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.

Focal nodular hyperplasia of the liver in longterm survivors of neuroblastoma: how much diagnostic imaging is necessary?

Objectives: Focal nodular hyperplasia of the liver is a tumor-like lesion, uncommon in children, but it has recently been more frequently observed in children treated for malignant diseases, especially neuroblastoma. The aetiology is unclear, the pathogenesis remains controversial. Focal nodular hyperplasia of the liver is suspected to be a sequela of tumor therapy.

Localized infant neuroblastomas often show spontaneous regression: results of the prospective trials NB95-S and NB97.

Purpose: The excellent prognosis of localized neuroblastoma in infants, the overdiagnosis observed in neuroblastoma screening studies, and several case reports of regression of localized neuroblastoma prompted us to initiate a prospective cooperative trial on observation of localized neuroblastoma without cytotoxic treatment.

Patients And Methods: For infants with localized neuroblastoma without MYCN amplification, chemotherapy was scheduled only in cases with threatening symptoms; otherwise, the tumor was either resected or observed by ultrasound and magnetic resonance imaging (MRI).

Results: Of 340 eligible participants, 190 underwent resection, 57 were treated with chemotherapy, and 93 were observed with gross residual tumor.

Review of image defined risk factors in localized neuroblastoma patients: Results of the GPOH NB97 trial.

Background: Recently, an international expert group proposed revision of the International Neuroblastoma Staging System (INSS). Localized disease can be classified as L1 without and as L2 with image defined risk factors (IDRF published in JCO 2005; 23:8483-8489). Our aim was to evaluate IDRF for the prediction of resectability, complications, and outcome.

True thymic hyperplasia associated with severe thymic cyst bleeding in a newborn: case report and review of the literature.

We report on a 5-week-old male infant with recurrent respiratory distress since birth and congenital thymic hyperplasia. Acute life-threatening thymic bleeding apparently from ruptured thymic cysts into the pleural spaces complicated the clinical situation. Thoracotomy and complete thymectomy were performed.

Prospective volumetric analysis of gallbladders in critically ill newborns: the impact of nutrition.

Background: Neonatal reference values of gallbladder size have been assessed in healthy newborns with enteral feeding regimen. Their applicability to critically ill patients under total parenteral nutrition (TPN) remains to be questioned.

Objective: Our aim was to evaluate the impact of short-term TPN versus enteral nutrition (EN), gender and birth weight on neonatal gallbladder volume.

Prospective sonographic evaluation of fentanyl side effects on the neonatal gallbladder.

Objective: In an effort to minimise the stress and pain of mechanically ventilated neonates, the application of opioids has increased markedly. Abdominal adverse effects of opioid analgesics are constipation and increased pressure in the biliary system. Our aim was to evaluate the impact of continuous intravenous infusion of fentanyl on the volume of the neonatal gallbladder and to assess potential gastrointestinal side effects.

Isolated femoral hypoplasia: an intrauterine differential diagnosis to campomelia.

The isolated form of femoral bowing is an important differential diagnosis of campomelia. Therefore, knowledge of isolated anomalies is fundamental for prenatal diagnosis, especially for the differential diagnosis from severe syndromes. Four cases are presented to discuss the differential diagnosis of femoral bowing including a review of the literature.

[Congenital lobar emphysema - a radiological diagnosis at first glance?].

Congenital lobar emphysema (CLE) is a malformation of the lung of unknown etiology. The characteristic overdistension of the affected lobe leads to compression and displacement of adjacent normal lung tissue and mediastinum. In the majority of cases, symptoms appear during the neonatal period or in early childhood.

Proton spectroscopic metabolite signal relaxation times in preterm infants: a prerequisite for quantitative spectroscopy in infant brain.

Purpose: To determine relaxation times of metabolite signals in proton magnetic resonance (MR) spectra of immature brain, which allow a correction of relaxation that is necessary for a quantitative evaluation of spectra acquired with long TE. Proton MR spectra acquired with long TE allow a better definition of metabolites as N-acetyl aspartate (NAA) and lactate especially in children.

Materials And Methods: Relaxation times were determined in the basal ganglia of 84 prematurely born infants at a postconceptional age of 37.

Nephrocalcinosis in preterm infants: a single center experience.

The risk of nephrocalcinosis in preterm infants is considerable, but conflicting numbers are given for the actual incidence (10-65%). Furosemide induced hypercalciuria is said to be the main risk factor. We examined retrospectively the incidence, causes and outcome of nephrocalcinosis in preterm infants born in our hospital from 1988 to 1998 ( n=2190).

[Primary intracranial germinoma. Problems in determining the diagnosis exemplified by 4 cases].

Primary intracranial germinoma is a possible cause of "idiopathic" diabetes insipidus in childhood and adolescence. The histopathologic appearance can be similar to inflammation, and may delay the final diagnosis. Further diagnostic difficulties are demonstrated by the use of anamnestic and clinical data as well as diagnostic imaging findings in four children with histologically proven primary intracranial germinoma.

Myositis ossificans traumatica in young children: report of three cases and review of the literature.

Myositis ossificans traumatica (MOT) is a rare musculoskeletal disorder in young children. Clinical and imaging presentation in the early stage of disease makes it difficult to differentiate between infection and musculoskeletal neoplasms, particularly in the absence of a history of trauma. Three cases of MOT in children under the age of 10 years, two with inferential trauma, are presented and the findings on different imaging modalities are discussed with reference to the existing literature.

Posterior pituitary ectopia: another hint toward a genetic etiology.

We report the MR and clinical findings of two patients with growth hormone deficiency and posterior pituitary ectopia (PPE). Possible causes of PPE are discussed.

Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature.

Background: Nodular regenerative hyperplasia (NRH) of the liver is a multi-acinar regenerative nodular lesion in a non-cirrhotic liver. It is a rare entity, especially in children, and remains of unknown aetiology.

Objective: NRH is often seen in association with other diseases or drug intake.

Right sternoclavicular dislocation after traumatic delivery: a case report.

Sternoclavicular (SC) dislocation is an injury that is very rare in the newborn. Thus far there have been no reports describing this in neonates after a traumatic birth injury. This condition can be difficult to differentiate from epiphyseal separation, which occurs more often in older children.

MR cholangiography in children with autosomal recessive polycystic kidney disease.

Background: Magnetic resonance cholangiography (MRC) is a relatively new, non-invasive imaging technique of the biliary tree that has shown good correlation with endoscopic retrograde cholangiopancreatography. The liver manifestation of autosomal recessive polycystic kidney disease (ARPKD) is congenital hepatic fibrosis (CHF). CHF may be accompanied by Caroli's disease, which is characterised by a non-obstructive dilation of the intrahepatic bile ducts.

Cerebral complications in Schimke immuno-osseous dysplasia.

Unlabelled: Schimke immuno-osseous dysplasia is a multisystem disorder consisting of spondylo-epiphysial dysplasia, progressive renal insufficiency due to focal segmental glomerulosclerosis, and immunodeficiency. Cerebrovascular complications have only been described in five patients. Here we report a patient with prominent neurological symptoms most likely caused by transient ischaemic attacks.

Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta.

CT angiography, or the spiral CT technique, is a promising minimally invasive method of visualising the arterial vascular system and can be applied in children in whom ultrasound, MRI and/or angiography or cardiac catheterisation cannot be performed, or where an exact diagnosis cannot be made. CT angiography should be considered in special cases as a diagnostic alternative to MRI, ultrasound and angiography. As an example of the possibilities of CT angiography, a case is described in which hypoplasia of the descending aorta was diagnosed and a postoperatively encountered perigraft reaction was demonstrated.

Proton magnetic resonance spectroscopy reflects metabolic decompensation in maple syrup urine disease.

Using localized proton magnetic resonance spectroscopy (1H-MRS), accumulation of branched-chain amino acids (BCAA) and their corresponding 2-oxo acids (BCOA) could be non-invasively demonstrated in the brain of a 9-year-old girl suffering from classical maple syrup urine disease. During acute metabolic decompensation, the compounds caused a signal at a chemical shift of 0.9 ppm which was assigned by in vitro experiments.

Infradiaphragmatic bronchogenic cyst with high CT numbers in a boy with primitive neuroectodermal tumor.

Abdominal bronchogenic cysts are very rare with less than 20 published cases. We report the case of a retroperitoneal bronchogenic cyst in a 12-year-old boy, who initially presented with a primitive neuroectodermal tumor of the pelvis. Computed tomography (CT) showed a small, hyperdense nonenhancing mass adjacent to the right crus of the diaphragm.