Catecholamine-Induced Cardiomyopathy Secondary to Pheochromocytoma Rescued With Percutaneous Left Ventricular Assist Device: Novel Application of the Impella CP in a Pediatric Patient.

Catecholamine-induced cardiomyopathy is an uncommon complication of pheochromocytoma. Pheochromocytoma is a rare tumor that predominantly occurs in adults, making catecholamine-induced cardiomyopathy secondary to pheochromocytoma in children an exceedingly rare presentation. Treatment typically consists of medical management followed by surgical resection.

Pediatric Undifferentiated Pleomorphic Sarcoma of the Cecum.

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm typically diagnosed in older adults and localized to the extremities or retroperitoneum. Because of poor response to therapy and high rates of recurrence, this neoplasm is associated with a poor prognosis. A 12-year-old female presented with weight loss, abdominal pain, fatigue, and diarrhea.

Drain options after vertebral body tethering.

Purpose: Since the introduction of vertebral body tethering (VBT) for adolescent idiopathic scoliosis (AIS), a variety of post-operative chest drainage systems have been utilized. Most surgeons use formal chest tubes with a Pleur-evac, while others use smaller bulb suction drains (e.g.

A pediatric surgeon's dilemma: does cholecystectomy improve symptoms of biliary dyskinesia?

Background: Biliary dyskinesia (BD) is a well-established gallbladder pathology in adult patients and rates of cholecystectomy for BD continue to rise in the United States. Many pediatric patients with vague abdominal pain of variable duration are evaluated for biliary dyskinesia. It remains unknown which cohort of pediatric patients diagnosed with BD are most likely to have sustained improvement in symptoms following laparoscopic cholecystectomy.

Surgical Safety Checklists in Children's Surgery: Surgeons' Attitudes and Review of the Literature.

Introduction: Surgical safety checklists (SSCs) aim to create a safe operating room environment for surgical patients. Provider attitudes toward checklists affect their ability to prevent harm. Pediatric surgeons' perceptions surrounding SSCs, and their role in improving patient safety, are unknown.

Torsion of a giant mesocolic lipoma in a child with Bannayan-Riley-Ruvalcaba syndrome.

A 6-year-old boy with Bannayan-Riley-Ruvalcaba syndrome (BRRS) presented to the emergency department with periumbilical abdominal pain for 12 h. A contrast-enhanced abdominal and pelvis CT examination revealed significant interval change in the size and appearance of a previously seen hypoattenuating right mesocolic fatty mass suggestive for lipoma, first observed 5 months prior. This lesion demonstrated new enhancing internal septations, a thickened capsule, interval development of adjacent mesenteric fat stranding and engorgement of the mesenteric vessels.

Animal models for prenatal gene therapy: rodent models for prenatal gene therapy.

Fetal gene transfer has been studied in various animal models, including rabbits, guinea pigs, cats, dogs, and nonhuman primate; however, the most common model is the rodent, particularly the mouse. There are numerous advantages to mouse models, including a short gestation time of around 20 days, large litter size usually of more than six pups, ease of colony maintenance due to the small physical size, and the relatively low expense of doing so. Moreover, the mouse genome is well defined, there are many transgenic models particularly of human monogenetic disorders, and mouse-specific biological reagents are readily available.

Use of manipulated stem cells for prenatal therapy.

Prenatal stem cell therapy has broad potential for therapeutic application. "Stem cells" of interest include multipotent adult-derived stem cells, cord blood, amniotic fluid, or fetal stem cells, and embryonic or induced pluripotent stem cells. Potential manipulations of stem cells prior to their administration may include harvest, processing, enrichment, expansion, and genetic transduction.

Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas.

Background: Large, prenatally diagnosed sacrococcygeal teratomas (SCTs) present a formidable challenge because of their unpredictable growth and propensity for complications. In our experience, even with aggressive serial imaging, many fetuses have died under a policy of "watchful waiting." We propose "early delivery" as the best option for selected cases of high-risk fetal SCT.

A tissue engineering approach for prenatal closure of myelomeningocele: comparison of gelatin sponge and microsphere scaffolds and bioactive protein coatings.

Myelomeningocele (MMC) is a common and devastating malformation. As an alternative to fetal surgical repair, tissue engineering has the potential to provide a less invasive approach for tissue coverage applicable at an earlier stage of gestation. We have previously evaluated the use of gelatin hydrogel composites composed of gelatin sponges and sheets as a platform for tissue coverage of the MMC defect in the retinoic acid induced fetal rat model of MMC.

Predicting the severity of congenital high airway obstruction syndrome.

Background: Congenital high airway obstruction syndrome (CHAOS) is caused by complete or near-complete obstruction of the fetal airway. Obstruction sets in motion a sequence of events that can ultimately lead to fetal demise. However, on rare occasions in utero airway decompression occurs, reversing syndromic findings and improving the prognosis.

Prenatal stem cell transplantation and gene therapy.

At the present time, the most likely and eminent application of stem cell therapy to the fetus is in utero hematopoietic stem cell transplantation (IUHCT), and this stem cell type will be discussed as a paradigm for all prenatal stem cell therapy. The authors feel that the most likely initial application of IUHCT will use adult HSC derived from bone marrow (BM) or peripheral blood (PB), and will focus this article on this specific approach. The article also reviews the experimental data that support the capacity of IUHCT to induce donor-specific tolerance.

Stem cell and genetic therapies for the fetus.

Advances in prenatal diagnosis have led to the prenatal management of a variety of congenital diseases. Although prenatal stem cell and gene therapy await clinical application, they offer tremendous potential for the treatment of many genetic disorders. Normal developmental events in the fetus offer unique biologic advantages for the engraftment of hematopoietic stem cells and efficient gene transfer that are not present after birth.