Establishment of a rescue program for anorectal malformations induced by retinoic acid in mice.

Aims Of Study: Retinoid-mediated signal transduction plays a crucial role in the embryogenesis of various organs. We previously reported the successful induction of anorectal malformations in mice using retinoic acid (RA). Retinoic acid controls the expression of essential target genes for cell differentiation, morphogenesis, and apoptosis through a complicated interaction in which RA receptors form heterodimers with retinoid X receptors.

Diagnostic impact of computed tomography cholangiography and magnetic resonance cholangiopancreatography on pancreaticobiliary maljunction.

Background/purpose: The aim of this study was to investigate the diagnostic potential of computed tomography cholangiography (CTC) and magnetic resonance cholangiopancreatography (MRCP) in children with pancreaticobiliary maljunction (PBM).

Methods: Fifty-three children with PBM were consecutively treated between 1997 and 2009. Among them, the patients who underwent CTC and/or MRCP preoperatively were enrolled in this study.

Laparoscopic versus open abdominoperineal rectoplasty for infants with high-type anorectal malformation.

Background/purpose: There has not been any study comparing laparoscopic abdominoperineal rectoplasty (ARP) with open ARP. This study investigated the true benefits of the laparoscopic approach in infants with high anorectal malformation.

Patients And Methods: A retrospective analysis was performed in 28 infants with high anorectal malformation treated between 1990 and 2007.

Usefulness of magnetic resonance imaging for congenital prepubic fistula.

Congenital prepubic fistula is a rare congenital anomaly. Complete removal of the fistular tract remains challenging because of the complicated course. Although conventional fistulography has been used widely as a diagnostic tool for congenital prepubic fistula, more detailed information such as accurate localization of the fistular end or relative position to the urinary tract cannot be preoperatively obtained because the conventional contrast studies have insufficient capability.

Predicting factors on the occurrence of cystic dilatation of intrahepatic biliary system in biliary atresia.

Purpose: Cystic dilatation of intrahepatic biliary system (CDIB) is an intractable complication of biliary atresia (BA). In this study, we investigated the predicting factors of CDIB development after jaundice resolved following hepatoportoenterostomy (HPE).

Methods: From 1988 to 2008, 28 (80.

Management strategies for infants with total intestinal aganglionosis.

Purpose: This study investigated appropriate management strategies for infants with total intestinal aganglionosis (TIA), focusing on surgical and medical managements.

Methods: Six infants with TIA or near TIA treated in our institution between 1980 and 2007 were reviewed retrospectively. Surgery was performed as a simple jejunostomy, 65 to 70 cm below the ligament of Treitz (LOT) in 2 infants, and 30 cm below LOT in 1 without extended myectomy-myotomy (EMM).

Significance of ductal plate malformation in the postoperative clinical course of biliary atresia.

Purpose: Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance.

Methods: Between 1988 and 2005, 31 patients with uncorrectable BA underwent hepatoportoenterostomy and steroid therapy.

The inv mouse as an experimental model of biliary atresia.

Background/purpose: The causation of biliary atresia (BA) remains unclear. However, ductal plate malformation (DPM), maldevelopment of the intrahepatic bile ducts, is 1 of the preferred theories. The inv homozygous mouse (inv mouse), created by insertional mutagenesis, shows situs inversus and jaundice.

Clinical significance of the confluence of the cystic duct in patients with anomalous arrangement of the pancreaticobiliary duct.

Background/purpose: In anomalous arrangement of the pancreaticobiliary duct (AAPBD), there is anatomical diversity of the cystic duct. In this study, we evaluated the influence of the level of insertion of the cystic duct into the extrahepatic bile duct on the pathophysiology of AAPBD.

Methods: Thirty-two children with AAPBD were examined using cholangiopancreatography.

Social quality of life for adult patients with anorectal malformations.

Background/purpose: Pediatric surgeons who performed the initial surgery on anorectal malformations (ARM) lose contact with the patients as they become adults. In the present study, we examined 20- to 40-year-old adult patients with a history of surgery for ARM and analyzed them from the points of social quality of life.

Patients And Methods: Twenty-nine patients with ARM, aged 20 to 40, were surveyed by questionnaire or personal interview.

Spontaneous rupture of choledochal cyst with pseudocyst formation-report on 2 cases and literature review.

Spontaneous rupture and subsequent bile peritonitis are rare complications of choledochal cysts. Of these complications, the formation of a biliary pseudocyst is an unusual form, and its preoperative diagnosis is difficult. In this report, we describe 2 cases showing spontaneous rupture with biliary pseudocyst formation.

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).

Estrogen receptor expression in anomalous arrangement of the pancreaticobiliary duct.

Background/purpose: Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is a malformation of the bile and pancreatic ducts which is commonly associated with congenital biliary dilatation and predominantly occurs in girls. Estrogen receptor (ER) is reported to modulate cholangiocyte proliferation and play a role in tumorigenesis of estrogen-dependent malignancies. The present study investigated the presence of ER in the gallbladder of patients with AAPBD to elucidate whether ER expression is correlated with sexual specificity and biliary histology.

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.

Intralobar pulmonary sequestration diagnosed by MR angiography.

A definitive diagnosis of pulmonary sequestration necessitates evidence of a systemic arterial supply to the sequestered lung segment by aortography. We report a case of intralobar pulmonary sequestration in an 11-month-old girl in whom the diagnosis was made by contrast-enhanced magnetic resonance (MR) angiography. Contrast-enhanced MR angiography is the most reliable noninvasive method for the definitive diagnosis of pulmonary sequestration in children.

Mucoepidermal carcinoma of the lung detected by positron emission tomography in a 5-year-old girl.

The authors describe a rare case of mucoepidermal carcinoma of the lung incidentally identified in preoperative assessments for inguinal hernia repair in a 5-year-old girl. This patient was referred for right external inguinal hernia, and a 3.0-cm round-shaped lesion was found in the right lower lung field of a chest x-ray film.

Surgical treatment for anomalous arrangement of the pancreaticobiliary duct with nondilatation of the common bile duct.

Background/purpose: For anomalous arrangement of the pancreaticobiliary duct (AAPBD) with nondilatation of the common bile duct (CBD), the optimal surgical procedure remains controversial. The authors investigated which procedure would be most effective for AAPBD with nondilatation of the CBD.

Methods: The authors encountered 60 children with AAPBD in our institution between 1979 and 2002.

Postoperative clinical evaluation following pylorus-preserving gastrectomy.

Background/aims: Recent pathological study demonstrated that extended lymphadenectomy is not always necessary for patients with early gastric cancer.

Methodology: Twenty-eight patients underwent pylorus-preserving gastrectomy. The clinicopathological findings of patients with pylorus-preserving gastrectomy were compared to those of 58 patients with conventional distal gastrectomy.

Prognostic value of lymph node staging in gastric cancer.

Background/aims: The latest TNM classification (5th edition) changed the definition of nodal staging from the anatomical localization to the total number of metastatic lymph nodes. This study was designed to evaluate and compare the prognostic significance of nodal staging between the two widely known staging systems, the TNM classification (TNM) and Japanese Classification for Gastric Cancer (JCGC).

Methodology: A total of 582 patients who underwent curative gastrectomy with extended lymphadenectomy for gastric cancer were reviewed retrospectively from hospital records.