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Paediatric liver transplantation: Queen Mary Hospital experience.

OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation.

Biliary Atresia↗

Multislice spiral CT angiography in evaluation of liver transplantation candidates.

BACKGROUND: Orthotopic liver transplantation has become the treatment of choice for patients with end-stage liver disease. This study was designed to study whether multislice spiral CT angiography (MSCTA) could be used for preoperative evaluation for orthotopic liver transplantation candidates. METHODS: Eighty consecutive potential candidates for liver transplantation were evaluated with dual-phase three-dimensional CT angiography (3DCTA). The arterial-phase was used to create vascular maps of the celiac axis (including the origin of the hepatic common artery, left gastric artery and splenic artery) and origin of the superior mesenteric artery. The portal venous-phase was used to analyze portal vein thrombosis and collateral vascularization of the portal vein. Statistical analyses were made using the chi-square test for differences between hepatic arterial anatomy of 80 patients and Michel's type I anatomy of 200 patients. Appearance of MSCTA and operative results of 16 patients were analyzed. RESULTS: Sixty-two patients (77.5%) showed conventional and 18 (22.5%) nonconventional hepatic arterial anatomy. A significant difference was found between the two groups in anatomy of the hepatic artery (P<0.05). Celiac axis stenosis was observed in 6 patients, SA aneurysm in 2, small-caliber hepatic arterial vessels in 2, and portal vein thrombosis in 15. Vascular structures of 16 operative patients were well defined. CONCLUSION: As a noninvasive examination, MSCTA can provide a comprehensive preoperative vascular evaluation for liver transplantation candidates.

Adult↗

[Extrahepatic portal vein thrombosis following laparoscopic cholecystectomy].

Postoperative portal vein thrombosis is a rare complication, which occurs most often after hepatic surgery, but has not yet been described in combination with laparoscopic cholecystectomy. We present a case where thrombotic occlusion of the portal vein was diagnosed on the 6th day following laparoscopic cholecystectomy. Treatment with high-dose anticoagulant and antibiotic therapy was successful and without further complications.

Anticoagulants↗

Thrombotic complications during L-asparaginase treatment for acute lymphocytic leukemia.

We report 2 new cases of thrombosis occurring in a cohort of 21 consecutive patients with acute lymphocytic leukemia treated with L-asparaginase (L-ase), 6,000 U/die s.c. or i.m. days 15-21 from start of chemotherapy, according to the GIMEMA LAL 0288 protocol. The first patient died of massive diffuse thromboembolism (thrombosis of sagittal sinus and of suprahepatic veins and pulmonary arteries; multiple hepatic and splenic infarctions) associated with markedly reduced levels of protein C, antithrombin III and plasminogen. In the second patient, portal vein thrombosis developed soon after the completion of L-ase. Antithrombin III was reduced, whereas protein C level was normal. Therapy with fresh frozen plasma and subcutaneous calcium heparin (12,500 U twice daily) proved successful, and 8 days later abdominal echotomography revealed the complete disappearance of the thrombus. The incidence of thrombosis is similar to that previously found in a cohort of consecutive patients treated at our Department with a different schedule and dosage of L-ase administration, and similar to that reported in previous series.

Adolescent↗

Esophageal endosclerosis in children.

During the past 6 years, 25 consecutive patients with esophageal variceal hemorrhage were treated by esophageal endosclerosis (direct injection of varices with a sclerosing agent). The primary disease in the 25 children was portal vein thrombosis (11 patients), biliary atresia (nine patients), and hepatic cirrhosis from cystic fibrosis (three patients), alpha 1-antitrypsin deficiency (one patient), and neonatal hepatitis (one patient). Thirteen patients were treated during acute, major variceal hemorrhage. Esophageal endosclerosis was repeated at regular intervals until all esophageal varices were obliterated. Twenty-one patients completed therapy. Four patients died: one of a complication of therapy and three of the primary disease. Other than the one death, complications were minor. Recurrent esophageal variceal hemorrhage has not been encountered in follow-up from 9 months to 6 years after completion of therapy.

Bile Ducts↗

[Embolization of the hepatic artery in the treatment of malignant tumors of the liver].

The results of therapeutic hepatic artery embolization (HAE) in 25 patients with malignant unresectable tumors of the liver are discussed. The proximal segments of the hepatic artery were occluded by metal spirals. Thrombosis of the portal vein was accepted as an absolute contraindication for HAE, and a tumor involving more than 50% of the volume of the liver and chronic hepatic insufficiency were considered relative contraindications. After the intervention signs of subjective improvement were noted in 73.9% and signs of objective improvement in 65.2% of cases. The mortality after HAE was 4.2%. The obtained results are evidence of the efficacy of proximal HAE as a method for the treatment of inoperable malignant tumors of the liver.

Adenoma, Bile Duct↗

Comparative study between living and cadaveric donors in pediatric liver transplantation.

UNLABELLED: We examined whether the results in living-related hepatic transplantation (LRLT) are better than those from a cadaveric donor (CDLT). MATERIAL AND METHODS: The last 27 consecutive LRLT, performed from 1998 to 2005, were compared with 27 CDLT matched for age, weight, date, and diagnosis. Grafts in LRLT group were left lateral segment (n = 22), left lobe (n = 3), and right lobe (n = 2). In the CDLT group, the grafts were split in situ (n = 10), hepatic reduction (n = 9) and whole liver (n = 8). We analyzed the actuarial survivals (grafts and children), retransplantation, primary nonfunction, initial graft malfunction (liver enzymes >2000 U/L), surgical complications, rejection, and resource consumption. RESULTS: Patient survivals at 6 months, 1 year, and 5 years were 100%, 96%, and 96% in LRLT and 100%, 100%, and 100% in CDLT (P = NS). Graft survivals were 93%, 89%, and 89% versus 96%, 96%, and 96%, respectively (P = NS). Complications were biliary complications (LRLT, 25% vs CDLT, 3%; P = .021); portal vein thrombosis (LRLT, 7% vs CDLT, 3%; NS), and hepatic artery thrombosis (LRLT, 0% vs CDLT, 3%; NS). The overall incidence of acute rejection was slightly higher (NS) in LRLT (LRLT, 18% vs CDLT, 11%; NS). Liver enzyme levels were higher in the CDLT group, but initial malfunction rate was not statistically different. Regarding resource consumption: blood product needs were higher in LRLT (P < .05) and hospital stay and ICU stay were longer, although not significantly, among LRLT. CONCLUSIONS: The results in LRLT among children are similar to those obtained in CDLT. We found a trend towards less initial graft malfunction in LRLT. Blood product needs were higher in LRLT. Hospital and ICU stay were longer, but not significantly different in LRLT. The benefits of LRLT are saving a scarce resource: a cadaveric donor liver graft.

Body Weight↗

Living related liver transplantation in infants and children: report of anesthetic care and early postoperative morbidity and mortality.

STUDY OBJECTIVE: To determine those infants at high risk for perioperative complications and mortality following living, related liver transplantation. DESIGN: Retrospective chart review. SETTING: Large metropolitan teaching hospital. MEASUREMENTS AND MAIN RESULTS: The charts and anesthetic records of the 12 infants and children who received the left lateral hepatic segment from a living relative the past 2 years at our institution were reviewed. The records were examined to determine the causes of perioperative morbidity and to identify patients at high risk for serious complications and mortality. All infants and children (mean +/- SD age, 29+/-30 months; weight, 13.6 +/-6.8 kg) survived the operation (8.3+/-1.7 hours) without intraoperative complications. The average blood loss, including 500 mL of recipient blood used to flush the liver before reperfusion, was 1483 +/-873 mL (119+/-70 mL/kg). Three infants developed portal vein thrombosis, and one of these infants also had hepatic artery thrombosis. The risk of vessel thrombosis was significantly higher (3/3 vs. 0/9; p<0.0045) in infants less than 9 kg body weight, as was the risk of death (2/3 vs. 0/9; p<0.045). Both children who died had vascular thrombosis. Other serious complications were bleeding, 6; infection, 7; acute rejection, 3; and bile leak, 2. CONCLUSIONS: Infants and children can successfully undergo living, related liver transplantation. However, the risks of vascular complications and death are greater in infants less than 9 kg body weight.

Anesthesia↗

[Budd-Chiari syndrome with medullar compression].

We report a case of Budd-Chiari syndrome caused by protein C deficiency. The association of thrombosis of the the inferior vein cava of the hepatic vein resulted in the development of epidural varices and a central and peripheral neurological syndrome. These symptoms, which are atypical in this disease, resolved after surgical treatment of the Budd-Chiari syndrome (cavoatrial and mesocaval stenting).

Adolescent↗

A comparative study of functional assays for tissue factor pathway inhibitor using normal plasma and clinical samples.

Tissue factor pathway inhibitor (TFPI) is a Kunitz-type inhibitor that regulates the initiation of tissue factor-mediated coagulation. Recent reports in the literature have described variable results using different methodologies for TFPI measurement. In this study, we used one clotting and two amidolytic methodologies to assess TFPI functional levels. The study groups included normal healthy donors as well as patients with acute hepatitis, diabetes, coronary artery bypass graft operations, deep vein thrombosis, and prior to and during heparin therapy. The aims were to compare the results obtained in normal plasma using different assay systems, to compare TFPI levels in a range of clinical samples, including those previously not determined using a clotting methodology, and to report TFPI levels in patient groups previously not investigated. The results clearly demonstrate poor correlation between functional TFPI values using the different methodologies, highlighting the requirement for standardization.

Biological Assay↗

Immunotherapy of hepatocellular carcinoma with autologous lymphokine-activated killer cells and/or recombinant interleukin-2.

Five patients with hepatocellular carcinoma were subjected to immunotherapy: three patients were treated by adoptive immunotherapy with lymphokine-activated killer (LAK) cells and recombinant interleukin-2 (rIL-2), and two patients by systemic administration of rIL-2 alone. In one patient with diffuse-type hepatocellular carcinoma and portal vein thrombosis who was treated by infusion of LAK cells (a total number of 1.5 x 10(10) cells/13 doses) and continuous rIL-2 administration (a total dose of 1.25 x 10(8) units) via a percutaneously placed hepatic arterial catheter, the size of the tumor reduced dramatically and the portal vein thrombosis retracted. In two patients who had LAK cells infused (totals of 6.6 x 10(9) cells/4 doses and 3.1 x 10(9) cells/2 doses, respectively) during hepatic angiogram followed by systemic administration of rIL-2 twice a day, no clinical improvement was noticed. In two patients who received rIL-2 alone systemically (total doses of 8.9 x 10(7) and 5.5 x 10(7) units, respectively), neither clinical improvement nor severe side effects were observed. The results suggest that adoptive immunotherapy combined with continuous local administration of rIL-2 via a percutaneously placed hepatic arterial catheter may be an effective therapy without apparent side effects for patients with hepatocellular carcinoma who cannot be treated by conventional cancer therapy.

Adult↗

[Multidisciplinary treatment for advanced hepatocellular carcinoma with portal vein tumor thrombosis and adrenal/lung metastases].

The patient was a 73-year-old male who was identified with an increase of serum PIVKA-II during a treatment for chronic hepatitis B. Hepatocellular carcinoma (HCC) of 60 mm in diameter with satellite nodules was diagnosed in segment 8 of the liver. In addition, portal vein tumor thrombosis (PVTT) of the right branch (Vp3) and metastases to bilateral lung and right adrenal gland were recognized. He received serial treatments with transcatheter arterial chemoembolization (TACE), radiation therapy and hepatic arterial chemotherapy with reservoir for primary liver tumor and PVTT. Soon after the treatments, PVTT was reduced in size and the serum level of PIVKA-II was decreased to 57 mAU/ml. After three months, the level of PIVKA-II had increased again and the size of the right adrenal metastasis grew to 50 mm in diameter. He received TACE to the right adrenal metastasis and percutaneous transhepatic portal chemoembolization to prevent further growth of PVTT. In spite of several treatments, the therapeutic effect was insufficient. Therefore, we performed right adrenalectomy and radio-frequency ablation of HCC in the liver S8. After the surgery, he received two times of TACE and the viable tumor had disappeared on CT and MRI. Prognosis of HCC with PVTT and distant metastasis is very poor. The two-year survival rate is less than 10%. However, it is possible to improve the prognosis of advanced HCC by multidisciplinary treatment with surgical intervention, local chemotherapy and radiation therapy.

Adrenal Gland Neoplasms↗

Surgical strategy in late-stage renal cell cancer with intracaval extension.

During the past two years several reports have substantially contributed to the surgical stratification of patients with late-stage renal cell cancer and simultaneously occurring intracaval neoplastic extension, and our knowledge about the clinical prognosis of the patients after nephrectomy with complete removal of the tumour thrombosis has been extended. Recognizing the importance of a tolerable perioperative and postoperative morbidity and mortality, it is now well documented in the literature that a multidisciplinary approach involving the combined use of deep hypothermia, cardiac arrest and cardiopulmonary bypass has dramatically improved the feasibility of surgical treatment of the venous extension above the hepatic veins. Furthermore, the diagnostic approach for patients with intracaval thrombosis, aiming at the reliable delineation of the cranial margin of venous extension, has been rationalized without compromising important diagnostic information required for the surgical stratification. This review assesses the current opinion of the surgical stratification of patients with renal cell cancer and intracaval neoplastic extension, the most reliable and least invasive diagnostic approach for the preoperative staging and, finally, the independent prognostic value of neoplastic venous extension for the clinical course of the disease.

Journal Article↗

Ultrasound-guided fine needle aspiration biopsy of portal vein thrombosis in liver cirrhosis: results in 15 patients.

Between 1988 and 1992 ultrasound-guided fine needle aspiration biopsies of thromboses in the main branches of the portal vein were carried out in 15 patients with liver cirrhosis. The aims of the study were to evaluate the usefulness, feasibility and diagnostic accuracy of this procedure in cirrhotics with known or suspected hepatocellular carcinoma. The procedure was carried out only in patients with a platelet count > or = 40,000/microL and prothrombin activity > or = 40%. A single pass, with a 22 gauge spinal needle, was performed in the portal vein lumen. Diagnosis of the aetiology of the portal vein thrombosis was obtained in all 15 cases. In 12 cases, a cytological diagnosis of hepatocellular carcinoma was made. In one case, the neoplastic cells aspirated were compatible with adenocarcinoma, and a subsequent colonoscopy confirmed the presence of colonic cancer. The material aspirated was compatible with chemically-induced thrombosis in one patient who had undergone several percutaneous ethanol injection sessions for treatment of hepatocellular carcinoma, and in the last case only blood was aspirated, thus ruling out the coexistence of hepatic cancer. We conclude that fine needle aspiration biopsy of portal vein thrombosis is a feasible, low risk procedure that facilitates the diagnosis of hepatocellular carcinoma when fine needle biopsy of focal liver lesions fails. Fine needle aspiration biopsy of portal vein thrombosis is also useful in excluding neoplastic aetiology of portal vein thrombosis.

Adenocarcinoma↗

Alveolar echinococcosis of the liver: sequelae of chronic inferior vena cava obstructions in the hepatic segment.

BACKGROUND: The clinical pattern and long-term course of chronic inferior vena cava (IVC) obstructions are variable and depend on the underlying cause, the segment involved, and the extension of secondary thrombosis. Pertinent data on IVC obstructions in well-defined series of patients are lacking. We report the sequelae of chronic IVC obstructions in the hepatic segment in 11 consecutive patients derived from a cohort of 104 patients with alveolar echinococcosis of the liver. METHODS: Based on the results of computed tomography scans, 11 patients (7 men, 4 women; mean age, 53.4 years) with IVC obstructions were selected from an ongoing prospective long-term chemotherapy trial comprising 104 patients with alveolar echinococcosis studied at yearly intervals according to a protocol. Obstruction of the IVC in the 11 patients existed for a mean duration of 8.6 years. In these patients, magnetic resonance imaging was performed to assess the morphologic features and extension of the IVC obstruction, as well as the collateral venous pathways. Patency and valvular function of the femoropopliteal veins were analyzed by color-coded duplex ultrasonography. RESULTS: Total occlusions of the IVC were evident in 8 patients (73%) and subtotal stenoses in 3 patients (27%). Only 4 patients (36%) exhibited signs and symptoms of chronic venous insufficiency of the lower extremities; 2 (18%) of the 4 had a history of swelling in the lower extremity. Seven patients (64%) had no lower extremity symptoms. One patient had a history of pulmonary embolism. Abdominal collateral veins were documented in 5 patients (45%) by using magnetic resonance imaging; however, they were clinically evident in only 3 patients (27%). In the 8 patients with IVC occlusion, thrombosis ended at the confluence of the hepatic veins. Obstruction of the IVC was limited to the hepatic segment in 2 patients (18%) and extended to the distal IVC or the iliofemoral veins in 6 patients (54%). Chronic venous insufficiency was present only if the femoropopliteal veins had been involved in the thrombotic process, showing residual venous obstruction, valvular incompetence, or both. Bilateral renal vein thrombosis with moderate proteinuria was observed in 2 patients (18%). The main collateral drainage was achieved through the ascending lumbar, azygos, and hemiazygos veins. CONCLUSIONS: In patients with alveolar echinococcosis, obstruction of the IVC in the hepatic segment often develops asymptomatically and rarely leads to the impairment of renal function. The collateral circulation fully compensates for obstruction of the IVC. Thrombotic involvement and valvular incompetence of the femoropopliteal veins seems to determine the development of chronic venous insufficiency of the lower extremities.

Adult↗

Color Doppler findings of gastrointestinal varices.

BACKGROUND: Compared with esophageal varices, gastrointestinal varices are relatively rare, but they are clinically important because they tend to bleed massively. Color Doppler sonography is now widely used to diagnose the collaterals, but few color Doppler findings of gastric or intestinal varices have been reported. The aim of this study was to investigate the sonographic and color Doppler findings of gastrointestinal varices and to determine the role of color Doppler sonography in the diagnosis of these varices. METHODS: We studied 30 patients who were diagnosed by endoscopy as having gastrointestinal varices (24 gastric, four duodenal, two intestinal) with color Doppler sonography and compared the results with the clinical data. The causes of gastric varices included liver cirrhosis (16/24, 66.7%), idiopathic portal hypertension (3/24, 12. 5%), chronic pancreatitis with splenic vein thrombosis (2/24, 8.3%), congenital biliary atresia (1/24, 4.2%), congenital hepatic fibrosis (1/24, 4.2%), and unknown (1/24, 4.2%). The causes of duodenal varices included idiopathic portal hypertension with portal thrombosis (3/4, 75%) and liver cirrhosis (1/4, 25%). RESULTS: The gastric wall at the fundus was thickened in 17 of 24 cases (70.8%) with gastric varices, and the duodenal wall was thickened in four of four cases (100%) with duodenal varices. Sonography revealed thrombosis in the splenic vein in two of two cases with gastric varices secondary to chronic pancreatitis and in the confluence of the superior mesenteric vein and the splenic vein in three of four cases with duodenal varices. Color Doppler sonography demonstrated multiple, slow constant blood flows in the thickened wall in 15 of 24 cases (62.5%) with gastric varices and in four of four cases (100%) with duodenal varices. It demonstrated accumulated slow constant blood flows in the cecum in the case with cecal varices. Color Doppler showed also the communication between the varices and the neighboring vascular system (superior mesenteric vein and inferior vena cava) in the case with cecal varices, but it did not directly reveal such a communication in the other 29 cases (96.7%). Color Doppler showed a hepatofugal flow in the left gastric vein in all the hemorrhagic gastric varicose patients with esophageal varices, but it showed a hepatopetal flow in the left gastric vein in the isolated nonhemorrhagic gastric varicose patients. CONCLUSION: Color Doppler sonography was very useful for the diagnosis of gastric and duodenal varices and for visualizing fine venous flows in the thickened gastric or duodenal wall. When it shows portal thrombosis in the confluence of the splenic vein and the superior mesenteric vein, duodenal varices should be suspected. The flow direction of the left gastric vein helps to differentiate hemorrhagic gastric varices from nonhemorrhagic ones.

Adolescent↗