Selective ligation of portal vein and hepatic artery for ruptured hepatocellular carcinoma in a 13-year-old boy.

Hepatocellular carcinoma (HCC) in children is rare, and the prognosis has been poor because of its advanced stage at diagnosis and unresponsiveness to chemotherapy. We report a 13-year-old boy with ruptured HCC in the left trisegment. When hemostasis of the ruptured surface was achieved in the emergency operation, the left branch of the portal vein and the left hepatic artery were ligated at the same time.

Evaluating mortality and disease severity in congenital diaphragmatic hernia using the McGoon and pulmonary artery indices.

Purpose: Lung hypoplasia is associated with mortality in congenital diaphragmatic hernia (CDH). However, the association between lung hypoplasia and disease severity is unclear. Early prediction of disease severity would provide parents with more precise information about the anticipated course of treatment, minimize treatment disruption, and maximize the efficient management of patients with CDH.

Repeat STEP procedure to establish enteral nutrition in an infant with short bowel syndrome.

The serial transverse enteroplasty (STEP) procedure is a safe and successful way to lengthen the small bowel in patients with short bowel syndrome. However, postoperative dilatation of the intestine may occur, which induces bacterial overgrowth and malabsorption leading to liver failure. We describe the case of an infant boy with short bowel syndrome caused by jejunal atresia requiring the STEP procedure twice.

A novel association of duodenal atresia, malrotation, segmental dilatation of the colon, and anorectal malformation.

We describe two female neonates who suffered from four gastrointestinal anomalies, including duodenal stenosis or atresia, malrotation, segmental dilatation of the colon, and anorectal malformation. Each patient was managed by two or three operations, resulting in good bowel movements. Since this is the first report of four gastrointestinal anomalies, these cases may provide clues to elucidate the etiology of gastrointestinal tract developmental abnormalities.

Prognostic significance of circulating tumor cells and bone marrow micrometastasis in advanced neuroblastoma.

Purpose: The aim of this study was to study the prognostic significance of circulating tumor cells (CTC) and the appropriate indications for aggressive surgery in advanced neuroblastoma.

Materials And Methods: Micrometastasis was sequentially explored using our reverse transcriptase-polymerase chain reaction method in 29 neuroblastoma patients (International Neuroblastoma Staging System stage 4, n = 24; stage 3, n = 5) who treated at our department with the united chemotherapeutic regimen since 1991. Their medical records and detection of CTC and/or the bone marrow micrometastasis were retrospectively reviewed then analyzed statistically.

An association of gastroschisis and fatal respiratory distress: does prenatal bile aspiration cause early-onset respiratory failure in neonates?

We present a neonate with gastroschisis and evidence of bile aspiration in utero, who developed severe respiratory distress that did not respond to postnatal intensive respiratory care. Although rare, a newborn with gastroschisis may develop severe respiratory distress due to bile aspiration in utero. Given the poor outcome in this case, we suggest a possible role for prenatal diagnosis and therapy.

High dose intravenous methylprednisolone resolves esophageal stricture resistant to balloon dilatation with intralesional injection of dexamethasone.

One of the most serious problems in patients with long-gap esophageal atresia or corrosive esophagitis is esophageal stricture, which may require esophageal resection and replacement. We describe two cases with persistent esophageal stricture successfully managed by high dose intravenous methylprednisolone following balloon dilatation. High-dose methylprednisolone with gradual tapering (daily 25, 15, 10, 5, 2 mg/kg for 4 days each) plus cimetidine and ampicillin for 1 week was intravenously administrated immediately after balloon dilatation of the esophageal stenosis.

The impact of strict infection control on survival rate of prenatally diagnosed isolated congenital diaphragmatic hernia.

Background/purpose: Although the prognosis of congenital diaphragmatic hernia (CDH) is determined by the degree of pulmonary hypoplasia, there may be an occult contribution of infection to outcomes. The purpose of this study is to evaluate the effects of our new supportive therapy to prevent infectious complications on mortality and morbidity of CDH.

Methods: Among 57 cases with CDH treated between 2002 and 2007, 43 prenatally diagnosed isolated cases were enrolled in this study.

Living donor liver transplantation with hyperreduced left lateral segments.

Liver transplantation is now an established technique to treat children with end-stage liver disease. Implantation of left lateral segment grafts (Couidaud's segments II and III) can be a problem in small infants because of a large-for-size graft. Reduced left lateral segmental liver transplantation has been recently introduced for small infants to mitigate the problem of large-for-size graft.

Aberrant venous flow measurement may predict the clinical behavior of a fetal extralobar pulmonary sequestration.

Objective: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by a massive pleural effusion, leading to tension hydrothorax and fetal hydrops. The goal of this study was to examine sonographic signs of venous obstruction in fetal EPS with or without pleural effusion.

Methods: Records of fetal ultrasound from 6 patients with EPS were reviewed with special attention to aberrant arterial and venous flow.

Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation.

This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor alpha and interleukin-1beta were within reference range.

Late complications after surgery in patients with neuroblastoma.

Purpose: The purpose of this study was 2-fold: to study the complications of neuroblastoma treatment and to establish surgical guidelines.

Materials And Methods: The medical records of 92 patients with neuroblastoma (stage 1 or 2, n = 33; stage 3, n = 21; stage 4, n = 31; stage 4S, n = 7) who had undergone surgery in our department between 1985 and 2001 were reviewed, with an emphasis on treatments and late complications.

Results: The disease-free survival rate was 98.

Clinicopathologic assessment of prenatally diagnosed lung diseases.

Purpose: To investigate the clinical features and pathologic diagnosis of prenatally diagnosed lung diseases.

Materials And Methods: The medical records of 28 fetuses with prenatally diagnosed lung diseases were reviewed with regard to perinatal courses, ultrasonographic measurement of the lesion volume ratio, and the histopathology of lung tissue.

Results: Of the 23 fetuses with a prenatal diagnosis of congenital cystic adenomatoid malformation (CCAM), 4 required an emergency lobectomy (1 prenatally and 3 postnatally), whereas the other 14 showed uneventful courses after birth.

Current role of liver transplantation for methylmalonic acidemia: a review of the literature.

To evaluate the current role of liver transplantation (LT) for methylmalonic acidemia (MMA), we reviewed the literature on outcomes of this treatment, and describe three of our own cases of living-donor liver transplantation (LDLT). The total number of LT cases identified was 18. Transplantation mode was deceased donor LT in 12, including five combined liver-kidney transplantations (CLKT) from deceased donors, and LDLT in six.

Venous arterialization in extralobar pulmonary sequestration associated with fetal hydrops.

Background/purpose: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by massive pleural effusion (PE) leading to fetal hydrops. The underlying mechanisms as well as the origin of the fluid are not well understood. This study was performed to find a histological hallmark of an EPS with massive PE.

Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy.

Background/purpose: The aims of this study were to analyze the outcomes of fetuses with congenital diaphragmatic hernia (CDH) treated by a lung-protective strategy using high-frequency oscillatory ventilation (HFOV) in a single center with a perinatology service and extracorporeal membrane oxygenation (ECMO) capability and to define the natural history of CDH in the era of lung-protective ventilation.

Methods: A retrospective chart review of 30 neonates with CDH seen between April 2002 and October 2004 was conducted. All fetuses with a prenatal diagnosis were evaluated by fetal magnetic resonance imaging to define the liver position, and those with a significant volume of the liver in the chest were regarded as liver-up.

Congenital bronchial atresia in infants and children.

Background/purpose: Congenital bronchial atresia (CBA) usually presents incidentally in asymptomatic young male adults but is rarely diagnosed in children. The aim of this study was to clarify the clinical characteristics of CBA in childhood and to describe the spectrum of this condition.

Methods: The clinical features in 29 patients with CBA, aged from 1 day to 13 years (median, 4 years), were reviewed retrospectively.

Tumor cell dynamics and metastasis in advanced neuroblastoma.

This study deals with the advancement process of neuroblastoma through clinical observations and circulating tumor cell exploration. Clinical feature, tumor biology, and circulating tumor cell detected by the previously described polymerase chain reaction (PCR) method were analyzed in 31 patients with advanced neuroblastoma treated in our department since 1991 through 2004. Treatment was completed in 28 patients, of whom 17 are alive without the disease and 11 died.

Lethal gastric rupture caused by acute gastric ulcer in a 6-year-old girl.

Gastric rupture is extremely rare in childhood beyond the neonatal period. We describe a previously healthy 6-year-old girl with a large laceration along the greater curvature on the posterior wall of the stomach. The patient was admitted to a neighboring hospital because of vertigo with 1-day history of intractable vomiting and epigastric pain.

Clonality analysis of childhood neuroblastoma by polymerase chain reaction for the human androgen receptor gene.

Neuroblastoma is a unique tumor that occurs during childhood. Tumors in infants less than one year of age often regress spontaneously and usually have an excellent prognosis, whereas those in older patients are aggressive, leading to a fatal outcome. To shed light on these unique aspects of this tumor, we investigated the clonality of neuroblastomas by analyzing the inactivation patterns through methylation of the human androgen receptor gene on the X chromosomes in female patients.

Life-threatening airway obstruction caused by mediastinal germinoma in a 9-year-old girl.

The authors report a case of a 9-year-old girl with a mediastinal germinoma that required emergent surgical extirpation because of tracheal compression and asphyxia. The tumor was successfully debulked under general anesthesia with the capability of extracorporeal circulation immediately available. Postoperatively, the tumor responded to adjuvant chemotherapy and irradiation and the patient survived without evidence of recurrence.

Prenatal diagnosis and management of abdominal diseases in pediatric surgery.

Purpose: The aim of this study was to investigate the prenatal courses and management of abdominal surgical diseases.

Methods: Of the 327 patients registered with our fetal treatment board since March 2002, 83 fetuses referred to the surgical department were enrolled for the current study. The prenatal diagnosis, sequential fetal images, and perinatal courses of these cases were reviewed retrospectively.

Renal angiomyolipoma in Japanese tuberous sclerosis patients.

Purpose: Renal angiomyolipoma (AML) is a benign neoplasm that may grow massive in tuberous sclerosis (TS) patients. The aim of this study was to document the characteristics of renal AML in Japanese TS patients.

Methods: Medical records of 29 TS patients followed up at the authors' center were reviewed for the presence, size, symptom, and treatment of renal AML.