Intestinal Perforation in Children as an Important Differential Diagnosis of Vascular Ehlers-Danlos Syndrome.

BACKGROUND Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders with heterogeneous clinical features associated with varying genetic mutations. EDS type IV, also known as vascular EDS (vEDS), is the rarest type but has fatal complications, including rupture of major vasculature and intestinal and uterine perforation. Intestinal perforation can be spontaneous or a consequence of long-standing constipation, a common symptom among patients with EDS. CASE REPORT We present a case of a 6-year-old boy with the previous diagnosis of vEDS who presented with colonic perforation from a stercoral ulcer. He underwent diagnostic laparoscopy and loop colostomy, with an uneventful postoperative course. Unfortunately, he developed a second colonic perforation 14 months after the initial episode and underwent total abdominal colectomy with end ileostomy. CONCLUSIONS Intestinal perforation is a well-documented and devastating complication of vEDS. However, spontaneous intestinal perforation is extremely rare in a young child. Therefore, the diagnosis of vEDS should be included in the differential diagnosis if a child presents with intestinal perforation. There is no clear guideline available for surgical management of colonic perforation in patients with vEDS, but total abdominal colectomy appears to provide the best chance of preventing recurrent perforation.

Information flow during pediatric trauma care transitions: things falling through the cracks.

Pediatric trauma is one of the leading causes of morbidity and mortality in children in the USA. Every year, nearly 10 million children are evaluated in emergency departments (EDs) for traumatic injuries, resulting in 250,000 hospital admissions and 10,000 deaths. Pediatric trauma care in hospitals is distributed across time and space, and particularly complex with involvement of large and fluid care teams. Several clinical teams (including emergency medicine, surgery, anesthesiology, and pediatric critical care) converge to help support trauma care in the ED; this co-location in the ED can help to support communication, coordination and cooperation of team members. The most severe trauma cases often need surgery in the operating room (OR) and are admitted to the pediatric intensive care unit (PICU). These care transitions in pediatric trauma can result in loss of information or transfer of incorrect information, which can negatively affect the care a child will receive. In this study, we interviewed 18 clinicians about communication and coordination during pediatric trauma care transitions between the ED, OR and PICU. After the interview was completed, we surveyed them about patient safety during these transitions. Results of our study show that, despite the fact that the many services and units involved in pediatric trauma cooperate well together during trauma cases, important patient care information is often lost when transitioning patients between units. To safely manage the transition of this fragile and complex population, we need to find ways to better manage the information flow during these transitions by, for instance, providing technological support to ensure shared mental models.

National variation in opioid prescribing after pediatric umbilical hernia repair.

BACKGROUND: Pediatric umbilical hernia repair is a common procedure that requires minimal tissue disruption. We examined variation in opioid prescription fills after repair of uncomplicated umbilical hernias to characterize the types and doses of medication used and persistent postsurgical use. METHODS: Using the Truven Health Analytics MarketScan© Research Database for June 2012-September 2015, we identified pediatric patients undergoing umbilical hernia repair. We excluded patients with obstruction, gangrene, an earlier repair or a concurrent surgical procedure, and those without available pharmacy claim data. Analyses describe filled outpatient prescriptions by age, geographic region, drug type, quantity, and second prescriptions/refills. RESULTS: Of 4,407 procedures performed, 2,292 patients (52%) filled a prescription for postoperative opioids (age 0-1 years: 21.6%, age 2-3 years: 51.5%, age 4-5 years: 54.3%, 6 years or older: 57.9% [P < .0001]). In the northeast United States, 42% of patients filled narcotic prescriptions, compared with 59% of patients in the south (P < .0001). Hydrocodone/acetaminophen was most commonly prescribed (51%), followed by codeine/acetaminophen (30%). Durations were ≤3 days (50%), 4-10 days (46%), and >10 days (4%). A total of 6% of patients filled a second opioid prescription within 30 days. CONCLUSION: Although many patients do not require opioids for umbilical hernia repair, most pediatric patients fill opioid prescriptions, including for prolonged courses and refills. Guidelines for appropriate prescribing of opioids after common, simple procedures, such as umbilical hernia repair, could improve the quality of care for children and impact the US epidemic of opioid abuse.

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Medical school does not prepare trainees for the reality of the practice of medicine, where book knowledge takes second place to the more pragmatic skills of time management, conflict resolution, and damage control. Junior residents, overwhelmed by the demands of daily floor work, can easily lose sight of the reasons that they went into medicine to begin with. Taken out of the context of the hospital, though, the opportunity to care for a patient one-on-one can be a vital reminder of the gift that is to know how to heal the sick. Reflecting on the opportunity to care for an accident victim at the scene, rather than in the hospital, reinforces to one young physician the remarkable thing that it is to be a physician.