Cranio-meta-diaphyseal dysplasia: 25 year follow-up and review of literature.

We report here on a 25-year follow-up of cranio-meta-diaphyseal dysplasia in a 31-year-old Caucasian male, who was reported in the literature at the age of 8 years [Langer et al. (1991); Skeletal Radiol 20:37-41]. He has hyperostotic craniofacial features with protruding lower jaw and midface hypoplasia. He has the typical radiographic features of wide long tubular bones without normal metaphyseal flaring and wide short tubular bones without normal diaphyseal constriction. We describe here his clinical and radiological findings and compare his case with those published in the literature. He is the oldest reported patient with this disorder giving some insight into the natural history of this rare skeletal dysplasia.

Critical evaluation of chest computed tomography scans for blunt descending thoracic aortic injury.

BACKGROUND: Although aortography has been the long-held "gold standard" for diagnosis of traumatic blunt aortic injury, advances in imaging technology offer less-invasive, more-rapid, and potentially more cost-effective evaluation. The purpose of this study was to review this hospital's experience with the screening and diagnosis of blunt aortic injury with emphasis on the critical evaluation of computed tomography (CT) scans for defining descending thoracic aortic injury. METHODS: A retrospective single-center analysis of all patients undergoing aortography to evaluate for blunt aortic injury between January 1, 1997, and August 31, 2004, was performed. A policy of relying on CT scans to definitively diagnose blunt aortic injury was not in force, and all patients with positive, equivocal, and negative screening CT scans with significant injury mechanism underwent subsequent aortography; this contributed to an unbiased analysis. A subgroup of patients imaged with the latest generation multislice CT scanners (July 1, 2003, to August 31, 2004) was separately analyzed with rapid three-dimensional reconstruction. RESULTS: Of 856 aortograms, 206 (24.1%) were preceded by chest CT scan. Of 31 patients with confirmed aortic injury, 20 had undergone CT scan with 16 positive for definite injury, 3 positive for possible injury, and 1 false-negative study. Of the 206 patients scanned, 114 (55.3%) showed possible injury, 76 (36.9%) were negative, and 16 (7.8%) were positive. Only 3 of the 114 with possible injury (2.6%) were true positives whereas 1 of the 76 negative scans (1.3%) was a false negative and all 16 positive scans were true positives. These data for CT scan imaging result in a sensitivity of 95%, a specificity of 40%, a positive predictive value of 15%, and a negative predictive value of 99%. CONCLUSIONS: Chest CT is an acceptable screening tool based on prerequisite high sensitivity and ease of performance in the trauma patient suspected of having a descending thoracic aortic injury. Although the excellent negative predictive value resulted in an algorithm change at this institution, there were a significant number of equivocal scans that required subsequent aortography. Three-dimensional software reconstruction of the aorta can aid in diagnosing blunt aortic injury when findings are equivocal, but there will continue to be artifacts and limitations that require aortography for clarification.

Mesenteric arteriographic findings in a patient with strongyloides stercoralis hyperinfection.

The authors present a case of a Latin American patient with systemic lupus erythematosus who was referred for a mesenteric arteriogram because of acute lower gastrointestinal bleeding. Multiple segments of dilation alternating with stenosis or spasm were noted in the superior mesenteric artery/inferior mesenteric artery distributions. At the time, these irregularities were thought to be representative of lupus vasculitis. Despite appropriate treatment for vasculitis, the patient continued to have bleeding episodes and ultimately died of multiple organ failure. Autopsy demonstrated no evidence of vasculitis, but did demonstrate the unexpected finding of Strongyloides stercoralis hyperinfection with vessel invasion.

Traumatic injury of the internal mammary artery: embolization versus surgical and nonoperative management.

The purpose of the study was to compare the outcomes, complications, and effectiveness of embolization versus surgical and nonoperative management in patients with injury to the internal mammary artery. Eighteen cases of angiographically proven internal mammary artery injury were identified by a retrospective review. Patient age range was 17-71 years (mean 34 years). Causes of vascular injury were equally divided (9 each) between penetrating and blunt trauma. Type of trauma, associated injury, plain film findings, treatment complications (immediate and delayed), and overall outcomes were assessed. Results of embolization versus surgical and nonoperative management were compared. Angiographic findings included occlusion, active hemorrhage, and pseudoaneurysm of the internal mammary artery. Of the 18 patients studied, 12 underwent embolization; 2 underwent surgical ligation, and 4 were managed by nonoperative observation. No patient died as a direct result of vascular injury; one died of renal failure unrelated to chest trauma and one other died of myocardial contusion. One patient who underwent embolization had delayed bleeding and two patients with conservative management developed a delayed hemothorax. This small series demonstrates that embolotherapy offers an effective, efficient, and safe alternative to conventional surgical management of internal mammary artery injuries.