Investigative MRI cholangiopancreatography for primary sclerosing cholangitis-type lesions in children with IBD.

OBJECTIVES: The aim of the study was to estimate the frequency of primary sclerosing cholangitis (PSC)-type lesions in children with inflammatory bowel disease (IBD) by means of magnetic resonance cholangiopancreatography (MRCP), and to investigate the association between a series of easily applicable data on the one hand and the presentation of such lesions at MRCP on the other hand. METHODS: Collected demographic, laboratory, and magnetic resonance enterography data from the records of 73 children with IBD were cross-sectionally related to the MRCP-based diagnosis. RESULTS: Around the time of MRCP, the distribution of IBD subtypes was 64.4%, 24.7%, and 11% for Crohn disease, indeterminate colitis, and ulcerative colitis, respectively. A total of 11 patients (15.1%) were identified with PSC-type lesions. Demographic and magnetic resonance enterography data were unrelated to the MRCP outcome. Biochemical abnormalities were of low prevalence (<50%) among patients with PSC. The abnormality prevalences of aspartate transaminase, alanine transaminase, and γ-glutamyl transferase were significantly higher in the PSC group, both at initial diagnosis of IBD and at the time of MRCP. Less-consistent results were documented for bilirubin and alkaline phosphatase, especially at initial diagnosis of IBD. CONCLUSIONS: The abnormality prevalences of aspartate transaminase, alanine transaminase, and γ-glutamyl transferase were significantly higher in the PSC group. Nevertheless, PSC-type lesions frequently occur in pediatric IBD, even if the biochemical profile is hardly indicative of this probability.

Use of fat suppression in R2 relaxometry with MRI for the quantification of tissue iron overload in beta-thalassemic patients.

PURPOSE: To assess the performance and results of R(2) relaxometry using a fat-suppressed (FS) multiecho sequence and compare these to conventional R(2) relaxometry in estimating tissue iron overload. MATERIALS AND METHODS: Relaxation rate values (R(2)=1/T2) of the liver, spleen, pancreas and vertebral bone marrow (VBM) were estimated in 21 patients with ß-thalassemia major, using a respiratory-triggered 16-echo Carr-Purcell-Meiboom-Gill (CPMG) spin-echo sequence before (R(2)) and after (R(2) FS) the application of chemically selective fat suppression. RESULTS: Hepatic and splenic R(2) FS values correlated with respective R(2) values (r=0.98 and r=0.96, P<.001), whereas correlations between R(2) FS and R(2) values for pancreas and VBM were not statistically significant. Bland-Altman plots show disagreement between R(2) and R(2) FS values, particularly for pancreas and VBM. Hepatic, pancreatic and VBM R(2) FS values correlated with serum ferritin (r=0.88, P<.001; r=0.51, P<.003; and r=0.75, P<.002, respectively). Hepatic R(2) FS values correlated with splenic R(2) FS (r=0.77, P<.03), pancreatic R(2) FS (r=0.61, P<.006) and VBM R(2) FS values (r=0.70, P<.001), whereas pancreatic R(2) FS values correlated also with VMB R(2) FS values. On the contrary, among the R(2) values of the above tissues, obtained without fat suppression, only hepatic R(2) values correlated with serum ferritin, whereas no correlation was documented between hepatic and pancreatic or VBM R(2) values. The application of fat suppression did not improve breathing or flow artifacts. CONCLUSION: Application of fat suppression in the standard CPMG sequence improved the capability of MRI in noninvasive quantification of iron, particularly in lipid-rich tissues, such as vertebral bone marrow (VBM) and pancreas.

Massive Uterine Leiomyoma in a Patient with Friedreich's Ataxia: Is There a Possible Association?

A possible association between Friedreich's ataxia (FA) and neoplastic development has been recognized. FA patients have low frataxin levels and insufficient response to oxidative stress. In these patients fibroblasts are characterized by a high rate of mutations. Herein, a case of a 39-year-old woman with FA tetraplegia, who was admitted in our department with intestinal obstruction due to a huge uterine tumor, is described. An abdominal CT revealed a huge intra-abdominal mass originating from the right cornu of the uterus. Tumor excision and adhesionlysis were performed. The histological examination of the tumor revealed a leiomyoma. FA patients seem to present with a variety of neoplasms uncommon for their young age. This is the first report of a leiomyoma originating from the genital system in a female patient with FA tetraplegia. Therefore it is important to identify neoplasms at an early stage in patients with FA and start immediate therapy.

Magnetic resonance imaging of the small bowel in children with idiopathic inflammatory bowel disease: evaluation of disease activity.

BACKGROUND: Examinations using ionizing radiation are frequently used in the evaluation of disease activity in children affected by idiopathic inflammatory bowel disease (IBD). OBJECTIVE: To develop an MR imaging protocol without the need for fluoroscopic insertion of an enteral tube and to assess the disease activity in children with IBD. MATERIALS AND METHODS: Included in the study were 37 children (22 girls and 15 boys; age range 7-15 years, mean 11.67 years) with IBD who underwent MR imaging of the small bowel. Of these 37 children, 32 had Crohn disease and 5 had indeterminate colitis. A water solution containing herbal fibres was administered orally or through a nasogastric tube. Patients were imaged on a 1.5-T MR scanner with T1-weighted and Tau2-weighted sequences followed by a dynamic study using 3-D T1-W images after intravenous administration of gadolinium. RESULTS: The percentage enhancement of the bowel wall was significantly increased in patients with abnormal C-reactive protein (CRP) values compared to patients with CRP values in the normal range (P<0.001). A relatively weak but significant correlation between percentage enhancement of the bowel wall and CRP values was noted during all phases of enhancement. CONCLUSION: This MR imaging protocol is a safe and well-tolerated method for evaluating disease activity and extraintestinal manifestations of IBD in children.

Assessment of iron distribution between liver, spleen, pancreas, bone marrow, and myocardium by means of R2 relaxometry with MRI in patients with beta-thalassemia major.

PURPOSE: To investigate the correlation between the degree of hepatic, splenic, pancreatic, vertebral bone marrow (VBM), and myocardial siderosis, as expressed by relaxation rate (R2 = 1/T2) values, in patients with thalassemia. MATERIALS AND METHODS: R2 relaxation rate values of liver, spleen, VBM, pancreas, and myocardium were estimated in 68 consecutive transfusion-dependent patients with beta-thalassemia major and 10 healthy controls using a respiratory triggered 16-echo Carr-Purcell-Meiboom-Gill (CPMG) spin echo sequence. RESULTS: Hepatic R2 values were significantly increased in all 68 patients; VBM, pancreatic, and myocardial R2 values were increased in 67/68, 35/47, and 47/61 patients, whereas five patients showed decreased pancreatic R2 attributed to fatty degeneration. Of the 39 nonsplenectomized patients, splenic R2 values were decreased in 30 and normal in nine patients. Hepatic R2 values correlated with splenic (r = 0.63, P < 0.001), VBM (r = 0.52, P < 0.001), but not with myocardial and pancreatic R2 values. CONCLUSION: Despite positive correlations between the degree of hepatic, splenic, and VBM siderosis, as expressed by respective R2 values, there was variability of iron distribution patterns in thalassemic patients. Unpredictable patterns of iron distribution may be seen, such as normal signal of the spleen in the presence of siderotic liver, resembling primary hemochromatosis. Fatty degeneration of the pancreas was not uncommon.

MR imaging findings of an atypical pulmonary hamartoma in a 12-year-old child.

We present the MR imaging findings in an atypical pulmonary hamartoma in a 12-year-old boy. CT showed no evidence of fat or calcifications in the tumour. It demonstrated peripheral rim enhancement on arterial-phase MR images due to a compressed respiratory epithelium, and progression to nearly homogeneous contrast enhancement on delayed-phase images. Small cystic spaces were identified on MR hydrographic and contrast-enhanced images formed by invaginating respiratory epithelium, a unique imaging finding in this condition.

Bright-dark ring sign in MR imaging of hepatic epithelioid hemangioendothelioma.

We describe the imaging findings in a rare case of multifocal nodular form of hepatic epithelioid hemangioendothelioma on ultrasound (US) and dynamic contrast-enhanced spiral computed tomography (CT) and MRI. The nodules showed multilayered target appearance on MR images with prominent peripheral rim with high signal intensity (SI) on T1-weighted and very low SI on T2-weighted images, corresponding to thrombosed vascular channels. The above combination of imaging findings is to our knowledge distinct and should suggest the diagnosis of hepatic epithelioid hemangioendothelioma.

High-resolution CT findings in mild pulmonary fat embolism.

OBJECTIVE: The aim of this article is to describe the high-resolution CT (HRCT) findings in mild cases of fat embolism syndrome (FES). MATERIAL AND METHODS: Nine patients with FES were examined with HRCT of the lungs (collimation, 1 mm/edge-enhancement algorithm). The median age of the patients was 26 years (range, 17 to 35 years). Five cases were included prospectively, and four cases were reviewed retrospectively. Of the major clinical criteria for FES, respiratory signs were present in six patients, CNS signs were present in two patients, and petechiae was present in six patients. HRCT patterns were recorded and analyzed. The type of injury and FES-associated clinical findings were also recorded. RESULTS: HRCT findings included ground-glass opacities in seven patients, associated with thickened interlobular septa in five patients and a patchy distribution resulting in a geographic appearance in four patients. A nodular pattern was observed in two patients. Resolution of the abnormalities occurred within 16.4 days (range, 7 to 25 days). CONCLUSION: The HRCT findings of mild fat embolism consist of bilateral ground-glass opacities and thickening of the interlobular septa. Centrilobular nodular opacities are present in some patients.