Plea for a standardized imaging approach to disorders of sex development in neonates: consensus proposal from European Society of Paediatric Radiology task force.

This consensus article elaborated by the European Society for Paediatric Radiology task force on gastrointestinal and genitourinary imaging is intended to standardize the imaging approach in newborns with disorders of sex development. These newborns represent a difficult and stressful situation necessitating a multidisciplinary team approach. Imaging plays an important role in the work-up but needs to be optimized and customized to the patient. Ultrasound plays the central role in assessing the genital anatomy. The examination must be conducted in a detailed and systematic way. It must include transabdominal and transperineal approaches with adapted high-resolution transducers. The pelvic cavity, the genital folds, the inguinal areas and the adrenals must be evaluated as well as the rest of the abdominal cavity. A reporting template is proposed. The indications of magnetic resonance imaging and cysto- and genitography are discussed as well as they may provide additional information. Imaging findings must be reported cautiously using neutral wording as much as possible.

Correction to: Plea for a standardized imaging approach to disorders of sex development in neonates: Consensus proposal from European Society of Paediatric Radiology task force.

The above article was published online with an incorrect author name.

European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

Very early onset inflammatory bowel disease (VEO-IBD) is defined as disease presenting before the age of 6. These children require a tailored imaging approach because conventional imaging studies can be difficult to perform at such a young age. Unlike inflammatory bowel disease in older children and adults, colonic disease predominates in VEO-IBD, and small-bowel disease is rare. Distinguishing Crohn disease from ulcerative colitis is challenging both clinically and on histology. Radiology offers the greatest utility for detecting small-bowel disease because it helps to distinguish the two main disease entities and guide clinical management. Small-bowel ultrasound is recommended as the first-line investigation because it requires relatively little preparation, is readily available and is generally well tolerated in young children. We present these recommendations, based on the current evidence for radiologic management in this group, and propose an imaging algorithm for investigating VEO-IBD.

Correction to: European Society of Paediatric Radiology abdominal imaging task force: statement on imaging in very early onset inflammatory bowel disease.

The above article was published online with incorrect author name. The right spelling should be Damjana Kljucevsek instead of Damjana Kjucevsek. The correct name is presented here.

Time-resolved functional 1H MR spectroscopic detection of glutamate concentration changes in the brain during acute heat pain stimulation.

Non-invasive in vivo detection of cortical neurotransmitter concentrations and their changes in the presence of pain may help to better understand the biochemical principles of pain processing in the brain. In the present study acute heat pain related changes of the excitatory neurotransmitter glutamate were investigated in the anterior insular cortex of healthy volunteers by means of time-resolved functional proton magnetic resonance spectroscopy ((1)H-MRS). Dynamic metabolite changes were estimated with a temporal resolution of five seconds by triggering data acquisition to the time course of the cyclic stimulus application. An overall increase of glutamate concentration up to 18% relative to the reference non-stimulus condition was observed during the application of short pain stimuli.

Differential amygdala activation to negative and positive emotional pictures during an indirect task.

The role of the amygdala for the processing of valence and arousal is a matter of debate. Using event-related functional magnetic resonance imaging, we tested valence-specific amygdala effects during attentional distraction. Subjects attended to a matching task in the foreground of neutral pictures, and of negative and positive pictures matched for arousal. Negative pictures elicited stronger amygdala activation than neutral or positive pictures, suggesting valence-specific amygdala responses under attentional load.

Brain activation to phobia-related words in phobic subjects.

Behavioural studies suggest that phobic subjects are hypersensitive in the processing of phobia-related linguistic stimuli. We used functional magnetic resonance imaging (fMRI) to investigate blood oxygen level dependent (BOLD) brain activation to phobia-relevant words in spider phobic and non-phobic subjects. Phobia-related versus phobia-unrelated words elicited increased activation in prefrontal cortex, insula, and posterior cingulate cortex in spider phobics, while these effects were absent in controls. Furthermore, between-group comparisons confirmed that differential activations within these brain regions were specifically due to increased responses to phobia-related stimuli in phobics. Our results provide first insights into brain activation patterns when phobics are confronted with phobia-specific linguistic information und suggest a neural network for the processing of these threatening stimuli.

MR Imaging in Wegener granulomatosis of the spinal cord.

Wegener granulomatosis is a multisystemic disorder characterized by necrotizing vasculitis that primarily involves the respiratory tract. The orbits, heart, skin, joints, and nervous system are frequently involved. We describe the MR imaging findings of Wegener granulomatosis in the cervical spine and correlate them with the histopathologic features. MR imaging showed epidural liquid masses surrounded by granulomatous masses that compressed the cervical myelin from behind.

Early diagnosis of cerebral involvement in Sturge-Weber syndrome using high-resolution BOLD MR venography.

Sturge-Weber syndrome (SWS) is a congenital disorder characterized by a vascular birthmark and neurological abnormalities. Typical imaging findings using MRI or CT are superficial cerebral calcification, atrophy and leptomeningeal enhancement. We present a neonate diagnosed with SWS because of a port-wine stain. In the absence of neurological symptoms the first MRI was performed when he was 4 months old, and follow-up MRI studies were performed after his first seizure at the age of 12 months. MRI was performed using standard sequences before and after administration of IV gadolinium. A high-resolution T2*-weighted, rf-spoiled 3D gradient-echo sequence with first-order flow compensation in all three directions was used for additional venographic imaging [blood-oxygen-level-dependent (BOLD) venography]. The initial conventional MRI sequences did not demonstrate any abnormality, but BOLD venography identified leptomeningeal internal veins. Follow-up MRI after the first onset of seizures demonstrated strong leptomeningeal enhancement, while BOLD venography revealed pathological medullary and subependymal veins as well as deep venous structures. At this time there were the first signs of atrophy and CT showed marginal calcifications. This report demonstrates that high-resolution BOLD MR venography allows early diagnosis of venous anomalies in SWS, making early therapeutic intervention possible.

Pediatric brain MRI in neurofibromatosis type I.

Neurofibromatosis (NF) is the most common of the phakomatoses, with a prevalence of 1 in 3-4,000. Many organ systems can be affected. In addition to multiple peripheral neurofibromas, NF I predisposed to CNS tumors including optic glioma, astrocytoma and plexiform neurofibroma. The purpose of this pictorial review is to illustrate characteristic brain MR imaging lesions in children with NF I and to give some recommendations about diagnostic imaging procedures in children suffering from NF I. Typical findings in brain MRI are hyperintense lesion on T2-weighted images, so-called unknown bright objects, which may be useful as an additional imaging criterion for NF I. Contrast administration is necessary in MR studies to maximize tumor detection and characterization, to add confidence to the diagnosis of benign probable myelin vacuolization, and to document stability of neoplasm on follow-up examinations. We recommend to perform serial MR imaging in children every 12 months. The frequency of follow-up in children with known brain tumors will vary with the tumor grade, biological activity and treatment.

Evaluation of bone-mineral density by digital X-ray radiogrammetry (DXR) in pediatric renal transplant recipients.

BACKGROUND: Loss of bone mass and increased fracture risk are known complications after renal transplantation in adults. Risk factors include donor source, dialysis status prior to transplantation, aetiology of renal disease, transplant rejection and drug therapy, particularly steroids. OBJECTIVE: In this preliminary study of quantification of bone loss in children after renal transplantion, we evaluated the applicability of digital X-ray radiogrammetry (DXR) of hand radiographs to estimate cortical bone mineral density (DXR-BMD). MATERIALS AND METHODS: A total of 23 renal transplant recipients (9 girls, 14 boys; age 6.5-20 years, median 16.3 years) underwent DXR measurements for calculation of DXR-BMD and metacarpal index (DXR-MCI) using radiographs of the non-dominant left hand. The duration between transplantation and the DXR evaluation, the duration of dialysis and medication were considered. The results were compared to a local age-matched and gender-matched reference data base. RESULTS: Our study revealed a significant decrease in bone mineral density compared to an age-matched and sex-matched normal population (P<0.05). In three patients the DXR-BMD was reduced more than -2.5 SD. In 12 patients the DXR-BMD was between -1 and -2.5 SD, and in 7 patients the DXR-BMD was in the normal range. In one patient, evaluation was not possible. Fractures were documented in three patients following transplantation. Reduced DXR-BMD was not significantly associated with immunosuppressive therapy or the duration of dialysis, and there was no significant correlation between DXR-BMD and the time between transplantation and DXR evaluation. CONCLUSIONS: Paediatric renal transplant patients show reduced DXR-BMD. In this preliminary study we demonstrated that DXR-BMD seems to be a reliable technique for quantification of demineralisation following renal transplantation in children.

Diffusion tensor imaging in children and adolescents with tuberous sclerosis.

BACKGROUND: Tuberous sclerosis (TS) is characterised by benign hamartomatous lesions in many organs. Diffusion tensor imaging (DTI) can detect microstructural changes in pathological processes. OBJECTIVE: To determine apparent diffusion coefficient (ADC) and fractional anisotropy (FA) maps in children with TS and to investigate the diffusion properties in cortical tubers, white-matter lesions, perilesional white matter, and contralateral normal-appearing white matter, and to compare the results with ADC and FA maps of normal age- and sex-matched volunteers. MATERIALS AND METHODS: Seven children and adolescents (age range 2-20 years) suffering from TS were included. MRI was performed on a 1.5-T scanner using a transmit/receive coil with T1-W and T2-W spin-echo and FLAIR sequences. DT images were acquired by using a single-shot echo-planar pulse sequence. Diffusion gradients were applied in six different directions with a b value of 1,000 s/mm(2). RESULTS: ADC was higher in cortical tubers than in the corresponding cortical location of controls. ADC values were higher and FA values were lower in white-matter lesions and perilesional white matter than in both the contralateral normal-appearing white matter of patients and in controls. There were no significant differences for both ADC and FA values in the normal-appearing white matter of patients with TS compared to controls. CONCLUSIONS: DTI provides important information about cortical tubers, white-matter abnormalities, and perilesional white matter in patients with TS.

Assessment of skeletal age at the wrist in children with a new ultrasound device.

BACKGROUND: Determination of skeletal development in children is important. The most common method of evaluation uses the standards of Greulich and Pyle (G and P) to assess the left hand radiograph. Numerous assessments may be made during follow-up. OBJECTIVE: The aim of our study was to compare the accuracy of a new sonographic method with the standard radiographic method. MATERIALS AND METHODS: Seventy consecutive patients (age 6-17 years; 34 girls, 36 boys) underwent radiography of the left hand, followed by sonographic examination of the same hand using the BonAge system (Sunlight Medical Ltd., Israel). This system evaluates the relationship between the velocity of sound passing thorough the distal radial and ulna epiphysis and growth, using gender- and ethnicity-based algorithms. One experienced paediatric radiologist analysed the radiograph and assigned bone age scores based on the G and P atlas for the whole left hand and for the distal radius alone. The radiologist was blinded to the chronological age (CA), height of the patient and the BonAge result. Correlation between BonAge and G and P was undertaken. RESULTS: In 65 patients, BonAge measurement could be performed successfully. In five patients, the scanning process was impossible using the ultrasound device. The r(2) (r is the Pearson correlation coefficient) of the BonAge ultrasound measurement and the G and P method was 0.82. The averaged accuracy (i.e. absolute difference in years between G and P reading and BonAge ultrasonic results) was calculated. Results were similar for boys and girls: 1.0+/-0.8 years for the whole left hand and 0.8+/-0.7 year for the distal radius. On average, the difference between BonAge and CA is the same as the difference between G and P and CA, i.e. 1.4 years. CONCLUSIONS: The BonAge device demonstrates the ability of ultrasound to produce an accurate assessment of bone age. The results are highly correlated with skeletal age evaluated conventionally using the G and P method. Obvious advantages of the ultrasound device are objectivity, lack of ionizing radiation, and easy accessibility.

Comparison of whole-body STIR-MRI and 99mTc-methylene-diphosphonate scintigraphy in children with suspected multifocal bone lesions.

The study was performed to compare whole-body short time inversion recovery (STIR) MR imaging and (99m)Tc-methylene diphosphonate planar scintigraphy in the examination of children with suspected multifocal skeletal malignant lesions. Sixteen patients with known or suspected malignant skeletal disease underwent both whole-body STIR MR imaging and bone scintigraphy. The lesions were described and numbered according to scintigraphic evaluation criteria. Thus, 16 regions were analyzed in each patient for the comparison between the two modalities. Histology was proven in the primary malignant regions. Follow-up MRIs were registered. Scintigraphy and MRI follow-up were evaluated as gold standard. A total of 139 different lesions was observed by both modalities. Baseline whole-body MRI revealed 119 bone lesions in 256 possible sites (46.5%); scintigraphy revealed only 58 lesions (22.6%). Congruence was observed in only four patients (25%). According to the location of the lesion, correlation was observed in 39/139 lesions (28%). In all, 57.5% of the lesions were detected only by MRI and 14.5% of the lesions were detected only by scintigraphy. Whole-body MRI was more sensitive (P<0.001). Of all lesions numbered which could be separated in the initial MRI, whole-body MRI detected 178 lesions in the patients. The results suggest that whole-body MRI using a STIR sequence is an effective radiation free method for examination of children with suspected multifocal bone lesions. MRI showed more lesions than conventional (99m)Tc-methylene diphosphonate scintigraphy. Therefore, whole-body MRI may be feasible as a screening modality for metastatic and skip lesions in osteosarcoma, PNET, Ewing sarcoma and Langerhans cell histiocytosis in children.