Meeting in the middle: pediatric abdominal wall reconstruction for omphalocele.

Background: Omphalocele is a congenital abdominal wall defect with an incidence of 1/4,200 births. Repair timing varies from the neonatal period to the first few years of life. Surgical technique has changed over the last two decades.

Screening for Child Abuse in Children With Isolated Skull Fractures.

Objective: Head trauma is the most common cause of death from child abuse, and each encounter for recurrent abuse is associated with greater morbidity. Isolated skull fractures (ISF) are often treated conservatively in the emergency department (ED). We determined patterns of physical abuse screening in a children's hospital ED for children with ISF.

Perioperative outcomes and the effects of anesthesia in congenital central hypoventilation patients.

Purpose: Patients with congenital central hypoventilation syndrome (CCHS) have autonomic dysfunction and lack ventilatory responses to hypoxemia and hypercarbia and thus are prone to adverse events during general anesthesia. The objective of this study was to describe the perioperative outcomes of patients with CCHS who were undergoing diaphragm pacer (DP) implantation surgeries under general anesthesia.

Methods: A retrospective cohort study was conducted on patients with CCHS who underwent DP implantation surgeries at CHLA between January 2000 and May 2016.

An unusual cause of diaphragm pacer failure in congenital central hypoventilation syndrome.

Unlabelled: Congenital central hypoventilation syndrome is a rare genetic disorder affecting ventilatory response to hypercapnia and/or hypoxemia. We describe a case of diaphragm pacing (DP) failure in a 38-year-old woman with congenital central hypoventilation syndrome who used DP as ventilatory support only during sleep for 24 years. Diagnostic evaluation began with examination of external DP equipment, but adjustment did not elicit adequate diaphragm contractions.

Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients.

Background: Congenital central hypoventilation syndrome (CCHS) is a rare disorder affecting central control of breathing. Thus, patients require lifelong assisted ventilation. Diaphragm pacing (DP) may permit decannulation in those who are ventilator dependent only during sleep.

Thoracoscopic placement of phrenic nerve pacers for diaphragm pacing in congenital central hypoventilation syndrome.

Purpose: Congenital central hypoventilation syndrome (CCHS), or Ondine's curse, is a rare disorder affecting central respiratory drive. Patients with this disorder fail to ventilate adequately and require lifelong ventilatory support. Diaphragm pacing is a form of ventilatory support which can improve mobility and/or remove the tracheostomy from CCHS patients.

Laparoscopic versus open treatment of congenital duodenal obstruction: multicenter short-term outcomes analysis.

Background: Laparoscopic repair of congenital duodenal obstruction has become popularized over the past decade. Comparative data on outcomes, however, are sparse. We hypothesized that laparoscopic repair of congenital duodenal obstruction could be performed with similar outcomes to traditional open repair.

Treatment of congenital pulmonary airway malformation induced hydrops fetalis via percutaneous sclerotherapy.

Large type II and III congenital pulmonary airway malformations (CPAMs) can cause pulmonary hypoplasia, non-immune hydrops fetalis and fetal demise. Fetal intervention is indicated if hydrops fetalis develops. In this report, we describe three cases of type II and III CPAMs complicated by hydrops and treated with percutaneous sclerotherapy by ethanolamine injection into the tumor.

State of the practice for pediatric surgery--career satisfaction and concerns. A report from the American Pediatric Surgical Association Task Force on Family Issues.

Background: There has been increasing interest and concern raised in the surgical literature regarding changes in the culture of surgical training and practice, and the impact these changes may have on surgeon stress and the appeal of a career in surgery. We surveyed pediatric surgeons and their partners to collect information on career satisfaction and work-family balance.

Methods: The American Pediatric Surgical Association Task Force on Family Issues developed separate survey instruments for both pediatric surgeons and their partners that requested demographic data and information regarding the impact of surgical training and practice on the surgeon's opportunity to be involved with his/her family.

Inversion herniotomy: a laparoscopic technique for female inguinal hernia repair.

Purpose: Laparoscopic inversion herniotomy (LIH) is a method of inguinal hernia repair for female patients. In this article, we report our technique and outcomes for this procedure. We propose that LIH is a valid alternative to conventional open herniorraphy.

L1CAM mutation in association with X-linked hydrocephalus and Hirschsprung's disease.

X-linked hydrocephalus (XLH) is characterized by increased intracranial ventricle size and head circumference secondary to aqueduct of Sylvius congenital stenosis. Exceedingly rare is the concurrence of XLH and Hirschsprung's disease (HSCR) with a theoretical incidence of 1 in 125-250 million cases. Herein, we are describing a case of a patient with concurrent XLH and HSCR.

Combined laparoscopic and thoracoscopic esophagectomy and gastric pull-up in a child.

Severe caustic burns to the esophagus may necessitate esophagectomy with replacement, if stictures form which are not amenable to dilation. Traditionally, the operation is performed via open transabdominal and/or thoracic approaches. Here we describe our minimally invasive approach, combining laparoscopy and thoracoscopy for esophagectomy, stomach tubularization and a cervical esophagogastrotomy.

Laparoscopic choledochal cyst excision: lessons learned in our experience.

Background: Choledochal cyst (CDC) is a rare biliary disorder. Surgical treatment consists of CDC excision and biliary-enteric reconstruction. Recently, some institutions have reported successful CDC excision by using minimally invasive techniques.

Thoracoscopic repair of neonatal diaphragmatic hernia.

Introduction: The use of minimally invasive surgery (MIS) in the neonatal population is increasing. Thoracoscopic intervention for congenital diaphragmatic hernia (CDH) is no exception. In this report, we describe our initial experience with thoracoscopic repair of left-sided diaphragmatic defects in neonates.

Contemporary management of lipoblastoma.

Purpose: Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms.

Methods: We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005.

Ethanol-lock technique for persistent bacteremia of long-term intravascular devices in pediatric patients.

Objectives: To use the ethanol-lock technique (in conjunction with systemic antibiotics) to salvage central lines from removal and to prevent persistence of catheter-related infections among pediatric patients with long-term intravascular devices.

Design: Medical records of patients treated with ethanol locks were retrospectively reviewed from June 1, 2004, through June 22, 2005.

Setting: Childrens Hospital Los Angeles, Los Angeles, Calif, a tertiary care pediatric hospital.