MRI for Response Assessment of Extensive Lymphatic Malformations in Children Treated With Sirolimus.

BACKGROUND. Extensive lymphatic malformations (LMs) may cause substantial morbidity. The mammalian target of rapamycin (mTOR) inhibitor sirolimus shows promise for treating vascular anomalies, although response assessment is not standardized. OBJECTIVE. The purpose of this study was to retrospectively characterize changes seen on MRI of children with extensive LMs treated with sirolimus. METHODS. Twenty-five children treated with sirolimus for extensive LMs were included. Baseline MRI was defined as the MRI examination performed closest to therapy initiation; follow-up MRI was defined as the most recent MRI examination performed while the patient was receiving therapy. Two pediatric radiologists independently determined MRI lesion volume by tracing lesion contours on all slices (normalized to patient body surface area expressed in square meters) and determined signal by placing an ROI on the dominant portion of the lesions (normalized to CSF signal) on baseline and follow-up T2-weighted MRI sequences. Interreader agreement was determined, and values were averaged for further analysis. Volume and signal changes were compared with patient, lesion, and treatment characteristics. RESULTS. The mean (± SD) interval between initiation of sirolimus treatment and follow-up MRI was 22.1 ± 13.8 months. The mean lesion volume index on baseline and follow-up MRI was 728 ± 970 and 345 ± 501 mL/m2, respectively (p < .001). Ninety-two percent of children showed a decrease in lesion volume index that was greater than 10% (mean volume change, -46.4% ± 28.2%). Volume change was inversely correlated with age (r = -0.466; p = .02). The mean volume change was -64.7% ± 25.4% in children younger than 2 years old versus -32.0% ± 21.6% in children 2 years old or older (p = .008). The mean volume change was -58.1% ± 24.0% for craniocervical lesions versus -35.5% ± 28.2% for lesions involving the trunk and/or extremities (p = .03). Mean lesion signal ratio on baseline and follow-up MRI was 0.81 ± 0.29 and 0.59 ± 0.26, respectively (p < .001). Mean signal ratio change was -23.8% ± 22.7%. Volume and signal changes were moderately correlated (r = 0.469; p = .02). Volume and signal changes were not associated with sex, lesion subtype, serum concentration of sirolimus, or the interval between sirolimus initiation and follow-up MRI (p > .05). Interreader agreement for volume index change was excellent (intraclass correlation coefficient, 0.983), and that for signal ratio change was moderate to good (intraclass correlation coefficient, 0.764). CONCLUSION. Sirolimus treatment of extensive LMs in children is associated with significant reductions in volume and signal on T2-weighted MRI. The decrease in volume is greater in younger children and craniocervical lesions. CLINICAL IMPACT. The results may facilitate development of standardized MRI-based criteria for assessing the response of vascular malformations to pharmacotherapy.

Morphologic and functional evaluation of duplicated renal collecting systems with MR urography: A descriptive analysis.

PURPOSE: To describe the morphology and function of duplicated collecting systems in pediatric patients undergoing functional MR urography (fMRU). METHODS: This is a HIPAA compliant IRB approved retrospective study of all patients with duplicated renal collecting systems undergoing fMRU at our institution between 2010 and 2017. Two pediatric radiologists evaluated the studies to determine the presence, morphology and function of duplicated collecting systems using both T2-weighted and dynamic post-contrast fat saturated T1-weighted images. Assessed morphologic features included pelvic and calyceal dilation, partial or complete ureteral duplication, ureteral dilation, ectopic ureteral insertion and ureteroceles. Functional analysis was carried out per moiety. RESULTS: A total of 86 examinations (63 girls; 23 boys), median age 2.6 years (Standard Deviation 6.4 years, interquartile range: 0.4-10.3 years) and 107 kidneys (39 right; 30 left and 19 bilateral), which yielded 214 evaluable moieties, were included in the final sample. One hundred and sixty-three (76.1%) of the moieties had normal morphological features and normal functional results (average calyceal transit time and renal transit time of 2 min 28 s and 3 min 16 s, respectively). The remaining 51 moieties (23.8%) were hypoplastic or dysplastic. Seventy-seven (35.9%) had pelvic and calyceal dilation. Slightly more than half of the kidneys had complete ureteral duplication (60/107; 56%); 50 (50/107, 46.7%) had ectopic ureters (23 intra- and 27 extravesical) and 9 (9/107, 8.4%) had ureteroceles. CONCLUSION: fMRU provides comprehensive information regarding the morphology and function of duplicated renal collecting systems in children. In particular, fMRU is useful for assessing barely or non-functioning renal poles and ectopic ureters.

Hepatic and Mesenteric Vasculitis as Presenting Manifestation of Mixed Cryoglobulinemia Related to Chronic Hepatitis C Virus Infection in a Female Patient.

Approximately 80% of patients with hepatitis C virus infection develop chronic liver disease as cirrhosis, and 40% develop autoimmune complications as mixed cryoglobulinemia (MC). Gastrointestinal involvement in MC is rare, and even more so is hepatic involvement. We report a case of an 87-year-old woman with a 10-year history of blood transfusion-acquired hepatitis C virus infection, without treatment. She consulted the emergency department for diffuse abdominal pain, associated with vomiting. After 2 weeks of hospitalization in the intensive care unit, a diagnosis of MC was made; cirrhosis and secondary mesenteric and hepatic vasculitis were confirmed by a diagnostic laparoscopy. Unfortunately the condition of the patient worsened with sepsis and resulted in death in the fourth week from admission. This case highlights the importance of having in mind gastrointestinal tract vasculitis as a medical cause of abdominal pain in patients with chronic hepatitis C virus infection and using data laboratory tests, images, and histopathologic studies to aid with the diagnosis.