Incidence,impact, and treatment of portal and hepatic venous complications following pediatric liver transplantation: A single‐center 12 year experience

Heffron TG, Pillen T, Smallwood G, Henry S, Sekar S, Casper K, Solis D, Tang W, Fasola C, Romero R. Incidence, impact, and treatment of portal and hepatic venous complications following pediatric liver transplantation: A single‐center 12 year experience.
Pediatr Transplantation 2010: 14:722–729. © 2010 John Wiley & Sons A/S. Abstract: PVT or PVS and HVOO are known causes of graft and patient loss after pediatric liver transplantation. Increased incidences of these complications have been reported in partial livers including DDSLT or LDLT. From 1997 to 2008, 241 consecutive pediatric patients received 271 hepatic grafts at a single center. Median follow‐up is 1856 days. Surgical technique, demographics, lab values, and radiologic imaging procedures were obtained utilizing OTTR^® to evaluate the relationship of portal and hepatic complications with risk factors, patient and graft survival. Grafts were composed of 115/271 (42.4%) partial livers of which 90 (33.2%) were DDSLT and 25 (9.2%) LDLT. Of 271 patients, 156 (57.6%) received whole‐sized grafts. There were six PVC in five patients with one patient requiring retransplantation (0.34%) and no patient deaths. Utilizing all three hepatic vein orifices on the recipient hepatic vena cava and the donor hepatic vein cut short enables a wide hepatic outflow tract unlikely to twist. None of the 241 patients developed early or late complications of the hepatic vein. None of the last 128 consecutive patients who received 144 grafts over seven and a half yr have developed either early or late complications of the hepatic or portal vein. Partial‐graft actuarial survival was similar to whole‐graft survival (87.2% vs. 85.3% at one yr; 76.6% vs. 80.2 at three yr; p = 0.488). Likewise, patient survival was similar between partial grafts and whole grafts (93.8% vs. 93.1% at one yr; 89.8% vs. 87.2% at three yr; p = 0.688) with median follow‐up of 1822 (±1334) days. Patients receiving partial livers were significantly younger and smaller than patients receiving whole livers (p < 0.001). Portal and hepatic venous complications may have negative effects on patient or graft survival after pediatric liver transplantation. In our series, there was one graft and no patient loss related to portal or hepatic venous complications after pediatric liver transplantation over 12 yr.     

Chemoresistant hepatoblastoma in a patient with mosaic trisomy 18 treated with orthotopic liver transplantation

We present a 9-month-old male with mosaic trisomy 18 with a right hepatic lobe mass. The tumor was completely resected and identified as pure fetal histology hepatoblastoma but contained increased mitotic activity. Adjuvant chemotherapy consisted of cisplatin, vincristine, and 5-fluorouracil. After the first and fourth cycles of chemotherapy, recurrent tumor developed. The patient underwent rescue orthotopic liver transplantation, and is currently alive without evidence of hepatoblastoma 28 months after transplantation. This report demonstrates the use of orthotopic liver transplantation in a child with mosaic trisomy 18 and hepatoblastoma.     

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.     

The efficacy of liver transplantation in malignant liver tumors associated with tyrosinemia: clinical and laboratory findings of five cases

To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.     

Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report

Backes AN, Tannuri ACA, de Mello ES, Gibelli NEM, de Castro Andrade W, Tannuri U. Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report. Abstract: Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor–recipient serostatus, especially in relation to the Epstein–Barr virus. The patient was a two‐yr‐old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor. Nine months after transplantation, one of the adult recipients developed an urothelial tumor in the kidney graft. Imaging tests were repeated monthly in the liver‐transplanted child and revealed no abnormalities. However, one yr and two months after the transplantation, the patient developed episodes of fever. At that time, imaging and liver biopsy showed a clear cell tumor of urothelial origin in the graft and the disease was limited to the liver. The patient underwent liver retransplantation, and she is currently free of tumor recurrence. Although rare, the occurrence of tumors in the post‐transplant period from cadaver donors, without previously diagnosed tumors, is one of the many problems encountered in the complex world of organ transplantation.     

Development of liver size and perfusion after reduced-size liver transplantation in children

The technique of segmental liver transplantation (s-LTx) provides a method to overcome the shortage of suitable livers for small recipients. Patient survival rates are parallel to those obtained with whole liver transplantation (w-LTx). For long-term rehabilitation, adaptive liver growth and adequate perfusion is crucial; however, morphometric and hemodynamic parameters in growing children with s-LTx are not available. Seventeen children who received a s-LTx and 25 with a w-LTx who had follow-up evaluation 1 and 2 yr after LTx were studied. Mean age at time of transplantation was 4.3 +/- 3.5 yr for s-LTx and 10.3 +/- 6.0 yr for w-LTx, mean height 98 +/- 21 cm and 122 +/- 30 cm respectively. At follow-up evaluation mean values for liver enzymes, bilirubin and prothrombin time were in the normal ranges for both groups. Liver dimensions were measured by gray scale ultrasound, and hemodynamic parameters by Doppler sonography in the portal vein and hepatic artery using an Acuson 128 machine. Maximal (Vmax), minimal (Vmin) and time-average velocity (TAV) were measured and the resistive index (RI) calculated. We found that 1 and 2 yr after LTx liver dimensions were at a mean in the upper normal range of healthy controls. Spleen size was above the normal range and did not show any tendency towards regression. Mean Vmax in the hepatic artery in s-LTx and w-LTx was 48 cm/sec vs. 28 cm/sec after 1 yr and 30 cm/sec vs. 35 cm/sec after 2 yr, the RI 0.66 vs. 0.55 and 0.59 vs. 0.73, respectively (p for all parameters > 0.05). Maximal portal vein flow was 25 cm/sec in s-LTx vs. 29 cm/sec in w-LTx. Blood flow calculated by vessel diameter and TAV showed no statistical difference between both groups. In conclusion, liver size after s-LTx and w-LTx was increased to the upper normal range, and portal vein blood flow velocities were within the normal range. Vmax in the hepatic artery was reduced in s-LTx; however, the reduction was to the same extent as in w-LTx. In the view of long-term functional adaptation, s-LTx is not inferior to w-LTx.     

Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience

Guiteau JJ, Cotton RT, Karpen SJ, O’Mahony CA, Goss JA. Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: The UNOS experience.
Pediatr Transplantation 2010: 14: 326–331. © 2009 John Wiley & Sons A/S. Abstract: Treatment for HEH does not follow a standardized algorithm. From clinical experience, it is assumed that pediatric patients with HEH will fare as well as other common pediatric liver tumors post‐OLT. The UNOS dataset was examined for patients with pediatric OLT between 1987 and 2007. Patients were grouped into non‐tumors, HB, HCC, HEH, and rare liver tumors. COD analysis was calculated using Fisher’s exact test. Patient, allograft, and recurrence‐free survival were compared using Kaplan–Meier curves and log‐rank tests. A total of 366 patients with pediatric OLT were identified with primary liver tumors (HB – 237, HCC – 58, HEH – 35, other – 36). HEH patient survival (five yr: 60.6%) was poorer than non‐tumor OLTpatient survival (five yr: 84.4%). Survival was worse when compared to HB (five yr: 72%) and rare liver tumors (five yr: 78.9%), but better than HCC (five yr: 53.5%). Allograft survival in HEH (five yr: 50.1%) lies between HB (five yr: 63.6%) and HCC (five yr: 42.8%). COD analysis demonstrates recurrence as a major cause in HB and HCC, but not for HEH or other liver tumors. The data suggest that patient survival may not be as high as previously believed and further investigation is warranted.     

Emotional and behavioral problems after pediatric liver transplantation: a quantitative assessment

Several uncertainties regarding psychological problems in children who underwent liver transplantation and the need to differentiate these disturbances from those related to the underlying previous chronic liver disease itself exist. This background triggered the present pilot study to investigate, using quantitative assessment methods, the incidence and the type of emotional and behavioral disturbances after liver transplantation. Sixteen liver transplant recipients (aged 5.7-14.4 yr) and 12 age-matched controls with stable chronic liver disease were assessed through the parent report form of Child Behavior Checklist/ 4-18. The mean time elapsed since transplantation was 8.1 yr. No patient or family had received psychological support during chronic liver disease or at any phase of the transplantation process. Transplanted children scored within borderline range for Internalizing and Total Behavioral Problems and within pathological range for Competences, except for the Activity Scale. Transplanted children showed more Total Behavioral (p = 0.005) and Externalizing Problems (p = 0.0005) than controls. Both groups scored within the pathological range for Total Competences with no significant differences between the two groups. Our findings suggest that in the absence of support programs a psychological risk does exist for a long period of time, after transplantation. Regarding Total Behavioral Problems and Externalizing Problems, this risk is higher than in children with chronic liver disease.     

Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein

Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.     

Hepatitis C viral recurrence in a pediatric patient following liver transplantation

Hepatitis C virus (HCV) infections are known to have a more benign course in children than in adults. Although the natural history of HCV recurrence after liver transplantation has been well studied in adult patients, much less is known about HCV recurrence after liver transplantation in pediatric patients. Herein, we report a case of a pediatric patient with HCV presumably acquired through vertical transmission. She underwent liver transplantation at 14 yr of age. The first three yr after liver transplantation were uneventful. However, in the past 12 months she has been hospitalized twice after developing ascites, hematemesis and esophagogastroduodenoscopy (EGD)-documented esophageal varices. Post-transplant biopsy has demonstrated chronic inflammation complicated with active hepatitis C and stage 2-3 scarring. This case report demonstrates the need for further epidemiologic studies to study the natural history of the rate of HCV recurrence after liver transplantation in the pediatric population.     

Liver transplantation for hepatocellular carcinoma in children

We present our experience with living-donor liver transplantation in the treatment of nine children with hepatocellular carcinoma. Between January 2001 and March 2007, we performed 81 liver transplantations in 79 children at our center. Nine of the 79 children (11.3%; mean age, 9.7 +/- 5.5 yr; age range, 12 months-16 yr; male-to-female ratio, 2:1) underwent an living-donor liver transplantation because of hepatocellular carcinoma. Two of nine children received right lobe grafts, three received left lateral segment grafts, and the remaining four children received a left lobe graft. According to the TNM staging system, two children had stage 1 carcinoma, three had stage 2, and four had stage 4A(1). The mean follow-up was 19.8 +/- 10.6 months (range: 7-32 months). There has been only one tumor recurrence, which occurred in the omentum 26 months after liver transplantation. There was no evidence of recurrence or AFP elevation in the other eight children. Both graft and patient survival rates are 100%. In conclusion, liver transplantation is a life-saving procedure for children with chronic liver disease with accompanying hepatocellular carcinoma. During follow-up of patients with chronic liver disease, serial AFP screening and combined radiologic imaging studies should be mandatory.     

Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful “Kasai” hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was found when she was 5-years old. Needle biopsy could not establish a definitive diagnosis. Because the tumor size was not changing, she was conservatively followed by abdominal echo at an outpatient clinic. She showed progressive liver dysfunction and ongoing cirrhosis, and so at the age of 10 years, she received living donor liver transplantation at our institute. The tumor was pathologically diagnosed as a FNH. Though a FNH in biliary atresia patients is extremely rare, there are only two cases reported in the literature; it should be considered in the differential diagnosis for a hepatic nodule during a long follow-up course in patients with biliary atresia.     

Liver transplantation for refractory severe pruritus related to widespread multifocal hepatic focal nodular hyperplasia (FNH) in a child: Case report and review of literature

Merli L, Grimaldi C, Monti L, Nobili V, Francalanci P, de Ville de Goyet J. Liver transplantation for refractory severe pruritus related to widespread multifocal hepatic focal nodular hyperplasia (FNH) in a child: Case report and review of literature.
Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: FNH is a rare and benign tumor of the liver. It is not a conventional indication for liver transplantation, and no transplant for FNH in a child has been reported to date. Multifocal FNH growing in adolescent age to a widespread tumor invading the whole liver and associated with severe refractory pruritus was an unusual indication for transplantation in a 13‐yr‐old girl. The operation and the follow‐up were uneventful, allowing full recovery and disappearance of pruritus.     

Hepatic artery thrombosis in pediatric liver transplantation: Graft salvage after thrombectomy

Hepatic artery thrombosis (HAT) is a devastating complication that may occur after orthotopic liver transplantation (OLT). A higher incidence has been reported in children. Salvage of the graft by thrombectomy has been suggested as an alternative to re-transplantation. In this study we report the outcome of three children who underwent thrombectomy for HAT. Between January 1992 and June 1998, 14 children (< 17 yrs of age) underwent liver transplantation. Three developed HAT (one a whole-liver graft recipient, age 17; two living-related graft recipients, ages 4 and 4.5 yr). In the first patient, thrombosis of the hepatic artery was associated with scattered areas of parenchymal necrosis on computed tomography. In the two living-related patients, HAT was found incidentally during re-exploration for bleeding (day 2 and day 10). Thrombectomy was performed in all three patients. At 18-24 months after thrombectomy, all three children had normal graft function. In the first patient, complete regeneration of the liver has been documented by computed tomography and a late asymptomatic recurrent thrombosis is suggested by absence of arterial flow on Doppler examination. The hepatic artery is patent in the two living-related recipients. One of these living-related recipients developed ischemic bile duct stricture and underwent successful percutaneous balloon dilatation. We conclude that long-term normal graft function can be achieved by thrombectomy in pediatric liver recipients with HAT, even in the presence of limited parenchymal damage.     

Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease

We reviewed the outcome of children undergoing living related liver transplantation (LRLT) for Wilson's disease (WD), and specifically addressed the potential risk associated with the use of donors who were heterozygous for the Wilson genetic defect. LRLTs were carried out in 11 children with WD, nine of whom presented with fulminant hepatic failure and two with end-stage hepatic insufficiency. The age of the patients ranged from 6 to 16 yr. Eight patients had hepatic encephalopathy and were plasmapheresed preoperatively. The donors (all parents: six fathers and five mothers) were all one-haplotype matched with their respective recipients, and were all therefore heterozygote carriers of the WD genetic defect. The serum ceruloplasmin levels were within normal limits in all donors (mean: 20.0 +/- 2.85 mg/dL). All recipients but one had low serum ceruloplasmin levels with a mean value of 11.6 +/- 7.36 mg/dL before transplantation. The serum ceruloplasmin levels had increased to an average of 21.0 +/- 3.76 mg/dL after LRLT at the latest evaluation, which ranged between 7 and 75 months after transplantation. A marked reduction in urinary copper excretion was observed in all recipients after transplantation. Of eight recipients presenting preoperatively with Kayser-Fleischer (K-F) rings, this abnormality resolved completely after LRLT in five patients and partially in three. All recipients are alive and remain well, and none have developed signs of recurrent WD after a mean follow-up period of 31 months (range 7-75 months). In conclusion, LRLT is an excellent choice for effective treatment of WD, and grafts chosen from heterozygote carriers of the condition do not appear to confer any risk of recurrence in the recipients.     

Long-term glomerular filtration rate following pediatric liver transplantation

In adult patients a significant proportion of chronic renal failure after liver transplantation (LTX) has been described. This was attributed mainly to nephrotoxicity caused by Calcineurin inhibitors (CNI). If these results are transferable to pediatric patients was the aim of this study. Forty-five pediatric patients with a LTX performed between 1988 and 2003 were evaluated. Glomerular filtration rate was calculated using the Schwartz formula (calculated GFR (cGFR) (mL/min/1.73 m2) = kx height (cm)/serum creatinine (mg/dL)). Median age at LTX was 4 yr (range 0.3-18.1). Pretransplant median cGFR was significantly elevated with 157.5 mL/min/1.73 m2. Within the first 3 months after LTX median cGFR normalized to a median value of 102.7 (p < 0.05 vs. pretransplant cGFR). During long-term follow-up median cGFR remained stable with calculated values of 108.0 two years and 112.6 five years after transplantation. Using a linear and an exponential one compartment mathematical modeling of renal function the calculated GFR was stable even for very long observation times (n > 10 yr). Liver insufficiency prior to transplantation was associated with glomerular hyperfiltration. After successful liver transplantation cGFR normalized within the first 3 month and, in contrast to the reported GFR impairment in adult liver transplant recipients, remained stable, even in long-term follow-up.     

Pediatric hepatocellular carcinoma in a developing country: Is the etiology changing?

HCC is the second most common malignant liver tumor of childhood. It typically affects children with a median age of 10–14 yr on background hepatitis B‐related liver disease and is often metastatic or locally advanced at diagnosis. Children below the age of five yr typically constitute <10% of all children with HCC. In these children, it occurs on a background of congenital or metabolic liver disease. The records of all children with HCC who presented to our department over a six‐yr study period were reviewed. Twelve patients with a median age of 5.9 yr (range 1.6–15.4) were diagnosed to have HCC. All patients underwent liver transplantation, and none were resected. Eleven patients had background congenital or metabolic liver disease. All five of those with hereditary tyrosinemia type 1 who presented to us were found to have HCC. No patient had hepatitis B‐related liver (HBV) disease. Eight (66.7%) patients had incidentally discovered HCC on examination of the explant. Incidentally discovered HCC were smaller, well differentiated, and did not show microvascular invasion compared to those diagnosed preoperatively. There was no recurrence with a median follow‐up of five months. The patient demographic for pediatric HCC is changing probably as a consequence of successful immunization against HBV. Younger patients with congenital and metabolic liver disease in whom liver transplantation is the ideal treatment are likely to constitute an ever‐increasing proportion of patients with pediatric HCC as HBV disease is controlled or eradicated.     

A case of Epstein–Barr virus–associated pulmonary leiomyosarcoma arising five yr after a pediatric renal transplant

Suzuki K, Urushihara N, Fukumoto K, Watanabe K, Wada N, Takaba E. A case of Epstein–Barr virus–associated pulmonary leiomyosarcoma arising five yr after a pediatric renal transplant. Pediatr Transplantation 2011: 15: E145–E148. © 2010 John Wiley & Sons A/S. Abstract: EBV‐associated SMTs in immuno‐compromised patients have recently been reported. We report on a case of EBV‐associated pulmonary leiomyosarcoma arising five yr after renal transplantation. The patient was an eight‐yr‐old girl, who received a living related kidney transplant from her mother. She had had bilateral giant Wilm’s tumors as an infant and underwent bilateral nephrectomy at one and two yr of age. At the age of seven, she suffered from bronchitis several times, and a year later, two nodules were detected in her left lung by X‐ray and computed tomography. We suspected a recurrence of Wilm’s tumor and performed surgical resection. The pathological finding was SMT with moderate mitosis and no evidence of Wilm’s tumor. The fact that the tumors were positive for EBER suggested an association with the EBV. Six months later, there was a recurrence in her left lung. Surgical resection was performed, and immunosuppressive agents were reduced. Two yr after the second operation, she is well with no recurrence. We report the first case of EBV‐associated pulmonary leiomyosarcoma in a pediatric patient after renal transplantation owing to a malignant tumor.     

Liver transplantation for type I and type IV glycogen storage disease

Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period. One patient had glycogen storage disease (GSD) type I (von Gierke disease) and seven patients had type IV GSD (Andersen disease). As previously reported [19], a 16.5-year-old-girl with GSD type I was successfully treated in 1982 by orthotopic liver transplantation under cyclosporine and steroid immunosuppression. The metabolic consequences of the disease have been eliminated, the renal function and size have remained normal, and the patient has lived a normal young adult life. A late portal venous thrombosis was treated successfully with a distal splenorenal shunt. Orthotopic liver transplantation was performed in seven children with type N GSD who had progressive hepatic failure. Two patients died early from technical complications. The other five have no evidence of recurrent hepatic amylopectinosis after 1.1–5.8 postoperative years. They have had good physical and intellectual maturation. Amylopectin was found in many extrahepatic tissues prior to surgery, but cardiopathy and skeletal myopathy have not developed after transplantation. Post-operative heart biopsies from patients showed either minimal amylopectin deposits as long as 4.5 years following transplantation or a dramatic reduction in sequential biopsies from one patient who initially had dense myocardial deposits. Serious hepatic derangement is seen most commonly in types I and IV GSD. Liver transplantation cures the hepatic manifestations of both types. The extrahepatic deposition of abnormal glycogen appears not to be problematic in type I disease, and while potentially more threatening in type IV disease, may actually exhibit signs of regression after hepatic allografting.