A rare malignant hepatic tumor of childhood: transitional liver cell tumor revisited.

Transitional liver cell tumor is an extremely rare entity and has a poor prognosis. It has similar histopathologic findings with hepatoblastoma and hepatocellular carcinoma. Up to now, only 10 cases have been reported in the literature. We report on an 8-year-old boy with histologically proven transitional liver cell tumor and describe the pertinent radiological findings.

Incidentally detected congenital giant left atrial appendage aneurysm in a child: MRI findings.

Left atrial appendage aneurysms are usually congenital and are very infrequent anomalies of the heart. They are very rarely diagnosed during childhood, with most cases symptomatic between the 2nd and 4th decades of life. Diagnosis is vitally important due to potential life-threatening complications. Surgical excision is the treatment of choice. Surgery reduces the risks of cardiac arrest, respiratory distress, arrhythmia, heart failure, thromboembolism, or rupture. We report the case of a 3-year-old boy with incidental diagnosis of a giant aneurysm of the left atrial appendage that was confirmed with magnetic resonance imaging and treated with surgery.

Radiological manifestations of abdominopelvic nerve tumours seen in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is the most common of the phakomatoses, which is inherited in an autosomal dominant pattern. Neurofibromas arise from Schwann cells and fibroblasts and plexiform neurofibromas are pathognomonic for NF1, which may arise in any peripheral nerve. The clinical expression of NF1 is extremely variable and gastrointestinal manifestations of NF1 are relatively uncommon. The purpose of this article is to illustrate the abdominal ultrasound, computed tomography, and magnetic resonance imaging findings of abdominopelvic nerve tumours seen in NF1 in both paediatric and adult patients.

Pulmonary agenesis and pulmonary sling anomaly in an infant with Down syndrome.

Pulmonary agenesis is a rare congenital anomaly presenting with normal karyotype in most of the cases. Rarely pulmonary agenesis is associated with chromosomal abnormalities and other genetic disorders such as Oculo-auriculo-vertebral spectrum, VACTERL association and velo-cardio-facial syndrome. This report presents a patient with pulmonary agenesis, pulmonary sling anomaly and Down syndrome.

A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery.

Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. He was treated with chemotherapy and extremity-sparing surgery. Amputation was avoided.

Non-invasive imaging of aberrant right subclavian artery pathologies and aberrant right vertebral artery.

Here, we review the CT and MR angiography findings of aberrant right subclavian and right vertebral arteries, with emphasis on the differences between these structures. In addition, non-invasive imaging findings of aberrant right subclavian artery pathologies, including arteritis, aneurysm and dissection, are discussed.

Visualization of the normal appendix in children by non-contrast MDCT.

OBJECTIVE: We studied the proportion of normal appendices, identified on non-contrast MDCT scans of the abdomen and pelvis in children with possible renal stones. MATERIALS AND METHODS: A total of 105 patients were included in the study, comprising 40 girls (38%) and 65 (62%) boys, with a mean age of 7.3 years. Non-enhanced abdominal computed tomographies were evaluated retrospectively, and the visualization, location, contents, diameter of the appendix, and the amount of abdominal fat were recorded. RESULTS: The appendix was clearly distinguished in 72 patients (68.5%). The difference in appendix visualization rates between patients with low and medium amounts of abdominal fat was statistically significant (p < 0.001). Visualization increased with age. The greatest external diameter was between 2.8 and 10 mm, with a mean of 5 +/- 1.34 mm. CONCLUSION: MDCT without contrast will be more useful when used in patients of 6 years old and over, for visualization of the appendix. A better visualization of retrocaecal appendix by MDCT provides a greater advantage over US. Prospective comparative studies will determine the role of this method in the diagnosis of acute appendicitis in pediatric patients.

Urinary epidermal and insulin-like growth factor excretion in autistic children.

Growth factors have been implicated in the pathogenesis of autism. We have investigated daily urinary excretion of insulin-like growth factor-1 (IGF-1), epidermal growth factor, and insulin-like growth factor binding protein-3 in autistic children (n=34, age 2-5 years) and age-matched control children (n=29). The mean urinary IGF-1 level was lower in the autism group than the control group (p=0.03). Height was normal. These findings suggest altered IGF-1 metabolism in young autistic children. The cause-effect relationship should be examined by longitudinal studies and insulin-like growth factor provocation tests.

MR angiography of left-sided cervical aortic arch with aberrant right subclavian artery.

Cervical aortic arch is a rare anomaly. We report the contrast-enhanced MR angiography features of left-sided cervical aortic arch with contralateral descending aorta and aberrant right subclavian artery in a 16-year-old girl.

Paediatric multidetector CT angiography: spectrum of congenital thoracic vascular anomalies.

Multidetector row CT (MDCT) is a non-invasive and rapid technique used for the evaluation of paediatric vascular diseases as an alternative to conventional angiography. Three-dimensional (3D) images allow excellent display of vascular anomalies that can be used as a vascular road map by surgeons. The aim of this pictorial review is to demonstrate diagnostic MDCT angiographic findings of various congenital thoracic vascular anomalies in paediatric patients. It is important to recognize these anomalies early for proper treatment and follow-up, and also to prevent morbidities and mortalities.

Bilateral hemorrhagic adrenal cysts in an incomplete form of Beckwith-Wiedemann syndrome: MRI and prenatal US findings.

Benign hemorrhagic adrenal cysts are a cause of subdiaphragmatic mass in the fetus and neonate with Beckwith-Wiedemann syndrome. Characteristic imaging features on ultrasonography, color Doppler, and magnetic resonance imaging help differentiate adrenal hemorrhage from neonatal neuroblastoma and help avoid unnecessary surgery in these patients. Bilateral adrenal hemorrhage is self-limiting, and spontaneous resolution is the usual outcome. This report presents this rare condition with prenatal ultrasonographic and magnetic resonance imaging findings and reviews the differential diagnosis of neonatal adrenal masses.

Pseudoaneurysm of the brachial artery in two infants following accidental arterial puncture.

A pseudoaneurysm is defined as an aneurysmatic sac surrounded byfibrous tissue instead of other vascular layers such as the muscular one. It is a rare incident in infants especially in the brachial artery. Blunt trauma and vascular access attempts are the most common etiologic factors. We present two infants with brachial artery pseudoaneurysm in the antecubital region following accidental arterial puncture.

Cellular congenital mesoblastic nephroma with contralateral medullary nephrocalcinosis.

Congenital mesoblastic nephroma is the most common renal mass in the newborn period and can present with atypical findings. Certain associated conditions such as hypercalcaemia, hypertension and reninism have been described. We report a cellular variant of congenital mesoblastic nephroma with hypercalcaemia and contralateral medullary nephrocalcinosis.

Inflammatory myofibroblastic tumour of the extrahepatic bile ducts: an unusual cause of obstructive jaundice in children.

Inflammatory myofibroblastic tumour is a rare entity in children with few reported series. The extrahepatic bile duct is an unusual location for this tumour. The authors report here the case of an 8-year-old girl presenting with obstructive jaundice due to inflammatory myofibroblastic tumour of the extrahepatic bile ducts with differential diagnosis of obstructive jaundice in children.

Pneumatosis intestinalis in an infant undergoing bone marrow transplantation for Wiskott-Aldrich syndrome.

A 7-month-old patient with Wiskott-Aldrich syndrome (WAS) developed pneumatosis intestinalis (PI) in the immediate post-transplant period after receiving paternal human leucocyte antigen (HLA) phenotypically matched bone marrow (BM). PI has been described in patients with congenital or acquired immunodeficiency states and after bone marrow transplantation (BMT). To our knowledge, the condition has not been described in WAS. The underlying bowel mucosa damage as a result of the history of massive rectal bleeding, the effects of the conditioning regimen, immunosuppression, neutropenia, and infection, may all have contributed to the development of PI. Although the condition resolved by conservative management alone, the patient developed Klebsiella pneumonia sepsis, interstitial pneumonitis, failed to engraft, and died on day +66 following a second infusion of stem cells mobilized from his father's peripheral blood.

Tumor volume or dynamic contrast-enhanced MRI for prediction of clinical outcome of Ewing sarcoma family of tumors.

BACKGROUND: The identification of risk factors that predict poor clinical outcome at the time of diagnosis could lead to intensified early therapy and improved outcome for pediatric patients with Ewing sarcoma family of tumors (ESFT). OBJECTIVE: To compare the effectiveness of static magnetic resonance (MR) imaging measurements of tumor volume with variables obtained by dynamic contrast-enhanced MR imaging (DEMRI) in predicting ESFT outcome. METHODS: MR examinations that included DEMRI were retrospectively reviewed. The analyses included 45 examinations of 21 patients with ESFT (performed from 1992 to 1996). Tumor volumes were measured on the static MR images, and the regions of interest were selected for DEMRI analysis. The relationships of static MR imaging and DEMRI variables with the probability of progression-free survival (PFS) and disease-free survival (DFS) were determined. RESULTS: Larger tumor volume at the time of diagnosis predicted poorer PFS and DFS estimates. No DEMRI variable predicted outcome. CONCLUSION: Determination of tumor volume by static MR imaging at the time of diagnosis is a simple and reliable method of predicting the clinical outcome of patients with ESFT. DEMRI is not as reliable a technique as static MR imaging for predicting the outcome of these patients.

Intrapancreatic duodenal duplication cyst with inversion of the superior mesenteric vessels: CT findings.

We present a case of intrapancreatic duodenal duplication cyst and inversion of the superior mesenteric vessels. CT findings of this association are discussed.

CT presentation of Wegener's granulomatosis in a child: rapidly progressive changes of pulmonary nodules to cavities.

We present a child with Wegener's granulomatosis who showed lung lesions progressing from nodules to cavities within a 1-month period on CT.