Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type 1 with Successful Transarterial Embolization

Vascular involvement in neurofibromatosis type 1 is rare but has the potential to be fatal. We report a case of a patient with spontaneous rupture of a left intercostal artery aneurysm, which presented as a massive left hemothorax and was successfully treated by transarterial coil embolization.

Sensitivity and specificity of 24-hour-delayed radiographs in demonstrating the transition zone of Hirschsprung disease

AbstractObjective: Our aims were to determine the sensitivity and specificity of the practice of 24-hour-delayed radiographs used to demonstrate the transition zone of Hirschsprung disease.Materials and methods: We collected the cases from all pediatric patients with suspected Hirschsprung disease who were referred for barium enema with a 24-hour-delayed radiographs from January 2000 through October 2006. All pediatricpatients had a final biopsy diagnosis. Both barium enema and 24-hour-delayed radiographs were restudied by an experienced radiologist unaware of the results of the pathology reports. The radiologist used a checklist of radiological criteria including transition zone, rectosigmoid index and mucosal irregularity patterns on barium enema and retention of contrast agent on delayed radiographs. The sensitivity and specificity of the findings were evaluated using 2x2 tables.Results:The study had 46 patients, 27 with and 19 without Hirschsprung disease. The sensitivity and specificity of the 24-hour-delayed radiographs for demonstrating the transition zone of Hirschsprung disease were about 80.8 and 40.0 percent, respectively. The sensitivity andspecificity of barium enema for demonstrating the transition zone of Hirschsprung disease were about 88.9 percent and 36.8 percent, respectively.Conclusion: 24-hour-delayed radiographs had high sensitivity and low specificity for demonstrating the transition zone of Hirschsprung disease, about the same as a barium enema.