This is the one of the largest retrospective study of pediatric GG. Identifying clinical variables, which could stratify these tumors into low- and high-risk groups might help to profile a risk-based therapeutic strategy. Collaborative multiinstitutional prospective studies are warranted to delineate treatment consensus and investigate prognostic factors.
Budd-Chiari syndrome refers to hepatic pathology secondary to diminished venous outflow, most commonly associated with venothrombotic disease. Clinically, patients with Budd-Chiari present with hepatomegaly, ascites, abdominal distension, and pain. On imaging, Budd-Chiari syndrome is hallmarked by occluded IVC and or hepatic veins, caudate lobe enlargement, heterogeneous liver enhancement, intrahepatic collaterals, and hypervascular nodules. Etiopathological factors for Budd-Chiari syndrome include several systemic thrombotic and nonthrombotic conditions that can cause venous outflow obstruction at hepatic veins and/or IVC. While the transjugular intrahepatic portosystemic shunt (TIPS) is used as a treatment option for Budd-Chiari syndrome, Budd-Chiari syndrome is not a well-known complication of TIPS procedure. We report a case of Budd-Chiari syndrome that occurred in a transplanted cirrhotic liver from malpositioned proximal portion of the TIPS in IVC causing occlusion of the ostia of hepatic veins which was subsequently diagnosed on contrast-enhanced CT.
A 43-year-old man arrived at the emergency department following a syncopal episode. Computed tomography and magnetic resonance images demonstrated a small interhemispheric, anterior parafalcine mass that mimicked a meningioma. Surgical excision and subsequent pathologic evaluation revealed an angioleiomyoma and the patient recovered without incident. Angioleiomyomas are classified as benign smooth muscle tumors and are classically seen in adult females arising in the soft tissues of the lower extremities. Although rare, these masses have been described in various intracranial locations, usually extra-axially. A comprehensive review of the literature and discussion are provided, emphasizing histopathologic and imaging features of this uncommon intracranial neoplasm.
Objective Our objective is to document the imaging appearance in the intracranial compartment at the time of the infants' first postnatal brain MR imaging after fetal repair for spinal dysraphisms. Methods Twenty-nine patients were evaluated on fetal and postnatal magnetic resonance imaging for a series of features of Chiari II malformation. Results Of the 29 infants, 55% had resolution of tonsillar ectopia, and 62% showed a dorsal outpouching of the near the foramen magnum on postnatal magnetic resonance imaging. The majority had persistence of Chiari II features including: prominent massa intermedia (93%), tectal beaking (93%), towering cerebellum (55%), flattening of the fourth ventricle (90%), hypoplastic tentorium (97%), and tonsillar hypoplasia (59%). Conclusions Normally positioned or minimally descended, oftentimes hypoplastic tonsils in the presence of a posterior fossa configuration typical of Chiari II, was the most common presentation. An additional documented feature was an outpouching of the dorsal thecal sac between the opisthion and the posterior arch of C1.
Purpose: To identify patterns of abnormalities using DaTscan ™ single photon emissioncomputed tomography (SPECT) imaging of patients with non-Parkinson's movement disorders. Materials and methods: A single institution retrospective evaluation was performed of consecutive patients who underwent DaTscan SPECT imaging for Parkinson's-like movement disorders, excluding those who had scan findings consistent with Parkinson's disease. Demographic and clinical data were collected for all patients. A single experienced reader graded the degree of decreased dopamine transporter activity using a semi-quantitative visual score. Additional quantitative analysis was utilized to generate z-scores in 101/104 patients. Data were analyzed to establish patterns and frequency of abnormalities on DaTscan. Correlation between quantitative and semi-quantitative visual scores was also performed. Results: A total of 104 patients were included, of which 77 patients (74%) had abnormal findings on semi-quantitative visual assessment of the DaTscan images and 27 patients (26%) had normal findings. Of the abnormalities, the majority were either mild or moderate decrease in tracer accumulation, in one or both putamina. The most common site of abnormality was the left posterior putamen followed by the right posterior putamen. Quantitative z-score analysis revealed that 14% of the patients exhibited an abnormality, while 86% had normal z-scores. Conclusion: Semi-quantitative visual DaTscan analysis of patients with non-Parkinson's movement disorders most commonly reveals mild to moderate decrease in dopamine transporter activity, primarily involving the posterior aspect of one of the putamina.
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