Fractures in children with neurofibromatosis type 1 from two NF clinics.

Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with osseous abnormalities occurring in up to one-third of patients. Several studies have documented osteopenia in both children and adults with NF1; however, the significance of lower bone mineral density (BMD) in relationship to fracture incidence is not well elucidated in NF1, particularly in children. We undertook a retrospective study to determine prevalence and location of fractures in children and adolescents with NF1, ages 5-20 years, using a standardized questionnaire. We surveyed 256 individuals with NF1 from two multidisciplinary NF centers and 178 controls without NF1 of similar ages and sex. Participants with known long bone dysplasia (LBD) were analyzed separately. Data collected included numbers and location of fractures, dietary calcium intake, and physical activity levels. There was no difference in prevalence of ever having a fracture between the NF1 group without LBD (22%) and the control group (25%); median number of fractures also did not differ. There were significant differences in fracture location with a higher frequency of fractures of the lower extremities in NF1 individuals without LBD compared to controls. Both NF1 cohorts had lower rates of physical activity than controls (P < 0.0001). Our data demonstrate that the likelihood of having had a fracture is not higher in young NF1 individuals without LBD in comparison to healthy controls. The lower physical activity level may have a "protective effect" for those with NF1, thus keeping their fracture incidence lower than expected for their relative degree of osteopenia.

Longitudinal analysis of hospital charges following injury in a level 1 pediatric trauma system.

BACKGROUND: The treatment of injured children contributes substantially to the financial burden of a health care system. The purpose of this study was to characterize these charges at a level-1 pediatric trauma center. METHODS: Financial data for children (<14 years) admitted for traumatic injury from 1/2009 to 12/2014 were analyzed. The charges of the index admission and first two years following discharge were evaluated. RESULTS: 5853 trauma patients were included with average annual charges of $11,128,730. The most common mechanisms of injury were fall (44%), sports (12%), and bike (9%). The median ISS was 6 (IQR 4-10) with a mortality rate of 1.8% and Z-score of 13.04 (p<0.001). The overall total charges per patient during the index admission were $9513. Spinal cord and major abdominal injuries had the greatest charges per patient ($55,560 and $23,618 respectively) primarily owing to hospital LOS. During the first year after discharge, the total charges per patient were $1733, of which spinal cord injury resulted in highest overall ($19,426), owing to inpatient rehabilitation. For all other injury patterns, mean total charges per patient were $2376 (range $791-$3573). CONCLUSIONS: The value proposition in health care requires us to define outcomes relative to costs. Injury severity, major injury location, and hospital length of stay are the highest contributors for the financial burden of pediatric traumatic injury, while inpatient readmissions and inpatient rehabilitation drove higher charges in the years following discharge. TYPE OF STUDY: Clinical Research Paper. LEVEL OF EVIDENCE: II - Cohort Study.

The utility of a "trauma 1 OP" activation at a level 1 pediatric trauma center.

PURPOSE: To expedite flow of injured children suspected to require operative intervention, a "trauma 1 OP" (T1OP) activation classification was created. The purpose of this study was to review this strategy at a level 1 pediatric trauma center. METHODS: A retrospective review of T1OP activations between 2003 and 2015 was performed. Children suspected of requiring neurosurgical intervention were classified as trauma 1 OP neuro (T1OP(N)). Comparisons were made to trauma 1 (T1) patients who required emergent operative intervention, excluding orthopedic injuries. RESULTS: Overall, 461 T1OP activations occurred (72% T1OP(N)) compared to 129 T1 activations requiring emergent surgery. Demographics were not significantly different between groups, although T1OP patients were slightly younger and more often experienced falls or were victims of abuse. Compared to T1 activations, T1OP activations had a significantly higher mortality rate (21% vs. 7%, p<0.001). Repeat head imaging was more common in the T1OP(N) group compared to imaged children in the T1 group (20% vs. 37%, p=0.05). T1OP(N) patients more often went directly to the OR (45% vs. 33%, p=0.02) and did so in a significantly faster period of time (32min vs. 53min, p<0.001). CONCLUSIONS: Use of the T1OP activations appropriately triaged surgical patients, resulting in significantly faster transport times to the OR. LEVEL OF EVIDENCE: II, prognosis study.

The use of angiography in pediatric blunt abdominal trauma patients.

BACKGROUND: Angiography is a common treatment used in adults with blunt abdominal trauma and/or severe pelvic fractures. The Committee on Trauma of the American College of Surgeons has recently advocated for this resource to be urgently available at pediatric trauma centers; however, its usefulness in the pediatric setting is unclear. The purpose of this study was to determine the incidence of angiography in the treatment of blunt abdominal trauma among injured children. METHODS: An analysis was performed using an established public use data set of children (younger than 18 years) treated at 20 participating trauma centers for blunt torso trauma through the Pediatric Emergency Care Applied Research Network. Patients who underwent angiography of the abdomen or pelvis were identified and analyzed. RESULTS: Of the 12,044 children evaluated for blunt abdominal trauma included within the data set, 973 sustained abdominopelvic injuries. Of these, only 26 (3%) underwent angiography. The median age was 14 years, 65% were males, with a mortality rate of 19%. Overall, 29 angiographic procedures were performed: 21 abdominal, 8 pelvic, with 3 patients undergoing both abdominal and pelvic. Eleven patients underwent embolization of a bleeding vessel, all of which were related to the spleen. No hepatic, renal, or pelvic vessels required embolization. The median time to angiography from emergency department evaluation was 7.3 hours. In addition to angiography, 50% also required surgical intervention, of which 31% underwent a laparotomy. Thirty-five percent of these patients required blood product transfusion, and 42% were admitted to the intensive care unit. CONCLUSION: The emergent use of angiography with embolization is uncommon in pediatric patients with blunt abdominal injuries. The requirement that pediatric trauma centers have access to interventional radiology within 30 minutes may be unnecessary. LEVEL OF EVIDENCE: Epidemiologic study, level III; therapeutic study, level IV.

Preventable pediatric intensive care unit admissions over a 13-year period at a level 1 pediatric trauma center.

BACKGROUND: No formal criteria exist to determine the need for admission of injured children to the pediatric intensive care unit. Our objective was to analyze trauma patient admissions to the PICU at a level 1 pediatric trauma center. METHODS: The trauma registry was analyzed between 2002 and 2015. A preventable PICU admission was defined as a child discharged home or transferred out of the PICU within 30h without surgical intervention, blood transfusion, or ventilator support. RESULTS: Of 16,209 children, 19% were admitted to the PICU: mean age 7.3years, median ISS 17, and overall mortality 7%. Per our definition, 36% were preventable PICU admissions of which 83% suffered a head injury. The preventable admissions were younger (6.9 vs. 7.6years, p<0.001) with a lower median ISS (16 vs. 21, p<0.001), shorter median PICU LOS (17 vs. 41h, p<0.001) and shorter median hospital LOS (51 vs. 121h, p<0.001). These admissions resulted in total facility charges of $9,981,454.76 with 54% produced by children with an isolated head injury. CONCLUSIONS: A significant number of children admitted to our PICU were classified as preventable. They carry a substantial economic burden to the health care system with an overutilization of resources. Methods to limit such admissions should be actively pursued.

Preventable transfers in pediatric trauma: A 10-year experience at a level I pediatric trauma center.

BACKGROUND: Injured children are often treated at one facility then transferred to another that specializes in pediatric trauma care. The purpose of this study was to identify and characterize potentially preventable transfers (PT) to a freestanding level-I pediatric trauma center. METHODS: Children with traumatic injuries transferred between 2003 and 2013 were retrospectively analyzed. A PT was defined as a child who was discharged within 36hours of arrival without surgical intervention or advanced imaging studies. RESULTS: During this period, 6380 children were transferred, with head injury being the most common injury. 61% had CT imaging performed before transfer. The mean age was 6.9years, mean injury severity score (ISS) 10.4, and median transfer distance 37miles. 27% of these transfers were classified as PT. Air transport was used in 15% at mean charge of $18,574. 29% were discharged from the emergency department. When compared, PTs were younger (6.0 vs. 7.2years, p<0.001), with lower median ISS (5 vs. 9, p<0.001), shorter median LOS (15 vs. 43.6hours, p<0.001), and less PICU admissions (6% vs. 34%, p<0.001). CONCLUSION: A significant number of pediatric trauma transfers can be classified as preventable. Reducing preventable transfers could offer opportunities for improving value in a trauma care system.

Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21.

BACKGROUND/PURPOSE: The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD). METHODS: A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000-2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011-2015) were performed. RESULTS: 26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD+T21 (p=0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD+T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD+T21 (p=0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD+T21). One child with HD+T21 was toilet trained by age 4years versus 12 with HD (p=0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD+T21. CONCLUSION: Enterocolitis rates in children with HD+T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent postoperative follow-up. LEVEL OF EVIDENCE: Retrospective Study - Level II.