Fatal mesenteric fibromuscular dysplasia: a case report and review of the literature.

Fibromuscular dysplasia is a rare nonatherosclerotic, noninflammatory angiopathy of uncertain etiology and high morbidity. Because of its propensity to affect medium-sized vessels in a variety of locations, presenting symptoms may vary substantially, resulting in a delayed or missed diagnosis. We describe a 57-year-old woman who, on multiple occasions, presented with progressive gastrointestinal symptoms and eventually underwent surgical revascularization for celiac and superior mesenteric artery stenosis of uncertain etiology. Her postoperative course was complicated by bowel ischemia, multiple organ failure, and death. Autopsy findings proved useful in determining the underlying disease process and cause of death. This case report and a review of the literature illustrate the high morbidity and mortality that are caused by mesenteric fibromuscular dysplasia, the challenge in establishing a correct diagnosis, and the importance of early detection and treatment.

Acquired progressive lymphangioma.

Acquired progressive lymphangioma is a very rare disorder that presents in childhood or early adolescence as a lymphatic proliferation that gradually enlarges. It most commonly involves an extremity and is typically characterized as an indurated plaque that may be brownish-red, violaceous, or yellow. We report the case of a 15-year-old boy with a 10-year history of acquired progressive lymphangioma on the sole.

Surgical versus nonsurgical treatment of empyema thoracis: an outcomes analysis.

BACKGROUND: Empyema thoracis (ET) is associated with substantial morbidity and mortality. The optimal means for draining the pleural space remains controversial but there may be increasing bias for less invasive strategies. This study compared outcome after a nonsurgical versus a surgical approach to ET. METHODS: Patients with ET over a 10-year period (n = 93) were reviewed and stratified into nonsurgical (thoracentesis and/or closed tube thoracostomy) and surgical (thoracotomy, decortication, and/or open window thoracostomy) groups based on pleural drainage techniques. Hospital course was analyzed except when altered by death (n = 12), noncompliance (n = 3), or severe comorbidities (n = 3). RESULTS: Seventy-five patients were stratified into nonsurgical (n = 32) and surgical (n = 43) groups. Demographics, comorbidities, signs and symptoms, and causative organisms were similar between groups. Mortality did not significantly differ in nonsurgical (16%) versus surgical (10%) groups (P = 0.7). Although delay in diagnosis and number of therapeutic interventions were nearly identical, the time to definitive therapy was longer in the surgical versus the nonsurgical group (18 +/- 3.8 versus 8.5 +/- 3.8 days, P = 0.023). The time to discharge after definitive therapy (20.0 +/- 3.5 versus 35.6 +/- 14.0 days, P < 0.001), and overall hospital stay (40.6 +/- 5.3 versus 47.4 +/- 15 days, P = 0.01) was significantly decreased in the surgical versus nonsurgical treatment groups, respectively. CONCLUSION: The treatment of ET is complex. Failure to adequately evacuate the pleural space and/or persistent signs of infection should prompt surgical intervention. Surgical therapy is preferred for advanced stages of ET. Delaying definitive surgical treatment is largely responsible for prolonging hospital course.

Treatment of squamous cell carcinoma in situ of the penis with 5% imiquimod cream: a case report.

A patient with widespread in situ squamous cell carcinoma of the glans penis was successfully managed with topical application of 5% imiquimod cream. Therapy with topical immune response modifiers may prove beneficial in cases of superficial cutaneous carcinoma and may, if future studies confirm our findings, play a role in settings in which more destructive techniques may result in cosmetic defects or functional impairment. The use of imiquimod for intraepithelial squamous cell carcinoma of the anogenital area seems particularly logical because of the strong correlation between this neoplasm and infection with human papillomavirus.

Relapse of thrombotic thrombocytopenic purpura associated with decreased VWF cleaving activity.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon syndrome characterized by reversible, systemic aggregation of platelets in the microcirculation and disseminated microvascular thrombosis. Surgery may precipitate TTP and has been associated with relapse in some patients. However, relapse of this life-threatening disorder is unpredictable. We report a patient with an antecedent history of TTP who experienced a relapse after elective cardiac surgery. In this case, decreased von Willebrand factor (vWF)-cleaving metalloproteinase activity and an inhibitor of this endogenous enzyme were demonstrated preoperatively. These findings suggest that decreased vWF-cleaving metalloproteinase activity and/or the presence of its inhibitor may predict an increased risk for surgical-associated relapse of TTP.