Revisiting magnetic resonance imaging pattern of Krabbe disease - Lessons from an Indian cohort.

CONTEXT: Krabbe disease shows considerable heterogeneity in clinical features and disease progression. Imaging phenotypes are equally heterogeneous but show distinct age-based patterns. It is important for radiologists to be familiar with the imaging spectrum to substantially contribute toward early diagnosis, prognostication, and therapeutic decisions. AIMS: The study aims to describe different magnetic resonance imaging (MRI) patterns observed in a cohort of children with Krabbe disease and to assess correlation with age-based clinical phenotypes. MATERIALS AND METHODS: This is a retrospective descriptive study done at the Departments of Radiodiagnosis and Neurological Sciences of our institution, a tertiary care hospital in Southern India. Imaging features of children diagnosed with Krabbe disease over a 10-year period (2009-2018) were collected and analyzed. RESULTS: A total of 38 MRI brain studies from 27 patients were analyzed. Four distinct MRI patterns were recognizable among the different clinical subtypes. All patients from the early and late infantile group showed deep cerebral and cerebellar white matter and dentate hilum involvement. Optic nerve thickening was, however, more common in the former group. Adult-onset subtype showed isolated involvement of corticospinal tract, posterior periventricular white matter, and callosal splenium with the absence of other supra- and infra-tentorial findings. Juvenile subgroup showed heterogeneous mixed pattern with 78% showing adult subtype pattern and 22% showing patchy involvement of deep cerebral white matter with dentate hilum signal changes. CONCLUSION: Krabbe disease shows distinct imaging features which correspond to different clinical age-based subtypes. This article reemphasizes these distinct imaging phenotypes, highlights a novel imaging appearance in juvenile Krabbe, and also alludes to the rare variant of saposin deficiency. Awareness of these patterns is essential in suggesting the appropriate diagnosis and guiding conclusive diagnostic workup. Large multicenter longitudinal studies are needed to further define the role of imaging in predicting the clinical course and thus to guide therapeutic options.

Contrast-enhanced Computed Tomography Imaging of Splenic Artery Aneurysms and Pseudoaneurysms: A Single-center Experience.

AIM: The aim of our study was to evaluate the computed tomography (CT) imaging features of splenic artery aneurysm and pseudoaneurysm and to identify the disease conditions related to the same. We also wanted to ascertain any relationship between these associated disease conditions and the imaging features of the aneurysms. MATERIALS AND METHODS: This retrospective study included patients diagnosed to have splenic artery aneurysms on contrast-enhanced CT examination between January 2001 and January 2016. Data were obtained from the picture archiving and communication system. The size, number, location, morphology, the presence of thrombosis, calcification, and rupture of the aneurysms were evaluated. RESULTS: A total of 45 patients were identified with a mean age of 45 years. Splenic artery aneurysms were idiopathic in 12 (26.6%) patients. In the remaining patients, the main associated disease conditions included pancreatitis 15 (33%), chronic liver disease with portal hypertension 8 (18%), and extrahepatic portal vein obstruction (EHPVO) 6 (13%). Statistically significant findings included the relationship between EHPVO and multiple aneurysms (P = 0.002), chronic liver disease and fusiform aneurysm (P = 0.008), and smaller size of idiopathic aneurysms (P < 0.001). CONCLUSION: Based on this study, splenic artery aneurysms were associated with a variety of etiologies. The characteristics of the aneurysms such as size, location, and morphology vary with the associated disease conditions. These variations may have implications for the management.

Transarterial chemoembolization in unresectable hepatocellular carcinoma--assessing the factors affecting the survival: An audit from a tertiary care center in northern India.

INTRODUCTION: Hepatocellular carcinoma (HCC) is the most common primary malignant tumor of the liver and third most common cause of cancer-related mortality. According to Barcelona Clinic Liver Cancer (BCLC) staging system, transarterial chemoembolization (TACE) is considered a recommended option for intermediate HCC. OBJECTIVES: The purposes of our study were to evaluate the various factors affecting the outcome of TACE, to study the efficacy of TACE by evaluating the imaging response by European Association for the Study of the Liver (EASL) necrosis criteria, and to study the survival of patients. METHODS: Fifty-three patients (M = 48, F = 5) with unresectable HCC and treated with TACE were included in our study. Baseline laboratory and imaging characteristics were obtained. Clinical and laboratory toxicities were assessed. EASL necrosis response criteria were used to determine imaging response. Survival from the time of the first chemoembolization treatment was calculated. Univariate, multivariate, and survival analyses were performed using Kaplan-Meier estimations. RESULTS: A total of 53 patients underwent chemoembolization. Median age was 63 years (range 21-81 years). Thirty-one patients (58.4 %) belonged to Child-Pugh class A, while 22 patients belonged to Child-Pugh class B. Thirty-five patients died during the study period. Median study period was 13 months. Overall survival rate at 1 year was 62.26 %. Prognostic factors found to be significant on multivariate analysis were Child-Pugh class, presence of metastasis, and initial tumor size, while on univariate analysis, portal vein thrombosis, EASL response, and baseline AFP levels were also significant. CONCLUSION: Chemoembolization was an effective and safe treatment in Child A and early Child B patients. Factors that had adverse effect on survival were Child class B, larger tumor size, presence of portal vein thrombosis and metastasis, and high baseline alpha-fetoprotein levels.