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.

A comparative study on contrast enhancement between 0.5- and 2.0-Tesla field strength in rabbit VX2 carcinomas.

RATIONALE AND OBJECTIVES: Theoretically, the degree of contrast enhancement in magnetic resonance (MR) is affected by magnetic field strength. The authors conducted this study to compare the degree of MR contrast enhancement between 0.5 Tesla (T) and 2.0 T in VX carcinomas of rabbits. METHODS: In 12 rabbits with VX2 tumors, both 0.5 T and 2.0 T MR imaging was performed within 1 day before and after intravenous injection of gadopentetate dimeglumine. Each rabbit received an initial standard dose (0.1 mmol/KG) followed by and additional standard dose (total, double dose) during the MR study at each field strength. The degree of contrast enhancement in the VX2 tumors was assessed quantitatively. RESULTS: With standard and double dose studies, overall mean tumor and muscle contrast-to-noise ratio was higher at 2.0 T than 0.5 T by 95% and 81%, respectively. The double dose study showed higher contrast-to-noise ratio than the standard dose study at both field strengths. CONCLUSIONS: Results indicate that the effect of contrast enhancement increases with field strength.

An experimental study of embolic effect according to infusion rate and concentration of suspension in transarterial particulate embolization.

RATIONALE AND OBJECTIVES: The authors evaluate the embolic effect according to infusion rate and concentration of particulate suspension, focusing on arterial occlusion level. METHODS: The renal arteries of 14 rabbits were embolized with 150 to 250 microns polyvinyl alcohol (PVA) particles, divided into four groups according to two different infusion rates (1 mg/second and 0.1 mg/second) and two different concentrations of suspension (10 mg/mL and 2.5 mg/mL). Arteriograms obtained immediately and a week after embolization were assessed for occlusion level. For the nephrograms obtained a week after embolization, the opacifying areas were graded from 0 to 4. Median coronal sections of each kidney specimen were investigated for the presence of peripheral infarct grossly and for the presence of PVA particles in the small artery microscopically. RESULTS: Arteriograms showed various occlusion levels. Using a 0 to 4 grading system, the opacifying area of the nephrogram obtained 1 week after embolization was noted to be smaller in the low infusion rate group (P < 0.05). In gross and microscopic pathologic examination, the number of cases with peripheral infarct or PVA particles in the small artery (< 300 microns) was greater in the group with the low infusion rate and low concentration (P < 0.05). CONCLUSIONS: In transarterial particulate embolization, slower infusion of more diluted suspension provides for a more distal arterial occlusion.

Influence of instrument settings on flow signal and background noise in power Doppler US. An experimental study using a flow phantom with hyperechoic background.

OBJECTIVE: To determine the influence of various power Doppler instrument settings on intensities of flow signal and background noise in flow with a tissue-equivalent phantom. METHODS: Power Doppler images were obtained with changing wall filter level (low, medium, high, and maximum), pulse repetition frequency (PRF; 500, 700, 1000, 1500, 3000, and 6000 Hz), and Doppler gain (60%, 70%, 80%, 90%, and 100%) at different flow velocities (13.3, 26.5, and 49.8 cm/sec). To make a quantitative comparison of different settings, the authors measured the intensities of flow signal and background noise in obtained power Doppler images using the scanner and a computer program, calculated signal-to-noise difference (SND; intensity of flow signal--intensity of background noise), and evaluated the relation between SND and power Doppler settings. RESULTS: The intensities of flow signal and background noise were proportional to flow velocity and power Doppler gain but were inversely proportional to PRF and wall filter level. At constant wall filter level (medium), changes of PRF and Doppler gain to the same directions resulted in a high SND. At constant PRF (1000 Hz), changes of wall filter and Doppler gain to the same directions also resulted in a high SND. However, at constant Doppler gain (80%), a high SND was obtained with changing wall filter level and PRF to the opposite directions. CONCLUSIONS: Three Doppler instrument settings--wall filter level, pulse repetition frequency, and Doppler gain--have reciprocal influences on SND.

Doppler sonography in experimentally induced acute renal failure in rabbits. Resistive index versus serum creatinine levels.

RATIONALE AND OBJECTIVES: The purpose of this study is to investigate the temporal relation between the change in the resistive index (RI) from renal Doppler sonography and that of serum creatinine values in the course of experimentally induced reversible acute renal failure (ARF) in rabbits. METHODS: Reversible ARF was induced in 10 New Zealand white rabbits by injecting a glycerol solution (6.0 mL/kg) into the thigh muscle. Doppler sonography was performed before injection, and 6, 12, and 24 hours, and 3, 5, 7, and 14 days after injection of glycerol. Blood samples for measuring serum creatinine levels were obtained in the same time sequence. Three rabbits, which died during the course of the experiment, were excluded from the study group. RESULTS: The RI elevated rapidly after glycerol injection, peaked at 12 hours, and then decreased to normal values by 7 days. Conversely, serum creatinine values were elevated and peaked at 1 day and then decreased to normal by 14 days. The change in the RI preceded the change in serum creatinine levels in the course of reversible ARF. There was a weak linear correlation between RI and serum creatinine levels, with a correlation coefficient of 0.33. CONCLUSION: Doppler sonography with measurement of resistive index may be useful in predicting the course of ARF.

Intratumoral vascularity of experimentally induced VX2 carcinoma: comparison of color Doppler sonography, power Doppler sonography, and microangiography.

RATIONALE AND OBJECTIVES: Tumor vascularity is useful for characterizing tumors and determining tumor management. The recent development of power Doppler sonography has enhanced the sensitivity of color Doppler imaging in the detection of blood flow because of low power noise and less angle dependence. The purpose of this study was to compare the capability of color and power Doppler sonography with that of microangiography for showing tumor vascularity of VX2 carcinoma. METHODS: Color and power Doppler sonography was performed on VX2 carcinomas in the rabbit thighs, and their findings were correlated with those of microangiography. For qualitative analysis, tumor vascularity was categorized into four items including distribution of tumor vessels, crowdedness of vessels, small vessels, (> or = 0.1 mm), and micro-vessels (< 0.1 mm). Tumor blood flow signals of color Doppler sonography and power Doppler sonography were graded as 3, 2, 1, and 0 and were compared with tumor vascularity on microangiography. For quantitative analysis, percentages of tumor vascular area per tumor area on each study were compared. RESULTS: The mean scores of tumor vascularity on power Doppler sonography were 2.87, 2.73, 2.93, and 2.73 in tumor vascular distribution, crowdedness of vessels, small vessels, and micro-vessels, respectively. Those on color Doppler sonography were 2.4, 2.2, 2.8, and 1.67, respectively. Power Doppler sonography was statistically superior to color Doppler sonography in displaying tumor vascular distribution (P < 0.05) and micro-vessels (P < 0.01). The means and medians of percentages of tumor vascular area per tumor area were 22.7% and 23.5% on microangiography, 17.9% and 21.4% on color Doppler sonography, and 36.4% and 34.7 % on power Doppler sonography, respectively. Percentages of tumor vascular area per tumor area on both color Doppler sonography (r = 0.70) and power Doppler sonography (r = 0.84) were well correlated with those on microangiography. CONCLUSIONS: Power Doppler sonography can demonstrate the tumor vascularity on microangiography relatively well, however, it tends to overestimate the blood flow signals. Color Doppler sonography may have some limitations in imaging tumor vascular distribution and micro-vessels and tends to underestimate tumor vascularity, but can display the blood flow direction. Therefore, power Doppler and color Doppler sonography could complement each other in demonstrating the tumor vascularity.

Short-term effects of a new intravascular nitinol stent in canine arteries.

RATIONALE AND OBJECTIVES: To evaluate the short-term effects of a new nitinol stent on canine arteries. METHODS: Eighteen nitinol mesh stents were placed in abdominal aortas, common iliac arteries, and renal arteries of six dogs. Angiography was performed to evaluate the patency rates and structural changes of arteries at 1 day, 3 weeks, 4 weeks, and 10 weeks after stent insertion. Gross and light microscopic examinations were performed after angiography. RESULTS: On angiography, the patency rate was 100%, and no thrombosis was observed. All side branches from stented segments were patent. The mean neointimal thickness over and between stent wires was 94 and 167 microns. No difference was found between the aorta and the small vessels. Histologically, the neointima was covered with endothelium and was composed of subintimal fibrosis with mild inflammation. CONCLUSIONS: The new type of nitinol mesh stent showed a high patency rate, with no thrombosis and relatively thin neointimal proliferation.

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.

Angiofibroma confined to the pterygoid muscle region: CT and MR demonstration.

We report a case of angiofibroma confined to the pterygoid muscle plane without involvement of the sphenopalatine foramen or nasopharynx. The lesion was demonstrated on CT, MR, and angiography with typical findings of a hypervascular mass and was surgically resected after embolization.

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.

Cystic expansile masses of the maxilla: differential diagnosis with CT and MR.

PURPOSE: To describe the CT and MR findings of various entities causing cystic expansile masses in the maxilla and the significance of the cortical bony plate between the lesions and sinus cavities in the differential diagnosis. METHODS: CT findings of 28 patients with cystic expansile masses of the maxilla, including 20 cases of maxillary mucoceles (17 postoperative mucoceles, 2 mucoceles of a septated compartment of the maxillary sinuses, and 1 maxillary antral mucocele with inflammatory ostial obstruction), 3 cases of fissural cyst, 4 cases of odontogenic cyst, and 1 case of maxillary cystic ameloblastoma, were reviewed. Six cases (4 postoperative mucoceles and 2 odontogenic cysts) were also examined with MR. RESULTS: A thin bony plate between the lesion and antral cavity was demonstrated in every extraantral lesion (ameloblastoma, fissural cysts, and odontogenic cysts) and distinguishing these abnormalities from the antral lesions (mucoceles). All mucoceles showed findings of antral lesions except 2 cases of mucoceles at the septated compartments of the sinuses, in which thin bony septa were identified between the lesions and remaining sinus cavities. Postoperative mucoceles showed hyperostotic and retracted bony walls caused by previous surgery and localized erosion of bony walls with localized bulging of the cystic masses in every case. CONCLUSION: In the differential diagnosis of cystic masses of the maxilla, careful identification of the bony walls of the antrum and bony septa may allow one to locate the origin of a mass as antral or extraantral. This is important to an appropriate differential diagnosis.

Fibrosing inflammatory pseudotumors involving the skull base: MR and CT manifestations with histopathologic comparison.

PURPOSE: To describe the MR and CT features of fibrosing inflammatory pseudotumors of the skull base region, and to document the MR signal intensity of the lesions with histopathologic comparison. METHODS: We reviewed the MR and CT studies of five patients with pathologically proved fibrosing inflammatory pseudotumor involving the skull base. Unenhanced spin-echo T1- and T2-weighted and contrast-enhanced T1-weighted MR images were obtained at 0.5 T in three patients and at 1.5 T in two patients. MR findings were correlated with histopathologic findings in all five cases, and the enhancement pattern was compared with CT findings in three cases. RESULTS: In three cases, the cavernous sinus was involved unilaterally, with adjacent extracranial infiltrative masses. In one case, both orbits, the cavernous sinuses, and the tentorium were involved with diffuse infiltrative lesions. One patient had an infiltrative nasopharyngeal mass; and in all five patients, MR images showed localized involvement of the skull base, with bone marrow replaced by tumor. The soft-tissue lesions were hypointense on T2-weighted images in all five cases and showed homogeneous contrast enhancement. Histopathologic studies revealed scanty inflammatory cell infiltration with densely fibrotic background in all cases. The hypointensity of the lesions on T2-weighted images seemed to be related to the degree of fibrosis. CONCLUSION: Fibrosing inflammatory pseudotumor shows characteristic MR findings of infiltrative lesion with bone destruction and hypointensity on T2-weighted images. The lack of mobile protons due to the fibrotic background and/or high cellularity of the lesions may be the reason for their hypointensity and weaker enhancement on MR images.

MR of childhood metachromatic leukodystrophy.

PURPOSE: To investigate the MR findings of childhood metachromatic leukodystrophy (MLD). METHODS: Nine MR imaging studies in seven children (five girls and two boys, 10 to 32 months old) with MLD were evaluated retrospectively for the extent and progression of white matter abnormalities and the presence of contrast enhancement. RESULTS: All seven cases showed symmetric, confluent high signal intensity on T2-weighted images in the periventricular white matter and centrum semiovale. A posterior predominance of white matter abnormalities was noted in all cases. Although initially spared from demyelination in all cases, in one case, the subcortical U fibers were later involved in demyelination of follow-up MR studies. Other sites of involvement were the genu (n = 5) and splenium (n = 6) of the corpus callosum, the posterior limbs of the internal capsule (n = 5), the descending pyramidal tracts (n = 4), the claustrum (n = 4), and the cerebral white matter (n = 2); diffuse brain atrophy was seen in two cases. No enhancement of the lesion was seen on any of the five postcontrast examinations. A "tigroid" pattern, previously described in cases of Pelizaeus-Merzbacher disease, was noted in the centrum semiovale in six cases. CONCLUSION: In late-infantile MLD, demyelination is more prominent in the occipital region. In addition to demyelination of the periventricular white matter, common manifestations include a "tigroid" pattern and involvement of the corpus callosum, the internal capsule, and the corticospinal tract.

Experimentally induced pulmonary arterial occlusion with detachable balloon in pigs: thin-section CT findings.

RATIONALE AND OBJECTIVES: The authors evaluated changes of lung attenuation in pigs, with special attention to the mosaic pattern of low attenuation, at thin-section computed tomography (CT) after obstruction of the proximal pulmonary artery with a detachable balloon. MATERIALS AND METHODS: In seven pigs, nine sites of the descending pulmonary artery were obstructed with detachable balloons. This-section CT scans of the lungs were obtained immediately (n = 9) and at 1 week (n = 5), 2 weeks (n = 1), 3 weeks (n = 2), 4 weeks (n = 1), 6 weeks (n = 1), 8 weeks (n = 1), and 12 weeks (n = 1) after pulmonary artery obstruction. RESULTS: No statistically significant difference was found between the measured lung attenuation of the normal lung and that of the lung distal to the obstruction. Of the nine sites of pulmonary artery obstruction, five (56%) showed an irregular area of increased lung attenuation without lobular architecture. The diameter of the pulmonary artery after obstruction, compared with the diameter before obstruction, decreased by a range of 13%-57% (mean, 35%) and by 0-67% (mean, 44%) at levels 1 cm and 2 cm distal to the obstruction, respectively. CONCLUSION: This experimental study reveals that regional low-attenuation areas do not develop for up to 12 weeks after the obstruction of proximal pulmonary artery, despite a marked decrease in the diameter of the pulmonary artery distal to the obstruction.

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.

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.

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.

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.