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We present an approach to safely expose the hepatic hilum for revision procedures after left lobe living donor liver transplantation. A 14-year-old adolescent girl who had undergone left lobe living donor liver transplantation experienced repeated episodes of cholangitis. Because treatment with interventional techniques failed, surgical revision was indicated. The right thoracoabdominal approach was selected to minimize dissection. Intraoperative findings showed adhesive kinking of the Roux-Y limb just distal to the bilioenteric anastomosis, and a side-to-side jejunojejunostomy was performed. The thoracoabdominal approach leads to easy and excellent reoperative exposure of the hilar site of a left lobe liver graft.

We present the cases of 3 children with huge undifferentiated sarcoma of the liver who were treated with surgical excision including liver transplantation as an option and adjuvant chemotherapy. All 3 patients were males aged 10, 13, and 15 years old. The size of the tumor was 10, 15, and 20 cm in diameter, respectively. The youngest patient is disease free and doing well 43 months after resection. The 13-year-old patient presented with tumor rupture and underwent operation. The primary tumor and the ruptured tissue fragments were removed and he was given postoperative chemotherapy. The patient is disease free and doing well 52 months after surgery. The oldest patient had an unresectable tumor in the hilar region. Preoperative chemotherapy was given but later discontinued owing to severe side effects. He underwent living donor liver transplantation followed by postoperative chemotherapy. The patient had recurrent tumor 24 months after transplantation that was excised at reoperation. He is doing well and is disease free 18 months after the second procedure. Complete removal of the tumor including total hepatectomy and transplantation when indicated and suitable pre- and/or postoperative chemotherapy is an effective treatment for children with undifferentiated sarcoma of the liver.

The survival outcome for patients with hepatoblastoma normally depends on the resectability of the tumor. In Japan, the pre and/or postoperative chemotherapy protocol using a combination of cisplatin (CDDP) and tetrahydropyranyl-Adriamycin (THP-ADR) has been the standard treatment since 1991. This study aims to assess exactly what influence the establishment of this chemotherapy protocol has had on both the tumor resectability and the outcome of patients with hepatoblastoma.From 1982 to 1997, 60 patients with hepatoblatoma were treated in the Kyushu area, Japan. Based on the pretreatment extent of disease (PRETEXT), the outcome and tumor resectability were compared between group A (1982 to 1990, n = 27, PRETEXT I:5, II:8, III:6, IV:8) and group B (1991 to 1997, n = 33, PRETEXT I:9, II:9, III:5, IV:10).The 5-year survival rates (group A and group B) were 33% and 73% for all cases (P.01), 100% and 89% for PRETEXT I, 38% and 89% for II (P.05), 17% and 80% for III (P.01), and 0% and 40% for IV (P.01), respectively. The 5-year survival rates for patients with metastases were 0% for group A (n = 5) and 57% for group B (n = 7; P.01). The rates of a complete resection of primary tumor were 48% for group A and 67% for group B. In particular, a significant difference was found regarding the complete resection rate between groups A and B in the patients with PRETEXT III (17% for group A and 80% for group B; P.01). In the patients with an incomplete tumor resection (14 for group A, 11 for group B), the 5-year survival rates were 0% for group A and 45% for group B (P.01).The optimal chemotherapeutic regimen of CDDP and THP-ADR was thus found to greatly contribute to the improved survival rate of hepatoblastoma patients. Preoperative chemotherapy resulted in an increased resectability of the tumor, whereas postoperative chemotherapy played an important role in the increased cure rate of cases with either an incomplete tumor resection or metastasis. However, refractory cases with PRETEXT IV or metastasis may still require the development of an even more effective treatment modality, including the use of blood stem cell transplantation.

The authors report 3 cases of liver transplantations in children between 4 and 10 years of age, complicated with malignant hepatic tumors after biliary atresia. The preoperative abdominal computed tomography (CT) scans of all 3 cases showed hepatic masses. The serum alpha-fetoprotein levels were elevated highly in 2 cases. After living-related liver transplantation (LRLT), the pathologic findings of the masses in the resected livers showed hepatocellular carcinoma in 2 cases and hepatoblastoma in the other. All cases were associated with biliary cirrhosis. The stage of the liver tumor in the 3 cases using the TNM system was IVA (T4, N0, M0), II (T2, N0, M0) and IVA (T4, N0, M0). Chemotherapy was used in all cases after liver transplantation, and all patients survived with no recurrence. The results suggest that even though malignant liver tumors rarely are complicated with biliary atresia in childhood, one should be alert to the occurrence of hepatic malignancy and perform routine screening of alpha-fetoprotein levels, abdominal CT scans, and magnetic resonance imagings.

Background and purpose The strategy used to treat pediatric renal tumors in Japan is based on the Japanese Wilms' Tumor Study (JWiTS) protocol, which was based on the National Wilms' Tumor Study (NWTS)-5 regimen. The regimen is characterized by an initial radical operation, followed by adjuvant chemotherapy and radiotherapy. Concerning the histological classification, a new classification based on the International Society of Pediatric Oncology (SIOP) classification was used beginning in 2008. The main points of revision are that the "blastemal predominant type" was classified as an independent category in the Wilms' tumor subtypes. The purpose of this study was to analyze the biological characteristics from the standpoint of the newly established histological classification. Materials and methods From 1971 to 2005, 174 cases of Wilms' tumors treated with an initial operation followed by adjuvant therapy were re-evaluated by the new histological classification. Histologically, all these materials showed no secondary changes associated with adjuvant therapy. Results According to the new classification, Wilms' tumors were classified into four subtypes, including the mixed type (n=112), epithelial type (n=17), mesenchymal type (n=15), and blastemal predominant type (n=26). The 5 year overall survival rates were as follows; mixed type (90.1%), epithelial type (100%), mesenchymal type (93.3%), and blastemal predominant type (65.4%). Conclusion The patients with blastemal predominant tumors demonstrated a significantly worse prognosis compared with those of other subtypes. The treatment strategy of blastemal predominant category should be distinguished from the other favorable subtypes.

During the last 31 months, 50 children between 3 months and 15 years of age have undergone living related liver transplantation (LRLT) for end-stage liver diseases (39 biliary atresia, 2 Budd-Chiari syndrome, 2 progressive intrahepatic cholestasis, 3 liver cirrhosis, 1 Wilson disease, 1 protoporphyria, 1 tyrosinemia, and 1 fulminant hepatitis). Combined FK-506 and low-dose steroids were routinely used for immunosuppression. There were seven deaths, two of which were related to infection (Candida pneumonia and Epstein-Barr virus [EBV]-associated lymphoproliferative syndrome [LPS]). Five patients had a bacterial infection, all of which were associated with surgical complications. Three patients had Candida infection, all of which were malnourished, had biliary atresia, and had been managed with prolonged antibiotics against obstinate ascending cholangitis. There were 14 symptomatic viral infections (1 herpes simplex virus, 1 herpes zoster virus, 5 cytomegalovirus [CMV], 6 EBV, and 1 EBV-associated LPS). Three of the five CMV infections appeared in patients whose graft was ABO-incompatible, who were managed with prophylactic OKT-3. Most of the viral infections (except 1 EBV-associated LPS) were minor and were treated successfully. The low incidence and successful treatment of CMV infection are related to the high compatibility and low incidence of allograft rejection in LRLT. Bacterial and fungal infections can be decreased by greater refinement of surgical technique and more aggressive preoperative management. Treatment of EBV infection is still an unsolved problem.

Survivors of childhood cancer have a higher risk of developing a secondary neoplasm in their lifetime. The increased risk of a second malignant neoplasm is related to treatment of the primary tumor and genetic predisposition. We describe a 19-year-old man with 2 hepatic masses, one of which was diagnosed as a hepatic angiomyolipoma and the other as focal nodular hyperplasia 14 years after the treatment of stage IV pelvic rhabdomyosarcoma. The combination of these tumors has not previously been reported in the literature.

Background and Purpose In some small infants who are transplanted with a large-for-size graft by living donor liver transplantation, an incisional hernia is intentionally made to decrease the tension on the graft. The procedure and timing for repair of this type of hernia were retrospectively evaluated. Patients and Methods Repair was carried out in 3 patients at 4 to 11 years after living donor liver transplantation. The preoperative, perioperative, and postoperative statuses were analyzed in each patient. Result Fascial closure was possible in all 3 patients. In 2 patients, separation of a component of the rectus sheath or a lower part of the major pectoral muscle was required for approximation of the fascia. One recipient had transient bile leakage that was treated successfully. Conclusion An intentionally made hernia should be as small as possible to facilitate easy primary closure at a later date. A procedure resembling the "clam-shell opening" method, which used a partly separated and extended sheet of the fascia, was feasible to avoid the requirement for an artificial mesh. Preschool ages may be suitable for easier approximation of the fascia.

Background/Purpose Since 1996, the standard treatment of Wilms tumors in Japan has been based on the regimen of the Japanese Wilms Tumor Study. However, in Japan, there have been no reports about Wilms tumors that analyzed the clinical features and patient outcome in a large series until now. This study aims to assess the clinical characteristics of patients with Wilms tumor with a favorable histology from a retrospective standpoint in the Kyushu area in Japan and, furthermore, to analyze the historical changes of clinical features and outcome from the 1980s to the 1990s. Methods Between 1982 and 1996, 90 cases of Wilms tumors with a favorable histology were registered in the Kyushu area. Regarding the clinical feature and outcome, they were divided into 2 groups (group A, 1982-1989, n=50; group B, 1990-1996, n=40). The outcome was analyzed based on the 5-year overall survival rate. Results The clinical features (age, sex, initial symptom, location, stage) demonstrated no definite differences between group A and group B. Regarding the operation, the rate of an initial complete resection in the early stages was significantly higher in group B than in group A. All stage V cases in group B undewent a bilateral tumor biopsy instead of a radical nephrectomy as the initial operation. The 5-year overall survival rate throughout the whole period was 87.8%, whereas the rates were 84.0% for group A and 90.0% for group B ( P = NS), respectively. Of particular note, the outcome of patients with stage I and stage V in group B substantially improved in comparison to that in group A. However, in advanced cases, no significant improvement in the outcome was noted. Conclusions This is the first report about the clinical features and outcome for Wilms tumors with a favorable histology in Japan from the 1980s to the 1990s. The present study suggested that in the early-stage cases, an initially complete resection followed by standard postoperative chemotherapy substantially improved the outcome of the patients in group B. In the stage V cases, the performance of renal salvage surgery may have positively contributed to the improvement in the outcome in group B. However, in the advanced stage cases, no definite improvement was noted. In the future, an improved efficacy of the treatments for Wilms tumors based on the standard protocol established by the Japanese Wilms Tumor Study in 1996 is expected in Japan.

Purpose: This study aims to assess the prognostic factors and optimal treatments for malignant germ cell tumors (MGCT) in childhood. Methods: Among 117 MGCT, the clinical features were analyzed. Regarding the histology, there were 89 embryonal carcinomas, 13 dysgerminomas, 4 choriocarcinomas, and 11 others. The prognostic factors and treatments were assessed based on the 5-year survival rate. Results: (1) Stage: 100% for stage I (n = 54), 75.0% for stage II (n = 4), 67.3% for stage III (n = 14), and 54.8% for stage IV (n = 33); Unknown: n=12. (2) Primary site: 93.4% for the testis (n = 52), 86.7% for the ovary (n = 31), 56.9% for the sacrococcygeal (n = 21), and 60.6% for others (n = 12); unknown: n=1. (3) Surgical intervention for primary tumor: 100% for stage I with a complete resection (n = 53), 78.4% for stage III, IV with a complete resection (n = 26), and 33.3% for stage III, IV with an incomplete resection (n = 21). (4) Type of chemotherapy for the stage III and IV: 83.9% for the PVB (cisplatin, vinblastin, bleomycin; n=13), 66.7% for the VAC (vincristine, actinomycin D, cyclophosphamide; n=6), and 47.1% for other regimens (n = 25). Conclusions: An early stage, a diagnosis under 1 year of age and a primary site in the gonads were favorable prognosis factors, whereas histologic findings of choriocarcinoma and liver or lung metastasis were unfavorable. Radical complete resection alone is a sufficient treatment for localized MGCT. The PVB regimen is optimal chemotherapy for advanced MGCT; however, high-risk cases still may require more aggressive treatment. J Pediatr Surg 37:1703-1706. Copyright 2002, Elsevier Science (USA). All rights reserved.

Background/Purpose: Ornithine transcarbamylase (OTC) deficiency is an X-chromosome-linked genetic disorder resulting in hyperammonemia hepatic dysfunction, coma, and serious neurological sequelae. This report describes an experience in treating this condition with living-related liver transplantation. Methods: Three children with OTC were treated with a living-related liver transplantation. Potential donors were evaluated with the allopurinol loading test to exclude heterozygotes. FK506 and low-dose steroids were used for immunosuppression. Auxiliary partial orthotopic transplantation (APOLT) was used in two of the cases. Results: All three children survived and are doing well without protein restriction. Conclusions: These observations suggest that in OTC removal of the native liver is not necessary because (1) a partial liver segment with normal enzyme activity corrects the hyperammonemia, (2) in case of graft failure the native liver is an available backup, and (3) the native liver has the ability to recover function. As long as living-related donors serve as the main source of donor organs, APOLT is a safer approach to liver transplantation in OTC.

To clarify the pathogenesis of pericholangitis in biliary atresia, immunohistochemical and ultrastructural methods were used to quantify the amount of collagen types III and IV, and alpha-smooth muscle actin-positive myofibroblast present in the pericholangial tissue. Patients were divided into two groups: the early-stage group (n = 7; 15 to 30 days old at the time of the Kasai operation), and the advanced-stage group (n = 14; 8 months to 6 years old at the time of liver transplantation). The liver specimen was harvested at the time of the Kasai operation in the early-stage group, and during liver transplantation in the advanced-stage group. Liver tissue from the donor was used as the control. The specimen was visually inspected and classified into three grades to express the extent of proliferation. An increase of collagen types III and IV, and myofibroblast in the pericholangial area was seen even in 2- to 4-week-old patients, which became marked with the progression of the fibrotic disease process. This suggests that pericholangitis occurs in the very early stage of biliary atresia, perhaps before the development of bile duct obstruction.

A case of pancreatoblastoma resected successfully after effective chemotherapy is discussed. Preoperative chemotherapy may be useful in patients with advanced unresectable pancreatoblastoma to permit complete resection of the tumor.

For the management of persistent rectal achalasia after the Soave endorectal pull-through procedure, we have used posterior sagittal myectomy of the remaining aganglionic rectal muscular cuff, and have had satisfactory outcomes in five patients. Via a posterior sagittal skin incision, the posterior aspect of the rectal muscular cuff is reached. With the striated muscular comlex retracted downward, the level of the dentate line is identified on the posterior wall of the rectum with the aid of the surgeon's finger inserted inside the anorectum. Two parallel longitudinal incisions are made on the rectal muscular cuff to create a muscular strip which is elevated and excised; the distal end of the myectomy strip is at the level of the dentate line and includes a part of the internal anal sphincter muscle. During the last 4 years, we performed this procedure in 5 patients with remarkable relief of constipation, distension, and enterocolitis. The advantages of this procedure include: (1) less technical difficulty than the transanal approach, (2) avoiding colostomy, and (3) promising results.

After hepatic portoenterostomy for biliary atresia, granulation that formed at the porta hepatis caused biliary obstruction in seven out of 27 patients (26%). Six of the seven developed the complication during the first 6 weeks after surgery. The mortality rate was 29% (2/7). Among the same group, the incidence and mortality rate of ascending cholangitis was 19% (5/27) and 20% (1/5), respectively. The features characteristic of biliary obstruction caused by granulation, as compared with those of ascending cholangitis, were the absence of signs of infection and the lack of response to medical treatment. Quantitative analysis of the healing process of an experimental hepatoenterostomy in the rat showed that the mean thickness of the granulation formed at the anastomotic site (area, 5 x 20 mm) decreased almost constantly--from 1.49 mm on day 7 to 0.64 mm on day 42. Mucosa covered 20% of the granulation on day 7, 55% on day 14, 63% on day 21, 76% on day 28, and 92% on day 42. The increase in coverage was greatest during the second postoperative week. The results suggested that the healing of the hepatoenteostomy should be almost complete within 6 weeks. The operative method and postoperative management to prevent excessive granulation should be chosen so as to decrease the incidence of postoperative biliary obstruction.