Clinical characteristics and conservative treatment of perineal groove.

Purpose: Perineal groove is a rare congenital anomaly of the perineum, and only a few papers describing a small number of cases have been reported in the medical literature. This study aimed to evaluate the clinical characteristics and proper management of perineal groove.

Methods: We performed a retrospective review of 26 pediatric patients who were diagnosed with perineal groove between January 2012 and October 2018 at our institution.

Laparoscopic repair of inguinal hernia in infants: Comparison with open hernia repair.

Purpose: This study aimed to evaluate the usefulness of laparoscopic repair of inguinal hernia (LR) in infants in comparison with open hernia repair (OR).

Methods: We retrospectively analyzed the clinical data of 465 infants treated for inguinal hernia from January 2006 to December 2015. Among them, 124 underwent LR and 341 underwent OR.

Trans-ethnic meta-analysis of genome-wide association studies for Hirschsprung disease.

Hirschsprung disease (HSCR) is the most common cause of neonatal intestinal obstruction. It is characterized by the absence of ganglia in the nerve plexuses of the lower gastrointestinal tract. So far, three common disease-susceptibility variants at the RET, SEMA3 and NRG1 loci have been detected through genome-wide association studies (GWAS) in Europeans and Asians to understand its genetic etiologies.

A genome-wide association analysis of chromosomal aberrations and Hirschsprung disease.

Hirschsprung disease (HSCR) is a neurocristopathy characterized by the absence of intramural ganglion cells along variable lengths of the gastrointestinal tract. Although the RET proto-oncogene is considered to be the main risk factor for HSCR, only about 30% of the HSCR cases can be explained by variations in previously known genes including RET. Recently, copy number variation (CNV) and loss of heterozygosity (LOH) have emerged as new ways to understand human genomic variation.

Association Analysis of SLC6A20 Polymorphisms With Hirschsprung Disease.

Purpose: Hirschsprung disease (HSCR) is a congenital and heterogeneous disorder, which is caused by no neuronal ganglion cells in part or all of distal gastrointestinal tract. Recently, our genome-wide association study has identified solute carrier family 6, proline IMINO transporter, member 20 (SLC6A20) as one of the potential risk factors for HSCR development. This study performed a replication study for the association of SLC6A20 polymorphisms with HSCR and an extended analysis to investigate further associations for subgroups and haplotypes.

Ultrasonographic assessment of optic nerve sheath diameter during pediatric laparoscopy.

This study investigated the extent of the raised intracranial pressure resulting from carbon dioxide (CO2) pneumoperitoneum by ultrasonographically measuring optic nerve sheath diameter (ONSD) in children undergoing laparoscopic surgery. Twenty-five children aged less than 9 y (53.1 ± 23.

Relationship between Anomalous Pancreaticobiliary Ductal Union and Pathologic Inflammation of Bile Duct in Choledochal Cyst.

Purpose: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years.

A genome-wide association study identifies potential susceptibility loci for Hirschsprung disease.

Hirschsprung disease (HSCR) is a congenital and heterogeneous disorder characterized by the absence of intramural nervous plexuses along variable lengths of the hindgut. Although RET is a well-established risk factor, a recent genome-wide association study (GWAS) of HSCR has identified NRG1 as an additional susceptibility locus. To discover additional risk loci, we performed a GWAS of 123 sporadic HSCR patients and 432 unaffected controls using a large-scale platform with coverage of over 1 million polymorphic markers.

The randomized crossover comparison of airway sealing with the laryngeal mask airway Supreme(™) at three different intracuff pressures in children.

Background: An intracuff pressure of 80 cm H2 O in the adult-sized laryngeal mask airway Supreme has been recommended to obtain a higher oropharyngeal leak pressure (OLP). However, the intracuff pressure for the higher OLP in the pediatric laryngeal mask airway Supreme could be different from that in the adult-sized laryngeal mask airway Supreme. Thus, we measured and compared OLP at three intracuff pressures of 40, 60, and 80 cm H2 O in the pediatric laryngeal mask airway Supreme.

Clinical characteristics and management of benign transient non-organic ileus of neonates: a single-center experience.

Purpose: The term benign transient non-organic ileus of neonates (BTNIN) is applied to neonates who present symptoms and plain radiographic findings of Hirschsprung's disease, but do not have aganglionic bowel and are managed well by conservative treatment. It can often be difficult to diagnose BTNIN because its initial symptoms are similar to those of Hirschsprung's disease. The aim of this study is to evaluate the clinical characteristics and proper treatment of BTNIN.

Calretinin and microtubule-associated protein-2 (MAP-2) immunohistochemistry in the diagnosis of Hirschsprung's disease.

Background/purpose: Identifying ganglion cells by rectal suction biopsy is a basic diagnostic tool for the diagnosis of Hirschsprung's disease (HD). However, the difficult interpretation of conventionally processed slides often necessitates ancillary staining methods. The aim of this study was to evaluate the usefulness of calretinin and microtubule-associated protein-2 (MAP-2) immunohistochemistry in the diagnosis of HD.

Minimally invasive surgery in infants with congenital diaphragmatic hernia: outcome and selection criteria.

Purpose: The aim of the study was to determine clinical indications for performing minimally invasive surgery (MIS) with acceptable results by reviewing our experience in congenital diaphragmatic hernia (CDH) repair and comparing outcomes of MIS with open surgery.

Methods: Medical records of patients who underwent CDH repair were reviewed retrospectively between January 2008 and December 2012, and outcomes were compared between MIS and open repair of CDH.

Results: From 2008 to 2012, 35 patients were operated on for CDH.

Comparison of 2 cuff inflation methods before insertion of laryngeal mask airway for safe use without cuff manometer in children.

Purpose: This prospective, randomized trial was conducted to establish whether the pediatric laryngeal mask airway (LMA) could be used without any concerns for abnormally high intra-cuff pressure when a cuff of the LMA was inflated with half the maximum recommended inflation volume or the resting volume before insertion.

Basic Procedures: Eighty children 0 to 9 years of age and weighing of 5 to 30 kg scheduled for general anesthesia were included. Before insertion, the cuff of the LMA was filled with half the maximum recommended inflation volume in the Half volume group, or the resting volume by opening the pilot balloon valve to atmospheric pressure in the Resting volume group.

Long-term outcome of left- vs right-sided antegrade continence enema.

Background/purpose: This study compared long-term outcomes of left- vs right-sided antegrade continence enema (ACE) procedures.

Methods: We retrospectively analyzed medical records and performed a follow-up telephone survey of patients who underwent the ACE procedure at Severance Children's Hospital between 1999 and 2011.

Results: A total of 49 patients underwent 51 ACE procedures.

A randomized controlled trial comparing Laryngeal Mask Airway removal during adequate anesthesia and after awakening in children aged 2 to 6 years.

Study Objective: To compare the frequency of airway complications during removal of the Laryngeal Mask Airway (LMA) in 2 to 6 year old pediatric patients.

Design: Prospective randomized study.

Setting: Operating room at a university hospital.

Clinical characteristics and treatment of esophageal atresia: a single institutional experience.

Purpose: Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. Subsequent to increased survival, postoperative morbidity has become an important issue in this disease. The aim of our study was to analyze our experience regarding the treatment of esophageal atresia.

Lessons and tips from the experience of pediatric robotic choledochal cyst resection.

Purpose: The laparoscopic surgery for a choledochal cyst in children is technically challenging because of its high degree of complexity despite its possibility. In an attempt to overcome this laparoscopic weakness and to facilitate the difficult steps in the minimally invasive surgery for choledochal cyst resection in children, we have performed the robot-assisted resection of the choledochal cyst. The aim of this study is to report lessons and tips obtained from our experience of the robot-assisted choledochal cyst resection in children.

Second serial transverse enteroplasty procedure in an infant with extreme short bowel syndrome.

The serial transverse enteroplasty (STEP) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome. The advantages of STEP include not only technical ease and simplicity, but also the ability to repeat the procedure. Herein, we report a case of extreme short bowel syndrome that was successfully treated by the second STEP procedure.

Anesthetic management for neonate with giant cystic hygroma involved upper airway -A case report-.

Significant differences exist between neonatal and adult airways. Anesthetic management of the airway may be challenging in neonate and young infant with large neck mass because these patients are at risk for sudden complete airway occlusion resulting in hypoventilation and hypoxemia. We experienced a 30-day-old baby presented with large cystic hygroma on the left side of neck.