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

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

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

Abstract 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

Cost analysis of pediatric robot-assisted and laparoscopic pyeloplasty

Purpose: An increasing percentage of pediatric pyeloplasties are being performed with assistance of the da Vinci® Surgical System. A review of the recent literature shows decreased operative times and length of hospital stays when robotic procedures are performed, although there are few published data comparing the cost of pediatric robotic and pure laparoscopic pyeloplasty. We reviewed a representative sample of pyeloplasties performed at our institution and performed a cost analysis. Materials and Methods: We retrospectively identified 23 robot-assisted and 23 laparoscopic pyeloplasties performed at our institution between August 2008 and April 2012. Total cost was calculated from direct and indirect costs provided by our billing department. Results: Robotic procedures were shorter than pure laparoscopic procedures (200 vs 265 minutes, p \u3c0.001) but there was no significant difference in the total cost of the 2 procedures ($15,337 vs$16,067, p \u3c0.46). When compared to laparoscopic cases, subgroup analysis demonstrated decreased operative times (140 vs 265 minutes, p \u3c0.00001) and total cost ($11,949 vs$16,067, p \u3c0.0001) in robotic cases where stents were placed in an antegrade fashion. Conclusions: With widespread use the cost of robotic instrumentation may decrease, and experience may further shorten operative times. However, it currently remains to be seen whether robotic technology will become a cost-effective replacement for pure laparoscopy in the management of pediatric ureteropelvic junction obstruction. © 2013 American Urological Association Education and Research, Inc

Video. Diagnostic laparoscopy and preoperative planning in ischiopagus tripus conjoined twins: a surgical first, with detailed demonstration of the complex anatomical relationships.

Diagnosis and management of conjoined twins are constantly evolving. New imaging techniques provide important anatomic details and help in planning the separation procedure. Despite these technological advances, however, the complex arrangement of conjoined organs is somewhat difficult to interpret, leaving unresolved questions at time of surgery. The authors present a video demonstrating laparoscopy as an adjunct in the preoperative planning of separation of ischiopagus tripus conjoined twins as well as illustrating the complex anatomy in a combination of video, drawings, and radiologic imaging. To date, this is the first time that laparoscopy has been used in preoperative assessment of conjoined twins. After elective cesarean delivery, ischiopagus tripus conjoined twins were thoroughly evaluated with conventional imaging, including plain radiographs and computed tomography scan with three-dimensional (3-D) reconstruction images. The anatomy of the gastrointestinal and genitourinary tracts was further defined with barium enema, retrograde pyelography, and cystoscopy. In addition to these tests, diagnostic laparoscopy was performed at time of tissue expander placement. An angled scope, introduced through a 5-mm umbilical port, was used to visualize the intraperitoneal organs as well as all accessible retroperitoneal structures. Laparoscopy provided useful information regarding the bowel distribution between the twins. In addition, it helped demonstrate the relationship of shared solid organs with other intra-abdominal structures and identify anatomic landmarks used in the subsequent separation of the twins. Finally, laparoscopy helped confirm the presence, number, and morphology of the internal female genitalia. Diagnostic laparoscopy is a useful tool in evaluation of ischiopagus tripus conjoined twins. It is an important adjunct to preoperative studies in preparing for an expeditious and safe separation procedure

Optimal Length of Follow-up for the Detection of Unsuccessful Pediatric Pyeloplasty: A Single-Center Experience

ObjectivesTo assess the optimal length of follow-up for patients undergoing both open and minimally invasive pyeloplasties to ensure prompt detection of a recurrent obstruction. There are no standard guidelines on ideal follow-up and imaging post-pediatric pyeloplasty currently.MethodsA retrospective chart review identified 264 patients (<18 years old) who underwent pyeloplasty for ureteropelvic junction obstruction between April 2002 and December 2014. Ultrasound was obtained every 3–4 months for the first year following pyeloplasty and thereafter at discretion of treating physician. Patient characteristics including symptoms and imaging were reviewed.ResultsOf the 264 patients, 72% were male with mean age of 51 months and follow-up of 26.8 months. Approximately 73% followed up to 3 years. Fourteen patients (5.3%) had a recurrent obstruction. Among the failures, 85% were diagnosed and underwent successful redo pyeloplasty within 3 years. Six infants had a recurrence (43% of all unsuccessful surgeries) and were diagnosed within 3 years of the initial surgery. Patients undergoing a minimally invasive procedure were less likely to be followed for more than 3 years compared to an open procedure (p < 0.001). Patients with severe hydronephrosis preoperatively were followed longer (p = 0.031). Age at surgery and type of surgical approach (p < 0.01) were significant predictors of length of follow-up in a negative binomial regression.ConclusionBased on the results, a minimum of 3 years of follow-up is necessary to detect the majority of recurrent obstructions. Those patients who have higher than average lengths of follow-up tend to be younger and/or underwent an open surgical approach