Breast Ultrasonography: Findings in Pediatric Patients.

Purpose To analyze referral indications, imaging findings and diagnoses in breast sonography in a division of pediatric radiology. Materials and Methods Breast ultrasound examinations of 270 patients were analyzed for referral reasons, imaging findings and final diagnoses (152 females, 118 males). The mean age of the patients was 8.8 years (range, 6 days-18 years). Each breast was examined systematically in two orthogonal probe orientations. Pathological findings were documented on two orthogonal imaging planes. Color Doppler ultrasonography was used additionally. Images and clinical data were reviewed in all cases. Results The most frequent referral reasons in female patients were breast enlargement (104 patients), palpable mass (24 patients) and mastodynia (23 patients). The most frequent diagnoses were normal gland tissue (101 patients), cysts (9 patients), augmented adipose tissue (7 patients) and hemangiomas (7 patients). The most frequent referral reasons in male patients were breast enlargement (106 patients), palpable mass (13 patients) and mastodynia (9 patients). The most frequent diagnoses were gland tissue (79 patients), augmented adipose tissue (24 patients) and cysts (10 patients). Only 2 malignant masses were diagnosed: A Burkitt lymphoma and a relapsed ALL. Conclusion Fear of breast cancer and permanent damage to the breast leads to low-threshold medical consultations and referrals. Sensitive handling is required especially in adolescent patients. Most disorders arise due to the variability of breast development. Ultrasound serves as a means to exclude significant diseases of the breast.

Permissive hypercapnia in extremely low birthweight infants (PHELBI): a randomised controlled multicentre trial.

BACKGROUND: Tolerating higher partial pressure of carbon dioxide (pCO2) in mechanically ventilated, extremely low birthweight infants might reduce ventilator-induced lung injury and bronchopulmonary dysplasia. We aimed to test the hypothesis that higher target ranges for pCO2 decrease the rate of bronchopulmonary dysplasia or death. METHODS: In this randomised multicentre trial, we recruited infants from 16 tertiary care perinatal centres in Germany with birthweight between 400 g and 1000 g and gestational age 23-28 weeks plus 6 days, who needed endotracheal intubation and mechanical ventilation within 24 h of birth. Infants were randomly assigned to either a high target or control group. The high target group aimed at pCO2 values of 55-65 mm Hg on postnatal days 1-3, 60-70 mm Hg on days 4-6, and 65-75 mm Hg on days 7-14, and the control target at pCO2 40-50 mmHg on days 1-3, 45-55 mm Hg on days 4-6, and 50-60 mm Hg on days 7-14. The primary outcome was death or moderate to severe bronchopulmonary dysplasia, defined as need for mechanical pressure support or supplemental oxygen at 36 weeks postmenstrual age. Cranial ultrasonograms were assessed centrally by a masked paediatric radiologist. This trial is registered with the ISRCTN registry, number ISRCTN56143743. RESULTS: Between March 1, 2008, and July 31, 2012, we recruited 362 patients of whom three dropped out, leaving 179 patients in the high target and 180 in the control group. The trial was stopped after an interim analysis (n=359). The rate of bronchopulmonary dysplasia or death in the high target group (65/179 [36%]) did not differ significantly from the control group (54/180 [30%]; p=0·18). Mortality was 25 (14%) in the high target group and 19 (11%; p=0·32) in the control group, grade 3-4 intraventricular haemorrhage was 26 (15%) and 21 (12%; p=0·30), and the rate of severe retinopathy recorded was 20 (11%) and 26 (14%; p=0·36). INTERPRETATION: Targeting a higher pCO2 did not decrease the rate of bronchopulmonary dysplasia or death in ventilated preterm infants. The rates of mortality, intraventricular haemorrhage, and retinopathy did not differ between groups. These results suggest that higher pCO2 targets than in the slightly hypercapnic control group do not confer increased benefits such as lung protection. FUNDING: Deutsche Forschungsgemeinschaft.

Measurement of real-time tissue elastography in a phantom model and comparison with transient elastography in pediatric patients with liver diseases.

PURPOSE: We aimed to determine the comparability of real-time tissue elastography (RTE) and transient elastography (TE) in pediatric patients with liver diseases. MATERIALS AND METHODS: RTE was performed on the Elasticity QA Phantom Model 049 (Computerized Imaging Reference Systems Company Inc., Norfolk, Virginia, USA), which has five areas with different levels of stiffness. RTE measurements of relative stiffness (MEAN [mean value of tissue elasticity], AREA [% of blue color-coded stiffer tissue]) in the phantom were compared with the phantom stiffness specified in kPa (measurement unit of TE). RTE and TE were performed on 147 pediatric patients with various liver diseases. A total of 109 measurements were valid. The participants had following diseases: metabolic liver disease (n=25), cystic fibrosis (n=20), hepatopathy of unknown origin (n=11), autoimmune hepatitis (n=12), Wilson's disease (n=11), and various liver parenchyma alterations (n=30). Correlations between RTE and TE measurements in the patients were calculated. In addition, RTE was performed on a control group (n=30), and the RTE values between the patient and control groups were compared. RESULTS: The RTE parameters showed good correlation in the phantom model with phantom stiffness (MEAN/kPa, r=-0.97; AREA/kPa, r=0.98). However, the correlation of RTE and TE was weak in the patient group (MEAN/kPa, r=-0.23; AREA/kPa, r=0.24). A significant difference was observed between the patient and control groups (MEAN, P = 5.32 e-7; AREA, P = 1.62 e-6). CONCLUSION: In the phantom model, RTE was correlated with kPa, confirming the presumed comparability of the methods. However, there was no direct correlation between RTE and TE in patients with defined liver diseases under real clinical conditions.

C1-esterase inhibitor deficiency in pediatric heart transplant recipients: incidence and findings on ultrasound.

BACKGROUND: Acquired angioedema of the bowel caused by a deficiency of C1-esterase inhibitor can lead to severe abdominal pain with sudden onset, mimicking an acute surgical abdomen. In contrast to hereditary angioedema, which usually manifests in childhood, acquired angioedema is broadly recognized to affect people older than 40 years. OBJECTIVE: To determine the incidence of acquired angioedema in a cohort of pediatric heart transplant recipients and assess imaging findings on ultrasonography. MATERIALS AND METHODS: A cohort of 207 children and adolescents who had undergone heart transplantation were assessed at regular follow-up examinations for incidence of acquired angioedema. All patients received ACE inhibitors and immune inhibitors. Control examinations carried out in 3-month intervals included history, assessment of clinical symptoms, physical examination, US of the abdomen and laboratory blood analysis. In addition, if clinical symptoms were newly encountered, children were admitted between regularly scheduled intervals. We analyzed results of abdominal US for pathological findings of the bowel, and we assessed imaging findings in children diagnosed with acquired angioedema. RESULTS: Acquired angioedema was diagnosed in 3/207 patients (2 girls ages 16 and 17 years and 1 boy age 9 months). These children presented with sudden onset of severe abdominal pain. The bowel wall was thickened in the presence of ascites. After a mean of 72 h, abdominal pains resolved. Thickening of bowel loops dissolved and ascites disappeared. CONCLUSION: Single episodes of acquired angioedema were encountered in 1.4% of our series of pediatric heart transplant recipients. Radiologists should be familiar with this disease so they can diagnose it on US imaging.

A pilot study of the characterization of hepatic tissue strain in children with cystic-fibrosis-associated liver disease (CFLD) by acoustic radiation force impulse imaging.

BACKGROUND: Progressive fibrotic alterations of liver tissue represent a major complication in children with cystic fibrosis. Correct assessment of cystic-fibrosis-associated liver disease (CFLD) in clinical routine is a challenging issue. Sonographic elastography based on acoustic radiation force impulse imaging (ARFI) is a new noninvasive approach for quantitatively assessing in vivo elasticity of biological tissues in many organs. OBJECTIVE: To characterize ARFI elastography as a diagnostic tool to assess alteration of liver tissue elasticity related to cystic fibrosis in children. MATERIALS AND METHODS: ARFI elastography and B-mode US imaging were performed in 36 children with cystic fibrosis. The children's clinical history and laboratory parameters were documented. According to the findings on conventional US, children were assigned to distinct groups indicating severity of hepatic tissue alterations. The relationship between US findings and respective elastography values was assessed. Additionally, differences between ARFI elastography values of each US group were statistically tested. RESULTS: Children with sonomorphologic characteristics of fibrotic tissue remodeling presented significantly increased values for tissue elasticity. Children with normal B-mode US or discrete signs of hepatic tissue alterations showed a tendency toward increased tissue stiffness indicating early tissue remodeling. CONCLUSION: Assessment of children with CFLD by means of ARFI elastography yields adequate results when compared to conventional US. For detection of early stages of liver disease with mild fibrotic reactions of hepatic tissue, ARFI elastography might offer diagnostic advantages over conventional US. Thus, liver stiffness measured by means of elastography might represent a valuable biological parameter for evaluation and follow-up of CFLD.

Radiation protection in pediatric radiology.

BACKGROUND: The German Federal Law on Radiation Control contains no special provisions for X-ray studies in children and adolescents, even though exposure to ionizing radiation must be kept especially low in young persons, because their tissues are highly radiosensitive. Children, who have many years left to live, are more likely than adults to develop radiation-induced cancer; also, as future parents, they are at risk for passing on radiation-induced genetic defects to the next generation. Whenever possible, radiological studies on children and adolescents should be of a type that does not involve ionizing radiation, such as ultrasonography or magnetic resonance imaging. Pediatric conventional X-rays and computerized tomography (CT) require special examining techniques and protocols that are adapted to the patient's age and to the indication for the study. METHODS: We selectively review the literature on pediatric dose reduction and discuss our own investigations on the subject as well. RESULTS: The essential technical prerequisites for lowering the dose of ionizing radiation in conventional X-ray studies include the proper setting of tube voltage, the use of tube filters, suitable patient positioning and fixation, variable use of a scattered-radiation grid, and a modern storage-plate system. In CT studies, the use of age- and indication-adapted protocols can lower radiation exposure by as much as 95%. CONCLUSION: There are now many ways to lower the exposure of children and adolescents to ionizing radiation without sacrificing diagnostic reliability. The main factors in lowering exposure are proper attention to clinical indications, the use of special X-ray protocols, the use of alternative imaging studies without ionizing radiation wherever possible, and the expertise of the examiner.

[Reconstruction of stab wounds from preoperative CT data--diagnostic report of a clinical forensic examination]. / Rekonstruktion von Einstichwunden anhand präoperativer CT-Daten--Befunderhebung im Rahmen einer klinisch-rechtsmedizinischen Untersuchung.

A young man with multiple penetrating wounds was admitted to a hospital for emergency treatment after a knife attack. The following day, a clinical forensic examination to document and interpret the injuries was ordered. However, by then, only the changed postoperative condition of the injuries could be assessed. Photos, detailed descriptions, or sketches of the original wounds had not been made. By using CT data sets that had been acquired prior to treatment, it was possible to localize and reconstruct the injuries inflicted to the skin, soft tissues and bones. With the aid of the attending physician, the injuries could be classified as stab wounds.

Conversion of a neonatal hepatic hemangioma to focal nodular hyperplasia.

Hepatic hemangioma and focal nodular hyperplasia are both frequently observed benign lesions of the liver. Whereas hepatic hemangioma is the most frequent benign liver tumor in children, focal nodular hyperplasia occurs predominantly in adult patients. Concomitance of both entities has been described in adults, suggesting a similar pathogenesis. We report on a 6-month-old child with a continuously shrinking hepatic hemangioma after interventional therapy and a growing hepatic mass 5 years later, which emerged as focal nodular hyperplasia at the site of the former hemangioma. Diagnostic and therapeutic strategies regarding this patient are discussed. The present case supports the theory that these two entities may share a similar pathomechanism.

Ovarian tumor in a 12-year old female with severe hypothyroidism: A case of Van Wyk and Grumbach syndrome.

We report a 12-year-old female presenting with an abdominal tumor. Diagnostic workup revealed giant bilateral ovarian cysts, severe hypothyroidism as well as an elevation of CA 125. We refrained from ovariectomy, which would be necessary for a malignant tumor, in view of an evident Van Wyk and Grumbach syndrome. The patient promptly responded to L-thyroxine with complete regression of all symptoms. Hypothyroidism should be considered in the evaluation of ovarian cysts. Although the Van Wyk and Grumbach syndrome is rare, it is crucial to rule it out in order to avoid unnecessary ovarian surgery when thyroid replacement is completely sufficient.

Evidence for involvement of the vitamin D receptor gene in idiopathic short stature via a genome-wide linkage study and subsequent association studies.

Stature is a highly heritable trait under both polygenic and major gene control. We aimed to identify genetic regions linked to idiopathic short stature (ISS) in childhood, through a whole genome scan in 92 families each with two affected children with ISS, including constitutional delay of growth and puberty and familial short stature. Linkage analysis was performed for ISS, height and bone age retardation. Chromosome 12q11 showed significant evidence of linkage to ISS and height (maximum non-parametric multipoint LOD scores 3.18 and 2.31 at 55-58 cM, between D12S1301 and D12S1048), especially in sister-sister pairs (LOD score of 1.9 for ISS in 22 pairs). These traits were also linked to chromosomes 1q12 and 2q36. The region on chromosome 12q11 had previously shown significant linkage to adult stature in several genome scans and harbors the vitamin D receptor gene, which has been associated with variation in height. A single nucleotide polymorphism (SNP) (rs10735810, FokI), which leads to a functionally relevant alteration at the protein level, showed preferential transmission of the transcriptionally more active G-allele to affected children (P=0.04) and seems to be responsible for the observed linkage (P=0.05, GIST test). Bone age retardation showed moderate linkage to chromosomes 19p11-q11 and 7p14 (LOD scores 1.69 at 57 cM and 1.42 at 50 cM), but there was no clear overlap with linkage regions for stature. In conclusion, we identified significant linkage, which might be due to a functional SNP in the vitamin D receptor (VDR) gene and could be responsible for up to 34% of ISS cases in the population.

Children with idiopathic short stature are poor eaters and have decreased body mass index.

OBJECTIVE: In children with idiopathic short stature (ISS), studies investigating body mass index (BMI) or parameters of satiety regulation are scarce, and studies analyzing eating behavior are lacking. METHODS: We recruited 214 children (123 index cases and 91 siblings) with ISS from 123 families. Affected children had to have a body height <5th percentile, or, in the case of siblings, the body height of 1 child had to be <5th percentile and the other <15th percentile. Medical histories were recorded by using structured and standardized interviews. Eating behavior was assessed by using the Child Eating Behavior Questionnaire. Percent energy intake as fat was assessed by using the Leeds Food Frequency Questionnaire. Endocrine markers of body weight regulation (leptin, ghrelin) were determined in serum. RESULTS: Compared with population norms, BMI was significantly lower (mean: -0.33 standard deviation score). Furthermore, there was decreased food responsiveness (mean Child Eating Behavior Questionnaire score: 1.9; population mean: 2.4), reduced enjoyment of food (3.2 vs 3.9), emotional undereating (2.6 vs 3.0), lower desire to drink (2.0 vs 2.8), and increased fussiness over food (3.2 vs 2.9). When the sample was subdivided into the 2 groups of "good" and "poor" eaters according to the mothers' assessment of the current eating behavior, reduction in BMI as well as the behavioral characteristics already delineated in the total sample were found to be even more consistent in the subgroup of poor eaters. In the total sample of our children, as well as in both subgroups, serum leptin (adjusted for gender, BMI, and Tanner stage) was found to be moderately raised but did not differ between poor and good eaters. Total serum ghrelin was not different between poor and good eaters. CONCLUSIONS: Our clinical, behavioral, and endocrinologic findings in patients with ISS point to an altered eating behavior that possibly contributes to their short stature.

Combined external beam and intraluminal radiotherapy for irresectable Klatskin tumors.

BACKGROUND: In most cases of proximal cholangiocarcinoma, curative surgery is not possible. Radiotherapy can be used for palliative treatment. We report our experience with combined external beam and intraluminal radiotherapy of advanced Klatskin's tumors. PATIENTS AND METHODS: 30 patients were treated for extrahepatic proximal bile duct cancer. Our schedule consisted of external beam radiotherapy (median dose 30 Gy) and a high-dose-rate brachytherapy boost (median dose 40 Gy) delivered in four of five fractions, which could be applied completely in twelve of our patients. 15 patients in the brachytherapy and nine patients in the non-brachytherapy group received additional low-dose chemotherapy with 5-fluorouracil. RESULTS: The brachytherapy boost dose improved the effect of external beam radiotherapy by increasing survival from a median of 3.9 months in the non-brachytherapy group to 9.1 months in the brachytherapy group. The effect was obvious in patients receiving a brachytherapy dose above 30 Gy, and in those without jaundice at the beginning of radiotherapy (p < 0.05). CONCLUSIONS: The poor prognosis in patients with advanced Klatskin's tumors may be improved by combination therapy, with the role of brachytherapy and chemotherapy still to be defined. Our results suggest that patients without jaundice should be offered brachytherapy, and that a full dose of more than 30 Gy should be applied.