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.

MR findings of desmoplastic cerebral astrocytoma of infancy.

Desmoplastic cerebral astrocytoma of infancy (DCAI) presents as a large supratentorial mass consisting of a central cystic component and an enhancing solid component associated with peripheral dural attachment. We report the unusual MR findings of a DCAI that differed from previously reported cases in terms of the presence of calcification, which is not considered a feature of this tumor, and the absence of an enhancing peripheral dural component.

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.

Herpes simplex encephalitis: MRI findings in two cases confirmed by polymerase chain reaction assay.

Herpes simplex virus (HSV) type I causes a fulminant necrotising meningoencephalitis distinguished from other encephalitides by its focal and often haemorrhagic nature. Specific antiviral therapy with acyclovir can significantly improve the prognosis. We present MRI findings of two cases of herpes simplex encephalitis (HSE) confirmed by PCR analysis, focusing on the serial changes after acyclovir therapy: gyral swelling, high signal intensity on T2-weighted images in the subfrontal region, temporal lobe and insula in the initial stage, then regional extension with enhancement and haemorrhage despite appropriate acyclovir therapy, and finally encephalomalacia and brain atrophy.

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.

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.

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.

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.

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.

Ultrasound of the adult hip.

The adult hip is a new and challenging site for evaluation with ultrasound (US). Clinically, diseases involving the hip region may be difficult to diagnose without the help of imaging. The hip region is a crossroad for numerous vascular, nervous, and muscular structures that pass between the trunk and the lower extremity. Thus, inflammatory processes and neoplasms may spread to and from the hip, buttock, thigh, pelvis, and retroperitoneum, and trauma may also effect the adjacent areas of the trunk and thigh. Because it is a crossroad, many conditions that are not specific to the hip occur in this area, including inguinal lymphadenopathy, pathology from the abdomen or the genitalia (intestinal hernia, inflammation, and infection), and even referred pain from spinal disorders. The goals of US imaging are the detection and localization of these pathological processes, the differentiation of intra-articular andextra-articular pathology, and the performance of diagnostic and therapeutic interventional procedures. This article reviews the hip anatomy, followed up by a discussion of the US evaluation and differential diagnosis of common pathology occurring in the hip area. This includes joint effusion, arthritis, loose bodies, bursitis, pseudoaneurysm, muscle and tendon diseases, as well as tumor and tumor-like lesions such as hematoma, abscess, and lymphadenopathy.

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.

Dynamic contrast-enhanced MR imaging of bacterial abscess and VX2 carcinoma in rabbits: comparison of gadopentetate dimeglumine and a macromolecular contrast agent.

OBJECTIVE: The objective of this study was to compare enhancement patterns of a blood-pool contrast agent, Gadomer-17, with those of gadopentetate dimeglumine in bacterial abscesses and VX2 carcinoma in rabbits. MATERIALS AND METHODS: Fourteen rabbits with experimentally induced bacterial abscesses and VX2 carcinoma in both thighs underwent dynamic contrast-enhanced MR imaging with Gadomer-17 and gadopentetate dimeglumine at a 24-hr interval. The enhancement ratios (postcontrast to precontrast signal intensities) of lesions in the same animal were assessed and correlated with microvessel density. RESULTS: For Gadomer-17, the enhancement ratio of the abscesses (1.66 +/- 0.39) peaked 15 min after the injection, while that of the carcinoma (2.05 +/- 0.16) peaked at 10 min. The enhancement ratios of the carcinoma were consistently higher than those of the abscesses up to 30 min. For gadopentetate dimeglumine, peak enhancement ratio of the abscesses (2.30 +/- 0.75) was seen 5 min after the injection, while that of the carcinoma (2.32 +/- 0.51) was seen at 3 min. The enhancement ratios of the carcinomas were significantly higher at 1 min, but significantly lower at 20-30 min, compared with those of the abscesses, as a result of rapid decrease of enhancement ratios in the carcinomas. The microvessel density was 9.8 +/- 5.2 vessels per field of view for the abscesses and 36.3 +/- 9.5 vessels per field of view for the carcinoma (p < 0.001). CONCLUSION: Delayed peak enhancement and slow decay were found in both bacterial abscess and VX2 carcinoma with Gadomer-17, whereas early peak enhancement and rapid decay were found especially in VX2 carcinoma with gadopentetate dimeglumine. Enhancement ratios on MR imaging with a blood-pool contrast agent correlated well with the microvessel density in bacterial abscess and VX2 carcinoma.

Transperineal ultrasonography in imperforate anus: identification of the internal fistula.

The purpose of this study was to assess the usefulness of transperineal ultrasonography in identifying the internal fistula in cases of imperforate anus. Transperineal ultrasonography was performed in 19 infants (13 neonates and 6 older infants; 13 were male and 6 were female) with imperforate anus to identify the internal fistula. Sagittal plane images were obtained through the anal dimple, and the internal connection of the rectal fistula was traced. The ultrasonographically traced internal fistula was compared with that observed on distal loopography after colostomy or with surgical findings. The internal fistula was identified as a hypoechoic linear tract, containing linear echogenicity in some cases. Of 19 patients, internal fistulas were correctly identified in 16 patients; these were rectourethral (n = 12), rectovaginal (n = 1), rectovestibular (n = 1), rectovesical (n = 1), and rectocloacal (n = 1). In three patients, internal fistulas were incorrectly defined; these cases consisted of rectovestibular (n = 2) and rectovaginal (n = 1) fistulas. Internal fistulas were correctly identified in all of the 13 male patients and in 3 of 6 female patients. Transperineal ultrasonography is an excellent diagnostic modality to define the type of the internal fistula in imperforate anus.

Metallic punctate densities in the breast after Chinese herbal treatment: mammographic findings.

PURPOSE: To describe the mammographic features of metallic punctate densities seen in women who were treated with the herb go-yak for breast abscess and to explain the cause of these findings. MATERIALS AND METHODS: Mammograms showing metallic punctate densities that appeared to be microcalcifications in 34 women were analyzed retrospectively with attention to the location, shape, distribution, and depth of the lesions. In all patients, go-yak was applied into the open wound after abscess drainage 6-42 years before mammography. In six patients, histopathologic specimens were obtained after needle localization. RESULTS: Metallic densities were in the subareolar or central breast in 24 (71%) of 34 patients. The shape was predominantly round or punctate in all patients, but rod-shaped or linear lesions were found in seven patients. The distribution and depth of lesions were variable, but they extended to the subcutaneous fat in 29 patients (85%). A high concentration of lead was found in the histopathologic specimens and herb samples. CONCLUSION: Lead deposits associated with go-yak treatment should be included in the differential diagnosis when the suspected microcalcifications are of unusually high density, are central in location, and extend into the subcutaneous fat in Asian women with a history of breast abscess.

Heterotopic pancreas of the stomach: CT findings correlated with pathologic findings in six patients.

BACKGROUND: The purpose of this study was to characterize the computed tomographic (CT) findings of heterotopic pancreas of the stomach. METHODS: CT scans of six surgically proven cases of heterotopic pancreas of the stomach were reviewed. Three were dynamic spiral CT scans, with both arterial dominant and late phase scans. In other three, both unenhanced and contrast-enhanced scans were obtained by using conventional techniques. Particular attention was given to the enhancement of the heterotopic pancreas. Pathologic and surgical findings were correlated with CT findings. RESULTS: The locations were in the gastric antrum in five cases and in the mid-body in one. Size ranged from 1 cm to 3 cm (mean = 2.1 cm). Three cases showed homogeneous, strong enhancement similar to the pancreas and consisted mainly of pancreatic acini with the same histologic features as the normal pancreas. Two cases showed poor enhancement and consisted mainly of ducts and hypertrophied muscle; pancreatic acini were a minor component. In one case appearing as a cystic lesion on CT, a pseudocyst was found with many ducts and some nests of pancreatic acini. CONCLUSIONS: Heterotopic pancreas of the stomach showed a diverse spectrum of CT findings. Good understanding of these CT findings may be helpful in making a correct diagnosis.

Pulmonary vasculitis: the spectrum of radiological findings.

Pulmonary vasculitis includes various disease entities with a wide range of clinical presentations and overlapping imaging features. Radiological findings of vasculitis in the angiitis-granulomatosis group are nodular and patchy opacities, whereas the principal feature of those diseases causing widespread capillaritis is diffuse air space consolidation. Aneurysms or stenoses of pulmonary arteries are seen in patients with Takayasu arteritis or Behçet's disease.

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.

Disseminated BCG infection in a patient with severe combined immunodeficiency.

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG) vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old girl with severe combined immunodeficiency. Plain radiographs showed multiple osteolytic lesions in the femora, tibiae, humerus, and phalanges. Abdominal sonography and CT scanning revealed multiple nodules in the spleen, and portocaval lymphadenopathy.

Airway reactivity to bronchoconstrictor and bronchodilator: assessment using thin-section and volumetric three-dimensional CT.

OBJECTIVE: To determine the extent to which thin-section and volumetric threedimensional CT can depict airway reactivity to bronchostimulator, and to assess the effect of different airway sizes on the degree of reactivity. MATERIALS AND METHODS: In eight dogs, thin-section CT scans were obtained before and after the administration of methacholine and ventolin. Cross-sectional areas of bronchi at multiple levels, as shown by axial CT, proximal airway volume as revealed by three-dimensional imaging, and peak airway pressure were measured. The significance of airway change induced by methacholine and ventolin, expressed by percentage changes in cross-sectional area, proximal airway volume, and peak airway pressure was statistically evaluated, as was correlation between the degree of airway reactivity and the area of airways. RESULTS: Cross-sectional areas of the bronchi decreased significantly after the administration of methacholine, and scans obtained after a delay of 5 minutes showed that normalization was insufficient. Ventolin induced a significant increase in cross-sectional areas and an increase in proximal airway volume, while the effect of methacholine on the latter was the opposite. Peak airway pressure increased after the administration of methacholine, and after a 5-minute delay its level was near that of the control state. Ventolin, however, induced no significant decrease. The degree of airway reactivity did not correlate with airway size. CONCLUSION: Thin-section and volumetric spiral CT with three-dimensional reconstruction can demonstrate airway reactivity to bronchostimulator. The degree of reactivity did not correlate with airway size.

Effectiveness of MR urography in the evaluation of kidney which failed to opacify during excretory urography: comparison with ultrasonography.

OBJECTIVE: The purpose of this study was to compare the effectiveness of MR urography (MRU) with that of ultrasonography (US) in the evaluation of urinary tract when this failed to opacify during excretory urography (EXU). MATERIALS AND METHODS: Twelve urinary tracts in 11 patients were studied. In each case, during EXU, the urinary system failed to opacify within one hour of the injection of contrast media, and US revealed dilatation of the pelvocalyceal system. Patients underwent MRU, using a HASTE sequence with the breath-hold technique; multi-slice acquisition was then performed, and the images were reconstructed using maximal intensity projection. Each set of images was evaluated by three radiologists to determine the presence, level, and cause of urinary tract obstruction. RESULTS: Obstruction was present in all twelve cases, and in all of these, MRU accurately demonstrated its level. In this respect, however, US was successful in only ten. The cause of obstruction was determined by MRU in eight cases, but by US in only six. In all of these six, MRU also successfully demonstrated the cause. CONCLUSION: MRU is an effective modality for evaluation of the urinary tract when this fails to opacify during EXU, and appears to be superior to US in demonstrating the level and cause of obstruction.