Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital anomaly characterized as aplasia or hypoplasia of uterus and vagina in women with normal development of secondary sex characteristics. It affects 1 in 4000-5000 female births. Women with this syndrome present with primary amenorrhoea. MRKH syndrome may be associated with renal, skeletal, cardiac and auditory anomalies. Women with MRKH syndrome may develop leiomyoma from a rudimentary uterus, though very rare. Initial investigation in women having MRKH syndrome with leiomyoma is ultrasonography (USG). However, CT and MRI are more accurate to evaluate the pelvic anatomy and pathologies.

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Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

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ObjectiveWe studied the correlation of quantified epicardial fat with severity of coronary artery disease in patients [suspected cases of coronary artery disease (CAD)] referred for computed tomography (CT) coronary angiography and established cutoffs for epicardial fat volume (EFV) for the presence of CAD and obstructive CAD.MethodsA prospective cum retrospective cross-sectional observational study was carried out on 950 Indian subjects (suspected cases of CAD) who were referred for coronary CT in the year 2013–2016. EFV was quantified using semiautomatic technique on multidetector coronary CT angiography. The presence of atherosclerotic plaques and degree of stenosis was assessed on coronary CT angiography scans. The correlation between quantified EFV and degree of stenosis was assessed. Multivariate analysis was also performed.ResultsA higher quantity of epicardial fat is found in patients with increasing severity of coronary artery stenosis. The EFV cutoff for the presence of CAD and obstructive CAD are 49.75 and 67.69 mL with area under the curve, sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of 0.68, 81%, 45.9%,58.24%, 72.2%, and 62.84% and 0.709, 64.9%, 66.4%, 35.84%, 86.55%, and 66%, respectively. EFV correlates with age, weight, and body mass index (BMI). Multivariate analysis revealed EFV to be an independent risk factor for the presence of CAD.ConclusionsHigher quantities of EFV are found in patients with greater degree of coronary artery stenosis. EFV correlates with age, weight, and BMI. EFV is an independent risk factor for CAD.