Adams-Oliver syndrome (AOS) is a rare disease characterized by congenital scalp defects, terminal transverse limb defects and cutis marmorata telangiectatica. A significant incidence of cardiac and vascular malformations has been reported, leading to the hypothesis of a vascular defect early involved in the pathogenesis. We report two members of the same family with previously diagnosed AOS based on clinical phenotype and later recognized to have pulmonary arterio-venous malformation (PAVM). None of the subjects fulfilled current diagnostic criteria of hereditary hemorrhagic telangiectasia, which is the most common cause of PAVM. The occurrence of PAVM in AOS lends support to the hypothesis that endothelial specific abnormalities could be a patho-physiological mechanism in its development. Therefore, the role of screening for PAVM in clinical management of subjects with AOS should deserve further studies.
The effectiveness of US-guided PLA for HCC tumors < or =4 cm turned out to be negatively affected by both operator-related (the beginning of the operator's experience with the technique) and tumor-related factors (non-naïve, infiltrating HCC tumors).
We would like to comment on the recent article by Dr. Wilson and colleagues [1] about discordance between contrast-enhanced sonography and CT and MRI as to patterns of enhancement of focal liver lesions. The authors should be commended on their effort to clarify this topic: albeit representing a small percentage of cases, patients with space-occupying liver lesions and discordant results between the contrast-based imaging techniques constitute a diagnostic challenge to their physicians.However, we have several questions. First, it is unclear from the Materials and Methods section what was the pathologic method of reference: percutaneous biopsy or histology on a surgical specimen? For example, in some cases of atypical liver adenoma, only surgery can definitely exclude liver malignancy [2].Second, did the authors evaluate whether discordant results occurred more often when older sonography, CT, or MRI machines were used? In other words, could the use of different equipment have influenced their results?Third, did the authors verify whether different histomorphology, vascular architecture, or both could explain why the same category of lesions (e.g., adenoma) behaved differently after the administration of contrast agents? Could lesion size affect the results as well?
Objective. Clinical and imaging (sonographic and computed tomographic [CT]) findings in 3 cases of giant mucocele of the appendix are described. Methods. Clinical records of 3 cases of giant mucocele of the appendix were reviewed. All patients had a basal B-mode sonographic examination and a contrast-enhanced sonographic examination using a second-generation low-mechanical index contrast medium. In all cases, a dual-phase spiral CT examination was carried out. Results. In 2 cases, the abdominal masses were discovered in asymptomatic patients; 1 patient had vague abdominal discomfort. A pathologic diagnosis of benign cystoadenoma was found at pathologic examination in all cases, and malignant pseudomyxoma peritonei was disclosed in 1 patient 1 year later. Common sonographic findings were as follows: (1) a huge abdominal mass with a maximum diameter ranging between 20 and 25 cm; (2) a thin hyperechoic border without either solid vegetations or signs of infiltration of surrounding tissues; (3) a complex internal echo structure with anechoic lacunae interspersed between curvilinear, wavy bands of echogenic material (the so-called sonographic onion skin sign); and (4) avascularity of the masses shown on contrast-enhanced sonography with a low-mechanical index medium. At CT, a well-circumscribed cysticlike mass of low attenuation was displayed in all cases. There was lack of enhancement during a dual-phase examination in 2 cases; in the other, a small peripheral area of faint enhancement was appreciated. Only in the latter case could CT reliably assess the origin of the mass. Conclusions. It is suggested that a combination of sonographic (namely the onion skin sign) and CT findings may aid in the correct preoperative diagnosis of giant mucocele of the appendix.
The right superior intercostal vein is visualised on CT examination as a circular opacity laterally at the right aspect of the vertebral body at the T4-T5 level. In venographic examination the RSIV appears to be formed by the confluence of the venous channels, the right second, third and fourth intercostal veins. In superior vena cava obstruction the RSIV is an important collateral pathway: the flow through the vein depends on the site of the obstruction, anterograde if the obstruction is superior or parallel to the level of the vena azygos, and retrograde if the obstruction is below the vena azygos.
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