Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.

Thymic carcinoma is exceptionally rare in children and it has never previously been associated with autoimmune disorders. The authors report the case of an 11-year-old boy with thymic carcinoma, hypertrophic pulmonary osteoarthropathy, and an autoimmune disease that resembled systemic lupus erythematosus. To their knowledge, this is the first case of such complex clinical findings. The tumor was of high grade histologically and the boy died after 1 year, in spite of chemotherapy and radiotherapy. A review is presented of the available medical literature on thymic malignancy in childhood.

The most likely etiology of benign obstruction of the superior vena cava (SVC) include fibrosing mediastinitis and iatrogenic etiologies such as sclerosis and obstruction caused by pacemakers and central venous catheter. Percutaneous stenting of SVC has been used with success both in malignant and benign superior vena cava syndrome; however, long-term follow-up of endovascular procedures is not well known. We present a case of a patient with complete occlusion of SVC of benign etiology, presenting dramatically with bilateral chylothorax and chylopericardium with cardiac tamponade, who underwent successful vena caval revascularization with thrombolytic therapy and placement of self-expanding metallic stent. The 42-month follow-up could encourage endovascular procedures even in SVC syndrome of benign etiology.

HighlightsHemobilia is a very rare emergency. Among non-iatrogenic causes pseudoaneurysm of cystic artery should be considered.Knowledge of anatomical variations of cystic artery is fundamental in hepatobiliary surgical and radiologic procedures.Because of extreme rarity of the aneurysms of cystic artery there are no guidelines about their management and treatment.

Intraductal papillary mucinous neoplasms (IPMNs) represent a group of cystic pancreatic neoplasms with large range of clinical behaviours, ranging from low-grade dysplasia or borderline lesions to invasive carcinomas. They can be grouped into lesions originating from the main pancreatic duct, main duct IPMNs (MD-IPMNs), and lesions which arise from secondary branches of parenchyma, denominated branch-duct IPMNs (BD-IPMNs). Management of these cystic lesions is essentially based on clinical and radiological features. The latter have been very well described in the last fifteen years, with many studies published in literature showing the main radiological features of IPMNs. Currently, the goal of imaging modalities is to identify “high-risk stigmata” or “worrisome feature” in the evaluation of pancreatic cysts. Marked dilatation of the main duct (>1 cm), large size (3–5 cm), and intramural nodules have been associated with increased risk of degeneration. BD-IPMNs could be observed as microcystic or macrocystic in appearance, with or without communication with main duct. Their imaging features are frequently overlapped with cystic neoplasms. The risk of progression for secondary IPMNs is lower, and subsequently an imaging based follow-up is very often proposed for these lesions.