Computed tomographic pulmonary angiography for diagnosis of invasive mold diseases in patients with hematological malignancies.

BACKGROUND: Invasive mold diseases (IMDs) of the lung remain a challenge for immunocompromised patients. Although timely diagnosis and treatment are crucial for the outcome of the infection, the poor sensitivity of microbiological techniques and the limited specificity of chest high-resolution computed tomography (HRCT) often delay definitive diagnosis of these infections. METHODS: To explore the diagnostic utility of computed tomographic pulmonary angiography (CTPA) for detecting angioinvasive patterns of pulmonary infection, we performed a single-center, prospective, nonrandomized trial involving 36 patients with hematological malignancies who had clinical suspicion of IMD, as defined by European Organization for Research and Treatment of Cancer/Mycosis Study Group diagnostic criteria. RESULTS: We found that 5 of 5 patients with proven IMD had CTPA-positive findings consistent with interruption of the arterial vessels (concordance, 100%). CTPA findings were positive in 5 of 7 patients with probable IMD (findings for 2 were considered false negative because lesions were too small or not evaluable). In 15 of 24 patients with a final diagnosis of possible IMD, CTPA findings were negative for 14 patients and were positive for 1 patient, who had septic emboli associated with Staphylococcus aureus bacteremia. CTPA findings were positive in the remaining 9 patients with a final diagnosis of possible IMD at the end of the study. CONCLUSIONS: We conclude that CTPA appears to be a promising tool to exclude the diagnosis of IMD in high-risk patients without specific findings on HRCT scans, and it is most useful in the presence of well-circumscribed lesions in which there is suspicion for IMD.

Occipital horn syndrome in a woman: skeletal radiological findings.

Ehlers-Danlos type IX syndrome, also called occipital horn syndrome (OHS), is a milder and rare form of Menkes disease where the patient reaches adulthood. As an X-linked disease, it typically occurs in male subjects, while female subjects are usually healthy carriers. OHS is mainly characterized by connective tissue disorders and slightly subnormal intelligence or signs of autonomic dysfunction are the only apparent neurological abnormalities, in connection with molecular defects in copper metabolism. Our purpose is to report on radiological skeletal findings that may be incidental or investigated when OHS is suspected and to underline the possible involvement and expression in the female. Moreover, the impact of skeletal findings is also highlighted in the prevention of serious complications of the disease.

Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe in an adult patient: a case report.

INTRODUCTION: Congenital cystic adenomatoid malformation of the lung is an uncommon cause of respiratory distress in neonates and babies. The disorder is usually diagnosed in the neonatal period and the first two years of life. This anomaly has been described in association with bronchopulmonary sequestration, extralobar intra-abdominal sequestration or bronchial atresia in live and stillborn babies. It is rarely encountered in adults, in whom the diagnosis is made incidentally from mass lesion features seen on chest radiographs. The oldest patients recorded with this malformation have been about 35 years old, and only 10% of primary diagnoses are made after the first year of life. Delayed diagnosis can be related to infection or serendipitous discovery. CASE PRESENTATION: We describe the radiological findings of a 34-year-old Caucasian woman with a clinical history of recurrent pneumonia, intermittent anterior pleuritic chest pain and haemoptysis. Congenital cystic adenomatoid malformation of the lung associated with bronchial atresia involving a different lobe was discovered. CONCLUSION: Although rare in adults, congenital cystic adenomatoid malformation should be suspected in adult patients who suffer from recurrent or persistent non-productive coughs. The discovery of an association of congenital cystic adenomatoid malformation with bronchial atresia in adulthood is rare but possible, even in different lobes.