Takuo Noda

Biliary complications after excisional procedure for choledochal cyst

During the last 25 years, from 1969 to 1994, the authors treated 97 choledochal cysts by surgical excision. Biliary reconstruction consisted of 67 hepaticoduodenostomies and 30 hepaticojejunostomies. The common hepatic duct was the site of anastomosis in 9 of the duodenostomies and 13 of the jejunostomies and of the bifurcation of the hepatic ducts in 58 duodenostomies and 17 jejunostomies. Reoperation was required in 10 cases because of recurrent cholangitis with intrahepatic gallstones. Biliary strictures were responsible for the cholangitis in 9 children with anastomoses at the level of the common hepatic duct and in 1 with an anastomosis at the level of the bifurcation. These results suggest that biliary complications develop because of anastomotic stricture or primary ductal stricture, and may be minimised by the creation of a wide anastomosis, which is best accomplished at the hepatic hilum.

Liver volume in children measured by computed tomography

Abstract Liver volume was measured by computed tomography in 54 children and young adults with no history of liver disease. Their ages ranged from 10 days to 22 years. The volume was calculated as follows: (1) the edges of the liver were traced on each scan image and the area was calculated by computer; (2) the areas were summed and multiplied by the scan interval in centimeters. The mean liver volume (± SD) was 178.2 ± 81.9 cm3 in infants (less than 12 months old) and 1114.3 ± 192.9 cm3 in adolescents (more than 16 years old). The mean liver volume in relation to body weight (± SD) was 34.1 ± 5.5 cm3/kg in infants and 20.2 ± 3.1 cm3/kg in adolescents. In general, liver volume increases rapidly in infants, gradually in schoolchildren, and not at all in adolescents. Volumetry might be clinically useful for evaluating the liver function in children and determining the graft size in liver transplantation.

Standard splenic volume in children and young adults measured from CT images.

The number of children and adolescents with hypersplenism is increasing as the number of long-term survivors undergoing successful Kasai operation for biliary atresia increases. The aim of this study was to determine the standard splenic volume in normal children and adolescents. We measured the splenic volumes with computed tomography (CT) images obtained from 49 Japanese children, adolescents, and young adults, ranging from 9 days to 25 years of age, and from 3.0 kg to 89.0 kg of body weight. The best correlation was observed between splenic volume (SV: cm3) and body weight (BW: kg) with a power curve regression; SV=6.516 BW0.797,r=0.924. The splenic volume and age (AG: years) also correlated well on a power curve; SV=41.879 AG0.411,r=0.897. The ratio of the splenic volume to the body weight (SV/BW: cm3/kg) decreased with age; from 4.5 cm3/kg at 1 month to 2.4 cm3/kg at 25 years of age, according to an exponential curve; SV/BW=4.473e−0.026AG,r=0.593. The above formulas are thus considered to be clinically useful, especially in the assessment of splenic size in children with hypersplenism both before and after partial splenic embolization.

Peritoneal Lavage Versus Drainage for Perforated Appendicitis in Children

A total of 231 children with acute appendicitis were treated at our hospitals during the 10 years between 1984 and 1993, 53 of whom had a perforated appendix. These 53 patients were randomly assigned to two groups at the time of surgery according to the different procedures performed. Thus, 29 children were managed by appendectomy followed by peritoneal lavage using a large amount of saline, and intravenous antibiotic therapy consisting of aminoglycoside and cephem (lavage group), while the other 24 children were treated by appendectomy with silicon tube drainage and the same systemic antibiotic therapy (drainage group). The mean length of hospitalization, and the mean durations of fever and the need for fasting after laparotomy in the lavage group were significantly less than those in the drainage group: 10.1 versus 18.8 days, 2.8 versus 7.7 days, and 1.8 versus 3.5 days, respectively. The operation wounds healed well in the lavage group due to the fact that there was no drain. Wound infections occurred in two children from the lavage group and six from the drainage group. Intra-abdominal abscesses occurred in two children from the drainage group. Accordingly, peritoneal lavage appears to be superior to intraperitoneal tube drainage for the management of perforated appendicitis in children.

Changes in splenic volume after partial splenic embolization in children

AIM The authors studied changes in splenic volume after partial splenic embolization (PSE) in children who had thrombocytopenia caused by splenomegaly. MATERIALS AND METHODS Five children with hypersplenism (ages of 1, 6, 7, 10, and 14 years) underwent PSE. The follow-up period ranged from 1 month to 8 years 9 months. Whole and infarcted splenic volumes were measured from computed tomography images obtained before and after PSE. RESULTS Within 2 weeks after PSE, the whole splenic volume increased to 110% to 140% of the pre-PSE volume. The infarcted area disappeared 3 to 4 months after PSE, and the whole splenic volume decreased markedly. The decreased volume tended to be stable during the follow-up period. The splenic enlargement before PSE ranged from 7.2 to 14.2 times the standard splenic volume for a corresponding body weight. The splenic volume did not become normal after PSE; it remained between 2 and 7 times larger than standard. Platelet counts stayed above 10 x 10(4) /mm3 when the splenic volume reduced to 2 times the standard volume. CONCLUSION The first 4 weeks after PSE is the most dangerous period for the development of splenic capsule. The infarcted parenchyma is absorbed 3 to 4 months after PSE, and the whole splenic volume is reduced. Thereafter, the splenic volume is stable for several years. Reembolization is not likely to be necessary in the majority of patients who have had splenic embolization with an infarction rate of > or = 80%.

Alveolar rhabdomyosarcoma of the lung in a child

The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the lung is presented. Brain metastasis and recurrence of the right pulmonary hilum and parietal pleura developed 6, 11, and 24 months (respectively) after tumor resection. Chemotherapy and radiotherapy were effective. Neuron-specific enolase was very helpful in detecting metastasis and disease recurrence. Primary pulmonary rhabdomyosarcoma can be divided into two groups: tumor in the normal lung, and tumor in cystic lesions of the lung.

Exploring the length of the common channel of pancreaticobiliary maljunction on magnetic resonance cholangiopancreatography

In the revised diagnostic criteria for pancreaticobiliary maljunction (PBM), PBM can be diagnosed from a long common channel by magnetic resonance cholangiopancreatography (MRCP). However, it is necessary to differentiate from high confluence of pancreaticobiliary ducts (HCPBD) with a relatively long common channel (≥6 mm) and effect of the sphincter in the pancreaticobiliary junction. This multicenter study aimed to explore definite value of the length of an abnormally long common channel, which enables to distinguish PBM from HCPBD on MRCP.

Modified Kasai's procedure for a choledochal cyst with a very narrow hilar duct

Excision has been widely recognized as the treatment of choice for choledochal cysts. For biliary reconstruction after cyst excision, we have strongly recommended hepaticoenterostomy at the hepatic hilum with a wide anastomotic stoma to prevent postoperative cholangitis. However, we recently treated two infants in whom a wide anastomotic stoma could not be made due to a narrow hilar duct. Therefore, a hepatic portojejunostomy with Roux-en-Y anastomosis (Kasais procedure) was performed after cyst excision in both case to permit free drainage of bile. The mucosa of the hilar duct was everted and fixed to the liver parenchyma to prevent stricture formation. Both babies have done well since the surgery.

Abstract 2448: Suppression of MYCN-driven neuroblastoma malignant phenotype by telomerase-targeted tumor suppressor p53 transactivation

Background: Neuroblastoma (NB) is a primary malignant tumor in the peripheral sympathetic nervous system. High-risk group of NB has an unfavorable clinical course, even after treatment with current chemotherapy regimens and aggressive surgical resection. Therefore, a novel therapeutic strategy is needed for the treatment of high-risk group of NB. Recent accumulating evidences in whole-genome sequencing analysis have shown that human telomerase reverse transcriptase (hTERT) gene rearrangement and MYCN gene amplification are predictive biomarkers for the high-risk group of NB, in which massive transcriptional activation of hTERT mRNA was frequently observed. We recently developed two types of hTERT-driven oncolytic adenoviruses, OBP-301 and OBP-702, in which the hTERT promoter drives the expression of the viral E1A and E1B genes for tumor-specific virus replication, and OBP-702 further expresses tumor suppressor p53 protein. In this study, we investigated whether OBP-301 and OBP-702 inhibit cell viability and MYCN protein expression in human NB cells with different MYCN status. Methods: We used 3 human NB cell lines with or without MYCN amplification, including IMR-32 (MYCN-amplified), LA-N-5 (MYCN-amplified), SK-N-SH (MYCN-not amplified). The antitumor effects of OBP-301, OBP-702, and Ad-p53, a replication-defective adenovirus expressing p53 gene, were evaluated using XTT assay. Virus-induced apoptosis and MYCN suppression were analyzed by Western blot analysis. Results: OBP-301, OBP-702, and Ad-p53 suppressed the viability of all 3 NB cell lines, whereas OBP-702 showed the most profound anti-tumor effect especially in MYCN-amplified NB cells. OBP-702 induced higher p53 expression than Ad-p53, resulting in the p53-mediated apoptotic cell death. Although MYCN expression in NB cells with MYCN amplification was downregulated after infection with all types of adenoviruses, the inhibitory effect of OBP-702 was the strongest among these adenoviruses. Conclusions: These results suggest that a hTERT-driven oncolytic adenovirus OBP-702 is a promising antitumor agent to induce profound apoptotic cell death through p53 transactivation and MYCN suppression in human NB cells with or without MYCN amplification. In vivo experiments are under way to investigate the antitumor effect of OBP-702 against xenograft NB tumors. Citation Format: Terutaka Tanimoto, Hiroshi Tazawa, Hiroshi Noso, Takanori Oyama, Yasuo Urata, Shunsuke Kagawa, Takuo Noda, Toshiyoshi Fujiwara. Suppression of MYCN-driven neuroblastoma malignant phenotype by telomerase-targeted tumor suppressor p53 transactivation. [abstract]. In: Proceedings of the 107th Annual Meeting of the American Association for Cancer Research; 2016 Apr 16-20; New Orleans, LA. Philadelphia (PA): AACR; Cancer Res 2016;76(14 Suppl):Abstract nr 2448.

Suspected early onset of congenital Langerhans cell histiocytosis involving ectopic cervical thymus and mediastinal thymus, simultaneously

To the Editor: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder characterized by accumulation of Langerhans cells [1,2]. Congenital LCH chiefly affects the skin and is selfhealing (Hashimoto–Pritzker disease), but rare involvements of the lung, bone, or liver have been reported [3]. Thymus is one of the uncommon organs involved by LCH [4]. Ectopic thymus is an extremely rare etiology of a neck mass in an infant which results from abnormal embryogenesis of the thymus [5,6]. We present a case of congenital LCH with simultaneous involvement of ectopic cervical thymus and mediastinal thymus. A 2-month-old female without any significant medical history through the prenatal and neonatal period was brought to a physician complaining of left submandibular swelling. On physical examination, there was an elastic, soft, and poorly movable mass in the left submandibular area. The mass was 3 cm in diameter and had a smooth surface. Computed tomography (CT) revealed cystic and solid components in the mass (Fig. 1A). An open biopsy of the mass was performed. The mass was well demarcated from surrounding tissues and was resected without macroscopic leaving residues. The pathological examination showed proliferation of CD1a positive cells with a round nuclear and rich cytoplasm in an eosinophil rich background (Fig. 1B–D). Diagnosis of LCH was made. Moreover, the resected mass contained ectopic thymic tissue whose architecture was partly effaced by infiltration of Langerhans cells (Fig. 1B and E). Reconfirmation of the CT image revealed the enlarged mediastinal thymus with multiple cysts and punctate calcifications which are specific findings of the thymic involvement of LCH (Fig. 1A) [7]. Although biopsy of the mediastinal thymic LCH