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Standard of care for unilateral nephroblastoma includes total nephrectomy (TN) with nodal sampling. We sought to compare the outcomes of TN and partial nephrectomy (PN). We performed a retrospective cohort study of TN and PN for nephroblastoma using the National Cancer Data Base. The outcomes included nodal sampling frequency, margin status, and survival. Categorical and continuous data were evaluated with χ2 and t tests, respectively ( P < 0.05). Generalized linear models evaluated nodal sampling and margin status. Cox regression compared survival. In total, 235 patients underwent PN and 3572 had TN. TN patients were 50 per cent more likely to undergo nodal sampling (RR: 1.47, 95% CI 1.30–1.66). There was no difference in margin status (RR: 0.91, 95% CI 0.65–1.28) or overall survival (HR 1.57; 95% CI 0.78–3.19). This study reports the largest review of patients with PN for unilateral nephroblastoma. PN patients had less nodal sampling but similar margin involvement and overall survival.

Pediatric breast malignancies are rare, and descriptions in the literature are limited. The purpose of our study was to compare pediatric and adult breast malignancy. We performed a retrospective cohort study using the National Cancer Data Base comparing patients ≤21 years to those >21 years at diagnosis (1998–2012). Generalized linear models estimated differences in demographic, tumor, and treatment characteristics. Cox regression was used to compare overall survival. Of 1,999,181 cases of invasive breast malignancies, 477 (0.02%) occurred in patients ≤21 years. Ninety-nine percent of adult patients had invasive carcinoma compared with 64.8% of pediatric patients with the remaining patients having sarcoma, malignant phyllodes, or malignancy not otherwise specified (p

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder of the Müllerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea. However, in some cases, such as the one we present here, menses are normal due to an obstructed hemivagina, and diagnosis can be delayed. We describe evaluation and surgical management of a 13-year-old girl with this condition who was diagnosed by computed tomography (CT) scan and confirmed by pelvic ultrasound and surgical exploration, as well as a review of the literature. A síndrome de Herlyn-Werner-Wunderlich (HWW) é uma doença congênita rara dos dutos müllerianos, em que há útero didelfo, hemivagina obstruída e agenesia renal unilateral. A apresentação clínica mais comum é como uma massa abdominal secundária a hematocolpo, dor e dismenorreia. Em alguns casos, porém, como no que apresentamos aqui, a menstruação é normal devido à obstrução de uma hemivagina, e o diagnóstico pode ser tardio. Descrevemos neste trabalho a avaliação e manejo cirúrgico de uma paciente de 13 anos com essa condição que foi diagnosticada com uso de tomografia computadorizada e confirmada por ultrassonografia pélvica e exploração cirúrgica, bem como uma revisão da literatura.

Background Though the volume-outcome relationship has been well-established in adults, low mortality rates and small sample sizes have precluded definitive demonstration in children. This study compares treatment-specific factors for children with nephroblastoma at high (HVC) versus low volume centers (LVC). Methods We performed a retrospective cohort study comparing patients ≤18years with unilateral nephroblastoma treated at HVCs and LVCs using the National Cancer Data Base (1998–2012). Definitions of HVCs included performing above the median, the upper two quartiles, and the highest decile of nephroblastoma resections. Outcomes included nodal sampling, margin status, time to chemotherapy and radiation, and survival. Statistical analyses included χ 2 , t-tests, generalized linear, and Cox regression models (p Results Of 2911 patients from 210 centers, 1443 (49.6%) were treated at HVCs. There was no difference in frequency of preoperative biopsy or days to radiation (p>0.05). High volume centers were more likely to perform nodal sampling (RR 1.04, 95%CI 1.01–1.08) and had fewer days to chemotherapy (RR 0.80, 95%CI 0.69–0.93). Five-year survival was similar (HVC: 0.93, 95%CI 0.92–0.94; LVC: 0.93, 95%CI 0.91–0.94). Conclusions HVCs were more likely to perform nodal sampling and had fewer days to chemotherapy. There was no difference in days to radiation or survival between centers. Level of evidence Level II (retrospective prognosis study).

Wheezing infants are frequently encountered in the emergency department. Bronchiolitis is the most commonly seen cause. Radiographs are not recommended in the routine management of bronchiolitis. We present the case of a young wheezing infant with a mildly abnormal chest x-ray whose cystic hygroma caused life-threatening respiratory distress soon after he was admitted to the hospital.

Sepsis is difficult to identify in patients treated with extracorporeal membrane oxygenation (ECMO). This study evaluates the usefulness of surveillance cultures obtained during ECMO. We retrospectively reviewed the records of 187 patients from four ECMO centers with birth weights 1,574 to 4,900 gm and gestational ages 33-43 weeks, over a 4 year interval. Most patients had surveillance blood cultures daily, and tracheal aspirates and urine culture every other day. Charts were reviewed for culture results before, during, and for the 7 days after ECMO, and clinical response to the culture results. A total of 2,423 cultures were obtained during 1,487 days of ECMO, of which 155 were positive (6.4%): 13 of 1,370 blood cultures (0.9%), 137 of 850 tracheal aspirate cultures (16%), and 5 of 203 urine cultures (2.3%). After 72 hours, tracheal aspirate cultures became positive with nosocomial organisms in 33 of 131 patients. None of 153 bacterial urine cultures were positive, and only one of 34 viral urine cultures were positive (CMV). We conclude that routine daily blood cultures are not useful in neonatal ECMO. Tracheal aspirate cultures may be helpful in the management of antibiotic therapy in patients on ECMO for more than 5 days. Routine bacterial urine cultures did not provide useful information.

Importance Melanoma in children is rare, accounting for approximately 2% of all pediatric malignant neoplasms. However, for the past 30 years, the incidence of melanoma in those younger than 20 years has been increasing. Location of the primary tumor has been shown to be an important prognostic factor, with melanomas of the scalp and neck conferring a worse prognosis than those originating at other sites. Objective To examine the survival, demographic, tumor, and treatment characteristics of pediatric head and neck melanoma. Design, Setting, and Participants We performed a retrospective cohort study using information from the National Cancer Data Base from January 1, 1998, to December 31, 2012, on pediatric (≤18 years) and adult (>18 years) patients with head and neck melanoma. Data analysis was conducted from August 1, 2015, to June 30, 2016. Exposure Pediatric age (≤18 years) at diagnosis of head and neck melanoma. Main Outcomes and Measures Survival differences were estimated using a Cox proportional hazards regression model. Surgical outcomes, including nodal sampling and margin status, were estimated with generalized linear models comparing pediatric and adult patients. Patient demographic, tumor, and treatment characteristics were estimated using t tests and χ 2 tests between pediatric and adult patients with head and neck melanoma for continuous and categorical data, respectively. Results Of the 84 744 patients with head and neck melanoma, 657 (0.8%) were 18 years or younger (mean [SD] age, 13.5 [4.7] years; 285 female and 372 male; 610 white). Pediatric and adult patients had similar demographics but different histologic subtypes (risk difference of pediatric vs adult patients: melanoma, not otherwise specified, 8.5% [95% CI, 4.7%-12.3%]; superficial spreading, 4.2% [95% CI, 0.89%-7.4%]; and lentigo maligna, –13.4% [95% CI, –14.1% to 12.6%]). Pediatric patients had tumors of similar mean depth to those in adult patients (pediatric, 1.54 mm; adult; 1.39 mm; absolute difference, 0.15 mm; [95% CI, –0.32 to 0.008]) and more frequent nodal metastases than did adult patients (risk difference of pediatric vs adult patients for stage T2, 23.9% [95% CI, 14.1%-33.6%]). Five-year survival among pediatric patients was higher for those with stage 1, 2, or 3 disease (absolute difference of pediatric vs adult patients: stage 1, 18% [95% CI, 9.7%-26.3%]; stage 2, 36% [95% CI, 25.3%-46.7%]; stage 3, 39% [95% CI, 26.8%-51.2%]; and stage 4, 2% [95% CI, –8.2% to 12.2%]). Conclusions and Relevance Although pediatric patients with head and neck melanoma present with similar tumor depth and more frequent nodal metastases than do adult patients, younger patients have higher overall survival.

Nasopharyngeal carcinoma (NPC) is endemic in some Asian regions but is uncommon in the United States. Little is known about the racial, demographic, and biological characteristics of the disease in pediatric patients.To improve understanding of the differences between pediatric and adult NPC and to determine whether race conferred a survival difference among pediatric patients with NPC.This retrospective cohort study included all 17 317 patients with a primary diagnosis of NCP in the National Cancer Data Base from January 1, 1998, to December 31, 2011. Of these, 699 patients were 21 years or younger (pediatric); 16 618 patients, older than 21 years (adult). Data were analyzed after data collection.Pediatric age at diagnosis of NPC.Demographic, tumor, and treatment characteristics of pediatric patients with NPC were compared with those of adults using the χ2 test for categorical variables. An adjusted Cox proportional hazards regression model was used to examine survival differences in pediatric patients relative to adult patients. In addition, the risk for pediatric mortality by race was estimated.Of the 17 317 patients, a total of 699 pediatric and 16 618 adult patients were identified with a primary diagnosis of NPC (female, 239 pediatric patients [34.2%] and 5153 adult patients [32.4%]). Pediatric patients were most commonly black (299 of 686 [43.6%]), whereas adults were most likely to be non-Hispanic white (9839 of 16 504 [60.0%]; P .001). Pediatric patients were less likely to be Asian (39 of 686 [5.7%]) than were adults (3226 of 16 405 [19.7%]; P .001). Pediatric patients were more likely to have regional nodal evaluation and to present with stage IV disease (227 of 643 [35.3%] and 330 of 565 [58.4%], respectively) than were adult patients (3748 of 15 631 [24.0%] and 6553 of 13 721 [47.8%], respectively; P .001 for both comparisons). Pediatric patients had a lower risk for mortality relative to adults (hazard ratio, 0.37; 95% CI, 0.25-0.56). No difference in mortality by racial group was found among pediatric patients (hazard ratio, 1.10; 95% CI, 0.82-1.40).Pediatric patients with NPC were more commonly black and presented more frequently with stage IV disease. Pediatric patients had a decreased mortality risk relative to adults, even after adjusting for covariables. Asian race was not associated with increased mortality in pediatric patients with NPC. Racial differences are not associated with an increased risk for mortality among pediatric patients.

Omental torsion is an underdiagnosed cause of abdominal pain in children. It resembles appendicitis, and the diagnosis is often made surgically. We review the presentation, treatment, and outcomes in the largest reported series to date. We recorded demographics, diagnostics, treatment, and histopathology in 18 children with omental torsion between May 2000 and 2007. We found a 4:1 male to female ratio and 85 per cent met criteria for obesity based on Centers for Disease Control and Prevention body mass index-for-age growth chart calculations. Fourteen of 18 (78%) presented with right lower quadrant pain concerning for appendicitis and seven of 18 (39%) with fever, nausea, or vomiting. Mild leukocytosis was found in 78 per cent of patients. All but one was taken to surgery with a diagnosis of appendicitis. Partial omentectomy, either open (50%) or laparoscopic (50%), was performed in all cases. The appendix, resected in 17 patients, was grossly normal. However, 30 per cent of specimens had histopathologic findings of appendicitis. Hospital discharge, after symptom resolution, averaged 33 hours. Surgeons should have a high index of suspicion for omental torsion when evaluating obese children for right lower quadrant pain. Both surgical approaches provide the diagnosis and treatment with minimal morbidity and rapid recovery. We advocate simultaneous appendectomy because appendicitis is often encountered with the torsion.

Background/Purpose: Intracranial hemorrhage (ICH) is a major concern during extracorporeal membrane oxygenation (ECMO). Daily cranial ultrasonography has been used by many ECMO centers as a diagnostic tool for both detecting and following ICH while infants are on bypass. The purpose of this patient review was to look at the usefulness of performing daily cranial ultrasonography (HUS) in infants on ECMO in detecting intraventricular hemorrhage of a magnitude sufficient to alter patient treatment. Methods: The authors reviewed retrospectively all of the records of all neonates treated with ECMO at the Hermann Children's Hospital, Wilford Hall USAF Medical Center, Cincinnati Children's Hospital, The University of Texas Medical Branch at Galveston, and Texas Children's Hospital between February 1986 to March 1995. Two hundred ninety-eight patients were placed on ECMO during this period. All patients had HUS before, and daily while on ECMO, and all were reviewed by the staff radiologists. A total of 2,518 HUS examinations were performed. Results: Fifty-two of 298 patients (17.5%) had an intraventricular hemorrhage seen on ultrasound scan. Nine of 52 patients (17.3%) had an ICH seen on the initial HUS examination before ECMO, all of which were grade I, and 43 of 52 patients (82.7$) had ICH while on ECMO. Of these ICH, 15 were grade I, 10 were grade II, 10 were grade III, and eight were grade IV. Forty of these ICH (93%) were diagnosed by HUS during the first 5 days of the ECMO course. Seven hundred eighty-six HUS were performed after day 5, at an estimated cost of $300,000 to $450,000 (charges), demonstrating three new intraventricular hemorrhages, one grade I, and one grade IV on day 7 and one grade I on day 8. Eight patients were taken off ECMO because of ICH diagnosed within the first 5 days. One patient was taken off ECMO because of ICH diagnosed after 5 days. This patient had clinical symptoms suggestive of ICH. Conclusions: Almost all ICH occur during the first 5 days of an ECMO course. Unless there is a clinical suspicion, it is not cost effective to perform HUS after the fifth day on ECMO, because subsequent HUS examinations are unlikely to yield information significant enough to alter management.