Continuous renal replacement therapy and therapeutic plasma exchange in pediatric liver failure.

Patients with acute liver failure (ALF) and acute on chronic liver failure (ACLF) have significant morbidity and mortality. They require extracorporeal blood purification modalities like continuous renal replacement therapy (CRRT) and therapeutic plasma exchange (TPE) as a bridge to recovery or liver transplantation. Limited data are available on the outcomes of patients treated with these therapies. This is a retrospective single-center study of 23 patients from 2015 to 2022 with ALF/ACLF who underwent CRRT and TPE. We aimed to describe the clinical characteristics and outcomes of these patients. Median (IQR) age was 0.93 years (0.57, 9.88), range 16 days to 20 years. Ten (43%) had ALF and 13 (57%) ACLF. Most (n = 19, 82%) started CRRT for hyperammonemia and/or hepatic encephalopathy and all received TPE for refractory coagulopathy. CRRT was started at a median of 2 days from ICU admission, and TPE started on the same day in most. The liver transplant was done in 17 (74%), and 2 recovered native liver function. Four patients, all with ACLF, died prior to ICU discharge without a liver transplant. The median peak ammonia pre-CRRT was 131 µmol/L for the whole cohort. The mean (SD) drop in ammonia after 48 h of CRRT was 95.45 (43.72) µmol/L in those who survived and 69.50 (21.70) µmol/L in those who did not (p 0.26). Those who survived had 0 median co-morbidities compared to 2.5 in non-survivors (aOR (95% CI) for mortality risk of 2.5 (1.1-5.7), p 0.028). Conclusion: In this cohort of 23 pediatric patients with ALF or ACLF who received CRRT and TPE, 83% survived with a liver transplant or recovered with their native liver. Survival was worse in those who had ACLF and those with co-morbid conditions. What is Known: •  Pediatric acute liver failure is associated with high mortality. •  Patients may require extracorporeal liver assist therapies (like CRRT, TPE, MARS, SPAD) to bridge them over to a transplant or recovery of native liver function. What is New: • Standard volume plasma exhange has not been evaluated against high volume plasma exchange for ALF. • The role, dose, and duration of therapeutic plasma exchange in patients with acute on chronic liver failure is not well described.

Optimizing pediatric liver transplantation: Evaluating the impact of donor age and graft type on patient survival outcome.

BACKGROUND: We examined the combined effects of donor age and graft type on pediatric liver transplantation outcomes with an aim to offer insights into the strategic utilization of these donor and graft options. METHODS: A retrospective analysis was conducted using a national database on 0-2-year-old (N = 2714) and 3-17-year-old (N = 2263) pediatric recipients. These recipients were categorized based on donor age (≥40 vs <40 years) and graft type. Survival outcomes were analyzed using the Kaplan-Meier and Cox proportional hazards models, followed by an intention-to-treat (ITT) analysis to examine overall patient survival. RESULTS: Living and younger donors generally resulted in better outcomes compared to deceased and older donors, respectively. This difference was more significant among younger recipients (0-2 years compared to 3-17 years). Despite this finding, ITT survival analysis showed that donor age and graft type did not impact survival with the exception of 0-2-year-old recipients who had an improved survival with a younger living donor graft. CONCLUSIONS: Timely transplantation has the largest impact on survival in pediatric recipients. Improving waitlist mortality requires uniform surgical expertise at many transplant centers to provide technical variant graft (TVG) options and shed the conservative mindset of seeking only the "best" graft for pediatric recipients.

Small-for-size syndrome in a 9.7 kg pediatric recipient with a whole liver graft.

BACKGROUND: Small-for-size syndrome (SFSS) in pediatric liver transplant recipients, particularly those weighing less than 10 kg, is rare. This report describes a case of a 15-month-old whole liver transplant recipient who suffered SFSS, and systematic literature review was performed to identify outcomes of such cases and potential risk factors for SFSS. CASE PRESENTATION: A 15-month-old toddler with a history of biliary atresia underwent a deceased donor whole liver transplant. The graft weighed 160 g, resulting in a graft-to-recipient weight ratio (GRWR) of 1.6%. The post-operative course was complicated by SFSS, characterized by massive ascites causing hemodynamic instability and compromised hepatic artery flow. Pharmacological intervention with octreotide was initiated, and the patient eventually recovered. CONCLUSION: In small pediatric recipients, especially those weighing less than 10 kg, the native liver body weight ratio (LBWR) is significantly higher. When selecting an appropriately sized graft for these recipients, this higher ratio should be taken into consideration. The literature review suggests that a GRWR of less than 2% is associated with a higher incidence of small-for-size syndrome in small pediatric recipients weighing less than 10 kg.

Critical elements of pediatric liver cancer surgery.

The appropriate management of pediatric liver malignancies, primarily hepatoblastoma and hepatocellular carcinoma, requires an in depth understanding of contemporary preoperative risk stratification, experience with advanced hepatobiliary surgery, and a good relationship with one's local or regional liver transplant center. While chemotherapy regimens have become more effective, operative indications more well-defined, and overall survival improved, the complexity of liver surgery in small children provides ample opportunity for protocol violation, inadequate resection, and iatrogenic morbidity. These guidelines represent the distillation of contemporary literature and expert opinion as a means to provide a framework for preoperative planning and intraoperative decision-making for the pediatric surgeon.

Transplant renal vein stent placement complicated by obstructive hematuria: A case report.

BACKGROUND: Renal vein stenosis is uncommon following transplantation. We report acute renal vein stenosis post-transplant treated with an endovascular stent and complicated by urinary obstruction from clot formation. METHODS: Retrospective case report. RESULTS: A 16-year-old female 3 years post-transplant suffered anuria post-stenting with renal ultrasound demonstrating obstructive clot in the collecting system, a previously unreported complication. Subsequent nephroureteral JJ stent placement resulted in high-volume urine output. CONCLUSION: This article underscores the high index of suspicion required for renal vein stenosis following transplantation and the need to monitor urine output closely following stent placement.

Employment search, initial employment experience, and career preferences of recent pediatric surgical fellowship graduates: An APSA survey, part of the right child/right surgeon initiative.

BACKGROUND: APSA's Right Child/Right Surgeon Initiative addresses issues concerning patient access to appropriate pediatric surgical care and workforce distribution. The APSA Workforce Committee sought to understand the experiences and motivations of recent graduates of Pediatric Surgery Training Programs entering the workforce. METHODS: Using APSA membership databases, we identified members who completed fellowship training from 2010 to 2019. An online survey was created using Survey Monkey, and invitations to participate were sent via email. RESULTS: 144 of 447 invited participants responded (32% response rate). 91% of respondents participated in dedicated research prior to fellowship, but only 64% perform research during their employment. 23% completed an additional clinical fellowship, but only 54% currently practice within the second field. When asked to identify the top three factors used to choose a position, the most common responses were "location or geography" (71%), "available mentorship" (53%), and "compensation and benefits" (37%). Describing their first position, 77% reported working in an academic institution, 78% reported working in a metropolitan/urban area, and 55% reported working in a free-standing children's hospital. 94% participate in General Surgery resident education, and 49% are faculty within a Pediatric Surgery fellowship. Overall, 92% of respondents were able to find the type of employment position that they had wanted. CONCLUSION: In our survey the overwhelming majority of young pediatric surgeons found the type of job they desired. Most report beginning their practice in more populated, urban areas within academic institutions. Geographic location and work environment played heavily into their employment decisions. These preferences could contribute to continued disparity in access to pediatric surgeons between urban and rural America and to dilution of experience for urban surgeons. Possible solutions include alternative incentive programs for employment in less populated areas or new training models for general surgeons in rural areas to train in fundamentals of Pediatric Surgery.

Do funding sources influence long-term patient survival in pediatric liver transplantation?

BACKGROUND: Socioeconomic status has been associated with inferior outcomes after multiple surgical procedures, but has not been well studied with respect to pediatric liver transplantation. This study evaluated the impact of insurance status (as a proxy for socioeconomic status) on patient and allograft survival in pediatric first-time liver transplant recipients. METHODS: Our retrospective analysis of the UNOS data base from January 2002 through September 2017 revealed 6997 pediatric patients undergoing first-time isolated liver transplantation. A mixed Cox proportional hazards model adjusted for donor, recipient, and program characteristics determined the RR of insurance status on allograft and patient survival. All results were considered significant at P < .05. All statistical results were obtained using R version 3.5.1 and coxme version 2.2-10. RESULTS: Medicaid status had a significant negative impact on long-term survival after controlling for multiple covariates. Pediatric patients undergoing first-time isolated liver transplantation with Medicaid insurance had a RR of 1.42 [confidence interval: 1.18-1.60] of post-transplant death. CONCLUSION: Pediatric patients undergoing first-time isolated liver transplantation have multiple risk factors that may impact long-term survival. Having Medicaid insurance almost doubles the chances of dying post-liver transplant. This patient population may require more global support post-transplant to improve long-term survival.

The impact of public health service increased risk donors in pediatric liver transplantation.

Many transplant programs are reluctant to use organs from deceased donors designated as "PHS increased risk" due to misconceptions regarding the quality of those organs. This study evaluated the impact of PHS increased risk donors on patient and allograft survival in pediatric patients undergoing liver transplantation. Retrospective analysis of the UNOS database from January 2005 through September 2017 revealed 5615 pediatric patients who underwent isolated liver transplantation; of these, 5057 patients received primary isolated liver transplants and 558 patients received isolated liver retransplants. PHS increased risk organs were used in 6.7% and 5.4% of the children receiving primary isolated and retransplant livers, respectively. Cox proportional hazards models adjusted for donor and recipient characteristics determined the relative risk of PHS status on allograft and patient survival. Sicker children (those in ICU [P < .001] and on life support [P = .04]) were more likely to receive PHS increased risk donor organs. There were no differences in overall patient (P = .61) or allograft (P = .68) survival between pediatric patients receiving PHS positive vs PHS negative deceased donor organs; adjusted models also demonstrated no statistically significant differences in patient or allograft survival. Excellent patient and allograft survival can be accomplished with PHS increased risk organs.

New Evidence Supporting Increased Use of Split Liver Transplantation.

BACKGROUND: Young children and small-statured candidates are dying on liver candidate waitlists. The Organ Procurement and Transplantation Network and United Network for Organ Sharing have proposed a split liver (SL) variance encouraging transplant programs to split more livers to aid these smaller statured candidates. METHODS: We evaluated the US experience of splitting donor livers during 2002-2016. The results of our analysis provide new evidence to support this variance. RESULTS: During 2002-2016, SL grafts from 935 donors were transplanted into 1870 recipients. Controlling for recipient factors and using time period and program identification as random variables, a multivariable mixed Cox proportional hazards model for graft failure revealed that donor aged 3-10 years had a relative risk (RR) of 3.94 (2.86-5.44), and donor aged >30 years had a RR of 1.94 (1.59-2.35) for graft failure. Donor-to-recipient body surface area ratio <0.90 had a RR of 1.40 (1.13-1.75). Programs with experience transplanting <23 SLs had a RR of 1.43 (1.21-1.75). The same program transplanting both split segments had a RR of 1.38 (1.20-1.59). CONCLUSIONS: Splitting protocols based on these findings could improve graft survival after SL transplantation, which would encourage programs to opt into splitting more livers. The new protocols may consider donor age restrictions, necessary program experience, donor with body surface area appropriate for recipient, and improved logistical factors to share segments between transplant programs. The result would likely be a drastic reduction in liver waitlist deaths for young children and small-statured candidates.

Endovascular treatment of surgical mesoportal and portosystemic shunt dysfunction in pediatric patients.

BACKGROUND: Published data describing the endovascular treatment of dysfunctional mesoportal and portosystemic shunts in the pediatric population are limited. OBJECTIVE: We sought to describe the treatment and follow-up of such shunts managed by interventional radiology at a single pediatric hospital. We hypothesized that stenotic and occluded pediatric portosystemic and mesoportal shunts can be maintained patent by interventional radiology in the moderate term. MATERIALS AND METHODS: We conducted a single-center retrospective study at a tertiary pediatric hospital. We included children with surgical mesoportal (meso-Rex) or portosystemic (mesocaval, splenorenal or splenocaval) shunts treated with attempted angioplasty or stenting from 2010 to 2018. Technical success was defined as catheterization and intervention upon the shunt with venographic evidence of flow improvement. The primary outcome variables were shunt patency at 1 month, 6 months, 12 months and 24 months post-procedure and freedom from reintervention. RESULTS: Twenty pediatric patients (11 boys, 9 girls; mean age 8.25 years, range 1.3-17 years) met inclusion criteria. Fifty-two interventions (primary and reintervention) on 13 splenorenal, 3 meso-Rex, 2 mesocaval and 2 splenocaval shunts were performed because of evidence of shunt failure, including gastrointestinal bleeding, hypersplenism, or radiographic evidence of a flow defect. The 11 stenotic shunts were treated with 100% technical success, while the remaining 9 occluded shunts were treated with 66.7% technical success. The mean number of reinterventions was 1.9 (standard deviation [SD] = 3.1) per child, which did not differ between stenotic and occluded shunts (P=0.24). Primary patency at 1-month, 6-month, 12-month and 24-months follow-up visits was 17/17 (100%), 10/16 (62.5%), 7/15 (46.7%) and 4/10 (40%), respectively. However, 100% of shunts were either primary patent or primary-assisted patent by endovascular reintervention. There were no cases of shunt occlusion following initial technical success. Finally, the median freedom from reintervention duration was 387 days (SD=821 days). CONCLUSION: Dysfunctional portosystemic surgical shunts are effectively managed by endovascular methods. While many shunts require reintervention, combined primary patency and assisted primary patency rates are excellent.

Immunosuppression in the pediatric transplant recipient.

The field of pediatric solid-organ transplantation has significantly evolved since its beginnings in the early 20th century. As advancements have led to the development of innovative surgical techniques and novel medication regimens, transplantation has now become a routine practice leading to an increase in the rates of organ recipients worldwide. The care of pediatric solid-organ transplant recipients differs from adults in several areas not only due to technically challenging surgeries, but mostly due to the complexity of their immunosuppression management. Although there is large variation of pediatric immunosuppression regimens worldwide, the use of calcineurin inhibitors, either tacrolimus or cyclosporine, still forms the backbone of immunosuppression regimens after solid-organ transplantation. Both medications are relatively well tolerated but are known to have long-term side effects, especially nephrotoxicity and neurotoxicity. The goal of care in long-term pediatric survivors of solid-organ transplant now aims to safely minimize exposure to immunosuppression and to achieve long-term graft tolerance.

Body mass index as a predictor of outcomes among pediatric kidney transplant recipient.

Controversies exist regarding the impact of obesity on patients undergoing kidney transplantation. We sought to estimate the association between BMI and patient outcomes (survival and graft function) among pediatric kidney transplant patients in the USA. We conducted a retrospective analysis of the United Network for Organ Sharing database (1987-2013), which revealed 13 014 pediatric patients (<18 years old) who underwent primary kidney transplantation. Patients were stratified into five BMI categories established by the World Health Organizations according to their Z score, which is based on age, gender and BMI. The -2, 0, and +2 categories were collapsed and served as the reference group, while the -3 (thin) and +3 (obese) categories were evaluated for differences in graft and patient survival. The survival rates between these categories were compared using the Kaplan-Meier estimator. Cox proportional hazards models were constructed to adjust for recipient and donor characteristics to estimate the risk of graft loss and mortality associated with BMI. Logistic regression models were estimated to evaluate whether there was an association between BMI and DGF. There were no differences in overall patient (P=.1655) or graft (P=.1688) survival between the severely thin, normal, and obese patients. Adjusted models also revealed no statistically significant differences in graft or patient survival. There were no differences in the odds of DGF (both unadjusted and adjusted) among the three groups. The prevalence of obesity is increasing among children who present for kidney transplant in the USA. In this national study of pediatric kidney transplant recipients, there was no difference in graft or patient survival and no differences in rates of DGF among obese children compared to normal and underweight children undergoing kidney transplantation.

Pediatric liver transplant portal vein anastomotic stenosis: correlation between ultrasound and transhepatic portal venography.

The objective of this study was to determine which transabdominal ultrasound parameters correlate with portal vein stenosis (PVS) on percutaneous transhepatic portal venography in pediatric liver transplant patients. A retrospective review was performed of percutaneous transhepatic portal venograms performed between 2005 and 2013. The findings were compared to those from ultrasounds performed before venography and at the baseline. Patients were stratified on the basis of the presence of significant PVS (group 1, >50% stenosis; group 2, ≤50% stenosis) on portal venography. Findings were compared to those for age-matched controls. Twenty portal venograms were performed for 12 pediatric patients. Thirteen of the 20 patients (65%) demonstrated significant PVS (>50%). The mean peak anastomotic velocity (PAV) was 253.6±96 cm/s in group 1, 169.7±48 cm/s in group 2, and 51.3±20 cm/s in the control group. PAV (r=0.672, P=0.002) was the only ultrasound variable that correlated with the presence of significant PVS. A receiver operating characteristic curve was generated from PAV and PVS data (area under the curve=0.75, P=0.08). A threshold velocity of 180 cm/s led to a sensitivity of 83% and a specificity of 71% in predicting significant PVS on portal venography. At the baseline, the mean PAV was 155.8±90 cm/s for group 1 and 69.5±33 cm/s for group 2 (P=0.08); for control subjects, it was 78.9±53 cm/s (P=0.06). PAV is the only measured ultrasound parameter that correlates with significant PVS on portal venography in pediatric liver transplant patients. An elevated baseline PAV may increase the risk of developing PVS.

Hepatocellular carcinoma in children: epidemiology and the impact of regional lymphadenectomy on surgical outcomes.

BACKGROUND: Factors influencing survival in children with HCC have not been studied. The objective of this study was to identify prognostic factors in pediatric HCC, and to determine whether regional lymphadenectomy is associated with improved survival. METHODS: We performed a retrospective cohort study using the Surveillance, Epidemiology and End Results (SEER) registry. All patients <20 years old diagnosed with HCC from 1973-2009 were included. Disease-specific survival was compared using Kaplan-Meier statistics and Cox proportional-hazards regression. RESULTS: We identified 238 patients (139 Male: 99 Female). Overall, 112 (47%) received an operation (resection/transplantation). Observed mortality and adjusted hazard of disease-specific death was greater for females (HR=2.07, p=0.013) and older children. Among operative patients, 44% were documented to have a regional lymphadenectomy. Although demographic factors did not differ between lymphadenectomy and non-lymphadenectomy groups, patients who underwent lymphadenectomy had a greater proportion of metastatic disease (24% vs. 15%) and fibrolamellar HCC (53% vs. 31%). Five-year survival for lymphadenectomy patients was superior to non-lymphadenectomy (70% vs. 57%). Adjusted mortality for lymphadenectomy was also improved relative to non-lymphadenectomy (HR=0.26, p=0.013). CONCLUSIONS: HCC in children is associated with poor survival, especially among children older than 4 years and girls. In surgical candidates, regional lymphadenectomy may be associated with improved survival.

Surgical treatment of primary liver tumors in children: outcomes analysis of resection and transplantation in the SEER database.

Adjusted survival outcomes following hepatic resection and transplantation for pediatric liver tumors have not been compared. To address this question, we conducted a retrospective cohort study using the SEER registry. While SEER lacks certain specifics regarding staging, chemotherapy, comorbidities, and recurrence, important hypothesis-generating data are available and were analyzed using Kaplan-Meier statistics and Cox proportional hazards regression. All SEER patients under the age of 20 yr undergoing surgery for HB (n = 318) or HCC (n = 80) between 1998 and 2009 were included. Of HB patients, 83.3% underwent resection and 16.7% transplantation. Advanced disease, vascular invasion, and satellite lesions were more common among transplant patients. Unadjusted five-yr survival was equivalent, as was the adjusted hazard of death for transplant relative to resection (HR = 0.58, p = 0.63). Of HCC patients, 75.0% underwent resection and 25.0% transplantation. Transplant patients had a higher prevalence of vascular invasion and satellite lesions. Five-yr survival was 53.4% after resection and 85.3% after transplant, and the adjusted hazard of death was significantly lower after transplantation (HR = 0.05, p = 0.045). While transplantation is generally reserved for unresectable tumors, the favorable survival seen in HCC patients suggests that liberalized transplant criteria might improve survival, although further prospective data are needed.

Donor and recipient size mismatch in adolescents undergoing living-donor renal transplantation affect long-term graft survival.

BACKGROUND: Controversies exist in the adult literature regarding the use of kidneys from small donors into larger recipients. Little is known regarding this issue in pediatric kidney transplantation. To assess the impact of donor/recipient size mismatch on long-term renal graft survival in pediatric patients undergoing living-donor renal transplantation. METHODS: We reviewed the United Network for Organ Sharing database from 1987 to 2010 for adolescent (11-18 years old) patients who underwent primary living-donor renal transplantation. According to donor/recipient body surface area (BSA) ratio, patients were stratified into two categories: BSA ratio <0.9 and ≥0.9. Graft survival rates were compared between these two groups using Kaplan-Meier survival curves and Cox proportional hazards models. RESULTS: Of the 1880 patients identified, 116 (6.2%) had a donor/recipient BSA ratio <0.9 and 1764 (93.8%) had a donor/recipient BSA ratio ≥0.9 group. BSA ratio <0.9 conferred an increased risk of graft loss (adjusted hazard ratio, 1.61; 95% confidence interval, 1.13-2.27; P=0.008). Patients with a donor/recipient BSA ratio ≥0.9 group had a significantly longer graft survival compared with those with a donor/recipient BSA ratio <0.9 after adjustment for donor age and gender, recipient age, gender, ethnicity, cause of renal failure, as well as clinical factors, such as cold and warm ischemia time and HLA mismatch. CONCLUSION: We conclude that low donor/recipient BSA ratio was associated with an increased risk of graft loss. Appropriate size matching conferred better long-term graft survival in adolescents receiving live-donor kidney transplants.

Intestinal lengthening and nutritional outcomes in children with short bowel syndrome.

BACKGROUND: Intestinal lengthening remains a treatment option in pediatric short bowel syndrome. However, clinical indications and nutritional outcomes from bowel lengthening are not well defined. METHODS: A retrospective review of a prospective database was conducted of patients who underwent bowel lengthening using serial transverse enteroplasty (STEP) at a single center. RESULTS: Sixteen children who underwent 19 STEP procedures were identified. STEP increased median small bowel length from 84 cm (range, 19 to 295 cm) to 103 cm (range, 24 to 375 cm) (P = .0001). Caloric provisions from parenteral nutrition were decreased after STEP (69% vs 0%, P < .01). Of 15 subjects in the nutritional analysis, 12 (80%) had improved enteral tolerance, and 9 (60%) achieved enteral autonomy after STEP. Six STEP procedures were not associated with improvements in enteral nutrition, and 5 (83%) were performed in children with gastroschisis. CONCLUSIONS: A majority of children with short bowel syndrome were weaned off parenteral nutrition after STEP. Gastroschisis may portend a less optimal outcome from the procedure.

Ultrashort bowel syndrome in children.

OBJECTIVE: Recent data have demonstrated improved survival in children with intestinal failure. We hypothesized that this trend would also be observed in children with ultrashort bowel syndrome. METHODS: A prospective database from Seattle Children's Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with 10 cm or less of small bowel enrolled in the program since 2005. Data are listed as median (range). RESULTS: Five patients were identified with a bowel length of 6 (1-10) cm and follow-up of 54 (43-61) months. All children have survived and are currently between 3.5 and 5.5 years of age. One patient underwent isolated intestinal transplantation and one patient is currently listed for intestinal transplantation. The transplanted child is fully enterally fed. The other patients remain at least partially dependent on parenteral nutrition. None of these patients have current evidence of parenteral nutrition-associated liver disease. Patients have required extensive care after referral to our program, including 18 (15-32) visits to the emergency room, 152 (114-273) days of inpatient care, and 6 (5-9) central line-associated blood stream infections. CONCLUSIONS: Long-term survival in children with ultrashort bowel length is possible after referral to an intestinal failure program, although extensive medical management is required. These children may be reasonable candidates for long-standing intestinal rehabilitation as a bridge to intestinal transplantation.

Hepatic artery thrombosis and liver malignancy in pediatric liver transplantation.

BACKGROUND: Hepatic artery thrombosis (HAT) remains a significant cause of graft failure and mortality after pediatric liver transplantation. Conditions not associated with hepatic failure, such as liver tumors, may be more prone to thrombotic problems after transplant. We hypothesized that liver transplant for hepatic malignancies may be associated with increased rates of HAT in the posttransplant period. METHODS: We conducted a retrospective review of pediatric patients (age, 0-21 years) who underwent primary liver transplantation at a free-standing children's hospital from 1990 to 2009. We reviewed cause of underlying liver disease, age, sex, weight, occurrence of HAT, use of antiplatelets and anticoagulants perioperatively, as well as reintervention, retransplant, and death. RESULTS: A total of 129 children underwent 146 liver transplants, and 15 (12%) patients developed HAT. Nine liver transplants were performed for hepatic malignancy, and 4 (44%) of these patients developed HAT (relative risk, 4.85; 95% confidence interval, 1.9-12.2; P = .0015). All 4 children with hepatic malignancy and HAT required reintervention, including 3 retransplants (75%). One of these patients died. CONCLUSIONS: Hepatic artery thrombosis occurs approximately 5 times more often and appears to be more morbid in children with hepatic malignancy after transplantation. Prospective evaluation of prophylactic anticoagulation regimens in the setting of hepatic malignancy requiring transplantation is warranted.

Midterm follow-up of transjugular intrahepatic portosystemic shunts using polytetrafluoroethylene endografts in children.

PURPOSE: To report the initial experience of a pediatric center's use of expanded polytetrafluoroethylene (ePTFE) endografts for the creation of transjugular intrahepatic portosystemic shunts (TIPSs) in children. MATERIALS AND METHODS: Twelve consecutive patients had a TIPS created, all with ePTFE endografts. Nine were children younger than 18 years of age: one infant (age 18 mo) and eight adolescent children with a median age of 14 years, 3 months (range, 10 y, 1 mo, to 17 y, 2 mo). All had gastric or esophageal varices. Acute variceal bleeding was the primary indication in four of nine cases (44%). Mean follow-up was 20 months (range, 4 d to 32 mo). RESULTS: Technical success rate was 100%. Seven TIPSs (78%) were created with a 10-mm-diameter endograft and two (22%) were created with an 8-mm-diameter endograft. Mean portosystemic gradient was reduced from 15.9 mm Hg to 5.8 mm Hg after TIPS creation. The devices were not dilated to their nominal diameter in seven of nine cases (78%). There were no major complications or mortalities associated with TIPS creation. There was one minor complication during follow-up in which a child developed progressively increasing lethargy. Primary patency rate by ultrasound during follow-up was 100%. There were no shunt dysfunctions or repeat interventions required, and no clinical recurrences. One child received a liver transplant 4 days after TIPS creation. CONCLUSIONS: TIPSs can be created in children with the use of ePTFE endografts with an excellent midterm primary patency rate and a low complication rate.