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Background: Adamantinoma is a rare slow-growing malignant bone tumour. Objective: To describe the imaging appearances of six childhood cases and review the published literature. Materials and methods: The database of the Working Group on Paediatric Oncology, Academic Medical Centre/Emma Children's Hospital Amsterdam, was searched for cases of adamantinoma. Additionally a literature study was performed to identify cases of adamantinoma in childhood. Results: We identified six local cases of adamantinoma of the long bones, two boys (age 3 and 8 years) and four girls (mean age 8.8 years, range 3.0-14.0 years). The location of the tumour was the tibia in five and the tibia and fibula in one patient. In two patients initially a different diagnosis was made, which led to a delay in appropriate treatment. None of the children showed pulmonary metastases and all underwent total gross resection. On follow-up (mean 6.1 years, range 1.6-12.0 years) all children remained disease-free. Besides a discussion of our six patients, imaging features, histopathology, surgical approach and a literature review of childhood adamantinomas is presented. Conclusion: Although the incidence of adamantinoma is low, it is important to recognize this rare bone tumour, since in the early stages of the disease adequate treatment will result in an excellent prognosis. Keywords Bone * Tumour * Adamantinoma * Radiography * CT * MRI * Children

Cherubism is a paediatric disease affecting the jaws. It is a close radiological mimic of giant cell lesions of the mandible. The radiographic features of this condition are well known; however, the MR imaging features have not been well described. We describe the radiographic, CT and MR imaging features of cherubism in a 5-year-old girl. We highlight the MR appearances of cherubic lesions and the ability of MRI to not only accurately depict the anatomical extent of the lesions, but also to reveal signal intensity changes in those areas that are apparently normal on radiographs and CT images. Keywords Cherubism * Mandible * Radiography * CT * MRI * Child

We report a case of a 12-year-old girl with a mesenteric pseudoaneurysm and abdominal tuberculosis presenting with massive lower gastrointestinal hemorrhage requiring multiple blood transfusions. The pseudoaneurysm was treated successfully with transarterial microcoil embolization. Pseudoaneurysms are a rare and life-threatening complication of tuberculosis and understanding the imaging characteristics and treatment options will help to guide appropriate therapy. Keywords Tuberculosis * Lower GI bleeding * Mesenteric pseudoaneurysm * Embolization therapy

Background: Image-guided core needle biopsy is widely used in paediatric oncology, but many protocols continue to discourage this practice. No published randomized studies compare image-guided needle biopsy with surgical techniques. Objective: To perform a systematic review of the literature on image-guided core needle biopsy in paediatric oncology. Materials and methods: Several computerized databases were searched using the terms [(needle OR core) AND (biops*[ti]) AND (paediatric OR pediatric OR child OR children OR childhood OR boy OR girl)[ti]] to identify series of more than five cases of needle core biopsy for tumour diagnosis in children. Data from included studies were combined to calculate pooled estimates of adequacy, accuracy and complication rates. Results: Thirteen studies fulfilled the inclusion criteria. Overall biopsy adequacy rate (defined as sufficient to make a diagnosis) was 94% (95% CI 92-96%). The diagnostic accuracy rate in cases with adequate material (defined as achieving the correct specific diagnosis) was 94% (95% CI 92-96%). Complications requiring treatment occurred in 1%. Conclusions: Available pooled data suggest that about 95% of image-guided needle core biopsies provide an adequate sample for diagnosis of malignant disease in childhood. In such cases, the pathological diagnosis is correct in about 95%. Complications are rare. Keywords Needle biopsy * Tumour * Diagnosis * Histopathology * Children

Minimally invasive image-guided therapy for children, also known as pediatric interventional radiology (PIR), is a new and exciting field of medicine. Two key elements that helped the rapid evolution and dissemination of this specialty were the creation of devices appropriate for the pediatric population and the development of more cost-effective and minimally invasive techniques. Despite its clear advantages to children, many questions are raised regarding who should be performing these procedures. Unfortunately, this is a gray zone with no clear answer. Surgeons fear that interventional radiologists will take over additional aspects of the surgical/procedural spectrum. Interventional radiologists, on the other hand, struggle to avoid becoming highly specialized technicians rather than physicians who are responsible for complete care of their patients. In this article, we briefly discuss some of the current aspects of minimally invasive image-guided therapy in children and innovations that are expected to be incorporated into clinical practice in the near future. Then, we approach the current interspecialty battles over the control of this field and suggest some solutions to these issues. Finally, we propose the development of a generation of physicians with both surgical and imaging skills. Keywords Pediatric * Interventional therapy * Surgery * Vascular * Resident education/training

Background: Germinoma is the most common and least malignant intracranial germ cell tumor, usually found in the midline. Germinoma that arises in the basal ganglia, called ectopic germinoma, is a rare and well-documented entity representing 5% to 10% of all intracranial germinomas. The association of cerebral and/or brain stem atrophy with basal ganglia germinoma on CT and MRI is found in 33% of the cases. Objective: To review the literature and describe the CT and MRI findings of basal ganglia germinoma in children, known as ectopic germinoma, with associated ipsilateral cerebral and brain stem hemiatrophy. Materials and methods: Three brain CT and six brain MRI studies performed in four children at two institutions were retrospectively reviewed. All patients were male (case 1, 14 years; case 2, 13 years; case 3, 9 years; case 4, 13 years), with pathologically proved germinoma arising in the basal ganglia, and associated ipsilateral cerebral and/or brain stem hemiatrophy on the first imaging study. It is important to note that three of these children presented with cognitive decline, psychosis and slowly progressive hemiparesis as their indication for imaging. Results: Imaging results on initial scans were varied. In all patients, the initial study showed ipsilateral cerebral and/or brain stem hemiatrophy, representing Wallerian degeneration. All patients who underwent CT imaging presented with a hyperdense or calcified lesion in the basal ganglia on unenhanced scans. Only one of these lesions had mass effect on the surrounding structures. In one of these patients a large, complex, heterogeneous mass appeared 15 months later. Initial MR showed focal or diffusely increased T2 signal in two cases and heterogeneous signal in the other two. Conclusion: The association of a focal lesion in the basal ganglia of children with progressive hemiparesis, neuropsychiatric symptoms and ipsilateral cerebral and/or brain stem hemiatrophy should prompt the diagnosis of ectopic germinoma, avoiding delay in the diagnosis and further irreversible clinical deterioration, in a malignancy with an otherwise favorable prognosis. In these patients, hemiatrophy preceding or accompanying the imaging depiction of a basal ganglia mass lesion is thought to be caused by a paraneoplastic process. Keywords Ectopic germinoma * Basal ganglia * Cerebral and brain stem atrophy * CT * MR

Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. Keywords Hearing loss * Temporal bone

Imaging techniques are indispensable for diagnosis and follow-up of paediatric pulmonary diseases. In the past, interest was focused on morphological aspects of pulmonary tissue. With the development of novel CT and MRI techniques, functional pulmonary imaging became available. In this review, the new techniques of cross-sectional functional imaging of the lung are presented and the value of these methods for investigating paediatric pulmonary diseases and their potential clinical applications are discussed. Keywords Lungs * Function * Ventilation * Multidetector CT * MRI * Children

We present the radiological and clinical aspects of a patient with advanced-stage Burkitt lymphoma who presented with an acute abdomen complicated by abdominal compartment syndrome. Keywords Abdominal compartment syndrome * Burkitt lymphoma * CT

Pulmonary complications remain the main cause of mortality in cystic fibrosis, but the presenting symptoms in children are often related to gastrointestinal or pancreaticobiliary disease. Furthermore, abdominal manifestations are now seen throughout childhood, from infancy to adolescence. The child might present in the neonatal period with meconium ileus or its attendant complications. The older child might present with distal intestinal obstruction syndrome or colonic stricture secondary to high doses of pancreatic enzyme replacement. Less-common gastrointestinal manifestations include intussusception, duodenitis and fecal impaction of the appendix. Most children also show evidence of exocrine pancreatic deficiency. Radiologically, the combination of fat deposition and pancreatic fibrosis leads to varying CTand MR appearances. A higher than normal incidence of pancreatic cysts and calcification is also seen. Decreased transport of water and chloride also increases the viscosity of bile, with subsequent obstruction of the biliary ductules. If extensive, this can progress to obstructive cirrhosis, portal hypertension and esophageal varices. Diffuse fatty infiltration, hypersplenism and gallstones are also commonly seen in these patients. We present a pictorial review of the radiological appearance of these abdominal manifestations. The conditions are dealt with individually, together with typical appearances in various imaging modalities. Keywords Cystic fibrosis * Abdominal imaging

The combination of thoracic aortic pseudoaneurysm and left ventricular aneurysm resulting from a single traumatic incident is an exceedingly rare occurrence. We present a 10-year-old girl who sustained significant blunt trauma to the chest after being involved in a rollover motor vehicle accident. The child underwent immediate repair of a transected aortic arch. An inferior wall left ventricular aneurysm developed 3 weeks later, and the patient underwent successful repair of the left ventricular aneurysm and a damaged mitral valve. The use of fast multidetector row CT, cardiac MRI, and echocardiography have improved our ability to diagnose these types of injuries and accurately image their anatomic relationships in the acute and perioperative settings. Keywords Aortic pseudoaneurysm * Ventricular aneurysm * Trauma

Idiopathic infantile arterial calcification (IIAC) is a rare condition characterized by extensive calcification and stenosis of large and medium-size arteries. The etiology of the disease is unknown. However, the inheritance pattern has been shown to be autosomal recessive. The clinical presentation is variable, including cardiac failure (most common clinical finding), hypertension, and respiratory failure. Plain radiography, sonography and MRI can aid in the diagnosis. We present a case in which contrast-enhanced MR angiography with breath-hold and cardiac gating techniques allowed complete evaluation of the extent of this disease. Keywords Idiopathic infantile arterial calcification * MRA * Diphosphonate

Hepatoblastoma is the most common malignant liver tumour of childhood. Accurate radiological staging is very important, especially in children who are treated according to the protocols of the International Childhood Liver Tumor Strategy Group (SIOPEL). These protocols use risk stratification, based almost entirely on imaging findings, to minimize the treatment for localized tumours and to intensify treatment for extensive tumours and those with extrahepatic spread. Keywords Liver * Tumour * Hepatoblastoma * MRI * Children

Background: Intracranial atypical teratoid/rhabdoid tumors (AT/RT) are rare and extremely aggressive neoplasms seen primarily in childhood. Imaging features are often considered non-specific. However, correct diagnosis of AT/RT is important because these tumors have a markedly different clinical prognosis and require more aggressive therapy. Objective: To determine the imaging features of AT/RT. Materials and methods: We retrospectively analyzed imaging findings in 11 patients with primary intracranial AT/RT presenting over a period of 5 years. CT (n=11), MR (n=7), clinical (n=11) and pathological (n=11) features were evaluated. FISH analysis showing monosomy of chromosome 22 (absence of bcr 22q11 locus) was available for three patients. Immunohistochemical staining for INI-1 (BAF47) was performed on all tumors. Results: There were 11 patients, 6 boys and 5 girls. The age of presentation varied from 1 month to 15 years (average age 3 years 8 months). Six tumors were located in the posterior fossa and five in the supratentorial compartment. The tumors showed a hyperdense solid component (64%) that showed moderate to marked enhancement with contrast medium. On MR imaging, the predominant signal pattern was isointensity on T1-weighted images (57%) and T2 shortening with heterogeneity on T2-weighted images (86%). All tumors were large in size (average 4.2 x 3.7 cm), and there was a tendency for calcification (36%), hemorrhage (46%), necrosis (46%) and perifocal edema (100%). There was also a high tendency for subarachnoid dissemination, with five patients (46%) demonstrating brain and/or spinal metastasis. At follow-up (n=7), six patients showed local recurrence. At the time of recurrence, all these patients showed extensive leptomeningeal spread of the disease in both intracranial and intraspinal compartments. Conclusion: There are no specific imaging features for intracranial AT/RT. But a high tendency toward large size, a hyperdense solid component on CT scan with calcification, hemorrhage, necrosis and subarachnoid spread suggest that this tumor should be considered in the differential diagnosis of large pediatric intracranial tumors. Keywords Brain * Neoplasms * Atypical teratoid/rhabdoid tumor

A variety of portal vein anomalies that occur in children can be identified by US, CT, MRI and portal venography. Although these abnormalities can also occur in adults, there are certain pathological processes and aberrations within the portal system that are specific to children. Knowledge of the embryology and anatomy of the portal vein is of benefit in the understanding of these anomalies. Identifying deviations from normal portal architecture is important in the work-up for surgery such as liver transplantation, and prior to interventional procedures such as stent placement or embolization. The aim of this paper is to summarize the various types of congenital and acquired portal vein abnormalities that occur in children, describe their radiological features and provide images to demonstrate the differences from normal portal venous anatomy. Keywords Portal vein anatomy * Congenital variations * Ultrasound * CT * MRI

Background: Accurate assessment of treatment response in children with intracranial pus collections is vital to guide appropriate therapy and reduce morbidity and mortality. Objective: To correlate serial MR-measurable changes in diffusion-weighted imaging (DWI) with clinical response to treatment. Materials and methods: We retrospectively reviewed clinical notes, conventional MR sequences and DWI in eight children with intracranial pus collections. Trace DWI signal intensity and apparent diffusion coefficient (ADC) values were compared at three time points: at initial diagnosis (eight children, 13 collections), at follow-up during continued clinical infection (three children, seven collections), and at follow-up when clinical infection had resolved (seven children, 12 collections). Results: At initial diagnosis all patients were septic and collections showed restricted diffusion (mean ADC 0.61[+ or -]0.15x[10.sup.-3][mm.sup.2]/s). Patients with persistent clinical sepsis at follow-up DWI had collections with persistent low ADC values (0.66[+ or -]0.21x10-3[mm.sup.2]/s), significantly (P Keywords Diffusion-weighted imaging * Abscess * Empyema * Children

Byline: Kristina Virkola (1), Maija Lappalainen (2), Leena Valanne (3), Marjaleena Koskiniemi (2) Abstract: A total of 44181 serum samples from 16733 pregnant women were analyzed for findings suggesting primary Toxoplasma infection. Thirty-seven newborns exposed to maternal primary Toxoplasma infection in utero were studied prospectively with ultrasound, CT, and MRI for signs of intrauterine infection. Their mothers had been treated during pregnancy, and all infants were treated. The children were assigned to three groups according to their mothers' serological status, and the radiological results were compared with the clinical outcome. Although radiological signs were scarce, ultrasound findings combined with maternal serology were found to be significantly related to clinical outcome. Author Affiliation: (1) Department of Radiology, Children's Hospital, University of Helsinki, Stenbackink 11, FIN-00290, Helsinki, Finland (2) Department of Virology, Haartman Institute, University of Helsinki, Helsinki, Finland (3) Department of Radiology, Meilahti Hospital, University of Helsinki, Helsinki, Finland Article History: Registration Date: 28/03/2009 Received Date: 19/10/1994 Accepted Date: 28/04/1996

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. Keywords Langerhans cell histiocytosis * Liver involvement * Children * Ultrasound * CT MRI*MRCP

A full-term male infant presented at birth with a hard swelling of the left knee. The lemon-sized lesion was fixed to the underlying knee muscles, while the overlying skin was stretched and shiny; there was no bruit. Radiography, sonography and MRI suggested a soft-tissue tumour. After surgical excision, histology showed the presence of fibrous and mesenchymal tissue, with mature adipose tissue. Fibrous hamartoma of infancy was diagnosed. Among soft-tissue tumours, fibrous hamartoma of infancy is a rare and benign lesion, occurring in the first 2 years of life. The tumour mainly affects the trunk, axilla, and upper extremities. This infant had unique involvement of the knee. The treatment of choice is local excision. Keywords Knee * Tumour * Fibrous hamartoma of infancy * Radiography * Ultrasound * MRI

We describe the ultrasonography, the contrast-enhanced computed tomography and the magnetic resonance imaging of subcutaneous fat necrosis in a newborn infant. Keywords Subcutaneous fat necrosis * Neonate * Magnetic resonance imaging * Ultrasonography * Computed tomography

Intraosseous haemangioma, an uncommon benign vascular tumour, is most commonly seen in adults and tends to involve the vertebrae and the skull. Lesions of flat bones are rare and the imaging findings in these patients are non-specific. We report a unique case of intraosseous haemangioma in the ilium of a 7-year-old girl studied by US, radiography, scintigraphy, CT and MRI. Keywords Pelvis * Ilium * Haemangioma * Radiography * CT * MRI * Child

We report a case of perforation, fistula formation, and small bowel obstruction in a 2-year-old child who had ingested 32 small magnets. Multiple magnets will attract one another through the bowel wall and lead to pressure necrosis with complications. We recommend early surgical intervention before the onset of gastrointestinal complications if ingested multiple magnets have not moved on the follow-up radiograph. Both clinicians and the lay population need to be aware that multiple magnets can be hazardous foreign bodies for children. Keywords Multiple magnet ingestion * Intestinal foreign body * Magnet ingestion * Small-bowel obstruction * Fistula perforation DOI 10.1007/s00247-005-0056-3