Current practice regarding the diagnosis and treatment of anorectal malformations in female patients: a multicenter questionnaire survey in Japan.

Purpose: This study aimed to investigate the current practices in the diagnosis and surgical management of anorectal malformations (ARMs) in female patients in Japan, specifically focusing on anovestibular fistula (AVF), rectovaginal fistula (RVF), and persistent cloaca (PC).

Methods: An anonymous online survey was conducted with 61 institutional members of the Japanese Study Group for Anorectal Anomalies.

Results: Sixty-one institutions (100%) completed the survey.

The clinical impact of macrophage polarity after Kasai portoenterostomy in biliary atresia.

Introduction: Biliary atresia (BA) is a cholestatic hepatopathy caused by fibrosing destruction of intrahepatic and extrahepatic bile ducts, and its etiology has not been clearly revealed. In BA, liver fibrosis progression is often observed even after Kasai portoenterostomy (KPE), and more than half of cases require liver transplantation in their lifetime in Japan. Macrophages play an important role in liver fibrosis progression and are classically divided into proinflammatory (M1) and fibrotic macrophages (M2), whose phenotypic transformation is called "macrophage polarity.

IL13 and periostin in active fibrogenic areas of the extrahepatic bile ducts in biliary atresia patients.

Background: The leading pathology of biliary atresia (BA) is inflammatory and fibrous obstruction of extrahepatic bile duct, but the pathogenesis remains unclear. IL13 is a cytokine associated with allergies and inflammatory fibrosis, and periostin induces fibrogenesis by stimulation with IL13. We analyzed the involvement of IL13 and periostin in inflammatory fibrosis in the extrahepatic bile duct of BA patients.

Maldevelopment of intrapulmonary bronchial cartilage in congenital diaphragmatic hernia.

Background: Pulmonary hypoplasia is an important cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). This study aimed to verify our hypothesis that the abnormal development of bronchial cartilage as well as alveolar immaturity, might play a central role in hypoplasia of the lung in human CDH.

Method: We retrospectively analyzed autopsied lungs from 10 CDH cases and compared with nine age-matched controls to assess the bronchial cartilage and alveolar maturity using morphological techniques.

The roles played by the MYCN, Trk, and ALK genes in neuroblastoma and neural development.

Neuroblastoma is one of the most frequent, yet distinctive and challenging childhood tumors. The uniqueness of this tumor depends on its biological markers, which classify neuroblastomas into favorable and unfavorable, with 5-year survival rates ranging from almost 100-30%. In this review, we focus on some biological factors that play major roles in neuroblastoma: MYCN, Trk, and ALK.

Thoracoscopic resection for mediastinal thymolipoma in a child.

Thymolipoma is a rare disease among benign tumors. We herein report the case of a child who underwent thoracoscopic resection of a large thymolipoma. A 3-year-old boy was diagnosed with an anterior mediastinal solid tumor.

Laparoscopic intraperitoneal onlay mesh for pediatric incisional hernia-a case report.

Background: The incidence of incisional hernia in pediatric patients is low in comparison with that reported in adults. In the pediatric population, primary closure has generally been favored. However, synthetic or biomedical mesh offers advantages in the repair of larger defects when primary closure is difficult.

Tumor-homing effect of human mesenchymal stem cells in a TH-MYCN mouse model of neuroblastoma.

Background: Human mesenchymal stem cells (hMSCs) are multipotent stem-like cells that are reported to have tumor-suppression effects and migration ability toward damaged tissues or tumors. The aim of this study was to analyze the tumor-homing ability of hMSCs and antitumor potency in a transgenic TH-MYCN mouse model of neuroblastoma (NB).

Methods: hMSCs (3×10) labeled with DiR, a lipophilic near-infrared dye, were intraperitoneally (i.

Surgical intervention and perioperative risk factors of retroperitoneal teratomas in children: a single institution experience.

Purpose: Retroperitoneal teratomas (RTs) are rare among germ cell tumors and predominantly occur in infants. RTs are often difficult to manage by perioperative management. In this study, we retrospectively reviewed our series of RTs.

Successful laparoscopic extirpation of a large omental lipoblastoma in a child.

Omental lipoblastoma is extremely rare among benign tumors. We herein report the case of a child who underwent laparoscopic extirpation of a large omental lipoblastoma. A 4-year-old girl was diagnosed with an intra-abdominal solid tumor.

Surgical intervention strategies for pediatric congenital cystic lesions of the lungs: A 20-year single-institution experience.

Background: The aim of this study was to assess surgical intervention strategies for congenital cystic lesions of the lungs (CCL), focusing on the safety of lung resection.

Materials And Methods: The clinical features of 27 children (CCAM, n=16; bronchial atresia, n=4; bronchogenic cyst, n=3; pulmonary sequestration, n=3; lobar emphysema, n=1) who were treated at our institution between 1995 and 2014 were analyzed.

Results: Of the 27 patients, 14 were asymptomatic, and 13 were symptomatic.

Double and synchronous trichobezoars causing small-bowel obstruction and detected by multidetector computed tomography: report of two cases.

A trichobezoar is a rare mass formed by the ingestion and accumulation of hair within the gastrointestinal tract, especially the stomach. Cases of an isolated gastric trichobezoar with extension into the duodenum or the jejunum have been reported; however, synchronous gastric and intestinal trichobezoars causing a small-bowel obstruction is very unusual. We report our experience of two such cases to demonstrate the efficiency of preoperative multidetector computed tomography in locating the double bezoars and assisting us in surgical decision making.

Clinical analysis of liver fibrosis in choledochal cyst.

Purpose: Although most patients with choledochal cyst (CC) have a favorable prognosis with prompt treatment, some of them are complicated with liver fibrosis, resulting in prolonged liver dysfunction even after definitive surgery. The aim of this study was to distinguish the high-risk group of liver fibrosis in patients with CC.

Methods: Fifteen patients who underwent liver biopsy during surgery for CC from 1981 to 2012 were enrolled in this study.

Impact of our new protocol on the outcome of the neonates with congenital diaphragmatic hernia.

Background/purpose: Congenital diaphragmatic hernia (CDH) remains a defiant challenge for pediatric surgeons. Since 2003, we developed a new protocol aiming for the better outcome. In this study, the usefulness of our new protocol was evaluated.

Infrared spectrophotometry of intraluminal meconium calculi in a neonate with imperforate anus and rectourethral fistula.

Background/purpose: Intraluminal meconium calculi are a rare cause of neonatal abdominal calcifications in patients with anorectal malformations. To investigate their pathogenesis, we performed infrared spectroscopic analysis of meconium-calcified lesions.

Methods: Meconium calculi were collected from the colostomy in a newborn patient with imperforate anus and rectourethral fistula.

Developmental study of tethered spinal cord in murine embryos with anorectal malformations.

Background/purpose: Tethered spinal cord is frequently associated with anorectal malformations (ARMs). However, it remains unknown how the tethered spinal cord develops and relates to the severity of ARM. We studied the development of the spinal cord in ARM mouse embryos induced by all-trans retinoic acid (ATRA).

Iodine 123 metaiodobenzylguanidine radio-guided navigation surgery for recurrent medullary thyroid carcinoma in a girl with multiple endocrine neoplasia type 2B.

Multiple endocrine neoplasia type 2B (MEN 2B) is an inherited cancerous syndrome characterized by medullary thyroid carcinoma (MTC), adrenal pheochromocytoma, marfanoid habitus, and enteric ganglioneuromatosis. In this syndrome, a high frequency of persistent elevation of the serum calcitonin level, a sensitive marker for MTC, after total thyroidectomy has been reported, and the prognosis of such patients depends upon complete resection of recurrent MTC by repeated surgery. The authors performed iodine 123 metaiodobenzylguanidine ((123)I-MIBG) radio-guided navigation surgery for recurrent MTC in a 14-year-old girl with MEN 2B.

Neurocutaneous melanosis associated with Hirschsprung's disease in a male neonate.

Hirschsprung's disease is an inherited disorder characterized by the absence of ganglion cells in the distal bowel. Neurocutaneous melanosis is a rare congenital syndrome characterized by proliferation of melanin-producing cells in the skin and leptomeninges. The authors described a newborn patient with neurocutaneous melanosis associated with Hirschsprung's disease.

Impaired expression of myogenic regulatory molecules in the pelvic floor muscles of murine embryos with anorectal malformations.

Background/purpose: Recent biological studies have elucidated the molecular mechanism of muscle development, in which various regulatory factors (myogenic regulatory factors [MRFs]) play key roles during embryogenesis. To investigate the development of anorectal malformations (ARMs), we studied MRF expressions in myogenic cells in the pelvic floor using murine embryos affected with ARM.

Methods: Anorectal malformation embryos were obtained from the 10.