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Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by deficiency of arylsulfatase A, leading to progressive demyelination in the central and peripheral nervous systems and, causing gross motor deterioration. This study aimed to analyze data related to neuroimaging and clinical phenotypes of MLD patients according to disease subtype. Patients diagnosed with MLD based on arylsulfatase A enzymatic activity, demyelination in brain MR findings, and/or pathogenic mutations were enrolled in this study. The medical charts of patients with confirmed MLD were retrospectively reviewed. We used a modified MRI scoring system and clinical status using the analogous designed scale. We analyzed the correlation between MRI score and clinical status in the two groups divided by late-infantile and juvenile type, and specific neuroimaging lesions. We detected a positive relationship between clinical function deterioration and MRI score (rho 0.59, p 0.002) in patients with MLD. A stronger positive relationship between clinical score and brain MRI scoring (rho 0.700, p 0.003) was found in the late-infantile type than in the juvenile type. A strong relationship was also seen in groups with high signal intensities in the pons and basal ganglia, and cerebellar atrophy, but not in patients with lesions in the midbrain. MLD with a high MRI score is associated with poor clinical function. Correlating modified MRI scores and clinical function scale may help predict the prognosis of patients with MLD for identifying treatment options and increasing patientsʼ quality of life.

Inspissated bile syndrome (IBS) is a relatively rare condition. Many treatment options are available, including medication, surgery, and surgical interventions, such as insertion of cholecystostomy drain, endoscopic retrograde cholangiopancreatography, internal biliary drainage, and percutaneous transhepatic biliary drainage (PTBD). We herein report the first case of IBS that was successfully treated with PTBD in a two-month-old infant in Korea. PTBD was initiated on postnatal day 72. On postnatal day 105, we confirmed complete improvement and successfully removed the catheters. This report suggests that PTBD is a viable and safe treatment option for obstructive jaundice in very young infants.

Purpose This study aimed to investigate associations of central obesity with sexual maturation and metabolic parameters in Korean girls with precocious puberty. Methods This retrospective study evaluated data from 72 girls under 8 years of age with a chief complaint of early breast development. The patients were categorized as central precocious puberty (CPP) subjects or non-CPP subjects based on their gonadotropin-releasing hormone stimulation test results. Visceral fat thickness (VFT) was measured using ultrasonography and defined as the distance from the linea alba to the aorta. Patient anthropometric, metabolic, and hormonal parameters were also evaluated. Results Increased VFT was correlated with an earlier onset of thelarche among all study subjects (r=-0.307, P=0.034). Overweight CPP subjects showed higher insulin resistance than normal weight CPP subjects. Insulin resistance was not significantly different between overweight and normal weight non-CPP subjects. VFT was not significantly different between CPP and non-CPP subjects (2.22±0.79 cm vs. 2.74±1.47 cm, P=0.169). However, overweight and obese CPP subjects (body mass index percentile>85%) had lower VFT than non-CPP obese subjects. Conclusion Central obesity, defined using ultrasonography-measured VFT, might be associated with early pubertal development in Korean girls. However, VFT was not higher in CPP than non-CPP patients and was not significantly correlated with insulin resistance. Further longitudinal studies with a larger cohort are needed.

Purpose Abdominal obesity is a fundamental factor underlying the development of metabolic syndrome. Because of radiation exposure and cost, computed tomography or dual-energy X-ray absorptiometry to evaluate abdominal adiposity are not appropriate in children. Authors evaluated whether ultrasound results could be an indicator of insulin resistance and nonalcoholic fatty liver disease (NAFLD). Methods We enrolled 73 subjects (aged 6–16 years) who were evaluated abdominal adiposity by ultrasound. Subcutaneous fat thickness was defined as the measurement from the skin-fat interface to the linea alba, and visceral fat thickness (VFT) was defined as the thickness from the linea alba to the aorta. Anthropometric and biochemical metabolic parameters were also collected and compared. The subjects who met 2 criteria, radiologic confirmed fatty liver and alanine aminotransferase >40, were diagnosed with NAFLD. Results There was a strong positive correlation between VFT and obesity. VFT was highly correlated with the homeostasis model assessment for insulin resistance score (r=0.403, P

Background: Analysis of serum biomarkers and magnetic resonance spectroscopy (MRS) are useful for monitoring disease progression in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). We evaluated the correlation of serum biomarkers and MRS parameters during changes associated with stroke-like episodes. Methods: In 13 symptomatic MELAS patients carrying the A3243G mutation, we retrospectively obtained 207 voxels from 41 MRS studies, which were divided into three groups according to the temporal association with stroke-like episodes. The MRS NAA/Cr, Cho/Cr, NAA/Cho ratios, the presence of a lactate peak, serum biomarkers, serum lactate level and the pyruvate (Lac/Pyr) ratio were determined. Results: In regions with acute infarcts, the severity of serum Lac/Pyr and that of the MRS lactate peak (P = 0.0007) correlated; serum lactate (P = 0.02), severity of elevated serum lactate (P = 0.04), and serum Lac/Pyr (P = 0.02) correlated weakly. In previously infarcted regions, the severity of the MRS lactate peak and serum Lac/Pyr (P = 0.03), as well as the severity of serum Lac/Pyr (P = 0.02) were weakly correlated. In structurally normal regions, we found a weak to moderate negative correlation between serum lactate and MRS NAA/Cr (P = 0.008), and between the severity of elevated serum lactate and MRS NAA/Cr (P = 0.002) as well as MRS NAA/Cho (P = 0.02). Conclusions: MRS parameters correlate with specific serum biomarkers, and are useful for monitoring changes in brain metabolites, particularly as related to stroke-like episodes.

OBJECTIVE To assess the feasibility of T2*-corrected fat-signal fraction (FF) map by using the three-echo volume interpolated breath-hold gradient echo (VIBE) Dixon sequence to differentiate between malignant marrow-replacing lesions and benign red marrow deposition of vertebrae. MATERIALS AND METHODS We assessed 32 lesions from 32 patients who underwent magnetic resonance imaging after being referred for assessment of a known or possible vertebral marrow abnormality. The lesions were divided into 21 malignant marrow-replacing lesions and 11 benign red marrow depositions. Three sequences for the parameter measurements were obtained by using a 1.5-T MR imaging scanner as follows: three-echo VIBE Dixon sequence for FF; conventional T1-weighted imaging for the lesion-disc ratio (LDR); pre- and post-gadolinium enhanced fat-suppressed T1-weighted images for the contrast-enhancement ratio (CER). A region of interest was drawn for each lesion for parameter measurements. The areas under the curve (AUC) of the parameters and their sensitivities and specificities at the most ideal cutoff values from receiver operating characteristic curve analysis were obtained. AUC, sensitivity, and specificity were respectively compared between FF and CER. RESULTS The AUCs of FF, LDR, and CER were 0.96, 0.80, and 0.72, respectively. In the comparison of diagnostic performance between the FF and CER, the FF showed a significantly larger AUC as compared to the CER (p = 0.030), although the difference of sensitivity (p = 0.157) and specificity (p = 0.157) were not significant. CONCLUSION Fat-signal fraction measurement using T2*-corrected three-echo VIBE Dixon sequence is feasible and has a more accurate diagnostic performance, than the CER, in distinguishing benign red marrow deposition from malignant bone marrow-replacing lesions.

Purpose: To describe the ultrasonographic (US) findings of type IIIa biliary atresia. Methods: We retrospectively reviewed a medical database of patients pathologically confirmed to have biliary atresia, Kasai type IIIa, between January 2002 and May 2013 (n=18). We evaluated US findings including the visible common bile duct (CBD), triangular cord thickness, gallbladder size and shape, and subcapsular flow on color Doppler US; laboratory data; and pathological hepatic fibrosis grades. We divided them into two groups-those with visible (group A) and invisible (group B) CBD on US-and compared all parameters between the two groups. Results: CBD was visible on US in five cases (27.8%; group A) and invisible in 13 cases (72.2%; group B). US was performed at an earlier age in group A than in group B (median, 27 days vs. 60 days; P=0.027) with the maximal age of 51 days. A comparison of the US findings revealed that the triangular cord thickness was smaller (4.1 mm vs. 4.9 mm; P=0.004) and the gallbladder length was larger (20.0 mm vs. 11.7 mm; P=0.021) in group A. The gallbladder shape did not differ between the two groups, and the subcapsular flow was positive in all cases of both groups. There was no significant difference in the laboratory data between the two groups. Upon pathological analysis, group A showed low-grade and group B showed low- to high-grade hepatic fibrosis. Conclusion: When CBD is visible on US in patients diagnosed with type IIIa biliary atresia, other US features could have a false negative status. A subcapsular flow on the color Doppler US would be noted in the type IIIa biliary atresia patients.

To evaluate the feasibility and age-related changes of shear wave velocity (SWV) in normal livers, kidneys, and spleens of children using acoustic radiation force impulse (ARFI) imaging.Healthy pediatric volunteers prospectively underwent abdominal ultrasonography and ARFI. The subjects were divided into three groups according to age: group 1:5 years old; group 2: 5-10 years old; and group 3:10 years old. The SWV was measured using a 4-9 MHz linear probe for group 1 and a 1-4 MHz convex probe for groups 2 and 3. Three valid SWV measurements were acquired for each organ.Two hundred and two children (92 male, 110 female) with an average age of 8.1 years (± 4.7) were included in this study and had a successful measurement rate of 97% (196/202). The mean SWVs were 1.12 m/s for the liver, 2.19 m/s for the right kidney, 2.33 m/s for the left kidney, and 2.25 m/s for the spleen. The SWVs for the right and left kidneys, and the spleen showed age-related changes in all children (p0.001). And the SWVs for the kidneys increased with age in group 1, and those for the liver changed with age in group 3.ARFI measurements are feasible for solid abdominal organs in children using high or low frequency probes. The mean ARFI SWV for the kidneys increased according to age in children less than 5 years of age and in the liver, it changed with age in children over 10.

Objective To investigate the validity of the 100 kVp setting in CT venography (CTV) in the diagnosis of deep vein thrombosis (DVT), and to evaluate the feasibility of reducing the amount of administered iodine in this setting. Materials and methods After receiving the contrast medium (CM) of 2.0 mL/kg, 88 patients underwent CTV of the pelvis and lower extremities by using one of four protocols: Group A, 120 kVp setting and 370 mgI/mL CM; group B, 120 kVp and 300 mgI/mL; group C, 100 kVp and 370 mgI/mL; group D, 100 kVp and 300 mgI/mL. The groups were evaluated for venous attenuation, vein-to-muscle contrast-to-noise ratio (CNR(VEIN)), DVT-to-vein contrast-to-noise ratio (CNR(DVT)), and subjective degree of venous enhancement and image quality. Results Venous attenuation and CNR(VEIN) were significantly higher in group C (144.3 Hounsfield unit [HU] and 11.9), but there was no significant difference between group A (118.0 HU and 8.2) and D (122.4 HU and 7.9). The attenuation value of DVT was not significantly different among the four groups, and group C had a higher absolute CNR(DVT) than the other groups. The overall diagnostic image quality and venous enhancement were significantly higher in group C, but there was no difference between groups A and D. Conclusion The 100 kVp setting in CTV substantially help improve venous enhancement and CNR(VEIN). Furthermore, it enables to reduce the amount of administered iodine while maintaining venous attenuation, as compared with the 120 kVp setting.

OBJECTIVE To investigate the interobserver and test-retest reproducibility of T1ρ and T2 measurements of lumbar intervertebral discs using 3T magnetic resonance imaging (MRI). MATERIALS AND METHODS This study included a total of 51 volunteers (female, 26; male, 25; mean age, 54 ± 16.3 years) who underwent lumbar spine MRI with a 3.0 T scanner. Amongst these subjects, 40 underwent repeat T1ρ and T2 measurement acquisitions with identical image protocol. Two observers independently performed the region of interest measurements in the nuclei pulposi of the discs from L1-2 through L5-S1 levels. Statistical analysis was performed using intraclass correlation coefficient (ICC) with a two-way random model of absolute agreement. Comparison of the ICC values was done after acquisition of ICC values using Z test. Statistical significance was defined as p value < 0.05. RESULTS The ICCs of interobserver reproducibility were 0.951 and 0.672 for T1ρ and T2 mapping, respectively. The ICCs of test-retest reproducibility (40 subjects) for T1ρ and T2 measurements were 0.922 and 0.617 for observer A and 0.914 and 0.628 for observer B, respectively. In the comparison of the aforementioned ICCs, ICCs of interobserver and test-retest reproducibility for T1ρ mapping were significantly higher than T2 mapping (p < 0.001). CONCLUSION The interobserver and test-retest reproducibility of T1ρ mapping were significantly higher than those of T2 mapping for the quantitative assessment of nuclei pulposi of lumbar intervertebral discs.

Objective To prospectively investigate the feasibility of an 80-kilovolt (peak) (kVp) protocol in computed tomographic venography for varicose veins of the lower extremities by comparison with conventional 120-kVp protocol. Methods Attenuation values and signal-to-noise ratio of iodine contrast medium (CM) were determined in a water phantom for 2 tube voltages (80 kVp and 120 kVp). Among 100 patients, 50 patients were scanned with 120 kVp and 150 effective milliampere second (mAs(eff)), and the other 50 patients were scanned with 80 kVp and 390 mAs(eff) after the administration of 1.7-mL/kg CM (370 mg of iodine per milliliter). The 2 groups were compared for venous attenuation, contrast-to-noise ratio, and subjective degree of venous enhancement, image noise, and overall diagnostic image quality. Results In the phantom, the attenuation value and signal-to-noise ratio value for iodine CM at 80 kVp were 63.8% and 33.0% higher, respectively, than those obtained at 120 kVp. The mean attenuation of the measured veins of the lower extremities was 148.3 Hounsfield units (HU) for the 80-kVp protocol and 94.8 HU for the 120-kVp protocol. Contrast-to-noise ratio was also significantly higher with the 80-kVp protocol. The overall diagnostic image quality of the 3-dimensional volume-rendered images was good with both protocols. The subjective score for venous enhancement was higher at the 80-kVp protocol. The mean volume computed tomography dose index of the 80-kVp (5.6 mGy) protocol was 23.3% lower than that of the 120-kVp (7.3 mGy) protocol. Conclusion The use of the 80-kVp protocol improved overall venous attenuation, especially in perforating vein, and provided similarly high diagnostic image quality with a lower radiation dose when compared to the conventional 120-kVp protocol.

Neoplasms and tumorlike lesions that originate from chest wall tissues are uncommon compared with tumors in other parts of the body, and unfamiliarity with these disease entities can cause diagnostic difficulties for radiologists. Furthermore, the imaging features of many of these tumors are nonspecific, particularly those that are locally aggressive. However, a systematic approach based on patient age, clinical history, lesion location, and characteristic imaging findings often helps limit the differential diagnosis. Primary chest wall tumors can be classified as bone or soft-tissue tumors, with the latter being further classified into adipocytic tumors, vascular tumors, peripheral nerve sheath tumors, cutaneous lesions, fibroblastic-myofibroblastic tumors, and so-called fibrohistiocytic tumors, largely based on the 2002 World Health Organization classification. Within each category, it is possible to further limit the differential diagnosis with cross-sectional imaging. Information on specific features (eg, mineralization, fibrosis, hemosiderin deposits) and imaging patterns (eg, the "target sign" and "fascicular sign" seen in neurogenic tumors) can aid in making the diagnosis. Radiologists can achieve a sufficiently specific diagnosis of bone tumors and soft-tissue tumors if typical findings are present.

Biliary atresia (BA) is a progressive, obliterative cholangiopathy that occurs in neonates with hepatic portoenterostomy the treatment of choice, but early surgery is important for optimum outcomes. MRI, including MR cholangiopancreatography (MRCP) may be a diagnostically useful alternative to US, but the heavily T2-weighted sequences used include not only bile duct signals, but also other heterogeneously high signal intensities from surrounding structures. To evaluate the effects of gadolinium when used to decrease background signal intensity on T2-weighted MR cholangiopancreatography (MRCP) in infants and to evaluate the qualitative improvement of the depiction of the common bile duct (CBD) for evaluating neonatal cholestasis. Our Institutional Review Board approved this prospective study. MRCP was performed with gadopentetate dimeglumine injection using a 1.5-T scanner. Pre- and postcontrast MRCP images were compared. Forty-nine infants (male:female = 21:28; age 0–12 months, mean 2.3) were included. The final diagnoses were biliary atresia (BA) in 28 cases and non-BA in 21. Quantitative analysis was conducted using region-of-interest measurements of mean signal intensities of the liver, pancreatic head and gallbladder (if defined). Qualitative analysis was performed by four radiologists who subjectively scored image confidence in the presence of CBD on a 4-point scale (0 for definitely absent, 1 for probably absent, 2 for probably present, and 3 for definitely present). The signal-to-noise ratios were significantly decreased in the liver and pancreatic head after contrast medium enhancement (mean 5.7→4.0 in liver and mean 44.9→12.7 in the pancreatic head; P

The degree of hepatic fibrosis in biliary atresia (BA) correlates with the prognosis of the disease and thus, early diagnosis of liver fibrosis is clinically important. Liver biopsy is the gold standard for the evaluation of liver fibrosis, but it is an invasive procedure requiring sedation in children. Therefore, it is desirable to identify a noninvasive method for diagnosis and follow-up of hepatic fibrosis. The purpose of this study is to evaluate the possibility of quantifying liver fibrosis in infants by T2 relaxation time measurements. The institutional review board approved this prospective study and parental informed consent was obtained. During MR cholangiopancreatography using a 1.5-T MR scanner in infants with neonatal cholestasis, T2 relaxation time of the liver was calculated with the mean signal intensities measured on images obtained using spin-echo sequences (TR/TE, 2,000/20, 40, 60, 80, 100, 120, 140, 160 ms). A normal control study was performed during spinal MRI in infants with anorectal malformation and normal liver enzyme profiles. A liver biopsy was obtained in the children with cholestasis. The correlation between histopathological fibrosis stage and T2 relaxation time was evaluated by Kendall’s Tau-b test. Twenty-five infants (male: female, 12:13; age range 0–11 months, mean 3.2 months), 14 with neonatal cholestasis (9 BA and 5 non-BA) and 11 normal controls were included in this study. Relaxation times (mean ± standard deviation [SD]) for the liver were 57.8 ms ± 8.8 in the normal control group (n = 11) and 56.8 ms ± 9.6 in the BA group (n = 9) without statistically significant differences (P = 0.811). T2 relaxation times were not significantly different between the low stage (≤F1) and high stage (≥F2) fibrosis (mean 57.8 vs 56.8; P = 0.934). T2 relaxation of a normal infant liver at 1.5-T had a mean value of 57.8 ms, which is comparable with adult data (46–57 ms). However, T2 relaxation time was not different in patients with BA and did not correlate with stage of fibrosis.

Spontaneous bile duct perforation (SBP) is rare in children. Early diagnosis is important because the condition can be treated surgically. The purpose of this study is to report MR cholangiopancreatography (MRCP) findings of SBP and to evaluate the usefulness of MRCP. Over the last 10 years, three children (1 boy, 2 girls; ages 3, 4 and 15 months) underwent US, MRCP and hepatobiliary scintigraphy preoperatively and were surgically confirmed to have extrahepatic bile duct perforation. US showed ascites in all children and a choledochal cyst in one. On MRCP, a moderate-to-large volume of ascites was seen in addition to a loculated fluid collection at the porta hepatis. MRCP also depicted the low insertion of the cystic duct and choledochal cyst in each case. Hepatobiliary scintigraphy showed bile leak from the region of the porta hepatis extending to the whole abdomen. According to the surgical findings, the perforation site was around the junction of the cystic duct and the common hepatic duct in all children. In children with SBP, MRCP can depict the loculated fluid collection adjacent to the perforation site and associated bile duct anomalies.

To describe color Doppler ultrasonographic (US) findings in livers of neonates with biliary atresia (BA) and to compare them with US findings in livers of neonates with non-BA and control subjects.Institutional review board approval was obtained; acquisition of informed consent was exempted. US and color Doppler US findings were retrospectively reviewed in 64 patients with neonatal cholestasis and 19 control subjects. BA and non-BA were confirmed in 29 and 35 patients, respectively. Three pediatric radiologists assessed US and color Doppler US images, independently documented their findings, and resolved discrepancies by consensus. Triangular cord (TC) sign, gallbladder length, and hepatic artery and portal vein diameters were evaluated on US images. The presence of hepatic subcapsular flow was evaluated on color Doppler US images. Diagnostic value of TC sign and hepatic subcapsular flow in the diagnosis of BA were evaluated. Significance of hepatic artery and portal vein diameters in each group was assessed.In the diagnosis of BA, sensitivity and specificity of the TC sign on US images were 62% and 100%, respectively. On color Doppler US images, hepatic subcapsular flow was detected in all patients with BA and in five patients with non-BA. At the first review, there was a discrepancy between radiologists in interpretation of hepatic subcapsular flow in patients with non-BA. However, consensus was reached at the second review. There was no hepatic subcapsular flow in control subjects. Sensitivity and specificity of hepatic subcapsular flow on color Doppler US images were 100% and 80%-86%, respectively, on the basis of individual interpretations of reviewers. Sensitivity and specificity of hepatic subcapsular flow on color Doppler US images were 100% and 86%, respectively, on the basis of consensus reading. Mean diameter of the hepatic artery in patients with BA (2.1 mm +/- 0.7 [standard deviation]) was significantly larger than that in patients with non-BA (1.5 mm +/- 0.4, P.001) and control subjects (1.5 mm +/- 0.4, P = .001).The presence of hepatic subcapsular flow is useful for differentiating between BA and other causes of neonatal jaundice.

Background: The presence and extent of osteolytic bone lesions in untreated patients with multiple myeloma are important factors in the staging of the disease, and the extent of bone lesions in multiple myeloma cases significantly influences decisions regarding therapy. Recently, fluorodeoxyglucose positron emission tomography (FDG-PET) and magnetic resonance imaging (MRI) have been used to detect bone marrow involvement in patients with multiple myeloma. Purpose: To compare the efficacy of FDG-PET and MRI for the detection of bone marrow infiltration into the spine in untreated patients with multiple myeloma. Material and Methods: Twenty-two patients with multiple myeloma underwent both FDG-PET and spine MRI. The examined spinal regions by MRI included 21 thoracic and lumbar spines, one lumbar spine, and 12 cervical spines. The following imaging sequences were performed: T1-weighted spin-echo MRI with and without fat suppression, and T2-weighted spin-echo MRI in the sagittal plane. In the patients with bone marrow abnormalities, an additional contrast-enhanced T1-weighted spin-echo MR image and a fat-suppressed T1-weighted spin-echo MR image were obtained. Patients were divided into three groups on the basis of the criteria defined by Durie and Salmon: stage I ( n=9), stage II ( n=3), and stage III ( n=10). The number and location of lesions detected in both FGD-PET and MRI were recorded, and the lesions were compared using the McNemar test. Bone marrow biopsy results, the patient's clinical examinations, and other imaging findings (MRI, FDG-PET, etc.) were used as references. Results: In stages I and II (37 lesions in 12 patients), FDG-PET and MRI detected lesions in 78% (29 of 37 lesions) and 86% (32 of 37 lesions), respectively. However, the difference between the abilities of FDG-PET and MRI to detect lesions was not statistically significant ( P=0.317). In stage III (101 lesions in 10 patients), FDG-PET and MRI detected lesions in 80% (81 of 101 lesions) and 92% (93 of 101 lesions), respectively. The difference between the abilities of FDG-PET and MRI to detect lesions was statistically significant ( P=0.038). Conclusion: MRI is superior to FDG-PET in detecting bone marrow involvement in the spine of patients with advanced multiple myeloma.

Purpose: The aim of this study was to compare the accuracy of fluorodeoxyglucose-positron emission tomography (FDG-PET) with bone scan for diagnosis of single bone metastasis using a semiquantitative method. Material and Methods: Seventy-six patients with suspected single bone metastasis, who underwent both FDG-PET and a bone scan, were selected. The number and location of lesions detected upon both FDG-PET and bone scan were recorded, and the lesions were compared using the McNemar test. For semiquantitative analysis, a maximum (max) standard uptake value (SUV) of 2.5 was used as the positive cutoff value for metastasis. The difference in max SUV value among 3 groups (osteolytic, osteoblastic, and benign lesions) was assessed using the Student-Newman-Keuls method. Biopsy results, other imaging findings (multirow detector computed tomography, magnetic resonance imaging), and the patient's clinical course were used as references. Results: There were 47 single bone metastases and 29 benign lesions. The sensitivity, specificity, and accuracy of bone scans for diagnosing bone metastases were 89%, 41%, and 71%, respectively, and those of FDG-PET were 85%, 52%, and 72%, respectively. These data were not significantly different (P > 0.05). Using a max SUV of 2.5 as the positive cutoff value for metastasis, the specificity and accuracy of FDG-PET, 83% for each, improved. When classifying bone metastasis as osteoblastic or osteolytic, the max SUV was significantly higher in osteolytic metastasis than in osteoblastic and benign lesions (P = 0.001). Conclusions: Fluorodeoxyglucose-positron emission tomography (FDG-PET) using the semiquantitative parameter SUV improves the diagnostic ability to differentiate between single bone metastases and benign lesions.

Gastrointestinal tract duplications are uncommon congenital abnormalities arising anywhere along the alimentary tract. The most common modalities used to image duplications are US and contrast medium examinations. CT and MRI are less often used, but can be helpful in difficult cases that require a multiplanar approach. In this article, we discuss and illustrate a wide spectrum of gastrointestinal tract duplications from the oesophagus to the rectum, and illustrate the associated abnormalities of gastrointestinal tract duplications.

Purpose: The aim of our study was to compare the efficacy of multidetector row computed tomography (MDCT) with magnetic resonance imaging (MRI) and fluorodeoxyglucose-positron emission tomography (FDG-PET) for detection of spinal bone marrow involvement in patients with multiple myeloma. Materials and Methods: Ten patients with multiple myeloma stage III underwent MDCT and MRI of the spine and FDG-PET. The number and location of lesions detected by 3 modalities were recorded, and a lesion-by-lesion analysis was completed, using McNemar test. For MDCT, image analysis was performed according to the type of lesion (established by Laroche et al), and the efficacy of lesion detection was compared with that of the MRI and FDG-PET. P values less than 0.05 were considered statistically significant. Results: The MDCT, MRI, and FDG-PET detected the following numbers of lesions: 102 of 140 vertebrae, 95 of 140 vertebrae, and 84 of 140 vertebrae, respectively. The difference between the abilities of MDCT and MRI to detect lesions was not statistically significant (P = 0.289). However, the difference in effectiveness between MDCT and FDG-PET was statistically significant (P < 0.001). For small osteolytic lesions, less than 5 mm, the difference in effectiveness between MDCT and MRI was also statistically significant (P = 0.031). Conclusions: The MDCT is very sensitive in detecting small osteolytic lesions in the spine, as compared with MRI and FDG-PET.

The formation of inguinal scrotal hernias is usually aided by factors that act to increase the intra-abdominal pressure, such as vigorous crying, prematurity, chronic lung disease, ascites, and bowel disease in neonates and children. 1 The diagnosis is made by physical examination without difficulty in most cases. However, in the fetus, because physical examination is not possible, and the frequency is probably much less frequent than after birth, the diagnosis of a hernia is more difficult than in the neonate or infant. The clues to diagnosis of an inguinoscrotal hernia in the fetus have been reported as peristaltic movement of herniated bowel and paucity of blood flow on prenatal sonography. 1 We report a case of a fetal inguinoscrotal hernia that appeared as a solid mass by sonographic examination. Contrary to previous reports, the mass had blood vessels in it, and no peristaltic movement was seen during the sonographic examination. The fetal magnetic resonance imaging (MRI) features and outcome of the fetus are also presented.

The purpose of this study was to determine whether MR cholangiopancreatography can accurately depict anomalous pancreaticobiliary ductal union in children with choledochal cysts.Twenty children (age range, 1 month-13 years; mean age, 4.6 years; all girls) who were diagnosed with choledochal cyst by sonography underwent MR cholangiopancreatography with a single-shot fast spin-echo sequence. The type of choledochal cyst and anomalous pancreaticobiliary ductal union were characterized on the basis of MR cholangiopancreatographic findings and were compared with the finding of intraoperative cholangiography.The type of choledochal cyst (type Ia, n = 4; type Ic, n = 7; type IVa, n = 7; type IVb, n = 2) determined on MR cholangiopancreatography correlated with that identified on intraoperative cholangiography in each patient. Anomalous pancreaticobiliary ductal union was detected by MR cholangiopancreatography and intraoperative cholangiography in 12 (60%) and 16 (80%) of 20 patients, respectively. The types of anomalous pancreaticobiliary ductal union as determined on MR cholangiopancreatography (type A, n = 2; type B, n = 7; type C, n = 3) were concordant with those of intraoperative cholangiography in 11 of 12 patients. In five of eight patients with choledochal cyst (type Ia, n = 1; type IVa, n = 5; type IVb, n = 2) in whom MR cholangiopancreatography could not depict anomalous pancreaticobiliary ductal union, anomalous pancreaticobiliary ductal union was documented on intraoperative cholangiography that was performed after choledochal cyst resection.MR cholangiopancreatography provides diagnostic information about anomalous pancreaticobiliary ductal union in children with choledochal cyst.

Purpose: The aim of this study was to evaluate the usefulness of magnetic resonance cholangiography (MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods: Forty-seven consecutive infants with cholestatic jaundice underwent single-shot MRC. The diagnosis of biliary atresia was made by MRC based on the nonvisualization of extrahepatic bile ducts and excluded on the basis of the complete visualization of extrahepatic bile ducts. The final diagnosis of biliary atresia (BA group, n=23) or nonbiliary atresia (NBA group, n=24) was established by operation or clinical follow-up until the jaundice resolved. Results: The extrahepatic bile ducts including the gallbladder, the cystic duct, the common bile duct, and the common hepatic duct were visualized in 23 of the 24 infants of the NBA group. The extrahepatic bile ducts, except the gallbladder, were not depicted in any infant of the BA group. MRC had an accuracy of 98%, sensitivity of 100% and specificity of 96%, for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions: MRC is a very reliable noninvasive imaging modality for the diagnosis of biliary atresia. In infants with cholestatic jaundice and considered for exploratory laparotomy, MRC is recommended to avoid unnecessary surgery. J Pediatr Surg 37:599-604. Copyright 2002, Elsevier Science (USA). All rights reserved.

New CT reconstruction techniques may help reduce the burden of ionizing radiation. To quantify radiation dose reduction when performing pediatric chest CT using a low-dose protocol and 50% adaptive statistical iterative reconstruction (ASIR) compared with age/gender-matched chest CT using a conventional dose protocol and reconstructed with filtered back projection (control group) and to determine its effect on image quality in normal weight and overweight children. We retrospectively reviewed 40 pediatric chest CT (M:F = 21:19; range: 0.1–17 years) in both groups. Radiation dose was compared between the two groups using paired Student’s t-test. Image quality including noise, sharpness, artifacts and diagnostic acceptability was subjectively assessed by three pediatric radiologists using a four-point scale (superior, average, suboptimal, unacceptable). Eight children in the ASIR group and seven in the control group were overweight. All radiation dose parameters were significantly lower in the ASIR group (P

In this chapter, we covered imaging findings and clinical manifestations of soft tissue disorders excluding inflammatory conditions. Soft tissue inflammatory disorders with/without infectious causes are covered elsewhere in this textbook. Soft tissue disorders are largely described in this chapter largely categorized as nonneoplastic and neoplastic diseases. As for nonneoplastic disorders, calcification, ossification, local and generalized vascular lesions, and compartment syndrome are discussed and/or illustrated. Brief description and illustrations on neoplastic disorders for both benign (fibromatosis colli, infantile myofibromatosis, nodular fasciitis, neurogenic tumor, lipoblastoma, pilomatricoma) and malignant (rhabdomyosarcoma, synovial sarcoma, infantile fibrosarcoma, and the other relatively rare malignant soft tissue tumors) neoplasm will follow.

We describe the findings on single-photon emission computed tomography (SPECT) in patients with perinatal asphyxia at term, with perirolandic cortico-subcortical changes on MRI, and to correlate them with clinical features. SPECT of 7 patients was obtained after injection of 185-370 MBq of Tc-99m-ECD (ethyl cysteinate dimer). The patients had spastic quadriplegia (7/7) with perinatal asphyxia (6/7) at term (7/7). The results were correlated with the MRI findings. Hypoperfusion of the perirolandic cortex was clearly seen on SPECT in all patients, even in two with subtle changes on MRI. SPECT demonstrated a more extensive area of involvement than MRI, notably in the cerebellum (in 4), the thalamus (in 7) and basal ganglia (in 5), where MRI failed to show any abnormalities.

It has been well documented that the extracellular matrix components fibronectin and laminin promote or regulate morphogenesis of the myocardial cells in mammalian heart. However, their chronological change of expression (or localization) in the human heart remains elusive. In this study, fibronectin and laminin in the left ventricle of forty-two human fetuses aged from 8 to 26 weeks gestation and left ventricular tissues obtained from a 2-week old infant and two adults were investigated by Western blot analyses and indirect immunofluorescence technique with monoclonal antibodies. In the fetal heart, fibronectins were present along the endocardium, epicardium, and linings of larger blood vessels. In 14-16 weeks gestation, fibronectin immunofluorescence became stronger but not evenly dispersed in the interstitium. After 24 weeks gestation, they were strongly positive only in the relatively larger blood vessels, as well as those in the infant and adult cardiac tissues. Laminins were strongly positive along the endocardium and basement membrane of the myocardial cells and fibroblasts during fetal life. After birth, laminins formed fine fibrillar network along the basement membrane in association with the transverse tubules of myocardial cell; these morphological characteristics remained in the adult cardiac tissues. These results indicate that fibronectin expression is relatively constant during fetal life but decreases after birth; in contrast, laminin expression is not age-dependent and constant throughout the life.

A peritonsillar abscess is one of the most commonly occurring deep space infections of the head and neck in adults and children. A peritonsillar abscess that appears in newborns, however, is extremely rare. The treatment of a peritonsillar abscess requires both the selection of appropriate antibiotics and the best procedure to remove the abscessed material. We report a case of a peritonsillar abscess in a 40-day-old infant who was treated with antibiotic therapy alone.

Eosinophilic fasciitis is a rare disease characterized by diffuse fasciitis with peripheral eosinophilia and progressive induration and thickening of the skin and soft tissues. We report a 19-year-old female who presented with pitting edema in both lower extremities. She had a history of excessive physical activity before her symptoms developed. Physical examination revealed 2+ pitting edema in both lower legs. She complained of mild pain in both knee joints and feet, with no tenderness or heating sensations. Laboratory results were unremarkable except for severe eosinophilia. Parasite infection, venous thrombosis, and cardiac and renal problems were excluded. A magnetic resonance imaging study of both lower extremities revealed increased signal intensity in the subcutaneous lesions, consistent with superficial inflammation of the fascia. Mixed perivenular lymphoplas macytic and eosinophilic infiltration in the subcutaneous lesion were observed on biopsy. The patient was treated with corticosteroids, resulting in remarkable improvement in both edema and eosinophilia.

The pulmonary interstitial emphysema (PIE) is a life-threatening illness in premature infants with mechanical ventilation. While most are managed conservatively, decompression would be necessary. Here, we report the first case of PIE treated by percutaneous catheter insertion in an extremely low birth weight (ELBW) infant in Korea. The patient, born with 660 g in 23+2 weeks of gestation, showed PIE in left lower lung on postnatal day 12. Percutaneous catheter insertion was performed on postnatal day 25. The size of PIE decreased, but didn't disappear completely. On postnatal day 42, we exchanged catheter and inserted additional catheter in pleural space. However, sudden desaturation and pneumothorax occurred on postnatal day 44. We changed catheter in pleural space, and pneumothorax and PIE improved. Finally, we successfully removed catheters, and weaned patient out. As in our case, percutaneous catheter insertion would be a useful option for ELBW infants with PIE.

Aim To compare the reliability of the conventional ultrasonography grading system for hydronephrosis as suggested by the Society for Fetal Urology (SFU) in 1993 and that developed by Onen in 2007. Materials and methods One hundred and eighty kidneys in 90 paediatric patients were assessed by four radiologists using each of the two grading systems twice. The SFU system was graded 0–4 (0 = no hydronephrosis; 1 = visualized only renal pelvis; 2 = plus a few caliceal dilatation; 3 = all calyceal dilatation; 4 = plus parenchymal thinning). The Onen system was graded 0–4 (0 = no hydronephrosis; 1 = only renal pelvic dilatation; 2 = plus caliceal dilatation; 3 = plus 50% renal parenchymal loss). Cohen's kappa statistic was used to estimate intra- and interobserver agreement. The weighted least-squares approach was used to compare the intra-observer agreement, and bootstrapping was used to compare the interobserver agreement between the two systems. Results Intra-observer agreement was substantial to almost perfect in both the SFU (κ 0.79–0.95) and the Onen (κ 0.66–0.97) grading system without difference. The overall interobserver agreement was substantial in both the SFU (κ 0.61–0.68) and the Onen (κ 0.66–0.76) grading system. However, interobserver agreement was fair to moderate for SFU grades 1 and 2 and Onen grades 2 and 3. Conclusion Both the SFU and Onen grading system are reliable with good intra- and interobserver agreement. However, decreased interobserver agreement was demonstrated for SFU grades 1 and 2 and Onen grades 2 and 3.

To retrospectively compare radiation dose and image quality of pediatric chest CT using a routine dose protocol reconstructed with filtered back projection (FBP) (the Routine study) and a low-dose protocol with 50% adaptive statistical iterative reconstruction (ASIR) (the ASIR study).We retrospectively reviewed chest CT performed in pediatric patients who underwent both the Routine study and the ASIR study on different days between January 2010 and August 2011. Volume CT dose indices (CTDIvol), dose length products (DLP), and effective doses were obtained to estimate radiation dose. The image quality was evaluated objectively as noise measured in the descending aorta and paraspinal muscle, and subjectively by three radiologists for noise, sharpness, artifacts, and diagnostic acceptability using a four-point scale. The paired Student's t-test and the Wilcoxon signed-rank test were used for statistical analysis.Twenty-six patients (M:F=13:13, mean age 11.7) were enrolled. The ASIR studies showed 60.3%, 56.2%, and 55.2% reductions in CTDIvol (from 18.73 to 7.43 mGy, P0.001), DLP (from 307.42 to 134.51 mGy×cm, P0.001), and effective dose (from 4.12 to 1.84 mSv, P0.001), respectively, compared with the Routine studies. The objective noise was higher in the paraspinal muscle of the ASIR studies (20.81 vs. 16.67, P=0.004), but was not different in the aorta (18.23 vs. 18.72, P=0.726). The subjective image quality demonstrated no difference between the two studies.A low-dose protocol with 50% ASIR allows radiation dose reduction in pediatric chest CT by more than 55% while maintaining image quality.

Background It remains to be determined whether spine-targeted computed tomography (thoracolumbar spine computed tomography [TLS-CT]) images and visceral organ-targeted CT (abdominopelvic [AP]-CT) images are comparable for the evaluation of thoracolumbar spinal fractures using 16-channel multidetector row CT. The elimination of an additional spine-targeted CT protocol would substantially reduce time, the storage burden, and potential patient radiation exposure. Methods A total of 420 vertebrae in 72 consecutive patients who underwent AP-CT to assess blunt traumatic injury and an additional CT examination using a TLS-CT protocol to evaluate spinal fractures were retrospectively evaluated. The AP-CT set (set A, reconstructed with using a wide display field of view [FOV] and a soft algorithm) and the TLS-CT set (set S, reconstructed using a narrow display FOV and a hard algorithm) were composed of axial plus reformatted sagittal or coronal images or both. Three radiologists independently reviewed all CT data retrospectively. Performances for detecting and typing fractures were compared by using areas under receiver operating characteristic curves and by determining concordance rates. Results The overall areas under the curves for sets S and A for fracture detection were 0.996 and 0.995, respectively; no significant difference was found between the two sets. Concordance rates for typing performance also showed no statistical significance between the two sets for any of the three observers. Conclusion Sixteen-channel multidetector row CT images reconstructed using a soft algorithm and a wide display FOV that cover the entire abdomen using a visceral organ-targeted protocol with 1.5-mm collimation are sufficient for the evaluation of spine fractures in trauma patients, given that multiplanar-reformatted images are provided.

Background: Magnetic resonance (MR) imaging and measurement of glycosaminoglycan (GAG) have potential for characterization of hyaline articular cartilage. Recently, some reports have demonstrated the potential of direct administration of contrast media for MR imaging of cartilage. Purpose: To prove the feasibility of intraarticular gadolinium-enhanced MR imaging of cartilage (iGEMRIC) and T1 relaxation mapping of the articular cartilage in vivo with intraarticular injection of Gd-DTPA2−. Material and Methods: Five healthy beagle dogs underwent MR imaging and T1 relaxation mapping of the knee joints of both hind legs. The delayed gadolinium-enhanced MR imaging of cartilage (dGEMRIC) and iGEMRIC techniques were interchanged with MR imaging. For dGEMRIC, a double routine dose of Gd-DTPA2− (0.2 mM/kg) was administered intravenously. For iGEMRIC, 2.5 and 1.25 mmol/l saline-diluted Gd-DTPA2− solutions were separately injected into the right and left knee joints, respectively, prior to MR imaging. Color-coded T1 maps of 20 femoral condyles were obtained from the dGEMRIC and iGEMRIC images. The signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and glycosaminoglycan (GAG) delineation of articular cartilage were compared between the dGEMRIC and iGEMRIC techniques. Results: The mean SNR was higher with dGEMRIC than with iGEMRIC, but the difference was not statistically significant ( P = 0.174). The mean (±SD) CNR was higher with iGEMRIC (−11.6±3.4) than with dGEMRIC (−16.7±4.0; P = 0.000), although the absolute value of the CNR was higher with dGEMRIC. The layering and gradient distribution of GAG were more clearly visualized on the iGEMRIC images. The mean scores of GAG delineation with dGEMRIC and iGEMRIC were 0.7±0.6 and 2.2±1.7, respectively. The iGEMRIC method better visualized GAG distribution ( P = 0.001). Conclusion: Although the SNR did not differ significantly between the iGEMRIC and dGEMRIC techniques, the color-coded T1 map produced with iGEMRIC allowed better cartilage evaluation. Thus, iGEMRIC exhibits the useful features of both MR arthrography and dGEMRIC, and provides a color-coded T1 map that is useful for diagnosing early articular cartilage damage.

Camurati-Engelmann disease (CED) is an autosomal dominant progressive diaphyseal dysplasia caused by mutations in the transforming growth factor-beta1 (TGFB1) gene. We report the first Korean family with an affected mother and son who were diagnosed with CED. The proband is a 19-yr-old male with a history of abnormal gait since the age of 2. He also suffered from proximal muscle weakness, pain in the extremities, and easy fatigability. Skeletal radiographs of the long bones revealed cortical, periosteal, and endosteal thickenings, predominantly affecting the diaphyses of the upper and lower extremities. No other bony abnormalities were noted in the skull and spine and no remarkable findings were seen on laboratory tests. The patient's mother had a long-standing history of mild limb pain. Under the impression of CED on radiographic studies, we performed mutation analysis. A heterozygous G to A transition at cDNA position +653 in exon 4 of the TGFB1 gene (R218H) was detected in the patient and his mother.

Skeletal muscle metastasis is a rare condition in renal cell carcinoma (RCC). We here present an extremely rare case of late recurrence in a patient with RCC who had multiple muscle metastases 19 years after radical nephrectomy. This case shows that a soft-tissue mass in a patient with a known history of RCC even after curative nephrectomy, particularly with hypervascularity, must be regarded as highly suspicious of skeletal muscle metastasis.

Objective To assess whether multi-echo Dixon magnetic resonance (MR) imaging with simultaneous T2* estimation and correction yields more accurate fat-signal fraction (FF) measurement of the lumbar paravertebral muscles, in comparison with non-T2*-corrected two-echo Dixon or T2*-corrected three-echo Dixon, using the FF measurements from single-voxel MR spectroscopy as the reference standard. Materials and Methods Sixty patients with low back pain underwent MR imaging with a 1.5T scanner. FF mapping images automatically obtained using T2*-corrected Dixon technique with two (non-T2*-corrected), three, and six echoes, were compared with images from single-voxel MR spectroscopy at the paravertebral muscles on levels L4 through L5. FFs were measured directly by two radiologists, who independently drew the region of interest on the mapping images from the three sequences. Results A total of 117 spectroscopic measurements were performed either bilaterally (57 of 60 subjects) or unilaterally (3 of 60 subjects). The mean spectroscopic FF was 14.3 ± 11.7% (range, 1.9-63.7%). Interobserver agreement was excellent between the two radiologists. Lin's concordance correlation between the spectroscopic findings and all the imaging-based FFs were statistically significant (p < 0.001). FFs obtained from the T2*-corrected six-echo Dixon sequences showed a significantly better concordance with the spectroscopic data, with its concordance correlation coefficient being 0.99 and 0.98 (p < 0.001), as compared with two- or three-echo methods. Conclusion T2*-corrected six-echo Dixon sequence would be a better option than two- or three-echo methods for noninvasive quantification of lumbar muscle fat quantification.

PURPOSE To evaluate the immediate and 1-year clinical outcomes of mechanical thrombectomy with use of the Arrow-Trerotola percutaneous thrombectomy device (PTD) with or without low-dose urokinase in the treatment of acute iliofemoral deep vein thrombosis (DVT) MATERIALS AND METHODS Mechanical thrombectomy with the PTD was performed in 25 patients with acute iliofemoral DVT. Thrombolytic therapy with low-dose urokinase was used in all patients without contraindications ( n = 20). Other therapies used in combination included inferior vena cava filter insertion ( n = 5), sheath aspiration thrombectomy ( n = 25), and angioplasty and stent placement ( n = 20) RESULTS Initial technical and clinical success was achieved in all cases. In the 20 patients who had no contraindications to the use of urokinase, the dosage of urokinase did not exceed 1 million IU (range, 360,000–1,000,000 IU; mean, 640,000 IU). The mean time of urokinase infusion was 16 hours (range, 12–20). In five patients who had a contraindication to the use of urokinase, mechanical thrombectomy with the PTD was successful without the use of urokinase. There were no major complications. Primary patency of the stent-implanted common iliac vein segment was achieved at 1 year in 17 of 20 patients (85%). The overall 1-year clinical success rate was 92% (23 of 25 patients). Valvular insufficiency occurred in two patients (8%) CONCLUSION The PTD is an effective mechanical thrombectomy device in the treatment of acute iliofemoral DVT with or without adjunctive urokinase thrombolysis

Background. Proper recognition, classification and assessment of the tracheobronchial anatomy are essential for surgical management planning in left pulmonary artery sling (LPAS). Objective. To demonstrate the various imaging modalities in diagnosing and assessing anatomical relationships with tracheobronchial tree in LPAS. Materials and methods. Seven patients (three infants, three children, one adult) with LPAS were evaluated. The confirmative imaging modalities included electron-beam tomography (EBT, n = 2), MRI (n = 1) and helical CT (n = 4). Other imaging modalities included plain chest radiographs (n = 7), barium oesophagogram (n = 2), echocardiography (n = 3), cardiac and pulmonary angiography (n = 2) and bronchoscopy (n = 5). Results. There were two cases of type IA, one case of type IIA and four cases of type IIB. All five cases of type II had a long segment of tracheobronchial stenosis. A long segment of thoracic tracheal stenosis was unusually associated in one case of type IA. All confirmative modalities were sufficient for diagnosing LPAS and assessing the tracheobronchial tree anomaly. However, helical CT could not obviate angiography due to associated congenital cardiac defects. Conclusions. Sectional imaging modalities of EBT, MRI and helical CT were all sufficient for diagnosing and assessing the anatomical relationships of the tracheobronchial tree in LPAS.

Mesenchymal hamartoma of the chest wall is a rare tumor with about 53 reported cases in the English literature. We reviewed six chest wall mesenchymal hamartomas in four patients, including two cases with multiple lesions, with specific focus on the radiologic and pathologic correlation. All cases occurred in neonates or infants with ages ranging from seven hours to seven months. They were diagnosed with plain chest radiographs (n=6), ultrasonography (n=2), chest CT scan (n=6), whole body bone scan (n=2) and MRI (n=3). All cases except a small one without cystic change showed the typical features of mesenchymal hamartoma radiographically and pathologically. Radiologically they were well-circumscribed masses with solid and cystic components with multiple fluid-fluid levels in association with single or multiple rib destruction or change. The CT scan showed the typical findings of chest wall hamartoma, and the MR showed heterogeneous signal intensities of the mass on T1- and T2-weighted images. The MR also revealed more concisely a secondary aneurysmal bone cyst formation with multiple fluid-fluid levels on the T2-weighted image. Microscopically, they showed alternating areas of cartilaginous islands and primitive appearing mesenchymal proliferation, which corresponded well with the solid component on the radiologic findings. The areas of bone formation and blood-filled cystic spaces matched the calcified or ossified densities and the cystic components, respectively. A small case without cystic change showed peculiar radiological and pathological findings resembling an osteochondroma. In conclusion, mesenchymal hamartoma of the chest wall in infancy is quite rare and sometimes can be misdiagnosed as malignancy due to the bone-destroying radiographic appearance and the highly cellular and mitotically active microscopic features, unless the radiologists and pathologists are aware of the characteristic clinical, radiological, and pathological findings. Imaging studies can usually make a correct diagnosis with good correlation to the pathologic findings.

To correlate a triangular area of high signal intensity in the porta hepatis on T2-weighted magnetic resonance (MR) cholangiograms of biliary atresia with ultrasonographic (US) and histopathologic findings in a portal mass observed during a Kasai procedure.Twenty-one consecutive neonates and infants (age range, 13-88 days; mean age, 59 days) with cholestasis underwent US and single-shot MR cholangiography. In 12 patients with biliary atresia diagnosed at histopathologic examination, MR cholangiographic findings in the porta hepatis were correlated with US and histopathologic findings in the portal mass.At US, eight of the 12 patients had round, linear, or tubular hypoechoic portions within a triangular cord; MR cholangiography revealed a triangular area of high signal intensity confined to the porta hepatis. Histopathologic examination of the portal mass revealed a cystic or cleftlike lesion surrounded by loose myxoid mesenchyme and platelike fetal bile ducts. Neither the large cystic lesion without ductal epithelium nor the small cleftlike lesion with scanty epithelium demonstrated bile staining. Similar areas of high signal intensity were not seen on T2-weighted images in the remaining patients (four with biliary atresia and nine with neonatal hepatitis).In biliary atresia, T2-weighted single-shot MR cholangiography can show a triangular area of high signal intensity in the porta hepatis that may represent cystic dilatation of the fetal bile duct.