, 342-1102 in CAD grafts). Both patient and graft survival in 3 months, 1 and 5 years were significantly superior in the LD group (patient survival, 97%, 91% and 89% vs. 82%, 70% and 62%, p < 0.001; graft survival, 92%, 89% and 77% vs. 66%, 59% and 52%, p < 0.005). The incidence of vascular complications (hepatic artery or portal vein thrombosis) and biliary complications (leak of stricture) was comparable (vascular, 10% vs. 7%, biliary, 16% vs. 9%). The incidence of poor early graft function (6% vs. 21%) and primary non-function (2% vs. 18%) was significantly lower in the LD group. CONCLUSION: Although presenting similar surgical complexity, the outcome of segmental grafts from LD is better than of reduced size or split cadaveric grafts.

Adolescent↗

Interventional or semi-interventional treatment for Budd-Chiari syndrome.

OBJECTIVE: Report the results of interventional or semi-interventional techniques for 173 patients with Budd-Chiari syndrome. METHOD: This group included 120 males and 53 females. The pathologic lesions composed of localized complete occlusion of inferior vena cava (IVC) (78), IVC stenosis (49), IVC membrane with a hole (37), membrane of hepatic vein (HV) (3), IVC thrombosis (4), IVC membrane with thrombosis (2) and IVC lesion with occlusion of HV (32). Treatment methods included that I: Percutaneous transinferior vena cava angioplasty (PTA) (76); II: IVC PTA with stent (59); III: Percutaneous transhepatic vein recanalization (3); IV: IVC thrombolysis through a catheter (4), V: Combined transcardiac and transfemoral venous membranotomy and balloon dilation (22); VI: V and stent (17); VII: Stenting during radical surgery (3); VIII: Additional operation after intervention (23). RESULTS: The immediate technique success rate for intervention was 90.1%, for the semi-intervention was 100%. The IVC pressure was reduced from 3 to 29 cmH2O. Complications occurred in 8 cases. The death rate was 2.9%. A follow-up study showed the recurrence rates were 14.5% in IVC PTA group, 1.7% in IVC PTA with stent, 18.2% in combined technique without stent and no recurrence was found in other groups. CONCLUSION: The PTA is the first choice for localized lesions. When elastic recoil occurs, immediate stenting is suggested. The semi-interventional approach is advised for PTA failure and more complicated cases. For those with both IVC lesion and occlusion of HV, the additional operation is needed after IVC intervention.

Adolescent↗

[Portal and splenic veins thrombosis reveling a miliary tuberculosis of the liver].

The etiologies of the portal vein trombosis are dominated by the neoplasic forms with hepatocellular carcinoma; we report a rare case of portal and splenic veins thrombosis revealing a liver military tuberculosis occuring in a HIV 1 infected patient. A 42 years old senegalese woman with no personal or family history of thrombosis was admitted for abdominal upper right quadran, and epigastric pain, with fever and important weight loss. Ultrasound identified endoluminal echogenic images in the portal and splenic veins. There were no lymph nodes or liver tumor. Evaluations of proteins C and S were normal and there was no anticardiolipin antibody. In searching the aetiology of the thrombosis, a liver biopsy was performed, and showed a miliary tuberculosis. an HIV 1 infection was later on diagnosed. The antituberculosis treatment associated with heparine therapy was successful, the thrombosis resolved entirely. This portal and splenic veins thromboses occuring on a miliary tuberculosis of the liver seems to be an exceptional situation. We did not found in the literature a similar case.lt points out the interset of liver biopsy in searching the aetiology and the early heparine therapy set up

Adult↗

Treatment of the Budd-Chiari syndrome with orthotopic liver transplantation and long-term anticoagulation.

The Budd-Chiari syndrome (BCS) with hepatic vein occlusion is a rare disorder that can effectively be treated with orthotopic liver transplantation. In this retrospective analysis we report on 7 patients who received 9 liver grafts for terminal BCS. One patient died after 4 months due to cytomegalovirus-pneumonia. The actuarial survival rate is 85.7% compared to more than 90% in all other 376 patients transplanted between September 1988 and April 1993 at our institution. Anticoagulation management consisted of early postoperative intravenous heparin and continuation with dicoumarin. One patient with thrombocytosis received hydroxyurea. Under this regimen one postoperative hemorrhage requiring laparotomy was observed. Discontinuation of oral anticoagulation due to gastrointestinal bleeding caused hepatic artery and portal vein thrombosis in 1 patient who had to be retransplanted. One recurrence requiring retransplantation as well was due to an insufficient dicoumarin intake. In conclusion, terminal BCS represents a good indication for orthotopic liver transplantation with a closely-monitored anticoagulation to avoid such adverse side effects as thrombosis and hemorrhage.

Adolescent↗

[Results of hepatic transplantation from donors older than 50 years of age. Comparison with other age groups].

We have performed 284 OLT and from 1990 we accept donors older than 50 years. We analyzed 100 consecutive OLT from 1990 in relation to donor age and we divided them in three groups: A) 10 liver transplants with donors < 16 years old; B) 76 liver transplants with donors between 16 and 49 years old, and C) 14 liver transplants with donors > 49 years old. The follow-up was more than 6 months. The liver preservation was carried out with Belzer solution and the immunosuppression was performed with cyclosporine A, corticoids and azathioprine. The C group donors were more stable haemodynamically (less dopamine dosage-p < 0.001- and less hours with arterial hypotension-p < 0.05-) and they died less of craneoencephalic traumatism than A group donors (p < 0.01). The C group donors didn't have more frequence of liver esteatosis or greater graft harvest lesions (p = n.s.). The cold ischaemia time was greater in C group vs A (p < 0.05) and its pretransplant clinical status was more serious than in A and B groups (p < 0.05). There were no significant differences between groups in relation to recipient status, frequence of hepatic artery or portal vein thrombosis, frequence of acute rejection, chronic rejection or biochemical graft evolution at first month. There were more primary graft non functions in B vs A (p < 0.05) and retransplants in B vs A and C (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗

[After-care by the internal medicine physician following liver transplantation].

Orthotopic liver transplantation offers the only therapeutic option for many patients with end-stage liver disease. In adults, the overall five-year survival following transplantation has increased dramatically from approximately 30% a decade ago to nearly 70% currently. Cyclosporine A became available in the 1970s and substantially improved immunosuppressive therapy. It is now the mainstay of the therapeutic approach to prevent graft rejection. The postoperative care of liver transplant recipients is one of the most exciting challenges in clinical medicine. On the basis of the experience and results of the Innsbruck liver transplant programme the management of liver transplant recipients will be discussed with regard to the typical time frame of complications. We present data of 87 liver transplantations in 84 patients during a 10-year observation period. At present, intraoperative mortality is fortunately close to zero. Most of the deaths occur in the immediate postoperative period. Major complications are haemorrhage, hepatic artery or portal vein thrombosis, biliary leakage or graft dysfunction. In addition, side-effects resulting from high-dose immunosuppressive therapy or from antibiotic and antiviral therapy add to problems in this early period. Opportunistic infections, chronic graft failure, disease recurrence and nephrotoxicity related to Cyclosporine A therapy are complications seen during long-term management of liver transplant patients. The differential diagnosis and the adequate management of these complications are a great challenge to every hepatologist.

Aftercare↗

Liver transplantation at the University of Chicago.

Over the past 5 years, we have employed several strategies to increase the donor pool for both the pediatric and adult populations. The innovative expansion of the donor pool with the use living-related donors for children and cadaveric, high-risk donors for adults has increased our ability to serve our recipients and transplant them at an earlier stage in the disease process, thereby improving survival. As Hepatitis C is now the leading indication for liver transplantation in the adult population, the investigation of the natural history of Hepatitis C prior to and after transplantation provides a major challenge and is currently a focus of both laboratory and clinical efforts. For those recipients of Hepatitis C-positive-donor livers, determining the role of recipient and donor genotypes in the progression of recurrent hepatitis should help define the proper utilization of these organs. For patients on CsA-based immunosuppression regimens who experience steroid-resistant rejection, tacrolimus has proved to be extremely effective in reversing the rejection episodes and maintaining normal graft function. The long-term results of this therapy appear to be superior to OKT3 therapy. The recipients of living-related liver transplantation continue to have a survival advantage in comparison to recipients of cadaveric grafts. The donor operation can be routinely performed with minimal risk. Because of the superior results achieved and minimal donor risks, we feel that providing the option of living-donor transplantation is ethically justified, and medically necessary. Despite the encouraging results from living-donor transplantation, unexpected complications including portal vein complications and hepatic artery thrombosis have forced technical modifications of the original technique which may have implications to pediatric liver transplantation in general. As the volume of pediatric liver transplants and the number of immmunosuppressive regimens have increased over the years, posttransplant lymphoproliferative disease has been identified as a problem which requires more inspection. We have determined that the severity of rejection and the subsequent treatment, and primary Epstein-Barr virus are the primary risk factors for developing of PTLD. Identification of the risk factors and early detection may provide some hope for treatment of the disease while allowing long-term graft function. Results of our preliminary data show that, following transplantation, 3/4 of the children or parents report minimal impairment with regard to developmental or physical milestones. Patients and their families, however, continue to report significant levels of stress in their lives and occasional pain. Further research on outcome needs to be performed on our pediatric recipients to ensure the long-term benefit of our efforts.

Academic Medical Centers↗

Branch retinal vein thrombosis and visual loss probably associated with pegylated interferon therapy of chronic hepatitis C.

Ophthalmological complications with interferon therapy are usually mild and reversible, not requiring the withdrawal of the treatment. We report a case of a patient who had visual loss probably associated with interferon therapy. Chronic hepatitis C virus infection (genotype 1a) was diagnosed in a 33-year old asymptomatic man. His past medical history was unremarkable and previous routine ophthalmologic check-up was normal. Pegylated interferon alpha and ribavirin were started. Three weeks later he reported painless reduction of vision. Ophthalmologic examination showed extensive intraretinal hemorrhages and cotton-wool spots, associated with inferior branch retinal vein thrombosis. Antiviral therapy was immediately discontinued, but one year later he persists with severely decreased visual acuity. This case illustrates the possibility of unpredictable and severe complications during pegylated interferon therapy.

Adult↗

Techniques for and outcome of liver transplantation in neonates and infants weighing up to 5 kilograms.

BACKGROUND: Neonates and small infants represent less than 5% of paediatric candidates for liver replacement. Most cases present under urgent conditions and receive grafts from large donors. Surgical techniques must be adapted for adequate graft preparation, vascular reconstruction, and abdominal closure. METHODS: Technical aspects and outcome of 15 liver transplantations in infants weighing less than 5 kg performed at our unit were analysed retrospectively. RESULTS: Liver transplantation was performed under urgent or highly urgent condition in 13 cases. Reduced or split liver grafts were used in all cases (median donor to recipient weight ratio, 9), including a monosegmental graft in 2 cases. In 10 cases, vascular reconstruction was done using a vascular conduit (5, 4, and 1 for artery, portal, and hepatic veins, respectively) and a delayed closure of the abdomen was necessary in 7 children. Postoperative complications were as follows: thrombosis of hepatic artery (n = 1) or portal vein (n = 1), gastrointestinal haemorrhage (n = 2), intraperitoneal bleeding (n = 1), biliary stricture (n = 2), septicaemia (n = 1). Two infants died of brain damage with a functioning graft. One child underwent retransplant for chronic rejection. CONCLUSIONS: Overall, survival rate is 60% (median follow-up, 34 months), which compares favourably with older patient groups when case mix is comparable.

Age Factors↗