Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children.

AIM: To investigate the imaging and clinical findings of central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RTs) in children. MATERIALS AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical records of 16 children with CNS AT/RTs were retrospectively reviewed. Tumour location, size, composition, enhancement pattern, peritumoural oedema, signal intensity (SI) on MRI and CT attenuation were evaluated. RESULTS: A total of 17 lesions from 16 patients (median age 2.3 years, age range 0.7-15 years) were included in the evaluation. Tumour location was infratentorial for 11 lesions and supratentorial for six lesions. The mean diameter of the largest dimension for a tumour was 4 cm. The tumour was mainly solid in 65% of cases, and solid and cystic or cystic and solid in 35% of cases. The solid component of the tumours had a homogeneous iso SI (n=15) on T2-weighted MRI images and iso SI (n=14) on T1-weighted images. Moderate to strong enhancement of the solid component was noted in most cases. In spite of a large tumour size, peritumoural oedema was minimal or mild except in four cases. Rapid growth of the tumour was demonstrated in three cases. Seven patients died from tumour progression, with a mean survival time of 8.4 months (range 2-12 months). CONCLUSION: Although the AT/RTs had non-specific imaging findings, the tumours tended to be large in size, have iso SI on T1 and T2-weighted MR images with prominent enhancement, and relatively mild peritumoural oedema. Rapid growth of the tumour was seen during the follow-up period.

Limited Dorsal Myeloschisis and Congenital Dermal Sinus: Comparison of Clinical and MR Imaging Features.

BACKGROUND AND PURPOSE: While limited dorsal myeloschisis is a distinctive form of spinal dysraphism, it may be confused with congenital dermal sinus. The aim of this study was to describe clinical and MR imaging findings of limited dorsal myeloschisis that can distinguish it from congenital dermal sinus. MATERIALS AND METHODS: We retrospectively reviewed the clinical and MR imaging findings of 12 patients with limited dorsal myeloschisis and 10 patients with congenital dermal sinus. Skin abnormalities, neurologic deficits, and infectious complication were evaluated on the basis of clinical information. We evaluated the following MR imaging features: visibility of the tract along the intrathecal course, attachment site of the tract, level of the conus medullaris, shape of the spinal cord, and presence of intradural lesions such as dermoid/epidermoid tumors. RESULTS: A crater covered with pale epithelium was the most common skin lesion in limited dorsal myeloschisis (10/12, 83%). Infectious complications were common in congenital dermal sinus (6/10, 60%), whereas none were found in limited dorsal myeloschisis (P = .003). The following MR imaging findings were significantly different between the 2 groups (P < .05): 1) higher visibility of the intrathecal tract in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), 2) the tract attached to the cord in limited dorsal myeloschisis (12/12, 100%) versus various tract attachments in congenital dermal sinus, 3) dorsal tenting of the cord in limited dorsal myeloschisis (10/12, 83%) versus in congenital dermal sinus (1/10, 10%), and 4) the presence of dermoid/epidermoid tumors in congenital dermal sinus (6/10, 60%) versus none in limited dorsal myeloschisis. CONCLUSIONS: Limited dorsal myeloschisis has distinct MR imaging features: a visible intrathecal tract with dorsal tenting of the cord at the tract-cord union. Limited dorsal myeloschisis was not associated with infection and dermoid/epidermoid tumors.

MR imaging of the brain in Wilson disease of childhood: findings before and after treatment with clinical correlation.

BACKGROUND AND PURPOSE: Although brain MR imaging findings in adult Wilson disease have been described in considerable detail, a paucity of information currently exists regarding brain MR imaging findings in pediatric Wilson disease. The purpose of this study was to analyze the brain MR imaging findings in Wilson disease of childhood at the initial stage and during follow-up after treatment and to correlate these observations with clinical response. METHODS: We evaluated 50 patients with pediatric Wilson disease. Fifty initial and 20 follow-up MR images from 15 patients following penicillamine treatment were analyzed retrospectively, and the data were correlated with clinical findings. RESULTS: Patients were categorized into 3 groups on the basis of initial MR imaging findings. Group I (n = 23) showed normal MR imaging findings. Group II (n = 15) was characterized by T1-weighted images with increased signal intensity in the globus pallidus (n = 15, 100%) followed by the putamen, midbrain, and caudate nucleus. Group III (n = 12) demonstrated T2-weighted images with increased signal intensity in the putamen (n = 10, 83%), followed by the caudate nucleus, globus pallidus, thalamus, midbrain, and pons. There was a significant difference in mean age, the presence of neurologic symptoms, and Child-Pugh classification among the 3 groups (P < .001). Following copper chelating therapy, the changes on follow-up MR imaging were strongly correlated with clinical response to treatment (P < .001). CONCLUSION: Brain MR imaging in children with Wilson disease can be categorized into distinct groups and demonstrated a significant correlation with clinical findings. Interval changes on follow-up MR imaging were also closely correlated with clinical findings and helpful in assessing the clinical response.

Quantitative Assessment of Neovascularization after Indirect Bypass Surgery: Color-Coded Digital Subtraction Angiography in Pediatric Moyamoya Disease.

BACKGROUND AND PURPOSE: For the postoperative follow-up in pediatric patients with Moyamoya disease, it is essential to evaluate the degree of neovascularization status. Our aim was to quantitatively assess the neovascularization status after bypass surgery in pediatric Moyamoya disease by using color-coded digital subtraction angiography. MATERIALS AND METHODS: Time-attenuation intensity curves were generated at ROIs corresponding to surgical flap sites from color-coded DSA images of the common carotid artery, internal carotid artery, and external carotid artery angiograms obtained pre- and postoperatively in 32 children with Moyamoya disease. Time-to-peak and area under the curve values were obtained. Postoperative changes in adjusted time-to-peak (ΔTTP) and ratios of adjusted area under the curve changes (ΔAUC ratio) of common carotid artery, ICA, and external carotid artery angiograms were compared across clinical and angiographic outcome groups. To analyze diagnostic performance, we categorized clinical outcomes into favorable and unfavorable groups. RESULTS: The ΔTTP at the common carotid artery increased among clinical and angiographic outcomes, in that order, with significant differences (P = .003 and .005, respectively). The ΔAUC ratio at the common carotid artery and external carotid artery also increased, in that order, among clinical and angiographic outcomes with a significant difference (all, P = .000). The ΔAUC ratio of ICA showed no significant difference among clinical and angiographic outcomes (P = .418 and .424, respectively). The ΔTTP for the common carotid artery of >1.27 seconds and the ΔAUC ratio of >33.5% for the common carotid artery and 504% for the external carotid artery are revealed as optimal cutoff values between favorable and unfavorable groups. CONCLUSIONS: Postoperative changes in quantitative values obtained with color-coded DSA software showed a significant correlation with outcome scores and can be used as objective parameters for predicting the outcome in pediatric Moyamoya disease, with an additional cutoff value calculated through the receiver operating characteristic curve.

MR imaging of changes of the growth plate after partial physeal removal and fat graft interposition in rabbits.

RATIONALE AND OBJECTIVES: The most important complication of skeletal injuries involving the growth plate is growth disturbance. The purpose of this study was to evaluate MR features of growth plate modification after fat graft interposition in growth plate injury and to correlate these findings with pathological findings. METHODS: A growth plate injury model was used in 12 skeletally immature rabbits. A longitudinal drill hole 5 mm in diameter was created in the central part of the growth plate in the distal femur, bilaterally. One side was filled with autologous fat, and the contralateral defect was left empty as a control. Magnetic resonance imaging was obtained 1, 3, and 6 months after surgery, and routine histological study was performed. The authors evaluated sequential changes in MR images and the histological basis of MR findings. RESULTS: In grafted femur, the signal intensity of the grafted area was lower than that of the surrounding bone on T2-weighted images at 1 month. The growth plate defect at 3 to 6 months was modified and had a proximally tapering appearance. The ratio of the growth plate defect was smaller in the grafted femur than in the control femur after surgery. Histologically, the fat-grafted area was replaced by fibrous connective tissue. In the control femur, a bony bridge was rectangular in the longitudinal direction and showed isosignal intensity with a rim of low signal intensity on T1-weighted imaging. Histologically, the defect was filled with mature fatty marrow with new bone formation in the control femur. CONCLUSIONS: The proximally pointing appearance and the low signal intensity of the grafted area on MR suggested fibrous degeneration of grafted fat that prevented solid bony bridge formation in experimentally induced growth plate injury.

Cerebellopontine angle ganglioglioma: MR findings.

We present a case of cerebellopontine (CP) angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. MR imaging demonstrated a mass of homogeneous low signal intensity in the left CP angle on T1-weighted images with no enhancement with gadolinium, and of relatively homogeneous high signal intensity on T2-weighted images.

Focal lesion in the splenium of the corpus callosum in epileptic patients: antiepileptic drug toxicity?

BACKGROUND AND PURPOSE: Discrete focal lesions in the splenium of the corpus callosum on MR images in epileptic patients have received little attention in the literature. Our purpose was to describe these lesions, which may be related to the toxicity of antiepileptic drugs (AEDs), and to discuss the possible mechanisms of their development. METHODS: We examined six patients with epilepsy whose brain MR imaging findings showed a discrete focal nonhemorrhagic lesion in the splenium of the corpus callosum. The medical records and MR images were reviewed retrospectively with respect to the patients' clinical history, medication, and laboratory findings to determine the etiology of the lesion. RESULTS: In all six patients MR imaging showed a focal lesion in the splenium of the corpus callosum, which was ovoid in shape and 15 to 19 mm in size. In the three patients who received contrast material, there was no enhancement of the lesion. Four of six patients had a history of medication with dilantin, in two of whom the level of serum dilantin was found to be elevated (22.3 micrograms/mL and 70.4 micrograms/mL, respectively). Vigabatrin was administered in three patients, one of whom took dilantin together with vigabatrin. In two patients, the focal lesion in the corpus callosum disappeared on follow-up MR images after withdrawal of dilantin and/or vigabatrin. CONCLUSION: A discrete, focal, ovoid, nonhemorrhagic lesion in the splenium of the corpus callosum may be seen on brain MR images of patients with epilepsy. The lesion is considered to be reversible demyelination related to AEDs toxicity.

MR of hippocampal sclerosis: comparison of qualitative and quantitative assessments.

PURPOSE: Our goal was to compare the diagnostic accuracy of subjective visual assessment versus MR volumetry in evaluating hippocampal sclerosis and to determine whether MR volumetry is needed in the lateralization of this disease process. METHODS: MR imaging findings were studied retrospectively in 48 patients who underwent surgery for temporal lobe epilepsy and were compared with findings at MR volumetry on an Allegro workstation. Both visual assessment and volumetry were carried out in a blinded fashion with oblique coronal T1-weighted three-dimensional MP-RAGE images obtained on either 1.0-T or 1.5-T units. Normal right-left volumetric differences were recorded in 30 control subjects. The optimum cutoff threshold value for right-left volumetric differences in the sensitivity and specificity of volumetric measurement was obtained from receiver operating characteristic analysis. RESULTS: Sensitivity, specificity, positive and negative predictive values, and accuracy of visual assessment were 86%, 83%, 86%, 83%, and 85%, respectively. For MR volumetry, with the optimum cutoff threshold value of right-left difference at 0.3 cm3, sensitivity, specificity, positive and negative predictive values, and accuracy were 81%, 82%, 87%, 83%, and 85%, respectively. CONCLUSION: Visual assessment was slightly superior to or similar to MR volumetry in assessing unilateral hippocampal sclerosis. MR volumetry of the hippocampus may not be needed for the evaluation of most cases of suspected hippocampal sclerosis.

Comparison of MR imaging with PET and ictal SPECT in 118 patients with intractable epilepsy.

BACKGROUND AND PURPOSE: MR imaging, PET, and ictal SPECT have been studied extensively as individual techniques in the localization of epileptogenic foci, but only a few comparative studies have been done. We evaluated the concordance rates of ictal video/EEG, MR imaging, PET, and ictal SPECT to compare the sensitivities of these imaging methods in the lateralization of epileptogenic foci. METHODS: The study included 118 consecutive patients who underwent surgery for medically intractable epilepsy and who were followed up for 12 months or more. MR imaging was compared retrospectively with ictal video/EEG, FDG-PET, ictal 99mTc-HMPAO SPECT, and invasive EEG as to their ability to localize the epileptogenic focus; the pathologic findings served as the standard of reference. RESULTS: MR imaging was concordant with video/EEG, PET, and ictal SPECT in 58%, 68%, and 58% of patients, respectively. With the pathologic diagnosis as the standard of reference, MR imaging, PET, and ictal SPECT correctly lateralized the lesion in 72%, 85%, and 73% of patients, respectively. Of the patients with good outcomes, MR imaging, PET, and ictal SPECT were correct in 77%, 86%, and 78%, respectively. In the good outcome group, MR imaging was concordant with PET and ictal SPECT in 73% and 62% of patients, respectively. Of 45 patients who underwent invasive EEG, MR imaging was concordant with the invasive study in 47%; PET in 58%; and ictal SPECT in 56%. Of 26 patients with normal MR findings, PET and ictal SPECT correctly lateralized the lesion in 80% and 55%, respectively. CONCLUSION: Overall concordance among the techniques is approximately two thirds or less in lateralizing epileptogenic foci. PET is the most sensitive, even though it provides a broad approximate nature of the epileptogenic zone, which is not adequate for precise surgical localization of epilepsy. PET and/or ictal SPECT may be used as complementary tools in cases of inconclusive lateralization with ictal video/EEG and MR imaging.

Sonography of acute osteomyelitis in rabbits with pathologic correlation.

RATIONALE AND OBJECTIVES: Ultrasonography (US) has a potential role in the diagnosis of osteomyelitis. The purpose of this study was to determine the characteristic sonographic features of acute osteomyelitis and correlate them with pathologic findings. MATERIALS AND METHODS: An experimental model of acute osteomyelitis was produced in the tibiae of 20 rabbits. Daily US and plain radiography were performed for 2 weeks. The authors evaluated periosteal reaction, subperiosteal fluid collection, and soft-tissue changes seen with US. A hypoechoic band and a hyperechoic line lying along the cortex were considered positive signs of subperiosteal fluid collection and periosteal reaction, respectively. The findings of periosteal reaction were compared for US and radiography, and pathologic findings were also correlated. RESULTS: The most common sonographic finding was a hypoechoic band along the cortex (21 [75%] of 28 tibiae), usually associated with a linear periosteal reaction (20 [71%] of 28). This juxtacortical abnormal echogenicity corresponded to periosteal elevation with loose fibrovascular connective tissue and granulation, associated with subperiosteal abscess formation. The periosteal reactions were detected with US before they were seen on radiographs. The periosteum showed gradual thickening during the disease process. In 50% of infected tibiae, inflammation or abscess formation was observed in the surrounding soft tissue. CONCLUSION: US readily demonstrates juxtacortical abnormal echogenicity and soft-tissue infection related to acute osteomyelitis. The abnormal echogenicity correlated well with the pathologic findings of periosteal reaction and subperiosteal abscess.

MR diagnosis of cerebellar infarction due to vertebral artery dissection in children.

Posterior circulation infarction is uncommon in children. We describe the clinical presentation and radiological findings in two children with cerebellar infarction resulting from dissection of the vertebral artery. We emphasize that vertebral artery injury should be considered in a child with acute symptoms and signs of ischaemia in the posterior circulation. MRI and MRA may be helpful in the diagnosis of cerebellar infarction and vertebral artery abnormality.

Acute eosinophilic pneumonia: radiographic and CT findings in six patients.

OBJECTIVE: Our objective was to assess the radiographic and CT findings of acute eosinophilic pneumonia. CONCLUSION: Initial and follow-up chest radiographs, chest CT scans (n = 5) and clinical data in six patients with acute eosinophilic pneumonia were reviewed by two chest radiologists. The predominant initial radiographic finding was diffuse bilateral reticular densities (four [67%] of six patients). Areas of ground-glass opacity were observed on CT scans in all patients (5 of 5) and were bilateral, random, and patchy in distribution in four (80%) of five patients. Smooth septal thickening and pleural effusions were observed in four patients. The disease manifested as rapid onset of severe dyspnea and fever and rapid resolution with (n = 3) or without (n = 3) steroid therapy. Bilateral reticular densities on chest radiographs and, on CT scans, ground-glass opacity with smooth septal thickening and pleural effusion associated with acute fever and dyspnea may suggest the diagnosis of acute eosinophilic pneumonia.

Radiological features of focal nodular hyperplasia of the liver in children.

BACKGROUND: Focal nodular hyperplasia (FNH) is an unusual hepatic tumour in children and should be distinguished from other hepatic lesions. OBJECTIVE: To describe the imaging characteristics of FNH in children. MATERIALS AND METHODS: We examined five patients (three boys and two girls, mean age 9.4 years) with pathologically confirmed FNH. The diagnosis was obtained by tumour resection (n = 4) and percutaneous needle biopsy (n = 1). One patient with multiple FNHs showed recurrent lesions after tumour resection. All patients were studied with US (including colour and power Doppler US [n = 3]) and CT. Dynamic enhanced CT scans were available in three patients. MRI (n = 2) or coeliac angiography (n = 1) was performed in three patients. RESULTS: Seven of eight FNH lesions in five patients were demonstrated by imaging. The average size of the lesions was 6.5 cm. Six lesions detected on US showed variable echogenicity with a central hyperechoic scar (n = 2). On Doppler examination, central or peripheral hypervascular areas were seen (n = 3). Six lesions detected on contrast-enhanced CT showed high attenuation (n = 4) or iso-attenuation (n = 2). On early phase scans, all the lesions (n = 3) showed high attenuation. Irregular linear or ovoid central scars were detected in two patients on CT. MR demonstrated three lesions in two patients, one of which had not been detected by US or CT. A central low signal intensity scar (n = 1) was seen on T2-weighted MRI. Coeliac angiography performed in one patient showed a hypervascular mass with homogeneous staining. CONCLUSION: FNH in children shows a wide spectrum of imaging findings on various radiological examinations and the typical central scar was not always seen on imaging studies. Dynamic enhanced CT obtained in the early phase and colour Doppler studies may be helpful in the diagnosis of FNH by allowing characterisation of tumour vascularity. FNH should be included in the differential diagnosis of liver mass in children.

Thoracic actinomycosis: CT findings.

PURPOSE: To characterize computed tomographic (CT) findings of thoracic actinomycosis. MATERIALS AND METHODS: Chest CT scans and radiographs obtained in 22 patients with histopathologically proved thoracic actinomycosis were retrospectively reviewed. All patients were immunocompetent; they were aged 12-73 years (mean, 42.6 years; 14 male, eight female). CT findings were correlated with histopathologic findings in nine patients who underwent surgery (lobectomy [n = 8] or segmental resection [n = 1]). RESULTS: All of the lesions were unilateral, with an average diameter of 6.5 cm (range, 2-12 cm). Patchy air-space consolidation (n = 20) or a mass (n = 2) was seen on CT scans. Fifteen (75%) of the 20 patients with air-space consolidation had central areas of low attenuation (5-30 mm in diameter) within the consolidation. Thirteen of the 15 patients underwent contrast medium-enhanced CT. Ten (77%) of the 13 patients showed ring-like rim enhancement. Adjacent pleural thickening was seen in 16 patients (73%). At histopathologic examination, central low-attenuation areas at CT were seen as microabscesses with sulfur granules or a dilated bronchus that contained inflammatory cells and Actinomyces colonies. Peripheral enhancement of the low-attenuation areas was wall of the microabscess or surrounding parenchyma composed of granulation tissue rich in vascularity. CONCLUSION: Findings of chronic segmental air-space consolidation that contained low-attenuation areas with peripheral enhancement or adjacent pleural thickening at CT were suggestive of thoracic actinomycosis.

Abdominal-wall myositis secondary to intra-arterial chemotherapy for femoral osteosarcoma.

With the increasing application of intra-arterial chemotherapy (IAC), new side-effects are encountered. We describe two children with proximal femoral osteosarcoma who developed focal myositis of the abdominal wall musculature after IAC. In both cases, myositis presented as abdominal pain and mimicked acute abdomen. US demonstrated asymmetrical thickening of abdominal-wall musculature in the right lower abdomen. This diagnosis should be considered when evaluating the patient with unexplained abdominal pain and a history of IAC.

Complex bronchopulmonary foregut malformation: extralobar pulmonary sequestration associated with a duplication cyst of mixed bronchogenic and oesophageal type.

We report a 13-year-old girl with an unusual, complex bronchopulmonary foregut malformation. The malformation included extralobar pulmonary sequestration and a duplication cyst of mixed bronchogenic and oesophageal type. Preoperative CT and MRI demonstrated the cystic and solid portions of the mass and indicated an aberrant vascular supply, suggesting the possibility of bronchopulmonary foregut malformation and several other differential diagnoses. A direct communication between the cyst and the bronchus of the sequestrated lung was found on pathological examination. This unusual combination of an extralobar pulmonary sequestration and a foregut cyst points to a common embryological pathogenesis.

Gangliocytoma of the spinal cord: a case report.

We present a case of intramedullary spinal gangliocytoma in a 7-year-old girl who presented with scoliosis and progressive weakness of both legs. The tumour involved the whole spinal cord and medulla oblongata and was composed of inner cystic and outer solid components. On MRI, the solid portion of the lesion showed strong enhancement at the thoracolumbar level and mild enhancement at the cervical and medullary levels. Histological examination of the surgical specimen showed neoplastic ganglion cells arranged irregularly in benign normocellular glial background, which made a diagnosis of gangliocytoma.

Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation.

Congenital abnormalities of the portal venous system are rare. There are few radiological descriptions of intrahepatic portosystemic venous shunt detected in the perinatal period. We report a congenital portosystemic shunt that was detected by US and treated with coil embolisation in the neonatal period.

Teratoid Wilms tumor in childhood: CT and ultrasonographic appearances.

Teratoid Wilms tumor is defined as an unusual variant of nephroblastoma, in which there is a significant diversity of cell types and tissues in a neoplasm, where areas of classic nephroblastoma tissue are also present. We report a case of teratoid Wilms tumor demonstrated as a unilateral cystic and solid renal mass containing fatty tissue on ultrasonography and computed tomography.