Minimally invasive repair of asymmetric pectus excavatum: An alternative technique to treating asymmetric morphology.

Minimally invasive repair of pectus excavatum or the Nuss procedure has become the standard operation for pectus excavatum repair. Pectus excavatum can be broadly divided into two categories: symmetric or asymmetric morphology. To optimize surgical outcomes of asymmetric pectus excavatum repair, previous work has proposed morphology-tailored bar shaping technique; the bar to be inserted is shaped asymmetrically to counter-balance the outer contour of the chest prior to the passage of the introducer across the chest.

Factors Impacting Long-Term Gastrostomy Tube Dependence in Infants with Congenital Heart Disease.

Background: Infants with congenital heart disease (CHD) often experience oral feeding intolerance requiring gastrostomy (GT). Complications related to GT use are common. The study aim was to identify factors associated with continued GT use at one-year.

A Novel Thoracic Ultrasound Measurement After Congenital Diaphragmatic Hernia Repair Identifies Decreased Diaphragmatic Excursion Associated With Adverse Respiratory and Surgical Outcomes.

Congenital diaphragmatic hernia (CDH) is a rare defect often associated with pulmonary hypoplasia and abnormal pulmonary vascular development. Even after successful hernia repair, pulmonary disease may persist into adulthood. Impaired diaphragmatic motility may lead to compromised respiratory function long after index repair.

NH-terminal deletion of specific phosphorylation sites on PHOX2B disrupts the formation of enteric neurons in vivo.

Mutations in the paired-like homeobox 2 b () gene are associated with congenital central hypoventilation syndrome (CCHS), which is a rare condition in which both autonomic dysregulation with hypoventilation and an enteric neuropathy may occur. The majority of patients with CCHS have a polyalanine repeat mutation (PARM) in PHOX2B, but a minority of patients have nonpolyalanine repeat mutations (NPARMs), some of which have been localized to exon 1. A nonsense mutation previously generated in a human pluripotent stem cell (hPSC) line results in an NH-terminus truncated product missing the first 17 or 20 amino acids, possibly due to translational reinitiation at an alternate ATG start site.

Laparoscopic Treatment of Slipping Rib Syndrome in Pediatric Patients.

Slipping rib syndrome (SRS) is an often unrecognized cause of lower chest and upper abdominal pain in children and adolescents. Surgical resection of the cartilaginous portions of the slipping rib often provides permanent pain relief, with the standard surgical approach being an open resection. A minimally invasive approach has not been reported previously; we report a novel laparoscopic technique for the treatment of SRS with satisfactory results.

A Giant Childhood Mesenteric Lipoblastoma With Extensive Maturation.

Abdominal lipoblastomas are uncommon soft tissue tumors in children and rarely arise from the mesentery. Due to intraabdominal location and slow growth, these masses can go unnoticed for long periods of time and often found on surgical exploration. We present a case of a 12-year-old male with years of abdominal distension accompanied by new onset early satiety that was found to have an intra-abdominal mass.

Postoperative Opioid Analgesia Impacts Resource Utilization in Infants Undergoing Pyloromyotomy.

Background: Opioid analgesia is often avoided in infants undergoing pyloromyotomy. Previous studies highlight an association between opioid use and prolonged hospitalization after pyloromyotomy. However, the impact of opioid use on healthcare resource utilization and cost is unknown.

R-Spondin 1 (RSPO1) Increases Mouse Intestinal Organoid Unit Size and Survival and Improves Tissue-Engineered Small Intestine Formation .

Cell therapy and tissue engineering has recently emerged as a new option for short bowel syndrome (SBS) treatment, generating tissue engineered small intestine (TESI) from organoid units (OU) and biodegradable scaffolds. The recombinant human R-Spondin 1 (rhRSPO1) protein may be a key player in this process due to its mitogenic activity in intestinal stem cells. Aiming at optimizing the TESI formation process and advancing this technology closer to the clinic, we evaluated the effects of rhRSPO1 protein on OU culture and TESI formation.

Induced pluripotent stem cell-derived enteric neural crest cells repopulate human aganglionic tissue-engineered intestine to form key components of the enteric nervous system.

Models for enteric neuropathies, in which intestinal nerves are absent or injured, are required to evaluate possible cell therapies. However, existing options, including transgenic mice, are variable and fragile. Here immunocompromised mice were implanted with human pluripotent stem cell-derived tissue-engineered small intestine 10 weeks prior to a second survival surgery in which enteric nervous system precursor cells, or saline controls, were injected into the human intestinal organoid-derived tissue-engineered small intestine and analyzed 4 weeks later.

Spleen Organoid Units Generate Functional Human and Mouse Tissue-Engineered Spleen in a Murine Model.

Splenectomy is common after trauma or hematologic disease, and alters immune protection against pathogens, which may lead to fulminant infection with high mortality. Yet the spleen has demonstrable regenerative capacity and cells might be recovered and reimplanted at the time of injury or excision to avoid these risks. Tissue-engineered spleen (TESp) was generated from mice (mTESp) or human donor spleen (hTESp) through implantation of spleen organoid units (spleen OU), in NOD/SCID mice with concurrent splenectomy, on a biodegradable scaffold.

Managing Procedural Pain in the Neonate Using an Opioid-sparing Approach.

Purpose: Pain in the neonate is often challenging to assess but important to control. Physicians often must balance the need for optimal pain control with the need to minimize oversedation and prolonged opioid use. Both inadequate pain control and overuse of opioids can have long-term consequences, including poor developmental outcomes.

The Association Between Opioid Use and Outcomes in Infants Undergoing Pyloromyotomy.

Purpose: The purpose of this study was to describe the frequency and variation of opioid use across hospitals in infants undergoing pyloromyotomy and to determine the impact of opioid use on postoperative outcomes.

Methods: A retrospective cohort study (2005-2015) was conducted by using the Pediatric Health Information System (PHIS) database, including infants (aged <6 months) with pyloric stenosis who underwent pyloromyotomy. Infants with significant comorbidities were excluded.

Emergent Repair of Paraesophageal Hernias and the Argument for Elective Repair.

Background And Objectives: A feared complication of large paraesophageal hernias is incarceration necessitating emergent repair. According to previous studies, patients who require an emergent operation are subject to increased morbidity compared with patients undergoing elective operations. In this study, we detail patients who underwent hernia repair emergently and compare their outcomes with elective patients.

A 10-Month-Old Infant Presenting With Signs of Precocious Puberty Secondary to a Sclerosing Stromal Tumor of the Ovary in the Absence of Hormonal Elevation.

Precocious puberty in an infant is an alarming and infrequent finding, making the differential diagnosis difficult for practitioners. Precocious puberty secondary to a sclerosing stromal tumor (SST) of the ovary is rare. We present a case of a child that began precocious puberty at 3 months of age including development of breast buds, pubic hair, growth spurt, and menarche 5 days prior to presenting to pediatric endocrinology at 10 months.

Simultaneous laparoscopic paraesophageal hernia repair and sleeve gastrectomy in the morbidly obese.

Background: Morbid obesity is associated with increased rates of hiatal and paraesophageal hernias. Although laparoscopic sleeve gastrectomy is gaining popularity as the procedure of choice for morbid obesity, there is little data regarding the management of paraesophageal hernias found intraoperatively. The aim of this study was to evaluate the feasibility and benefits of a combined sleeve gastrectomy and paraesophageal hernia repair in morbidly obese patients.

Periodontal infection, systemic inflammation, and insulin resistance: results from the continuous National Health and Nutrition Examination Survey (NHANES) 1999-2004.

Objective: Adverse microbial exposures might contribute to diabetogenesis. We hypothesized that clinical periodontal disease (a manifestation of microbial exposures in dysbiotic biofilms) would be related to insulin resistance among diabetes-free participants. The roles of inflammatory mediation and effect modification were also studied.