Syndromic incidence of ovarian carcinoma after liver transplantation, with special reference to anteceding breast cancer.

Ovarian cancer is the gynecologic malignancy with the highest number of deaths in the United States. Previous studies had found a decreased incidence of female gynecological malignancies after liver transplantation. In order to estimate the incidence of ovarian carcinoma after liver transplantation, we evaluated 1708 consecutive liver transplant recipients from 1984 to 2001. Of them, 770 (43%) were female. Routine follow-ups were performed at 1, 2, 5, and 10 years after transplantation. There were two cases of ovarian carcinoma. Both occurred in recipients with a previous history of breast cancer. Based on these data, we conclude that the incidence of ovarian cancer is 1:385 among all female liver transplant recipients, and 1:6.5 among those with a history of pretransplant breast cancer. We recommend that regular check-ups should be undertaken, especially in the population at highest risk.

Unusually high incidence of positive HTLV I/II results among young female organ donors in the peripartum period.

HTLV I and II are unusual retroviruses associated with multiple neurologic and hematologic disorders. We observed an unusually high incidence of HTLV I-II seropositivity among young and middle-aged female organ donors, especially among those in the peripartum period. Ethical issues may arise when informing the families as well as when deciding whether to use organs from these donors. Further confirmatory tests may be difficult to obtain because of time and economic constraints associated with organ procurement.

Correlation between intraoperative blood flows and hepatic artery strictures in liver transplantation.

Hepatic artery strictures (HASs) may be a source of morbidity and mortality in liver transplant recipients. This study evaluated the potential correlation between intraoperative arterial and venous blood flows measured after implantation of the liver allograft and the occurrence of postoperative HASs requiring repair. Prospectively collected data from 1,038 patients with complete data sets who underwent initial orthotopic liver transplantations between December 1984 and December 1999 were used. Electromagnetic flow measurements were routinely obtained in these cases. Hepatic artery and portal vein patency were reassessed routinely according to our protocol in the first postoperative day by Doppler ultrasound. When considered hemodynamically significant, strictures were corrected. There was a 6.2% incidence (n = 64) of hepatic artery stenosis in our transplant population. When considered as a whole, the hepatic artery stenosis group had lower intraoperative flow volumes than transplant recipients who did not develop strictures (mean flows, 452 v 518 mL/min, respectively; P =.025). The hepatic artery stenosis group also had lower intraoperative portal vein flows compared with the group without hepatic artery stenosis (1.80 v 2.11 L/min, respectively; P =.0043). Strictures were less frequent among transplant recipients with cryptogenic cirrhosis. We did not observe differences among the groups for retransplantation or patient and graft survival. In our series, there was a 6.2% incidence of postoperative HASs. We observed a significant association between intraoperative hepatic artery and portal vein flows and postoperative HASs.

Incidence and recurrence of autoimmune/alloimmune hepatitis in liver transplant recipients.

We prospectively collected data on 1,429 liver transplant recipients between December 1984 and December 1998. Fifty-five patients (3.8%; 10 men, 45 women; median age, 44.5 +/- 13 [SD] years) with autoimmune hepatitis (AIH) underwent orthotopic liver transplantation (OLT). Transplant recipients with AIH were younger, more likely to be women, and had a greater likelihood of rejection in the first 3, 6, and 12 months. There was no difference in patient survival or graft survival. There were 11 biopsy-proven recurrences (1 man, 10 women) of AIH after OLT. Almost half the episodes occurred within the first year after OLT. No patient required re-OLT because of recurrent disease. AIH has an incidence of 4% and a recurrence rate of 20% in OLT. Transplant recipients are more likely to be young women and have an increased incidence of acute cellular rejection (ACR) during the first post-OLT year. Recurrence should be suspected in those with abnormal liver function test results in the absence of ACR, especially during the first year after OLT. We cannot establish with certainty whether the observed process represents recurrence of the original autoimmune disease, an alloimmune phenomenon, or allograft dysfunction mimicking AIH.

Liver transplantation for cholestasis associated with cystic fibrosis in the pediatric population.

The most common hepatic complications of cystic fibrosis (CF) are steatosis, fibrosis, biliary cirrhosis, atretic gallbladder, cholelithiasis, and sclerosing cholangitis. Cholestatic liver disease is a slow progressive disorder, but will stabilize for many patients. CF patients may suffer from the consequences of their liver disease and without liver transplantation, variceal hemorrhage, malnutrition, or end-stage liver disease can lead to death. Prospective data were collected and reviewed on 311 liver transplants performed in 283 patients at the Children's Medical Center of Dallas between October 1984 and November 2000. Ten children received an orthotopic liver transplant (OTLX) for end-stage liver disease associated with cystic fibrosis. Pulmonary function tests were obtained preoperatively in all cases. There were nine boys and one girl. Six are currently alive, and four are dead. Both patient and graft survival was 5.75 yr. Among those currently alive, mean patient and graft survival is 7.71 yr (range 0.10-12.62 yr). Mean patient and graft survival of those who died was 2.35 yr (range 0.78-5.33 yr). No survivor required re-transplantation and currently, all have normal serum aminotransferase values. Chronic sinusitis was not a significant pre- or post-transplant morbidity, although systematic radiographic evaluation of the sinuses did not occur. Pulmonary deaths occurred in three patients from pulmonary hemorrhage, pulmonary infection with Aspergillus and Candida glabrata, and acute bronchopneumonia associated with polymicrobial sepsis because of Pseudomonas, Klebsiella, and Candida albicans 1.44, 0.78, and 1.83 yr, respectively, after transplantation. The fourth death was associated with chronic rejection, and occurred 5.33 yr after transplantation. All non-survivors were below the 5th percentile for height and weight at the time of liver transplantation. Mean age at transplantation was 9.72 yr (range 1.23-19.09, median 9.61). Survivors were transplanted at a younger age than non-survivors (mean of 9.21 yr vs. 10.66 yr), and had shorter waiting times from diagnosis of end-stage liver disease to transplantation (6.87 months vs. 13.83 months). Eighty percentage (n = 8) of patients had pretransplant variceal bleeds (83% of survivors, 75% of non-survivors). While all non-survivors had a history of meconium ileus and preoperative need of pancreatic enzymes, only 67% of those alive experienced these complications. Preoperative forced vital capacity FVC was 103% for survivors and 95% for non-survivors. The corresponding numbers for forced expiratory flow (FEF) 25-75 were 74-84% respectively. Preoperative Aspergillus was identified in 30% of patients (n = 3). Two of these patients are alive. Cystic fibrosis constitutes an indication for 3.5% of pediatric liver transplants. Evaluation and transplantation for end-stage liver disease associated with cystic fibrosis should be undertaken at an early age. Most deaths were associated with pulmonary/septic events, and occurred less than 2 yr after OLTX. Those children who did not survive had poor growth and nutrition, prolonged waiting times prior to transplantation, were transplanted at an older age, and had a higher incidence of pancreatic insufficiency and meconium ileus. The presence of Aspergillus in the sputum does not constitute a contraindication for OLTX.

Treatment of unresectable hepatoblastoma with liver transplantation in the pediatric population.

The purpose of our study was to evaluate the outcome of children who underwent liver transplantation as treatment for unresectable hepatoblastoma. We prospectively collected data on 311 consecutive liver transplants performed at Children's Medical Center of Dallas between October 1984 and November 2000. There were nine recipients (five boys, four girls) with a diagnosis of unresectable hepatoblastoma. Postoperative survival of those currently alive ranged from 6 months to 16 years (mean 6.4 years, median 7.7 years). All recipients received preoperative chemotherapy: 67% received postoperative chemotherapy. Mean AFP level prior to transplantation was 1 448000 ng/mL. Mean age at diagnosis was 0.81 years. Mean age at transplantation was 1.87 years. Only two patients experienced acute cellular rejection in the postoperative period. There was a total of three deaths and one recurrence. The only instance in which AFP levels did not decrease to low or undetectable levels post-transplantation was in the patient with recurrent tumor. Liver transplantation has an established role in the treatment of hepatoblastoma. It accounted for 3% of pediatric liver transplants, and provided the only opportunity for survival in otherwise incurable patients. Early diagnosis and treatment were found to be associated with better results. Response to chemotherapy may be an important factor influencing survival. Rising AFP levels after transplantation are associated with recurrence.

Thrombendvenectomy for organized portal vein thrombosis at the time of liver transplantation.

OBJECTIVE: To determine the efficacy of portal thrombendvenectomy in cases of portal vein thrombosis at the time of orthotopic liver transplantation. SUMMARY BACKGROUND DATA: Portal vein thrombosis (PVT) has been reported to have an incidence of 2% to 39% in end-stage liver disease. Multiple techniques have been suggested to treat this finding. Several reports have suggested suboptimal results after liver transplantation in recipients with PVT. METHODS: The authors prospectively collected data on 1,546 patients who underwent an initial orthotopic liver transplant at the authors' institution between December 1984 and October 1999. There were 820 male patients and 726 female patients. All recipients received either cyclosporine or tacrolimus immunosuppression. Intraoperative flows of the portal vein and hepatic artery were routinely measured. Duplex sonography was routinely performed on the first postoperative day and routinely 1, 2, 5, and 10 years after transplantation. Eighty-five patients underwent thrombendvenectomy for organized thrombus partially or completely occluding the portal vein. Postoperative treatment included low-molecular-weight dextran for 48 hours and daily aspirin for 3 months. There were 53 male patients and 32 female patients. The PVT group was compared with a control group consisting of transplant recipients without PVT. RESULTS: When compared with the control group, PVT patients were older at the time of transplantation and had a higher incidence of liver disease secondary to cryptogenic cirrhosis and Laennec's cirrhosis. There were no significant differences among both groups for 1-, 3-, and 6-year patient and graft survival rates. CONCLUSIONS: Thrombendvenectomy provides a rapid resolution of an otherwise complex problem. It is the authors' procedure of choice in cases of organized PVT at the time of transplantation. Operative time and length of stay in the intensive care unit are not prolonged, and patient and graft survival rates are not compromised.