Giant hepatic adenoma with atypical features in a patient on oxcarbazepine therapy.

An association between oxcarbazepine therapy and hepatic adenoma (HA) has been documented in animal models but not observed in humans. The authors report a case of a 16-year-old girl on oxcarbazepine therapy for seizure disorder who presented with a giant HA. Pathology of the HA was notable for marked periductal fibrosis and glycoprotein inclusions in the nontumor liver. The patient was not on oral contraceptives and has no other known risk factors for HA.

Timed Pediatric Risk of Mortality Scores predict outcomes in pediatric liver transplant recipients.

More reliable methods are needed to identify children at risk for poor outcomes following liver transplantation. The Pediatric Risk of Mortality (PRISM) Score is a physiology-based scoring system used to quantify risk of mortality in pediatric intensive care unit (ICU) populations. We evaluated the PRISM Score as a predictor of outcomes including survival in the pediatric liver transplant (LT) population. We retrospectively reviewed the records of 67 consecutive LTs performed between August 1997 and February 2000 at an urban, tertiary children's hospital in Chicago, IL, USA. Four PRISM Scores were calculated to determine which periods were most meaningful. A Classic PRISM Score was calculated during first 24 h of ICU admission, and three PRISM Scores were timed with the patient's transplant: a pre-LT PRISM Score (24 h prior to transplant whether in ICU or not), a 24-h post-LT PRISM Score and a 48-h post-LT PRISM Score. These PRISM Scores and other predictors including transplant number, UNOS status and PELD Score were compared with outcomes including survival using univariate methods. The pre-LT, the 24- and the 48-h PRISM Score were associated with the post-LT number of ventilated days (p < 0.05), ICU days (p < 0.05) and with 1-yr survival (p < 0.04). The PRISM Scores were not related to the post-LT hospital length of stay (LOS) or to 1-yr re-transplantation. The PELD Score correlated with the post-LT hospital LOS, but was not associated with mortality or with the ICU LOS. A patient's UNOS status and Classic PRISM Score were not associated with any of the outcomes measured. PRISM Scores are valid predictors of outcome including survival in pediatric LT recipients. These findings help to demonstrate the importance in this population of a patient's general physiologic condition and its influence on the overall hospital course and survival.

Living donor liver transplantation for children with liver failure and concurrent multiple organ system failure.

Liver transplantation for pediatric patients in liver failure and multiple organ system failure (MOSF) often results in poor patient survival. Progression of organ failure occurs while awaiting a cadaveric allograft. Therefore, we considered living donor liver transplantation (LDLT) in this critically ill group of children and report our initial results with comparison to a similar group who received cadaveric donation (CAD). A retrospective chart review was performed on all pediatric liver transplant recipients who met criteria for MOSF at the time of transplantation. Data collection involved pretransplantation patient profiles, as well as postoperative complications and patient survival. Eight patients in MOSF received living donor transplants and 11 patients received a cadaveric allograft. Mean wait time was 3.5 days in the LDLT group and 6.5 days in the CAD group. Pretransplantation patient profiles and postoperative complications were similar between groups. Mean cold ischemia times were 3.8 hours in the LDLT group and 7.9 hours in the CAD group (P = .0002). Thirty-day and 6-month survival rates of the LDLT group were 88% and 63% compared with 45% and 27% in the CAD group, respectively. Living donor transplant recipients in MOSF had decreased wait times to transplantation, as well as decreased cold ischemia times, compared with cadaveric transplant recipients. Patients in the LDLT group had markedly improved survival compared with the CAD group. Timely transplantation before worsening organ failure may account for these findings.

A new technique for resecting 'unresectable' liver tumors.

BACKGROUND/PURPOSE: Liver tumors that surround the three major hepatic veins traditionally have been considered unresectable. This report describes an extended atypical left hepatectomy technique for tumors around the major hepatic veins. METHODS: Three children with tumors surrounding the 3 hepatic veins underwent intraoperative evaluation for extended atypical left hepatectomy. The left hepatic artery, left branch of the portal vein, and the 3 hepatic veins are occluded with vascular clamps. Perfusion of the remaining liver is through the right hepatic artery and portal vein into the retrohepatic vena cava via the retro hepatic veins. If the liver remains soft and does not become mottled, division of the 3 hepatic veins and resection of the tumor are carried out. RESULTS: Extended atypical left hepatectomy was successful in 2 children. Bile leak occurred in 1 instance and healed spontaneously. Both patients had transiently elevated serum bilirubin and transaminase levels and an elevated prothrombin time for 2 weeks. Both survived after treatment with chemotherapy. In the third child the liver became tense and mottled, and the procedure was abandoned. CONCLUSIONS: Successful extended atypical left hepatectomy depends on the ability of the retro hepatic veins to adequately drain blood into the vena cava after interruption (clamping) of the main hepatic veins. If the liver becomes mottled and tense the procedure must be abandoned and the patient should be considered for hepatic transplantation.

NOF-11: a one-year pediatric randomized double-blind comparison of neoral versus sandimmune in orthotopic liver transplantation.

BACKGROUND: Although cyclosporine (CsA) has been a mainstay in liver transplantation immunosuppression the original formulation [Sandimmune (SIM)] has variable absorption, particularly in children. Neoral is a new formulation of CsA that may have improved biovailability that would be advantageous in children. This study was undertaken to assess the pharmacokinetics (PK) and effects on outcome of Neoral versus Sandimmune (SIM) in primary pediatric liver transplant recipients. METHODS: Thirty-two patients were randomized to receive Neoral (17 patients) or SIM (15 patients) in the early posttransplant period (days 1-7) in a double-blind fashion. Intravenous CsA was instituted immediately posttransplant followed by Neoral or SIM as soon as the patient was tolerating oral fluids (days 1-7). PK were compared after the first dose (1-7 days), 3 weeks, and 6 and 12 months posttransplant. In addition, side effects, effect of age and food on absorption, and rejection episodes were assessed by intent to treat analysis. Notable characteristics of this study include the use of a central laboratory for all sample analyses and the assessment of renal function using radioisotopic evaluation of glomerular filtration rates. RESULTS: At baseline the two groups were comparable. Neoral resulted in higher peak levels of CsA and total drug exposure with comparable time to peak drug levels at days 1-7 and week 3. This trend was maintained at 6 and 12 months. Time on i.v. CsA was reduced in the Neoral group (8.4 vs. 11.1 days) and the weight adjusted daily dose of SIM required to achieve target trough levels was about 2-fold more than Neoral from day 22 onward. In addition, biopsy proven and treated and steroid-resistant rejection episodes were fewer in the Neoral group (6 vs. 12; P=0.01 and 1 vs. 8: P=0.004, respectively). Side effects were comparable in both treatment groups. CONCLUSIONS: Neoral was well tolerated and had greater biovailability than SIM without any increase in the incidence of side effects. In addition fewer episodes of rejection were observed with Neoral versus SIM. We conclude that Neoral is the CsA formulation of choice for use in pediatric liver transplant recipients.

Distal splenorenal shunts for the treatment of severe thrombocytopenia from portal hypertension in children.

Profound thrombocytopenia resulting from portal hypertension may exacerbate gastrointestinal bleeding, precipitate spontaneous bleeding, preclude surgical intervention for associated disorders, and severely limit life-style because of the danger of splenic injury. Although splenectomy can reverse the thrombocytopenia, the procedure should be avoided in children. We reviewed our experience with distal splenorenal shunting (DSRS) in children, particularly when performed for the sole purpose of reversing severe thrombocytopenia resulting from portal hypertension. DSRS was performed in 11 children between the ages of 7 and 15 years: five for severe thrombocytopenia (group 1), four for advanced hypersplenism and congenital hepatic fibrosis prior to renal transplantation (group 2), and two for esophageal bleeding (group 3). One child in group 1 with severe heart disease and Child's class C cirrhosis due to hepatitis C died of progressive cardiac failure and was excluded from further analysis. Of the eight remaining patients in groups 1 and 2, four children had congenital hepatic fibrosis, two had portal vein thrombosis, one had hepatitis B, and one had Wilson's disease. After DSRS, the mean platelet count increased from 37,000 +/- 18,000 to 137,600 +/- 81,000 (P = 0.01). The platelet count improved significantly in all seven children with presinusoidal portal hypertension or stable cirrhosis but did not increase in the child with hepatitis B and Child's class B cirrhosis. The white blood cell count increased from an average of 3.3 +/- 1.1 to 5.4 +/- 2.6 (P= 0.02). There were no postoperative complications in this group. The improved platelet count allowed the four children with congenital hepatic fibrosis and renal failure to undergo renal transplantation with full posttransplant immunosuppression including azathioprine. Postoperative Doppler ultrasound examination demonstrated shunt patency at 6 months in all cases. Spleen size decreased appreciably in all children in groups 1 and 2. All children were able to resume full activity including contact sports. In summary, DSRS effectively controls profound thrombocytopenia resulting from presinusoidal portal hypertension or stable cirrhosis without sacrificing the spleen and should be the treatment of choice for this condition.

Delayed hemorrhage after nonoperative management of blunt hepatic trauma in children: a rare but significant event.

PURPOSE: Nonoperative management of blunt hepatic injury (BHI) has become widely accepted in hemodynamically stable children without ongoing transfusion requirements. However, late hemorrhage, especially after discharge from the hospital can be devastating. The authors report the occurrence of serious late hemorrhage and the sentinel signs and symptoms in children at risk for this complication. METHODS: Nonoperative management of hemodynamically stable children included computed tomography (CT) evaluation on admission and hospitalization with bed rest for 7 days, regardless of injury grade. Activity was restricted for 3 months after discharge. Hepatic injuries were classified according to grade, amount of hemoperitoneum, and periportal hypoattenuation. RESULTS: Over 5 years, nonoperative management was successful in 74 of 75 children. One child returned to the hospital 3 days after discharge with recurrent hemorrhage necessitating surgical control. Review of the CT findings demonstrated that he was the only child with severe liver injury in all four classifications. A second child, initially treated at an outside hospital, presented 10 days after injury with ongoing bleeding and died despite surgical intervention. Only the two children with delayed bleeding had persistent right abdominal and shoulder discomfort in the week after BHI. CONCLUSIONS: Our findings support nonoperative management of BHI. However, late hemorrhage heralded by persistence of right abdominal and shoulder pain may occur in children with severe hepatic trauma and high injury severity scores in multiple classifications.

Candida infection in pediatric liver transplant recipients.

A retrospective review of 100 liver transplantations in 98 children was performed to determine the incidence of infection caused by Candida organism in these patients and to identify risk factors that may predispose to serious fungal infection. Thirty-one infections caused by Candida organisms developed during the initial 28 days posttransplantation: 19 were definite invasive infections (one deep site or one positive blood culture), 2 were probable invasive infections (three superficial sites), and 10 were urinary tract infections. Eleven of 19 patients had fungemia or a disseminated infection (two noncontiguous deep organs involved and/or positive blood cultures) and 8 of 19 had peritoneal candidiasis. Infection caused by Candida organisms was a contributing factor to mortality in 7 of 21 patients (case fatality rate of 33%) with invasive infection. Risk factors that were predictive for invasive infection by univariate analysis included the following: pretransplantation antibiotic therapy, length of transplant operation, transfusion requirement, number of days in the intensive care unit, number of days intubated, number of concurrent bacterial infections, number of antibiotics administered, number of laparotomies performed posttransplantation, retransplantation, hepatic artery thrombosis, bile leaks, and renal and respiratory failure. By logistic regression analysis, bile leak, hepatic artery thrombosis, preoperative steroid use, transfusion requirement, and the number of days intubated were identified as independent risk factors for invasive infection caused by Candida organisms. The use of prophylactic antifungal agents in high-risk patients may be important in reducing the serious morbidity and mortality associated with sepsis caused by Candida organisms in pediatric liver transplant recipients.

Growth in children following liver transplantation.

Although liver transplantation (OLT) has become standard therapy for end-stage liver disease in children, growth after OLT remains an area of concern. We reviewed our experience with growth after OLT at the Hospital for Sick Children in 83 patients who survived at least 1 yr post-transplant. Our aims were to describe the success rate in steroid cessation in patients after transplantation, to examine the effect of transplantation on subsequent growth, to see if steroid reduction had a beneficial effect on growth, and to quantify the risk of stopping steroids on rejection. Patients below age 5 yr were weaned off steroids more easily than those over age 5: 19.2% vs. 0% (p<0.05), 65.9% vs. 50%, and 79.5% vs. 37.5% (p<0.05) at post-transplant years 1, 2, and 3, respectively. Pre-transplant, 30% of patients were below the third percentiles for height and weight. Post-transplant, there was a steady improvement in the distribution of patients above the 3rd percentile, so that by post-transplant year 6, only 5% were below the 3rd percentile. Height and height velocity percentiles were found to correlate inversely with total yearly steroid dose (mg/kg) at post-transplant years 2, 3 and 6 (p<0.05). In 60% of patients, steroids were successfully discontinued. In these patients, height and height velocity percentiles have achieved a near normal distribution with 40% and 46% of patients above the 50th percentile for height and height velocity percentiles, respectively. No grafts were lost to rejection in those off steroids, and all rejection episodes were easily reversed. We conclude that the majority of children can be weaned off steroids successfully after OLT and that growth in those children in the presence of good graft function is near normal.

Single-stage repair of Hirschsprung's disease: a comparison of 109 patients over 5 years.

Current management of Hirschsprung's disease (HD) typically involves staged therapy, which necessitates multiple hospital admissions and associated costs. The authors therefore investigated the course and outcome of treating such children using a single-staged (SS) approach, and compared them with those treated via multiple-staged (MS) therapy. The cases of one hundred nine consecutive patients who presented with HD from 1991 to 1996 were reviewed. Four patients were excluded (two unrelated deaths, two with small intestinal aganglionosis). Twenty-one of the remaining 105 patients underwent SS repair. Both groups were similar in gender, age at diagnosis, and frequency of comorbidities. Repair was possible in 100% of the SS patients. Complications, including enterocolitis, occurred in 63% of patients, and did not significantly differ between groups. The outcome in SS patients was unaffected by whether the repair was performed before or after 30 days of life. The outcome was unaffected by operative weight in either group. By contrast, the number of hospital admissions and total length of stay was significantly higher in the MS group, which resulted in a twofold increase in total costs associated with MS repair compared with SS repair. These data indicate that primary repair of HD is efficacious (even in the newborn), with morbidity equal to MS repair, and requires fewer hospital admissions. The significant savings to the patient and the health care system suggest that SS repair may be an improved strategy for treating HD.

The impact of a previous Kasai procedure on liver transplantation for biliary atresia.

The usual treatment for biliary atresia is a Kasai procedure followed by liver transplantation when indicated. Although primary transplantation for biliary atresia without a previous Kasai procedure is occasionally advocated, it is rarely performed. This review was undertaken to evaluate the impact of a Kasai procedure on the morbidity and mortality of patients who went on to need a liver transplant. Sixty-three patients with biliary atresia were included in this review. Fifty seven patients underwent transplantation: eight patients had a liver transplant only (group 1), and 49 patients underwent a Kasai procedure before transplantation (group 2). Six patients died before receiving a transplant. Time spent on the waiting list for liver transplant was longer in group 2 than in group 1 (170.3 +/- 24.6 days versus 63.3 +/- 7.1 days, P < .05). The patients in group 1 were younger (0.7 +/- 0.2 versus 2.3 +/- 0.4 years) and smaller (6.9 +/- 0.4 kg versus 11.6 +/- 1.2 kg) than the patients in group 2 (P = .07). There was no difference in pretransplant urgency status between the two groups. The mean duration of the transplant operation was shorter in group 1 patients (476.8 +/- 53.3 minutes) compared with group 2 (593.9 +/- 29.3 minutes, P = .06). Group 1 patients received 199.8 +/- 46.2 mL/kg blood transfusion intraoperatively, and group 2 patients had twice that amount, 466 +/- 122.5 mL/kg. No patients in group 1 experienced postoperative bowel perforations or required reoperation for bleeding. In group 2 however, 11 of 49 (22.4%) experienced bowel perforations and 7 of 49 (14.2%) required reoperation for bleeding. There was no difference in nonsurgical complications between the two groups. Long-term survival was equal in the 2 groups: six of eight patients (75%) in group 1 and 36 of 49 (74%) in group 2. The marked increase in complications noted in group 2 patients did not reach statistical significance because of the much smaller number of patients in group 1. These results suggest that patients with biliary atresia have fewer complications after transplantation if a Kasai procedure is not performed before the transplant, and that a more careful selection of surgical options available in treating patients with biliary atresia is required.

Biliary atresia: should all patients undergo a portoenterostomy?

PURPOSE: The management of noncorrectable extra hepatic biliary atresia includes portoenterostomy, although the results of the surgery are variable. This study was done to develop criteria that could successfully predict the outcome of surgery based on preoperative data, including percutaneous liver biopsy, allowing a more selective approach to the care of these babies. METHODS: The charts and biopsy results of 31 patients who underwent a Kasai procedure for biliary atresia between 1984 and 1994 were reviewed. Values for preoperative albumin, bilirubin, age of patient at Kasai, and lowest postoperative bilirubin were recorded. Surgical success was defined as postoperative bilirubin that returned to normal. A pathologist blinded to the child's eventual outcome graded the pre-Kasai needle liver biopsy results according to duct proliferation, ductal plate lesion, bile in ducts, lobular inflammation, giant cells, syncitial giant cells, focal necrosis, bridging necrosis, hepatocyte ballooning, bile in zone 1, 2, and 3, cholangitis, and end-stage cirrhosis. Clinical outcome was then predicted. RESULTS: Success after portoenterostomy could not reliably be predicted based on gender, age at Kasai, preoperative bilirubin or albumin levels. Histological criteria, however, predicted outcome in 27 of 31 patients (P < .01). Fifteen of 17 clinical successes were correctly predicted; as were 12 of 14 clinical failures (sensitivity, 86%; specificity, 88%). Individually, the presence of syncitial giant cells, lobular inflammation, focal necrosis, bridging necrosis, and cholangitis, were each associated with failure of the portoenterostomy (P < .05). Bile in zone 1 was associated with clinical success of the procedure (P < .05). CONCLUSIONS: Based on the predictive information available in a liver biopsy, we conclude that those patients who will not benefit from a Kasai procedure can be identified preoperatively, and channeled immediately to transplantation.

Excess zinc associated with severe progressive cholestasis in Cree and Ojibwa-Cree children.

BACKGROUND: High hepatic copper concentrations have been reported in several liver disorders. We report six Native Canadian children with severe chronic cholestatic liver disease, who had excess hepatic copper and zinc. METHODS: The children, aged 22 months to 8 years, came from northern Ontario, Canada. All were referred for possible liver transplantation because of end-stage liver disease. We examined explanted liver samples (or liver biopsy material in one case) by scanning transmission electronmicroscopic (STEM) X-ray elemental microanalysis and atomic absorption spectrophotometry. Samples from four controls (two with no liver pathology, one with biliary atresia, and one with Wilson's disease) were also analysed by atomic absorption spectrophotometry. FINDINGS: The explanted livers showed similar distinctive signs of advanced biliary cirrhosis, and on electronmicroscopy there were dense deposits in enlarged lysosomes and in cytoplasm. Hepatic copper concentrations were many times higher in the five patients with measurements (47.6-56.9 microgram/g dry weight) than in two samples of normal control liver tissue (2.3 and 2.9 microgram/g). Similarly, hepatic zinc concentrations were many times higher in the patients than in controls (104-128 vs 1.9-3.2 microgram/g dry weight). INTERPRETATION: The excess copper may be due to chronic cholestasis but the excess zinc is unexplained. Since three of the patients are related (shared grandparents), a genetic disorder of metal metabolism is possible, but we cannot exclude environmental factors.

The University of Toronto liver transplant program.

The University of Toronto liver transplant program began in 1985 at a time when the procedure had already evolved from an experimental form of surgery to an accepted treatment for many forms of liver failure. The program was established not only to provide clinical care for patients but also to address academically the barriers which impeded success. The program brought together experts in medicine, surgery, pathology, and the basic sciences of immunology, virology and molecular biology. Our group has had a special interest in transplantation for viral hepatitis. We demonstrated the role of HBV DNA as a prospective factor in both viral recurrence and survival. We further studied a number of agents to prevent re-infection including PGE, HBIG and more recently lamivudine. Although the short-term results of transplantation for HCV appear excellent, reinfection of the graft and development of chronic hepatitis and cirrhosis may make long-term results problematic. Therefore, we have directed attention to studies of pathogenesis and treatment of HCV in liver transplantation. Our studies have demonstrated a unique role for ribavirin as an immunomodulatory agent which can benefit the course of posttransplant HCV. Future studies will examine combination therapy in an attempt to eradicate the virus. Our group also has been interested in PNF and FHF and have demonstrated a positive effect of PGE in this setting. As we look to the future, the greatest challenges facing transplantation are the shortage of organ donors and the toxic effects of long-term immunosuppression. Our group now has established research efforts both in tolerance induction and xenotransplantation which we feel are necessary to make transplantation an effective, universal treatment for end stage organ failure.

Initial experience with one-stage endorectal pull-through procedures for Hirschsprung's disease.

To evaluate our experience with one-stage endorectal pull-through (ERPT) procedures (without colostomy) for Hirschsprung's disease (HD), we compared 7 such patients to a cohrt of 20 consecutive patients undergoing EROT folowing colostomy. Reasons for exclusion from hte cohort group included: (1) bowel obstruction requiring operation before 1 month of age; (2) presentation with enterocolitis (EC), intestinal perforation, or massive fecal distension; (3) long-segment disease; or (4) severe associated anomalies. The 7 male patients in the single-stage ERPT group first presented at a median age of 16 days (4 days-2.5 years) and were managed by regular digital dilation or colonic irrigation for between 1 and 6 months (median 2 months) prior to surgery. Single-stage procedures were performed at a median age and weight of 4 months (2 months-2.5 years) and 6.4 kg (4.5-13.8 kg), respectively Median hospitalization for these patients was 9 dyas. Postoperative complications occurred in 2 patients (29%), and included anastomotic stricture requiring outpatient dilation, and 1 case or recurrent EC that responded to a course of anal dilations. The cohort group (14 M, 6 F) presented at a median of 15 months. All underwent colostomy as a primary procedure. ERPT was deferred until a median age of 21 months, and the hospitalization after pull-through averaged 10 dyas (20 days including stay after colostomy). Four patient (20%) developed complications requiring reoperation following the initial colostomy. Complications after ERPT occurred in 5 patients (25%) and included 1 death from fulminant Hirschsprung's EC. Other complications included 1 anastomotic stricture and 2 mucosal prolapses requiring anoplasty. Long-term functional results were similar in both groups. Despit our limited experience, we conclude that one-stage ERPT can be safely performed in infants, including those under 3 months of age, with rectosigmoid HD. Total duration of hospitalization is reduced and colostomy complications are avoided. Functional outcome appears to be comparable between patients treated in one or two stages.

Colonic atresia combined with Hirschsprung's disease: a diagnostic and therapeutic challenge.

Three new cases of patients with co-occurrence of colonic atresia and Hirschsprung's disease and a review of the literature (n = 5) are presented in this report. All patients (n = 8) except one were full-term infants who had no other significant anomalies. The preterm infant had associated tetralogy of Fallot in addition to Hirschsprung's disease and colonic atresia. Six patients had atresia of the ascending colon, and two had atresia of the colon to splenic flexure. All colonic atresias were diagnosed neonatally; however, there was mean delay of 15.4 months (range, 1 mo to 5 years) in diagnosing associated Hirschsprung's disease. There were two deaths. A careful examination of the resected specimen to rule out Hirschsprung's disease is recommended. Performing a rectal biopsy must be considered for patients who initially were treated for colonic atresia and who have a slow return of normal gut function.

Unconventional treatment of neuroenteric cyst in a newborn.

Neuroenteric cysts are uncommon congenital malformations that can require early surgical treatment. The authors report on an unusual treatment of a very large neuroenteric cyst that involved most of the small bowel and extended into the chest. A 1-day-old boy was admitted because of abdominal distension. The prenatal ultrasound results at 8 and 36 weeks had been normal. Examination showed right upper quadrant fullness and mild respiratory distress. A malformed sternum and asymmetric upper thoracic vertebra were seen on the initial x-rays. The possibility of a midthoracic right paravertebral mass was raised. Abdominal ultrasound findings were consistent with a large bowel duplication cyst. Laboratory results were all normal except the bilirubin level, which was (59 mmol/L). During laparotomy, the second part of the duodenum was found to enter a dilated cyst, and the terminal ileum arose from the cyst. The total length of the intact small bowel was 20 cm including a competent ileocecal valve. The site of the biliary duct entering the cyst was not clear. The surgical procedure involved partial resection of the anterior wall of the cyst, creation of an enteric tube from the posterior cyst wall to communicate between the duodenum and the ileum, and addition of another 25 cm of length to the small bowel. An enterocutaneous fistula was created with the proximal portion of the cyst because the site of the papilla of Vater was suspected to enter this part of the cyst. A postoperative HIDA scan showed good uptake with no excretion into the gut or the proximal pouch.(ABSTRACT TRUNCATED AT 250 WORDS)

Prevention of biliary cirrhosis following hepatic arterial thrombosis after liver transplantation in children by using ursodeoxycholic acid.

Hepatic artery thrombosis (HAT) after liver transplantation is a severe complication that often requires retransplantation. The authors have adopted a different approach, aimed at treating the perioperative HAT complications aggressively and early, with ursodeoxycholic acid (UDCA), to try to preserve the original graft. Eighty-six liver transplants were performed in 73 children (age range, 4.5 months to 17.5 years; median, 2.6 years). HAT occurred eight times, in seven patients (9.3%). Patients with HAT were significantly younger and smaller (mean age, 0.8 +/- 0.4 v 4.8 +/- 5.3 years; P < .02; mean weight, 7.4 +/- 0.8 v 18.7 +/- 16.2 kg; P < .05). The incidence of HAT varied significantly according to the method of arterial reconstruction used: 4 of 16 (25%) when a donor iliac artery interposition graft to the aorta was used, 4 of 61 (6.6%) when the native hepatic artery was used, and 0 of 9 when the donor celiac axis was anastomosed directly to the aorta (P < .05). The incidence of HAT was not significantly different when reduced-size grafts were used. Early retransplantation was performed in three of the eight patients; two survived. All other patients were treated for 4 to 6 weeks with broad-spectrum antibiotics and amphotericin. Five patients were treated with UDCA, three immediately after the acute event and two after 4 and 6 months (respectively) post-HAT. The patients who had UDCA immediately post-HAT had histologically normal liver biopsy specimens. Results of liver function tests have been normal. One of these patients required transhepatic stenting of a common bile duct stricture for several months.(ABSTRACT TRUNCATED AT 250 WORDS)