Longitudinal Echocardiographic Evaluation of an Unusual Presentation of X-Linked Myxomatous Valvular Dystrophy Caused by Filamin A Mutation.

Polyvalvar myxomatous valve degeneration is a clinical pathology rarely encountered during cardiac anesthesia, but, when present, most commonly occurs in the context of a connective tissue disorder. Filamin A mutations have begun to be recognized as a source of progressive myxomatous mitral and tricuspid valve degeneration. These lesions can be diagnosed by echo, but their clinical presentation can be equivocal. We present a patient with significant echocardiographic findings of mitral and tricuspid valvar regurgitation, aortic dilatation, and intraoperative findings of aortic ectasia. In our case, a detailed family history led to a preoperative echocardiogram revealing myxomatous mitral and tricuspid valve degeneration with significant regurgitation and aortic dilatation. Genetic evaluation led to the diagnosis of a Filamin A mutation. Pre- and postrepair echocardiographic assessments of valvar function played a key role in the management of this patient. Continued surveillance of his aortic dilation and evaluation of postrepair valve function warrants close follow-up with a high likelihood for further surgical intervention.

Development and spontaneous regression of a de novo posterior communicating artery aneurysm in a child following gamma knife stereotactic radiosurgical obliteration of a cerebral AVM: Case report and review of the literature.

Reports of the development of true intracranial aneurysms after radiosurgery are exceedingly rare and unconvincing. We report the case of a young boy who developed a posterior communicating artery aneurysm after the Gamma Knife radiosurgical treatment of a thalamic AVM. This ten year-old presented with left upper extremity tremors. MRI/MRA revealed a right thalamic AVM. No intracranial aneurysms were found on initial angiography. The patient underwent two-stage endovascular embolization of his AVM followed by Gamma Knife radiosurgical treatment (18 Gy delivered to the 50% isodose line). Follow up angiogram at 38 months confirmed obliteration of the AVM, but revealed a new 2.2 x 1.5 mm aneurysm located at the origin of the right posterior communicating artery. A MRA obtained 5 months later (43 months after treatment) demonstrated regression of the aneurysm. This is the first report of the development of an intracranial aneurysm after Gamma Knife radiosurgery in a child and the first description to include pre and post-treatment angiography that clearly demonstrates interval aneurysm development. Although the aneurysm showed subsequent regression during continued follow-up, this report illustrates the critical importance of post-treatment angiography for patients with intracranial AVM treated in this manner.

Diet reconstruction in antebellum Baltimore: Insights from dental microwear analysis.

Construction in the City of Baltimore during 1996 led to the recovery of human skeletal remains dating from 1792 to 1856. Historical research indicates that the skeletal remains come from two adjacent graveyards: Christ's Church Episcopalian Cemetery and the Potters Field East. The different socioeconomic status of the internees in each cemetery suggests the possibility of marked contrasts in lifestyle, health, and diet. To shed further light on these possibilities, analyses of microscopic wear patterns on teeth, or dental microwear analyses, were undertaken. A sample from Spanish Florida was used to help interpret the results. Epoxy casts of incisor and molar teeth were placed in an SEM and photomicrographs of clean wear facets were taken. The photomicrographs were digitized using the software package Microware 4.02. Statistical analyses of rank transformed data consisted of single-factor ANOVA, followed by post hoc tests. No significant differences were found between Christ's Church and Potters Field East samples for any of the variables examined in either molar or incisor teeth. However, differences between each Baltimore sample and the La Florida samples give suggestions of possible diet differences in antebellum Baltimore. The mosaic of differences between the Baltimore and La Florida samples probably reflects the wide variety of foods available to antebellum Baltimoreans as well as the relative lack of abrasives in their diet.