Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals.

Background/purpose: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades.

Methods: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015.

Epithelial and ganglionic distribution at the distal rectal end in anorectal malformations: could it play a role in anastomotic adaptation?

Purpose: In anorectal malformations (ARMs), the epithelium of the distal rectal end is not well described. We histomorphologically evaluated epithelial and ganglionic distribution in the distal rectal end of ARMs resected during anorectoplasty to assess similarities and differences with normal anal canal structure.

Methods: In this single-center retrospective study, specimens from 60 ARM patients (27 males, 33 females) treated between 2008 and 2019 were evaluated.

Histological features of complete tracheal rings in congenital tracheal stenosis.

Purpose: Congenital tracheal stenosis is a disease in which complete tracheal cartilage rings (CTCR) cause airway narrowing. Although tracheal cartilage malformation has been suggested as a cause of CTCR, no histological studies have been performed. Here, we report a comparison of the tissues from CTCR and normal tracheal cartilage.

AFP-L3 as a Prognostic Predictor of Recurrence in Hepatoblastoma: A Pilot Study.

The α-fetoprotein (AFP) level is a sensitive biomarker of active hepatoblastoma (HB). This study aimed to clarify whether the Lens culinaris agglutinin A-reactive fraction of AFP (AFP-L3) after complete resection is a prognostic predictor of HB recurrence. Fourteen HB patients who underwent complete resection of HB were divided into the recurrence group (RG, n=4) and the non-recurrence group (NRG, n=10).

Point mutagenesis in mouse reveals contrasting pathogenetic effects between MEN2B- and Hirschsprung disease-associated missense mutations of the RET gene.

Missense mutations of the RET gene have been identified in both multiple endocrine neoplasia (MEN) type 2A/B and Hirschsprung disease (HSCR: congenital absence of the enteric nervous system, ENS). Current consensus holds that MEN2A/B and HSCR are caused by activating and inactivating RET mutations, respectively. However, the biological significance of RET missense mutations in vivo has not been fully elucidated.

Two infant cases of intraperitoneal arterial hemorrhage due to a duplication cyst: a case report.

Background: Intraperitoneal arterial hemorrhage without trauma is extremely rare. We report two infant cases of intraperitoneal arterial hemorrhage due to intestinal duplication.

Case Presentation: In case 1, a 2-month-old girl experienced sudden intraperitoneal hemorrhage from the middle colic artery with no apparent trauma.

Switch to Extracorporeal Membrane Oxygenation During Cardiac and Tracheal Repair.

Simultaneous repair of congenital tracheal and cardiovascular lesions remains challenging in small patients. We describe two infants weighing less than 3 kg who underwent successful tracheoplasty with concomitant correction of complex heart anomalies. In both operations, cardiopulmonary bypass was switched to extracorporeal membrane oxygenation after cardiac repair to optimize hemostatic function with transfusion and maintain activated clotting time at 200 to 240 seconds.

Mechanism of pancreatic juice reflux in pancreaticobiliary maljunction: A fluid dynamics model experiment.

Background: Pancreatic juice reflux to the common bile duct and gallbladder is observed in the pancreaticobiliary maljunction (PBM), and various pathological conditions occur in the biliary tract. However, the mechanism of pancreatic juice reflux has not been discussed yet. This study aimed to investigate the mechanism of this phenomenon from the perspective of the fluid dynamics theory.

Insulin therapy for hyperglycemia in neonatal sepsis using a preterm mouse model.

Background: Stress-induced hyperglycemia is a frequent complication of neonatal sepsis. Hyperglycemia induces oxidative stress and immunosuppression. We investigated the glucose kinetics and effect of insulin administration during stress-induced hyperglycemia in a neonatal sepsis mouse model.

Risk factors for the recurrence of perineal canal.

Purpose: The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC).

Methods: Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated.

Mice conditionally expressing RET(C618F) mutation display C cell hyperplasia and hyperganglionosis of the enteric nervous system.

Medullary thyroid carcinoma (MTC) develops from hyperplasia of thyroid C cells and represents one of the major causes of thyroid cancer mortality. Mutations in the cysteine-rich domain (CRD) of the RET gene are the most prevalent genetic cause of MTC. The current consensus holds that such cysteine mutations cause ligand-independent dimerization and constitutive activation of RET.

Dilatation of the common channel in pediatric congenital biliary dilatation remaining after radical operation.

Background: In congenital biliary dilatation, the protein plug is likely impacted, especially in a dilated common channel. However, nobody has discussed whether this dilatation of common channel remains after radical operation. The aim of this study was to investigate the situation of the dilated common channel after radical operation.

Clinical features and risk factors of bile duct perforation associated with pediatric congenital biliary dilatation.

Purpose: This study aimed to investigate the clinical features and risk factors of bile duct perforation in pediatric congenital biliary dilatation (CBD) patients.

Methods: CBD patients, whose initial symptom was abdominal pain, were enrolled in this study and were divided into perforated and non-perforated groups. The clinical features of the perforated group were investigated.

Experimental validation of laryngotracheal growth and recurrent laryngeal nerve preservation after partial cricotracheal resection in a growing rabbit model.

Purpose: The aim of this study was to confirm laryngotracheal growth and recurrent laryngeal nerve (RLN) preservation after partial cricotracheal resection (PCTR) in a growing rabbit model by performing the procedure in pediatric animals.

Methods: Six female Japanese white rabbits, 12 weeks of age, underwent PCTR. The course of the RLN was evaluated during surgery (n = 3).

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia.

Background: Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM.

Methods: Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively.

3-Dimensional computed tomography imaging of the ring-sling complex with non-operative survival case in a 10-year-old female.

We report a case of a 10-year-old female patient who survived ring-sling complex without surgery. The patient had congenital wheezing from the neonatal period and was treated after a tentative diagnosis of infantile asthma. The patient suffered from allergy and was hospitalized several times due to severe wheezing, and when she was 22 months old, she was diagnosed with ring-sling complex.

A case of biliary atresia with pancreaticobiliary maljunction.

Background: The pathogenesis of biliary atresia (BA) is still unknown. There are several reports on the etiology of BA, including pancreaticobiliary maljunction (PBM). We experienced a case of Kasai type IIIa BA with PBM, in which we found elevation of pancreatic enzymes in the gallbladder.

Management of congenital tracheal stenosis in the neonatal period.

Purpose: Few reports have focused on the management of congenital tracheal stenosis (CTS) in the neonatal period. The aim of this study was to determine appropriate management strategies for CTS in the neonatal period.

Methods: The medical records of eight neonatal patients with CTS at a single institution between January 2007 and December 2016 were retrospectively reviewed.

Pediatric airway surgery.

Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation.

Surgical Management of Duplication of the Pituitary Gland-Plus Syndrome With Epignathus, Cleft Palate, Duplication of Mandible, and Lobulated Tongue.

A 1-day-old male infant was referred to our department for evaluation of multiple malformations in his oral cavity. He was diagnosed duplication of the pituitary gland-plus syndrome with epignathus, cleft palate, duplication of the mandible, and a lobulated tongue. A thumb-sized mass lesion was visible on the hard palate.