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Background Limited resources and increased patient care demands have strained nurse-physician relationships in our hospital's neurosurgical intensive care unit, leading to low morale and adversarial dynamics. Studies exploring benefits of coaching interprofessional teamwork demonstrate performance improvements. Therefore, a coaching program designed to improve nurse-physician teamwork was initiated by the neurosurgery department of the hospital's affiliated university. Objective To assess the impact of a coaching program for nurses and physicians on workplace performance in a neurosurgical intensive care unit at a level 1 trauma center. Methods A coach was incorporated into everyday activities on the neurosurgical unit. After 3 months of observations, specific interdisciplinary initiatives were implemented to foster a more positive workplace environment. Nurses' perceptions before and after the initiatives were measured and compared using appropriate statistical tools. Results A significant improvement in relationships was found in 6 of 7 targeted categories after the program had been in place for 5 months. The results were sustained at 1 year. Conclusion A coaching program is an effective method of improving nurse-physician relationships, leading to enhanced workplace performance.

The appropriate imaging for pediatric patients being evaluated for suspected physical abuse depends on the age of the child, the presence of neurologic signs and symptoms, evidence of thoracic or abdominopelvic injuries, and whether the injuries are discrepant with the clinical history. The clinical presentations reviewed consider these factors and provide evidence-based consensus recommendations by the ACR Appropriateness Criteria ® Expert Panel on Pediatric Imaging.

Pancreatic problems that are commonly seen in infants and children, such as annular pancreas, may remain occult until adulthood; therefore, much about the pediatric pancreas can be informative to the adult surgical specialist. Pediatric management strategies, such as the nonoperative management of pancreatic trauma, have been found to be effective in this patient population, and the adult clinician must make an informed decision whether to extend these approaches to adult patients. Common surgical pancreatic conditions seen in infancy, childhood, and adolescence include annular pancreas, duodenal atresia, congenital hyperinsulinism of infancy, pancreas divisum, chronic pancreatitis, tumors, and trauma.

The majority of intermediate-risk rhabdomyosarcoma (RMS) patients have gross residual disease (Group III) after their first operative procedure. It is currently not known if local control rates can be maintained when, following induction chemotherapy, the radiation therapy (RT) dose is decreased after a delayed primary excision (DPE). To answer this question we evaluated patients enrolled on COG D9803 (1999-2005) who had Group III tumors of the bladder dome, extremity or trunk (thorax, abdomen and pelvis) were candidates for DPE at Week 12 if the primary tumor appeared resectable. RT dose was then adjusted by the completeness of DPE: no evidence of disease 36 Gy, microscopic residual 41.4 Gy and gross residual disease (GRD) 50.4 Gy. A total of 161 Group III patients were evaluated (24 bladder dome, 63 extremity and 74 trunk). Seventy-three patients (45%) underwent DPE which achieved removal of all gross disease in 61 (84%) who were then eligible for reduced RT dose (43/73 received 36 Gy, 19/73 received 41.4 Gy). The local 5-year failure rate (0% for bladder dome, 7% for extremity and 20% for trunk) was similar to IRS-IV, which did not encourage DPE and did not allow for DPE adapted RT dose reduction. In conclusion, DPE was performed in 45% of Group III RMS patients with tumors at select anatomic sites (bladder dome, extremity and trunk) and 84% of those who had DPE were eligible for RT dose reduction. Local control outcomes were similar to historic results with RT alone.

The decision for aggressive reoperation after discovery of an appendiceal carcinoid is generally based upon criteria such as size, grade, degree of involvement of the mesoappendix or the appendiceal base, lymphovascular invasion, and the presence of goblet cell or adenocarcinoid features. No guidelines currently exist for the management of perforated appendiceal carcinoids. We present a case of perforated appendiceal carcinoid that was subsequently treated with right hemicolectomy, and we review the pertinent literature.

Purpose To compare response rates for two schedules of irinotecan with vincristine in patients with rhabdomyosarcoma at first relapse or disease progression. Patients and Methods Patients with first relapse or progression of rhabdomyosarcoma and an unfavorable prognosis were randomly assigned to one of two treatment schedules of irinotecan with vincristine: regimen 1A included irinotecan 20 mg/m2/d intravenously for 5 days at weeks 1, 2, 4, and 5 with vincristine 1.5 mg/m2 administered intravenously on day 1 of weeks 1, 2, 4, and 5; regimen 1B included irinotecan 50 mg/m2/d intravenously for 5 days at weeks 1 and 4 with vincristine as in regimen 1A. Disease response was assessed at week 6. Those with responsive disease continued to receive 44 weeks of multiagent chemotherapy that incorporated the assigned irinotecan-vincristine regimen. Results Ninety-two eligible patients were randomly assigned (1A, 45; 1B, 47). Response could be assessed in 89 patients (1A, 42; 1B, 47). There were five complete responses and six partial responses on regimen 1A (response rate, 26%; 95% CI, 16% to 42%) and 17 partial responses on regimen 1B (response rate, 37%; 95% CI, 25% to 51%; P = .36). Neutropenia was less common on regimen 1A (P = .04). One-year failure-free and overall survival rates for regimen 1A were 37% (95% CI, 23% to 51%) and 55% (95% CI, 39% to 69%), respectively, and for 1B, they were 38% (95% CI, 25% to 53%) and 60% (95% CI, 44% to 72%). Conclusion There was no difference in the response rates between the two irinotecan-vincristine schedules. We recommend the shorter, more convenient regimen (1B) for further investigation.

Purpose The purpose of this study was to compare the outcome of patients with intermediate-risk rhabdomyosarcoma (RMS) treated with standard VAC (vincristine, dactinomycin, and cyclophosphamide) chemotherapy to that of patients treated with VAC alternating with vincristine, topotecan, and cyclophosphamide (VAC/VTC). Patients and Methods Patients were randomly assigned to 39 weeks of VAC versus VAC/VTC; local therapy began after week 12. Patients with parameningeal RMS with intracranial extension (PME) were treated with VAC and immediate x-ray therapy. The primary study end point was failure-free survival (FFS). The study was designed with 80% power (5% two-sided α level) to detect an increase in 5-year FFS from 64% to 75% with VAC/VTC. Results A total of 617 eligible patients were entered onto the study: 264 were randomly assigned to VAC and 252 to VAC/VTC; 101 PME patients were nonrandomly treated with VAC. Treatment strata were embryonal RMS, stage 2/3, group III (33%); embryonal RMS, group IV, less than age 10 years (7%); alveolar RMS or undifferentiated sarcoma (UDS), stage 1 or group I (17%); alveolar RMS/UDS (27%); and PME (16%). At a median follow-up of 4.3 years, 4-year FFS was 73% with VAC and 68% with VAC/VTC (P = .3). There was no difference in effect of VAC versus VAC/VTC across risk groups. The frequency of second malignancies was similar between the two treatment groups. Conclusion For intermediate-risk RMS, VAC/VTC does not significantly improve FFS compared with VAC.

Purpose: Few studies address the surgical correction of pectus excavatum (PE) in patients with connective tissue disease (CTD). We have identified the preoperative characteristics, postoperative complications, and outcomes of patients with CTD undergoing bar repair of PE and compared these outcomes to a control group without CTD. Methods: A retrospective review of patients undergoing primary repair of PE with a bar procedure from 1997 to 2006 identified 22 patients with CTD. Of those, 20 (90.9%) had their bars removed. We identified 223 patients of similar age without CTD whose bars were removed. Data collected included demographics, preoperative symptoms, operative characteristics, and postoperative outcomes. Results: Among those with CTD, the median age at repair was 15.5 years, with a mean pectus index of 4.0 ± 1.4. Three patients (13.6%) experienced bar displacement or upper sternal depression requiring surgical revision. Only 1 patient recurred after bar removal. Rates of bar displacement, upper sternal depression, and recurrence were not statistically different than those in the comparison group. Conclusions: Patients with CTD benefit from primary bar repair of PE and experience excellent operative outcomes after repair, with complication rates being no different than those found in similarly aged control patients.

Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood. Patient age, size, histologic finding, and site of the tumor are primary determinants of prognosis in RMS. Chest wall RMS is a site in which the limitations of surgical excision are realized. We aim to determine the impact of surgical excision in chest wall RMS.A retrospective chart review was conducted of all 130 pediatric patients enrolled in the Intergroup Rhabdomyosarcoma Study (IRS) with chest wall rhabdomyosarcoma from the first (I) through fourth (IV) IRS with follow-up to June 2005. Median follow-up was 12.1 years (4.6-27.2 years).There was a significant improvement in failure-free survival (FFS) and overall survival (OS) between the first IRS study, I, and IRS-IV. The estimated FFS and OS at 5 years in IRS I was 30% and 40%, respectively, compared to 68% and 78%, respectively, in IRS-IV (P = .03 and P = .05, respectively). There was no association between histologic finding or size and FFS or OS. However, all patients who presented without metastasis had an FFS and OS of 49% and 61%, respectively, compared with metastatic patients, 7% and 7%, respectively (P.001). Five-year FFS of group I, II, and III patients was 52%, 52%, and 45%, respectively, and OS was 65%, 60%, and 59%, respectively. There was no significant difference in 5-year FFS or OS in patients who had a complete resection (group I), complete resection with positive microscopic margins (group II), or biopsy or partial resection only (group III). In groups I to III patients, the local and regional failure rate at 5 years is 25% and 6%, respectively.The most significant impact on outcome in chest wall RMS patients is metastatic disease at diagnosis. The locoregional failure rate is high but does not appear to impact survival. Alternative treatment strategies are needed for chest wall RMS, but aggressive surgical excision may not be necessary.

Embryonal rhabdomyosarcoma, botryoid variant, is a malignant tumor that usually arises in the urinary bladder and other mucosal sites. This is a case of a 4-year-old girl presenting with a mass initially thought to arise within the urinary bladder. Macroscopic and microscopic examination proved that this botryoid rhabdomyosarcoma had its origin within the left ureter.

Introduction. Choosing surgery as a career is declining among U.S. medical students. The 8-wk third year surgery clerkship at our institution can be an intense learning experience, and we hypothesized that during this clerkship medical student quality-of-life would drop significantly from baseline, and that this drop would be greater among certain subgroups, such as women students not interested in pursuing a surgical career, and those who place a high value on a controllable lifestyle. Methods. At clerkship orientation (baseline), students were asked to complete a survey that measured quality-of-life on an 84-point scale, and depression on a 40-point scale. The quality-of-life scale was composed of select questions from the Medical Outcomes Study, and the Harvard Department of Psychiatry/ NDSD brief screening instrument was used to measure depression. Students were also asked the typical number of hours they slept per night. Demographics, attitude toward a controllable lifestyle, and top three specialties of interest were also gathered at baseline. On week 6 of the clerkship, students were surveyed on the same quality-of -life and depression scales, and asked average hours of sleep per night for the previous week. Results. From June 2005 through December 2006, 143 of 177 (81%) students agreed to participate, and after exclusions for missing data, 137 students were included in the analysis. Sixty-nine students were women (51%), and the average age was 25.8 (sd 2.6). Mean quality-of-life at baseline was 57.0 (sd 11.3) and at week 6 was 50.4 (sd 10.1) representing a statistically significant average decline of 6.6 points (P < 0.0001). Mean depression at baseline was 14.4 (sd 3.8) and at week 6 was 15.1 (sd 3.6), representing a small but significant average decline of 0.7 points (P = 0.0155). Mean sleep at baseline was 6.3 h/night (sd 0.9) and at week 6 was 5.7 h/night (sd 1.2), representing a statistically significant average decline of 0.6 h/night (P < 0.0001). Declines were similar on all outcomes between men versus women, those who ranked surgery in their top three career choices versus those who did not, and those who ranked controllable lifestyle as "very important" versus all other categories. Conclusion. Quality-of-life and sleep declines and depression increases significantly in third-year medical students from orientation to week 6 of their surgery clerkship at our institution. We look forward to studying quality-of-life on other clerkships for comparison, assessing whether the magnitude of this decline in quality-of-life predicts students avoiding a future career in surgery, and testing interventions to prevent this decline in quality-of-life during the clerkship.

Background This study was undertaken to define outcomes after laparoscopic Heller myotomy with anterior fundoplication in pediatric patients and compare their outcomes with those in adults. Study Design A total of 337 patients have undergone laparoscopic Heller myotomy with anterior fundoplication since 1992, and were prospectively followed; 14 were pediatric patients of median age 17 years (range 11 to 19 years). Symptoms noted by pediatric patients before and after myotomy were compared with symptoms of 56 concurrently treated adults (4 treated adults for each pediatric patient) of median age 48 years. Among many symptoms, patients scored the severity and frequency of dysphagia, chest pain, regurgitation, choking, vomiting, and heartburn before and after myotomy using a Likert scale, ranging from 0 (never/not bothersome) to 10 (always/very bothersome). Followups were 38 months, 42 months±33.1. Data are reported as median, mean ± SD. Results For pediatric patients, length of stay after myotomy was 2 days, 3 days±2.9 versus 2 days, 2±2.1 for adults. Before myotomy, symptom frequency and severity were similar between groups. After myotomy, symptom frequency and severity were similar between pediatric and adult patients, except for the frequency of chest pain. Conclusions Achalasia can produce disabling symptoms, which were similar between pediatric and adult patients before myotomy. Laparoscopic Heller myotomy with anterior fundoplication ameliorated symptoms of achalasia in all patients, with postmyotomy symptoms similar between pediatric and adult patients. Laparoscopic Heller myotomy dramatically improved symptoms of achalasia in pediatric patients and its use is encouraged.

OBJECTIVE. Children with moderate to severe traumatic brain injury (TBI) show early neurobehavioral deficits that can persist several years after injury. Despite the negative impact that TBI can have on a child's physical, cognitive, and psychosocial well-being, only 1 study to date has documented the receipt of health care services after acute care and the needs of children after TBI. The purpose of this study was to document the health care use and needs of children after a TBI and to identify factors that are associated with unmet or unrecognized health care needs during the first year after injury. METHODS. The health care use and needs of children who sustained a TBI were obtained via telephone interview with a primary caregiver at 2 and 12 months after injury. Of the 330 who enrolled in the study, 302 (92%) completed the 3-month and 288 (87%) completed the 12-month follow-up interviews. The health care needs of each child were categorized as no need, met need, unmet need, or unrecognized need on the basis of the child's use of post-acute services, the caregiver's report of unmet need, and the caregiver's report of the child's functioning as measured by the Pediatric Quality of Life Inventory (PedsQL). Regardless of the use of services or level of function, children of caregivers who reported an unmet need for a health care service were defined as having unmet need. Children who were categorized as having no needs were defined as those who did not receive services; whose caregiver did not report unmet need for a service; and the whose physical, socioemotional, and cognitive functioning was reported to be normal by the caregiver. Children with met needs were those who used services in a particular domain and whose caregivers did not report need for additional services. Finally, children with unrecognized needs were those whose caregiver reported cognitive, physical, or socioemotional dysfunction; who were not receiving services to address the dysfunction; and whose caregiver did not report unmet need for services. Polytomous logistic regression was used to model unmet and unrecognized need at 3 and 12 months after injury as a function of child, family, and injury characteristics. RESULTS. At 3 months after injury, 62% of the study sample reported receiving at least 1 outpatient health care service. Most frequently, children visited a doctor (56%) or a physical therapist (27%); however, 37% of caregivers reported that their child did not see a physician at all during the first year after injury. At 3 and 12 months after injury, 26% and 31% of children, respectively, had unmet/unrecognized health care needs. The most frequent type of unmet or unrecognized need was for cognitive services. The top 3 reasons for unmet need at 3 and 12 months were (1) not recommended by doctor (34% and 31%); (2) not recommended/provided by school (16% and 17%); and (3) cost too much (16% and 16%). Factors that were associated with unmet or unrecognized need changed over time. At 3 months after injury, the caregivers of children with a preexisting psychosocial condition were 3 times more likely to report unmet need compared with children who did not have one. Also, female caregivers were significantly more likely to report unmet need compared with male caregivers. Finally, the caregivers of children with Medicaid were almost 2 times more likely to report unmet need compared with children who were covered by commercial insurance. The only factor that was associated with unrecognized need at 3 months after injury was abnormal family functioning. At 12 months after injury, although TBI severity was not significant, children who sustained a major associated injury were 2 times more likely to report unmet need compared with children who did not. Consistent with the 3-month results, the caregivers of children with Medicaid were significantly more likely to report unmet needs at 1 year after injury. In addition to poor family functioning's being associated with unrecognized need, nonwhite children were significantly more likely to have unrecognized needs at 1 year compared with white children. CONCLUSIONS. A substantial proportion of children with TBI had unmet or unrecognized health care needs during the first year after injury. It is recommended that pediatricians be involved in the post-acute care follow-up of children with TBI to ensure that the injured child's needs are being addressed in a timely and appropriate manner. One of the recommendations that trauma center providers should make on hospital discharge is that the parent/primary caregiver schedule a visit with the child's pediatrician regardless of the post-acute services that the child may be receiving. Because unmet and unrecognized need was highest for cognitive services, it is important to screen for cognitive dysfunction in the primary care setting. Finally, because the health care needs of children with TBI change over time, it is important for pediatricians to monitor their recovery to ensure that children with TBI receive the services that they need to restore their health after injury.

A 13-year-old African-American girl was admitted to the hospital for surgery. She was diagnosed with Type I neurofibromatosis at the age of 1 year after she was noted to have multiple café au lait spots. Her past medical history included a history of neurofibroma in the base of the brain, treated with radiation therapy and ventriculoperitoneal shunt, as well as a recent diagnosis of bilateral optic gliomas, treated with chemotherapy. Family history was negative for neurofibromatosis.

Objectives To review our experience with the management of high-grade (grade IV and V) renal injuries to clarify the role of conservative management. Methods From 1991 to 2003, 79 consecutive patients (age range 2 to 14 years) with renal injuries were treated in an urban level I pediatric trauma center. Twenty children were identified as having high-grade renal injury (grade IV, 10 children and grade V, 10 children). The mechanism of injury was blunt trauma in 17 patients (85%) and penetrating trauma in 3 (15%). Results Of the 10 patients with grade IV injury, 8 (80%) were successfully treated conservatively with bedrest and catheter drainage. Two patients with persistent urine leaks required ureteral stenting, and one subsequently required open operative repair. The initial radiographic findings in both patients demonstrated complete renal fracture with retained vasculature to both renal segments. All 10 patients with grade V injury required open operative management and only 3 (30%) achieved long-term renal salvage. Conclusions Most children with grade IV renal injury can be treated conservatively. Patients with complete renal fracture or significant urinary extravasation on initial radiographic imaging may be less likely to undergo spontaneous resolution. Patients with a persistent urinary leak can be successfully treated with internal drainage. Grade V injuries are associated with an increased risk of requiring open operative intervention, and the renal preservation rates are low.

ObjectiveTo evaluate whether girls have better outcomes after traumatic brain injury than boys.DesignRetrospective cohort study.SettingUniversity hospital.PatientsA 16,586 patient subset of the National Pediatric Trauma Registry with nonpenetrating traumatic brain injury.InterventionsRetrospective r

Malignant peritoneal mesothelioma is a rare malignancy of the serosal membranes that is difficult to diagnose and carries a poor prognosis. Progression of presenting symptoms is slow and nonspecific. Diffuse peritoneal seeding by the neoplasm can present silently so that physical abnormalities may not be detected until the disease is far advanced. Standard laboratory and radiographic studies can also be inconclusive. We report a case of an adolescent female whose clinical presentation and initial response to management were consistent with inflammatory bowel disease (IBD) but who was ultimately diagnosed with malignant peritoneal mesothelioma.

Background. The variable clinical profile observed in critically ill patients is the result of multiple factors. Genetic determinants have recently been shown as confounding factors in the response to injury. However, other elements, such as the environment and the type of injury, could modify this response. The objective of this investigation was to study the effect of combining insults and different genetic backgrounds on the inflammatory response. Materials. Male mice, C57BL/6J (B6) and A/J, were randomized to undergo cecal ligation and single puncture (CLP) or sham operation (SOP). After 24 h of recovery, mice were randomized again into two groups, one group was injected with bacterial lipopolysaccharide (LPS; 15 mg/kg) and the other was injected with normal saline (NS). An additional experimental group included mice that were not operated (NOP) and injected with LPS. Mice were evaluated by plasma cytokine content. Results. The combination of insults resulted in an apparent additive effect for some cytokines, such as interleukin (IL) 6. In contrast, tumor necrosis factor α (TNF-α) was considerably lower in the combined injury group with respect to injection of LPS alone. There was no relevant difference in IL-10 levels between any group, except that its decay was slower in the CLP + LPS group. Overall, cytokine levels were different between B6 and A/J mice indicating a genetic contribution. Conclusions. These results indicate that the response to stress is the combination of the type of injury and the genetic background of the subject. These observations also illustrate the difficulty in predicting the inflammatory response and underlying mechanism based on cytokine plasma levels.

Background Although age

PURPOSE: To identify which patients with rhabdomyosarcoma and microscopic residual disease (group II) are likely to not respond to therapy. PATIENTS AND METHODS: Six hundred ninety-five patients with group II tumors received chemotherapy and 90% received radiation therapy on Intergroup Rhabdomyosarcoma Study (IRS)-I to IRS-IV (1972 to 1997). Tumors were subgrouped depending on the presence of microscopic residual disease only (subgroup IIa), resected positive regional lymph nodes, (subgroup IIb), or microscopic residual disease and resected positive regional lymph nodes (subgroup IIc). RESULTS: Overall, the 5-year failure-free survival rate (FFSR) was 73%, and patients with embryonal rhabdomyosarcoma treated on IRS-IV fared especially well (5-year FFSR, 93%; n = 90). Five-year FFSRs differed significantly by subgroup (IIa, 75% and n = 506; IIb, 74% and n = 101; IIc, 58% and n = 88; P = .0037) and treatment (IRS-I, 68%; IRS-II, 67%; IRS-III, 75%; IRS-IV, 87%; P < .001). Multivariate analysis revealed positive associations between primary site (favorable), histology (embryonal), subgroup IIa or IIb, treatment (IRS-III/IV), and better FFSRs. Patterns of treatment failure revealed local failure to be 8%, regional failure, 4%, and distant failure, 14%. The relapse pattern noted over the course of IRS-I to IRS-IV shows a decrease in the systemic relapse rates, particularly for patients with embryonal histology, suggesting that improvement in FFSRs is primarily a result of improved chemotherapy. CONCLUSION: Group II rhabdomyosarcoma has an excellent prognosis with contemporary therapy as used in IRS-III/IV, and those less likely to respond can be identified using prognostic factors: histology, subgroup, and primary site. Patients with embryonal rhabdomyosarcoma are generally cured, although patients with alveolar rhabdomyosarcoma or undifferentiated sarcoma, particularly subgroup IIc at unfavorable sites, continue to need better therapy.

Purpose: This study was designed to estimate the partial and complete response rates (CR and PR) of two novel drug pairs (vincristine and melphalan vs. ifosfamide and etoposide) and to improve overall survival of previously untreated patients with metastatic rhabdomyosarcoma. Patients and Methods: One hundred twenty-eight patients were randomly assigned to phase II window therapy consisting of vincristine and melphalan (VM-containing regimen) or ifosfamide and etoposide (IE-containing regimen). Brief window therapy (12 wks) was immediately followed-up by vincristine, dactinomycin, and cyclophosphamide (VAC), chemotherapy, surgery, and irradiation, with continuation of either VM or IE in patients with initial response. Major endpoints were initial CR and PR rates after the phase II window phase of therapy, failure-free survival (FFS), and survival. Results: Patients who received the VM-containing regimen experienced significantly more anemia, neutropenia, thrombocytopenia, and had more cyclophosphamide dose reductions. The initial PR and CR rates were not significantly different for patients treated with either regimen (VM, 74%; IE, 79%; P 0.428). However, FFS and overall survival (OS) at 3 years were significantly better with the IE-containing regimen (FFS: 33% vs. 19%; P 0.043; OS: 55% vs. 27%; P 0.012). Conclusions: Although the VM-containing regimen produced a high response rate, inclusion of melphalan appeared to limit the cyclophosphamide dose that could be administered, and ultimately, this regimen was associated with a significantly worse outcome than was the IE-containing regimen. Also, the IEcontaining regimen was associated with a gratifyingly high survival rate at 3 years (55%), which is significantly higher than has been observed on any previous Intergroup Rhabdomyosarcoma Study Group regimen for similar patients. We believe that this promising outcome indicates that this drug pair merits further randomized testing in metastatic rhabdomyosarcoma.

This article is the offshoot of a Pediatric Oncology Group (POG) seminar presented at the Adams Mark Hotel, Denver, Colorado, Friday, May 21, 1999, titled “The Frozen Section in Pediatric Solid Tumors—Crucial Issues.” There were eight presenters who spoke on a wide range of topics that included historical perspectives of the frozen section and discussion of the following systems: brain, renal, germ cell, bone, soft tissue, and lymph nodes. To complement these presentations, a pediatric surgeon explained his concern and philosophy regarding the use of frozen sections, and a lawyer tackled the issues and risks in rendering a frozen section diagnosis. We think that this review covers all the important aspects of the frozen section in our current practice of pediatric pathology.

BACKGROUND Rhabdomyosarcoma (RMS) of the parotid region is rare and to the authors' knowledge little information is available regarding the site of tumor origin, clinical presentation, and outcome in these patients. Therefore, the authors reviewed the files of all patients with RMS of the parotid region who were registered on the Intergroup Rhabdomyosarcoma Studies (IRS) I–IV. METHODS Patient charts and the Intergroup Rhabdomyosarcoma Study Group (IRSG) database were reviewed. RESULTS Sixty-two patients presenting with a mass in the parotid region were identified. None of the tumors was localized exclusively to the parotid gland, so the primary site was referred to as the “parotid region.” The tumor invaded a parameningeal site in 30 patients. These cases have been designated as parameningeal-parotid tumors to distinguish them from 32 cases that did not invade a parameningeal site and were designated as nonparameningeal-parotid tumors. The majority of patients had Group III tumors in both the nonparameningeal-parotid and parameningeal-parotid subgroups. However, although there were 16 patients with Group I or II tumors in the nonparameningeal-parotid subgroup, no patients with Group I or II tumors were found in the parameningeal-parotid subgroup (P = 0.001). Fifty-six of 62 patients (90%) received radiotherapy. The parameningeal primary site designation resulted in intensification of both chemotherapy and radiotherapy for patients with parameningeal-parotid RMS. The 5-year failure-free survival rate was 81% and the 5-year survival rate was 84%. There were no deaths reported among patients with Group I or II tumors. The 5-year failure-free survival did not appear to differ when comparing patients with parameningeal-parotid tumors with patients with nonparameningeal-parotid tumors (P = 0.21). CONCLUSIONS Treatment as defined by the IRS protocols has been reported to be highly effective for patients with RMS of the parotid region. Outcome for the more aggressively treated patients with parameningeal-parotid RMS appears similar to that for patients with nonparameningeal-parotid RMS. Cancer 2001;92:3135–46. © 2001 American Cancer Society.

Purpose: The aim of this study was to examine the authors' experience with patients who have floating sternum after correction of pectus excavatum via the classical Ravitch procedure. A floating sternum is defined as a sternum in which the only attachment to the chest wall is its superior (cranial) border, and in which the body is secured only by the manubrium and whatever lateral and inferior fibrous bands are present. Typically, a floating sternum is caused by either extensive resection of the costal cartilages and perichondrium during correction of pectus excavatum or failure of proper regrowth of these cartilages. Methods: The authors retrospectively assessed the charts of all patients diagnosed with a floating sternum noting age at original correction of pectus excavatum, time from original correction of pectus excavatum to diagnosis of floating sternum, age at correction of floating sternum, complaints before stabilization of the sternum, methods of repair, and postoperative complications. Results: Between July 1993 and June 1999, floating sternum was diagnosed in 7 patients. The mean age of patients who underwent operative correction of a floating sternum was 28.9 years (range, 16 to 42 years). The mean time interval between original correction of pectus excavatum, or "redo," and diagnosis of a floating sternum was 9.9 years (range, 2 to 20 years). Complaints before correction of the floating sternum included sternal pain and instability, exercise intolerance, and difficulty breathing. Operative repair consisted of mobilizing the lateral and inferior edges of the sternum, detaching the fibrous perichondrium, performing anterior sternal osteotomies, and finally supporting the sternum with substernal Adkins struts. All 7 patients had successful stabilization of the sternum. Two of 7 patients underwent 2 procedures to successfully stabilize the sternum. One patient has Adkins struts still in place because of hematopoetic malignancy. Six of 7 patients are now without symptoms. Conclusions: A floating sternum is a morbid phenomenon that may manifest many years after the original procedure. It can cause significant sternal pain, chest wall instability, and respiratory dysfunction, which are the hallmark indications for correction. Repair of a floating sternum can be accomplished successfully. J Pediatr Surg 36:159-164. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: Rhabdomyosarcoma (RMS) of the biliary tract is rare, and, in addition to multiagent chemotherapy with or without radiotherapy (RT), some investigators recommend aggressive surgery. To assess the role of surgery, records of all 25 eligible patients with biliary RMS enrolled in IRSG studies I through IV from 1972 to 1998 were reviewed. Methods: Treatment included surgery with or without vincristine, dactinomycin, cyclophosphamide, doxorubicin, cisplatin, etoposide, ifosfamide, and with or without RT. Data evaluated included clinical presentation, treatment, complications, and outcome. Results: Diagnostic imaging identified the primary tumor but failed to identify regional metastases. Despite aggressive surgery, gross total resection at diagnosis was possible in only 6 cases, 2 of which had negative surgical margins. Although only 6 (29%) patients without distant metastases underwent gross total resection, estimated 5-year survival rate was 78% (95% CI 58%, 97%). Infectious complications were common and frequently associated with external biliary drains. Five (20%) died within the first 2 months, 3 of sepsis. Conclusions: Surgery is critical for establishing an accurate diagnosis and determining the extent of regional disease. Gross total resection is rarely possible despite aggressive surgery, and outcome is good despite residual disease after surgery. External biliary drains increase the risk of postoperative infectious complications. J Pediatr Surg 35:309-316. Copyright © 2000 by W.B. Saunders Company.

This study compares the effects of carbon dioxide pneumoperitoneum versus laparotomy on cellular-mediated immune response in a murine model.Sixty-eight female C3H/He mice were sensitized to keyhole limpet hemocyanin (KLH) and to a mouse mammary carcinoma cell line (MC2) before surgery. Animals were randomized into 4 groups: group I, anesthesia (control); group II, pneumoperitoneum with carbon dioxide; group III, extraperitoneal wound; group IV, laparotomy. All animals were challenged subsequently with KLH and MC2 tumor cells. Delayed-type hypersensitivity skin reaction (DTH) to KLH was measured on postoperative days (PODs) 1, 2, 4, and 5. Tumor growth was assessed weekly as an indicator of postoperative cellular immune response.Compared with preoperative values, postoperative DTH skin reactions were significantly less for all PODs in groups III and IV (P.05), on POD 1 and 4 in group II (P.05) and POD 4 for group I (P.05). Group IV showed significantly fewer DTH skin reactions for all PODs compared with groups I and II (P.05) and all PODs except on day 2 compared with group III (P.05). Tumor growth was significantly increased at postoperative week 2 (n = 3/17 mice) and 3 (n = 4/17 mice) in group IV, when compared with groups I and II (P.05).Cellular immunity is preserved after carbon dioxide pneumoperitoneum compared with extraperitoneal incisions and laparotomy as measured by DTH and the ability to reject an immunogenictumor.

Injury is the leading cause of death among children more than 1 year of age—in fact, it exceeds all other causes of death, combined, for children. 4,11,12 In the past 25 years, prevention initiatives and systems of trauma care have reduced the morbidity and mortality rates of injured children, 77,84,139,163 but injury remains the leading health problem for children. 23 Currently, mortality rates and care of minor injury in regional pediatric trauma centers are no different than those of adult centers with pediatric commitment, 10 but the overall survival rate for children with certain severe critical illnesses or injuries is better in regional pediatric centers. 7,43,89,118,129,130,189 These more severe injuries include isolated head injury, multisystem injury, and isolated blunt abdominal injury. The establishment of statewide trauma systems for children has also reduced the risk for death from severe injury. 84 Children's hospitals provide not only tertiary medical and nursing care but also support services distinct from adult hospitals. Despite the special needs of injured children, the large number of injuries precludes care solely by children's hospitals and adult centers with pediatric commitment. 10,77,155 Thus, how physicians treat injured children may be the primary theme to lead us into the new millennium. 55,155 The concept of "trauma as a national disease" 2,92 led to the founding of emergency medical services. 111 The establishment of pediatric trauma centers, in 1973, 41,76 called attention to the special needs of injured children, and standards of their care were finally implemented in 1982. 9 Most importantly, a landmark conference for childhood trauma, emergency medical services for children, 73 focused for the first time on the overlooked emergency health needs of injured children. An integrated systems approach to the management of life-threatening childhood illnesses and injury was developed as a result of emergency medical services for children. This led to nationwide awareness of the unique health needs of children and physicians' responsibility for prevention and injury control. 133,155 Subsequently, the National Pediatric Trauma Registry (NPTR), sponsored by the National Institute of Disability and Rehabilitation Research and by the American Pediatric Surgical Association, was created in 1984 to serve as a multi-institutional database for injuries in children aged 0 to 19 years. 178 To date, 85,000 childhood injuries ranging from life-threatening to minor trauma have been entered into the registry (personal communication, DiScala, 1999). It provides physicians with information regarding epidemiology, demographics, mechanisms, and patterns of common childhood injuries that have facilitated standards of management, prevention strategies, and injury-control interventions. Federal, state, and community interest in childhood trauma is, as yet, sporadic and thus not adequate for the magnitude of the problem. Gaps in effective care still exist in the current algorithm for the optimal care of injured children. This article reviews epidemiology, injuries unique to children, components of acute care, and prevention.

Many poor-risk neuroblastomas and tumours of the Ewing's sarcoma family (ET) recur despite autologous transplants. Recurrence may be due to tumor cells contained in the BM harvests or PBSC harvests. The objectives of this prospective study were to: (1) determine the incidence and degree of tumor cell contamination in paired BM and PBSC harvests; and (2) determine the efficacy of tumor cell purging by immunomagnetic CD34+ cell selection. 198 samples from 11 consecutive patients with neuroblastoma or Ewing's sarcoma were analyzed. We assayed tumor contamination by RT-PCR assay for PGP 9.5, plus immunohistochemistry for neuroblastoma-specific antigens (the latter in neuroblastoma only). None of these patients had tumor cells detected in their BM by clinical histology immediately before BM or PBSC harvests. However, 82% of PBSC and 89% of backup BM harvests were contaminated with tumor by RT-PCR and/or immunocytochemistry assays. Unselected PBSC and BM harvests contained similar quantities of tumor cells (median, approximately 200000 cells). Cyclophosphamide plus G-CSF mobilization did not affect the incidence or level of contamination in PBSC harvests, as compared to blood obtained before mobilization. Immunomagnetic CD34+ cell selection depleted tumor cells by a median of 3.0 logs for PBSC, and 2.6 logs for BM harvests.

The purpose of this study is to delineate the effect of different operative procedures on the cell-mediated immune response in a pediatric animal model using the delayed type hypersensitivity (DTH) skin test.Sprague Dawley rats (1 week old) were sensitized against keyhole limpet hemocyanin (KLH). Animals were challenged 2 weeks later by an intradermal injection of KLH (0.3 mg) in sterile saline. Rats with positive DTH skin reactions at 24 and 48 hours after challenge (baseline) were divided randomly into five groups (n = 10 each): group I, unmanipulated control; group II, anesthesia; group III, anesthesia and midline extraperitoneal incision; Group IV, anesthesia and laparoscopy (pneumoperitoneum with carbon dioxide); Group V, anesthesia and midline laparotomy. Before each procedure (day 0) and on postoperative days 3 and 6, animals were again challenged intradermally with KLH (0.3 mg). DTH skin reaction was evaluated 24 and 48 hours later.A statistically significant difference (P.05) in DTH skin reaction at 24 and 48 hours was observed between postoperative days 1 to 5 in the extraperitoneal and laparotomy groups with respect to baseline and the control group. Statistically significant differences were found in postoperative days 1, 4, and 5 between laparoscopy and laparotomy. The laparoscopy group showed a statistically significant decrease in DTH skin induration on postoperative day 2 when compared with the control group. At postoperative day 7 and 8 there was no statistical difference in DTH skin response comparing baseline values or between groups.These results suggest that in a pediatric animal model, abdominal surgical procedures accompanied by extensive tissue dissection produce a cellular immunosuppression, lasting up to 7 days, which is not observed in less invasive procedures. Observations concerning lesser immunosuppressive effects of laparoscopy when compared with laparotomy in adult models, as previously described by our laboratory, were also found in this pediatric model.

Previous studies have found that the Injury Prevention Priority Score (IPPS) provides a reliable and valid method to gauge the relative importance of different injury causal mechanisms at individual trauma centers. This study examines its applicability to prioritizing injury mechanisms on a national level and within defined pediatric age groups.A total of 47,158 patients (age17) in the National Pediatric Trauma Registry were grouped into common injury mechanisms based on ICD-9 E-Codes. Patients also were stratified by age group. IPPS was calculated for each mechanism and within each age group.Falls of all types account for the greatest number of injuries (n = 15,042; 32%), whereas child abuse results in the most severe injuries (mean Injury Severity Score, 13.3) However, the most significant mechanisms of injury, according to IPPS, were motor vehicle crashes followed by pedestrian struck by motor vehicles. Certain age groups had specific injury problems including child abuse in infants and assault and gun injuries in adolescents.IPPS provides an objective, quantitative method for determining injury prevention priorities based on both frequency and severity at the national level. It also is sensitive to age-related changes in different mechanisms of injury.

Pancreatic ductal injuries in children are rare, and ductal transections presenting in a delayed or subacute fashion are seldom reported. We describe two cases of traumatic pancreatic ductal transection secondary to physical abuse, both of which presented late to medical care. Both were managed successfully without pancreatic resection. Judicious application of non-resectional management can yield favorable outcomes in this subset of pediatric patients.

In an effort to maximize staff utilization, all pediatric trauma patients were triaged by emergency room personnel to one of two tiers, based on information reported by prehospital providers over radiotelephones. A total of 952 patients less than 15 years of age were evaluated during a 1-year period. The triage criteria had a sensitivity of 86% in predicting which trauma patients would require operating room and/or pediatric intensive care, while maintaining a specificity of 90%. Fifteen patients died; however, by TRISS methodology there were no unexpected deaths and four unexpected survivors. All eventual deaths were initially captured from field data by the severely injured triage criteria. The study data suggest that physician-controlled two-tiered field triage criteria can safely serve to maximize staff utilization in the emergency room.

Traumatic diaphragmatic rupture is a rare childhood injury and is often difficult to diagnose. This is particularly true in infants. We present the case of a 3-month-old infant with traumatic rupture of the right diaphragm that became clinically apparent only after extubation. The diagnosis can often be made on the basis of chest radiography and clinical signs. Surgical treatment is required. Some phrenic nerve injuries can be anticipated and late sequelae may result.

BACKGROUND: The objectives of this study were to compare tumor volume and patient weight versus traditional factors of tumor size (greatest dimension) and patient age and to determine which parameters best discriminated outcome among pediatric patients with intermediate-risk rhabdomyosarcoma (RMS). METHODS: Complete information was available for 370 patients with nonmetastatic RMS who were enrolled in the Children's Oncology Group (COG) intermediate-risk study D9803 (1999-2005). The Kaplan-Meier method was used to estimate survival distributions. A recursive partitioning model was used to identify prognostic factors that were associated with event-free survival (EFS). Cox proportional hazards regression models were used to estimate the association between patient characteristics and the risk of failure or death. RESULTS: For all patients with intermediate-risk RMS, a recursive partitioning algorithm for EFS suggested that prognostic groups should be defined optimally by tumor volume (with a transition point at 20 cm3), patient weight (with a transition point at 50 kg), and embryonal histology. Tumor volume and patient weight added significant outcome information to the standard prognostic factors, including greatest tumor dimension and patient age (P = .02). The ability to resect the tumor completely was not associated significantly with the size of the patient, and patient weight did not significantly modify the association between tumor volume and EFS after adjustment for standard risk factors (P = .2). CONCLUSIONS: The factors that had the strongest association with EFS were tumor volume, patient weight, and histology. On the basis of regression modeling, tumor volume and patient weight were superior predictors of outcome compared with greatest tumor dimension and patient age in children with intermediate-risk RMS. The current results indicated that the prognostic performance of tumor volume and patient weight should be assessed in an independent prospective study. Cancer 2011. © 2010 American Cancer Society.

A case of pleuropulmonary blastoma in a 13-year-old child, exposed to the Chernobyl disaster while in-utero, is presented and discussed by both clinician and pathologist, in this traditional clinical-pathologic conference. The discussion includes the differential diagnoses of chest mass in children.

Developmental dysplasia of the hip (DDH) affects 1.5 of every 1,000 caucasian Americans and less frequently affects African Americans. Developmental dysplasia of the hip comprises a spectrum of abnormalities, ranging from laxity of the joint and mild subluxation to fixed dislocation. Early diagnosis of DDH usually leads to low-risk treatment with a harness. Late diagnosis of DDH in children may lead to increased surgical intervention and complications. Late diagnosis of DDH in adults can result in debilitating end-stage degenerative hip joint disease. Screening decreases the incidence of late diagnosis of DDH. Clinical evaluation for DDH should be performed periodically at each well-baby visit until the age of 12 months. There is no consensus on imaging screening for DDH. Consideration for screening with ultrasound is balanced between the benefits of early detection of DDH and the increased treatment and cost factors. In addition, randomized trials evaluating primary ultrasound screening did not find significant decrease in late diagnosis of DDH. In the United States, hip ultrasound is selectively performed in infants with risk factors, such as family history of DDH, breech presentation, and inconclusive findings on physical examination. Ultrasound for DDH should be performed after 2 weeks of age because laxity is common after birth and often resolves itself. A pelvic radiograph can optimally be performed after the age of 4 months, when most infants will have ossification centers of the femoral heads.

OBJECTIVE. Traumatic brain injury has a substantial impact on caregivers. This study describes the burden experienced by caregivers of children with traumatic brain injury and examines the relationship between child functioning and family burden during the first year after injury.PATIENTS AND METHODS. Children aged 5 to 15 years hospitalized for traumatic brain injury at 4 participating trauma centers were eligible. Caregivers completed baseline and 3- and 12-month telephone interviews measuring the child's health-related quality of life using the Pediatric Quality of Life Inventory. The emotional impact scale of the Child Health Questionnaire was used to identify caregivers with substantial distress, including general worry or interference with family routine. Caregiver perceptions of whether health care needs were met or unmet and days missed from work were also measured.RESULTS. A total of 330 subjects enrolled; follow-up was conducted with 312 at 3 months and 288 at 12 months. Most subjects were white (68%) and male (69%). Abnormal Pediatric Quality of Life Inventory subscores were related to substantial caregiver burden (either general worry or interference in routine). These abnormalities were reported by >75% of patients at 3 months and persisted to 1 year in some patients. Parental perception of unmet health care needs was strongly related to family burden outcomes, with up to 69% of this subset of parents reporting substantial worry, and nearly one quarter reporting interference with daily routine/concentration 1 year after injury. Child dysfunction predicted parental burden at 3 and 12 months. Burden was greater when health care need was unmet. Abnormalities on the Pediatric Quality of Life Inventory predicted the amount of work missed by parents, especially in the presence of unmet needs.CONCLUSIONS. Caregivers are more likely to report family burden problems when child functioning is poorer and health care needs are unmet. Improved identification and provision of services is a potentially modifiable factor that may decrease family burden after pediatric traumatic brain injury.

The roles of pediatric surgeons in the treatment of RMS have changed signi”cantly through the years,as other adjuvant therapies have become more ef”cacious.The purpose of this manuscript is todescribe the current surgical therapy recommen-dations of the Soft Tissue Sarcoma Committee of theChildren’s Oncology Group (COG),formerly knownas the Intergroup Rhabdomyosarcoma Group(IRSG).

An upper sternal depression following Lorenz bar repair of pectus excavatum (PE) represents a partial recurrence and poses a difficult problem for the surgeon. There is no published experience detailing the management options or best course of therapy for this complication. This study presents our institutional experience in treating eight patients with this specific subtype of recurrence and we discuss intraoperative considerations which aid in the identification and better management of this deformity. A retrospective review (1997-2006) of patients undergoing primary repair of PE with a Lorenz bar procedure identified eight patients who experienced upper sternal depression with the bar still in place following initial repair of PE. All patients were revised with the insertion of a second bar to elevate the upper sternal depression. Data collected for each patient included demographics, preoperative symptoms, operative characteristics, and postoperative outcomes. The mean age at the time of Lorenz bar repair and surgical revision was 20.8 +/- 9.5 and 21.5 +/- 10.1 years, respectively. A majority of patients (87.5%) were male. The mean time to reoperation was 23.8 +/- 11.8 months. Following this second procedure, no patient has experienced bar displacement, recurrence of the upper sternal depression, or has required a third procedure. Our limited experience supports the use of a second Lorenz bar in the treatment of upper sternal depression after bar correction of a PE deformity. Appropriate recognition and treatment of this entity will advance patient outcomes and satisfaction after surgery for PE deformities.

Primary graft failure and vascular thromboses are frequent complications of liver transplantation, yet the mechanisms responsible remain unclear. Previous work from our laboratory has shown that hepatic reperfusion injury results in damage at the microvessel level. The present study was performed to determine whether an increased susceptibility of immature animals to microvascular injury during reperfusion might be a contributing factor in these complications. Suckling (35 to 50 g) or adult (250 to 400 g) rats were subjected to 30 or 60 minutes of hepatic ischemia to the left and median lobes followed by 90 minutes of reperfusion. Control animals were sham-operated, time-matched rats. At the end of reperfusion, fluorescein-labeled albumin was injected systemically to mark perfused sinusoids. Frozen sections of liver biopsies were viewed under fluorescence microscopy. The perfused sinusoid density was determined by point count analysis and expressed as the number of intersections of perfused sinusoids with 25 randomly oriented points superimposed on the sinusoid field. In sham-operated rats, at both 30 and 60 minutes, there were no differences between sucklings and adults. After 30 minutes of ischemia and 90 minutes of reperfusion, adults showed a significantly decreased density of perfused sinusoids (4.5 +/- 0.1 intersections per field) when compared with suckling rats (6.0 +/- 0.3 intersections per field, P less than .001). However, in rats subjected to 60 minutes of ischemia followed by 90 minutes of reperfusion, the microvascular injury was more severe in suckling rats (2.7 +/- 0.2 intersections per field) than in adults (4.7 +/- 0.2 intersections per field, P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

To document health-related quality of life (HRQOL) of children with traumatic brain injury (TBI) and to examine the relationship between TBI severity and HRQOL during the first year after injury.Prospective cohort study.Four pediatric level I trauma centers.Children with TBI (n = 330).Traumatic brain injury.A primary caregiver completed telephone interviews at baseline, 3 months, and 12 months to measure the child's HRQOL using the Pediatric Quality of Life Inventory. The HRQOL outcomes were modeled as a function of injury, patient characteristics, and family characteristics using longitudinal, multivariable regression.A considerable proportion of children had impaired HRQOL at 3 months (42% of children) and 12 months (40% of children) after injury. Multiple dimensions of HRQOL were negatively affected among children with moderate or severe TBI (decrease of 3.7 to 17.6) (P.05) and did not improve significantly over time. Concomitant lower extremity fractures and spinal injuries resulted in large declines in overall HRQOL, particularly at 3 months after injury (decrease of 12.9 and 8.1, respectively) (P.05). The HRQOL scores were also reduced by preexisting psychosocial conditions (decrease of 2.9 to 12.3), impaired family functioning (decrease of 5.1 to 6.8), having Medicaid coverage or being uninsured (decrease of 3.1 to 5.5), and single-parent households (decrease of 3.2 to 3.4) (P.05).Moderate or severe TBI resulted in measurable declines in children's HRQOL after injury. Injury-related factors impacted HRQOL more compared with patient and family characteristics during the first year after injury.

Primitive neuroectodermal tumors of the kidney are rare neoplasms that may mimic renal cell carcinoma, especially in the presence of locally advanced or metastatic disease. Although several cases have been identified as primary renal tumors, this is the first report of metastatic spread of a primitive neuroectodermal tumor to the kidney.

Background/Purpose The purpose of this study was to determine the frequency of pectus excavatum and associated conditions in a large autopsy series. It also sought to determine whether there were different survival patterns for pectus excavatum patients than for patients without pectus excavatum. Methods A computer-assisted search of autopsy files maintained by Johns Hopkins University was conducted, dating from 1889 to 2001. Each patient's Autopsy Pathology Information System report was reviewed for diagnosis and comorbid conditions. To determine whether there were differences in survival patterns, we tested whether pectus excavatum patients survived longer than controls, using a standard epidemiological method. Each patient in the autopsy series was compared with the 2 patients entered in the autopsy database chronologically immediately before and the 2 patients immediately after the case. A Kaplan-Meier survival analysis was conducted. Results Pectus excavatum was identified at autopsy in 62 of 50,496 cases. Of these 62 patients, 17 were 65 years or older and appeared to have died of causes unrelated to pectus excavatum, the oldest being 91 years. Twenty-one were between the ages of 14 and 65 years and were found to have coexisting conditions or syndromes. Six were between the ages of 1 and 4 years. One of the 6 died in 1947 because of complications from pectus repair. No autopsied patient with pectus excavatum died between the ages of 5 and 14 years. Eighteen were infants younger than 1 year, and all 18 died because of conditions unrelated to pectus excavatum. There were no reported cases of pectus excavatum before 1947, and the severity of deformity could not be determined from the autopsy data. Survival analysis indicated that pectus excavatum patients had a different survival than the controls. Pectus excavatum patients tended to die earlier ( P = .0001). However, pectus excavatum patients who survived past the age of 56 years tended to survive longer than their matched controls ( P = .0001). Conclusion Although there were no histological abnormalities noted in the cartilage of the pectus excavatum patient's conditions, pectus excavatum was associated with several connective tissue abnormalities. Analysis is consistent with the theory that this condition can impact survival.

Background/Purpose There is currently no evidence-based screening instrument to assist in the detection of physical child abuse patients. The screening index for physical child abuse (SIPCA) was previously developed as a potentially new tool for this need. It is a scale that assigns point values, on the basis of variable weights from logistic regression models, to age and patterns of injuries (including fracture of base or vault of skull, contusion of eye, rib fracture, intracranial bleeding, multiple burns), with higher scores indicating greater suspicion for abuse. The purpose of this study is to validate this new tool in another independent data set. Methods A cross-sectional hospital discharge database from 1961 hospitals in 17 states is used (n = 58558). Children aged 14 years or younger with International Classification of Diseases, Ninth Revision, Clinical Modification codes 800 to 959 are included for analysis. Child abuse cases are identified by E codes and certain International Classification of Diseases, Ninth Revision, Clinical Modification codes in the 995.5x range. Screening index for physical child abuse performance is evaluated by discrimination (receiver operating characteristic) and goodness of fit (pseudo r 2 ). Results A total of 447 abused patients (0.76%) was identified. The receiver operating characteristic of SIPCA in this data set is 0.89 as compared with 0.86 in the development data set. The pseudo r 2 of SIPCA in this data set is 0.26 as compared with 0.28 in the development data set. A SIPCA score of 3 has a sensitivity of 86.6% and a specificity of 80.5% for detecting physical abuse; raising the threshold to a score of 4 improves the specificity to 93.1% but at a loss of sensitivity to 71.8%. Conclusions The validity of the new SIPCA instrument is supported by its performance in an independently derived data set. A score of 3 on SIPCA represents a balanced trade off in the sensitivity and specificity of the instrument in detecting physical abuse and is an optimal threshold above which to begin considering abuse in differential diagnosis. Application of the instrument could assist clinicians in detecting physical child abuse cases among pediatric trauma patients.

Purpose To better understand outcomes in children with rhabdomyosarcoma (RMS) and lung-only metastatic disease, the authors reviewed the experience from Intergroup Rhabdomyosarcoma Studies IV Pilot and IV. Methods Patients with lung-only (n = 46) vs other sites of metastatic disease (n = 234) were reviewed using patient charts and the database of Children's Oncology Group (COG). Results Sixteen percent of patients with RMS and metastatic disease had isolated lung metastases. Thirty-one (67%) had more than 5 metastatic lung lesions. These were bilateral in 34 (74%). Only 6 patients were biopsied at diagnosis. Sixteen children (35%) did not receive any lung radiotherapy. Patients that received lung radiotherapy had fewer lung recurrences ( P = .04), although this has no significant impact on overall survival (OAS, 47% radiotherapy vs 31% no radiotherapy). Compared with patients with other sites of metastatic disease, patients with lung-only metastases have a greater proportion of favorable histology (67% vs 39%, P = .0017), negative nodal involvement (67% vs 32%, P = .0013), and parameningeal primaries (39% vs 12%) and a smaller proportion of extremity primaries (20% vs 33%, P = .0005 for site of primary tumor). Overall survival at 4 years for lung-only metastases was not significantly different from other single-site metastasis (42% vs 34%). Survival was not improved for unilateral disease or fewer than 5 metastatic lesions. Factors associated with diminished OAS include unfavorable histology ( P = .0001) and age >10 years ( P = .015). Conclusions Children with RMS and lung-only metastases usually present with extensive bilateral disease that is frequently not biopsied nor given protocol-recommended radiotherapy (XRT). However, outcome is comparable, although slightly better, than patients with other single-site metastasis.

The incidence of child abuse is approximately 10% of all children presenting to an emergency department (ED), with a mortality rate less than 1%. By contrast, the characteristics of the subset of abused children presenting to a pediatric trauma service (PTS) is not well defined.This study was a retrospective evaluation of prospectively collected information from an urban Level I pediatric trauma registry from 1990 to 2002 (n = 11,919). Child abuse cases and their perpetrators were identified by E-codes. Patterns of injuries were examined by integer International Classification of Diseases, Ninth Revision codes, and diagnostic model was evaluated by discrimination and goodness-of-fit.A total of 171 cases of child abuse (1.4%) were identified, and the majority were boys (59%, p0.05 vs. nonabuse cases). The median age of the abused cohort was younger than 1 year old, and the number of abuse cases did not differ over time (mean, 11 per year.) Abused children present with a higher median Injury Severity Score (10 vs. 4, p0.01), more severe injuries of the head and integument, longer hospital lengths of stay (4 vs. 1 day, p0.01), and a higher mortality rate (12% vs. 2%, p0.01). The following variables emerged with significant association to abuse: fracture of base or vault of skull, contusion of eye, rib fracture, intracranial bleeding, multiple burns, and age. A new Diagnostic Index for Physical Child Abuse was created.Significant characteristics of the abused children in this pediatric trauma service include higher Injury Severity Score (especially in the head and integument), requirement for longer lengths of stay, and a nearly 10-times higher risk of death compared with the ED population. The Diagnostic Index for Physical Child Abuse is proposed as a new tool to assist in the identification of child abuse among pediatric trauma patients. An epidemiologic triangle for child abuse is described, with different prevalence and severity of child abuse seen at different levels of our health care system, starting with primary care providers, followed by the ED, the PTS, and ultimately the medical examiners. The number of cases decreases from the bottom to the top of the health care system, but the mortality rate increases as abuse escalates through the triangle. This establishes the PTS as possibly the final gatekeeper before an abused case becomes a fatality. These data emphasize the need for rigorous registry evaluation and subsequent evidence-based prevention initiatives.