Takuro Kazama

Impact of age at Kasai operation on short- and long–term outcomes of type III biliary atresia at a single institution

PURPOSE We reevaluated the impact of age at Kasai operation on the short- and long-term outcomes of type III biliary atresia (BA). PATIENTS AND METHODS From 1953 to 2009, 242 patients with type III BA underwent Kasai operation at ages ranging between 12 and 421 days (average, 79.7 days). The relationship between age at Kasai operation and jaundice disappearance rates (JDRs), and 10-, 20-, and 30-year native liver survival rates (NLSRs) were assessed retrospectively (JDR [%] = the number of patients in whom jaundice disappeared/the number of patients in each group × 100). RESULTS Age at Kasai operation had a significant impact on the JDRs (P < .001). However, there was no statistical relationship between long-term NLSR of the patients in whom jaundice disappeared after Kasai operation and operative age. From the results of the cumulative NLSRs estimated by Kaplan-Meier method, each survival rate was quite dependent on the age at operation until 30 years after Kasai operation, but the difference became much smaller in the later period provided age at operation was 4 months or younger. CONCLUSION The operative age as a prognostic factor might be less significant in the long-term outcome than in the short-term outcome.

Postnatal management of prenatally diagnosed biliary cystic malformation

PURPOSE Recent advances in ultrasonography have increased prenatal diagnosis of biliary atresia (BA) and choledochal cyst (CC). These conditions are not easy to distinguish before or just after birth. This study investigated diagnostic and therapeutic problems in prenatal diagnosis of BA and CC. METHODS We retrospectively studied clinical characteristics and progression of hepatobiliary cysts in 10 patients (4 cases of BA, 6 cases of CC) from the time of diagnosis. Chronological changes in cyst size and gallbladder morphology were assessed and measured sequentially by ultrasonography. RESULTS Three cases of BA were type I cyst and 1 case was type III-d. All cases of CC were type Ia. Cyst size decreased between birth and surgery in BA but increased in CC. The gallbladder appeared atrophic in BA. There was no significant difference in gestational age or cyst size at prenatal diagnosis, changes in cyst size between birth and surgery, and degree of liver fibrosis. CONCLUSIONS BA should be suspected if cyst size decreases before and after birth and the gallbladder atrophies after birth. Cholangiography is the only reliable method to differentiate BA from CC. Neonatal surgery is indicated for CC with icterus and liver dysfunction.

Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia

Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non-Hodgkin lymphomas and chronic lymphocytic leukemia, but it is rare in children with acute lymphoblastic leukemia (ALL). Here, we report a 7-year-old girl with chronic ITP during early intensive phase of chemotherapy for ALL. She underwent splenectomy because thrombocytopenia had persisted even after treatment with intravenous immunoglobulin (IVIG), steroids, vincristine, rituximab, and anti-D antibody. After splenectomy, her platelet count had recovered, and maintenance therapy could be resumed with a support of IVIG. To our knowledge, this is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient.

Technical standardization of Kasai portoenterostomy for biliary atresia

BACKGROUND The purpose of this study was to assess the clinical outcome of patients treated with the current technique of Kasai procedure compared with that of those treated with previous procedures. METHODS We retrospectively assessed the outcomes of 256 patients with biliary atresia who underwent Kasai portoenterostomy at our hospital between 1972 and 2014. Patients were divided into four groups: group 1 (1972-1981, double Roux-en Y, n=91), group 2 (1982-1991, Suruga II, n=80), group 3 (1992-2000, double-valve Roux-en Y, n=46), and group 4 (2001-2014, total removal of the extrahepatic biliary remnants at hepatic capsule and Roux-en Y reconstruction with a spur valve, n=39). Clinical outcomes were compared between the four groups. RESULTS In groups 1, 2, 3, and 4, the rate of jaundice clearance was 65.9%, 77.5%, 63.0%, and 87.2%, respectively; incidence of early cholangitis was 60.4%, 53.8%, 37.0%, and 23.1%, respectively; requirement for redo Kasai surgery was 15.4%, 37.5%, 17.4%, and 5.1%, respectively; 10-year native liver survival rate was 53.8%, 60.1%, 44.1%, and 73.7%, respectively; and 10-year overall survival rate was 55.0%, 72.3%, 86.7%, and 97.3%, respectively. CONCLUSION The standardized Kasai procedure was associated with favorable outcomes. Long-term outcomes remain to be evaluated. LEVEL OF EVIDENCE Case-control/treatment study, level III.

Living-related intestinal transplantation for a patient with hypoganglionosis

Abstract:  A 14‐yr‐old boy with total parenteral nutrition‐dependent short‐bowel syndrome associated with hypoganglionosis underwent the LR‐IT by using a 150 cm segment of distal ileum taken from a healthy donor. The graft vessels were connected to infrarenal aorta and inferior vena cava. The immunosuppressive regimen consisted of daclizumab, tacrolimus, and steroid. The graft surveillance for ACR was accomplished using zoom endoscopy and mucosal biopsy. The blood trough level of tacrolimus was maintained between 20 and 25 ng/mL for the first 2 months, followed by 15–20 ng/mL thereafter. The 50 mg of daclizumab was administered on the day of operation, and same dosage was repeated at 2‐wk intervals. The first ACR occurred on POD‐9 and was progressive, and required a 14‐day course of OKT‐3 injection. After the treatment with OKT‐3, the graft recovered from the ACR, and began to function well enough to discontinue the intravenous nutrition on POD‐55. No infectious complication has occurred. The patient was discharged in POD‐112, and currently tolerates full oral intake without requiring intravenous nutritional or fluid supplementation. The donor was discharged without any complications. The LR‐IT could successfully be performed with minimal risk to the donor, and it can be a treatment of choice for patients with short‐gut syndrome associated with hypoganglionosis.

The role of pulmonary metastasectomy for hepatoblastoma in children with metastasis at diagnosis: Results from the JPLT-2 study

BACKGROUND/PURPOSE The purpose of this study was to clarify the role of pulmonary metastasectomy in hepatoblastomas with lung metastasis at diagnosis. We reviewed cases enrolled in the JPLT-2 study. METHODS A total of 360 cases with hepatoblastoma were enrolled. The clinical courses and outcome of 60 cases with pulmonary metastasis at diagnosis were reviewed, focusing on metastasectomy. RESULTS Induction chemotherapy resulted in eradication of nodules in 26, residual nodules in 33, and early treatment-related death in one. Of the 33 cases with residual nodules, 11 underwent complete resection of the lung lesions, and among these, progression was reported in five. Complete resection of the liver tumor was not achieved in two of these. Three underwent incomplete resection of lung nodules, eventually leading to progression. Twelve cases with incomplete or no liver tumor resection progressed regardless of the status of lung lesions. Contrarily, among patients who underwent complete resection of the liver tumor, half were cured without metastasectomy. CONCLUSIONS Metastasectomy for residual pulmonary nodules after induction chemotherapy is effective provided that the liver tumor could be completely resected. TYPE OF STUDY Prospective Cohort Study. LEVEL OF EVIDENCE Level II.

Estimated glomerular filtration rate after nephrectomy for Wilms tumor

The aim of this study was to assess long‐term residual kidney function after unilateral nephrectomy for non‐syndromic Wilms tumor (NSWT).

Mediastinal tumor resection via open or video-assisted surgery in 31 pediatric cases: Experiences at a single institution

BACKGROUND Video-assisted thoracic surgery (VATS) has been increasingly used for the diagnosis and treatment of thoracic diseases. Pediatric mediastinal tumors are often difficult to resect because of their proximity to important organs. For this reason, VATS is not established as a standard surgical approach for such tumors. We compared the efficacy and safety of mediastinal tumor resection in children using open thoracotomy (OT) and VATS. METHODS A total of 31 children underwent mediastinal tumor resection (21 by VATS and 10 by OT). These nonrandomized approaches were retrospectively compared for incidence of operative complications, morbidity, and mortality. RESULTS Tumor sizes and operation times were similar between groups. However, the VATS group required significantly fewer blood transfusions and shorter durations of thoracic drainage and hospital stays (p<0.05). Complications greater than grade 2 on the Clavien-Dindo classification occurred in four patients treated by VATS and OT. No delayed effects were noted in the survivors of either group. A patient with clear cell sarcoma of the kidney who underwent OT relapsed and died. CONCLUSION Although complication rates were similar between the two approaches, VATS was suggested as less invasive because of fewer blood transfusions required, and shorter thoracic drainage and hospital stay durations.