Informed Consent in Decision-Making in Pediatric Practice.

Informed consent should be seen as an essential part of health care practice; parental permission and childhood assent is an active process that engages patients, both adults and children, in their health care. Pediatric practice is unique in that developmental maturation allows, over time, for increasing inclusion of the child's and adolescent's opinion in medical decision-making in clinical practice and research. This technical report, which accompanies the policy statement "Informed Consent in Decision-Making in Pediatric Practice" was written to provide a broader background on the nature of informed consent, surrogate decision-making in pediatric practice, information on child and adolescent decision-making, and special issues in adolescent informed consent, assent, and refusal. It is anticipated that this information will help provide support for the recommendations included in the policy statement.

Guidelines for the Development of Comprehensive Care Centers for Congenital Adrenal Hyperplasia: Guidance from the CARES Foundation Initiative.

Patients with rare and complex diseases such as congenital adrenal hyperplasia (CAH) often receive fragmented and inadequate care unless efforts are coordinated among providers. Translating the concepts of the medical home and comprehensive health care for individuals with CAH offers many benefits for the affected individuals and their families. This manuscript represents the recommendations of a 1.5 day meeting held in September 2009 to discuss the ideal goals for comprehensive care centers for newborns, infants, children, adolescents, and adults with CAH. Participants included pediatric endocrinologists, internal medicine and reproductive endocrinologists, pediatric urologists, pediatric surgeons, psychologists, and pediatric endocrine nurse educators. One unique aspect of this meeting was the active participation of individuals personally affected by CAH as patients or parents of patients. Representatives of Health Research and Services Administration (HRSA), New York-Mid-Atlantic Consortium for Genetics and Newborn Screening Services (NYMAC), and National Newborn Screening and Genetics Resource Center (NNSGRC) also participated. Thus, this document should serve as a "roadmap" for the development phases of comprehensive care centers (CCC) for individuals and families affected by CAH.

Multimedia article: endoscopically guided thoracoscopic esophagectomy for stricture in a child.

PURPOSE: Caustic ingestion is a common cause for acquired esophageal strictures in children. Thoracoscopic esophagectomy can be very useful in this setting, particularly for short segments of disease [1-4]. Thus far, the use of endoscopy to guide resection margins has not been described. METHODS: A 6-year-old boy developed a tight, short esophageal stricture from a lye ingestion injury at the age of 4 years. He had a gastrostomy tube placed at the time for supplemental feedings and subsequently failed attempts at antegrade and retrograde esophageal dilatation. This video demonstrates an endoscopically guided (endoscopes simultaneously passed retrograde via the gastrostomy and antegrade through the oropharynx) thoracoscopic esophagectomy and primary anastomosis management of the stricture. METHODS: In the left lateral decubitus position, four 5-mm ports were placed in the right chest in the fifth intercostal space, anterior-axillary line; sixth intercostal space, midaxillary line; fourth intercostal space, midaxillary line; and seventh intercostal space, posterior axillary line. A 5-mm 30 degrees Storz telescope and 4.9-mm and 9.6-mm Olympus endoscopes were used. The area of esophageal stricture was identified using the endoscopes and thoracoscopically dissected circumferentially. The vagus nerves were identified and circumferentially freed from the strictured esophageal segment. Primary resection and anastomosis was performed using intracorporeal sutures and then tested for leak using the endoscope via the gastrostomy site. RESULTS: Esophagram on postoperative day (POD) 7 revealed no leak and a widely patent anastomosis. He was discharged home on a soft diet on POD 8 and continues to feed orally over 1 year following his operation. CONCLUSION: As demonstrated by our video, endoscopy is a useful adjunct in the performance of a thoracoscopic esophagectomy for short esophageal stricture. It is particularly helpful because it provides direct visualization of the compromised lumen and allows for a more precise resection.