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Marcello De Santis

Publications and source records attributed to Marcello De Santis.

5 recordsLinked to original sources
PubMedDate not supplied

Cardinal vein isomerism: an embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect.

BACKGROUND: A persistent left superior vena cava (PLSVC) is a relatively frequent systemic venous anomaly associated with congenital heart defects. This anomaly has been explained with the persistence of the left superior cardinal vein. PLSVC usually drains into the right atrium, via coronary sinus, but it joins the left atrium in approximately 8% of the cases either directly in the setting of atrial isomerism, or via an unroofed coronary sinus, or through a coronary sinus type atrial septal defect. CASE REPORT: We describe a case of an adult patient with atria in the situs solitus, PLSVC draining into the left atrium, atresia of coronary sinus without atrial septal defect, and with additional cardiac anomalies (ventricular septal defect and discrete subaortic stenosis). CONCLUSION: A possible embryological explanation to this case rises from a right partial isomerism of the superior cardinal veins, which gives reason for both the coexistence of the PLSVC draining into the left atrium and the absence of coronary sinus, atrial septal defect, or coronary sinus ostium.

Adult↗
PubMedDate not supplied

Congenital bronchial atresia (CBA). A critical review of CBA as a disease entity and presentation of a case series.

PURPOSE: To analyse the state of the art of diagnostic imaging in the rare disease entity known as congenital bronchial atresia (CBA) and to suggest new guidelines for diagnosis. MATERIALS AND METHODS: From January 2002 to December 2003 we examined 6 patients, 4 males and 2 females. Four of them complained of relapsing bronchitis; one patient was admitted with a diagnosis of pulmonary abscess. All patients underwent chest X-ray and MDCT. RESULTS: Five patients presented at chest x-ray the typical association of hilar mass and distal parenchymal hyperinflation that raised the suspicion of CBA; the remaining patient presented an area of parenchymal translucency that prompted us to perform MDCT, which showed severe stenosis of the segmental bronchus. CBA involved the left upper lobe (LUL) in 4 patients and the right upper lobe (RUL) in the remaining 2. In 50% of cases there were associated anomalies: distal bronchiectasis, bronchogenic cyst, anomaly of branching of bronchial tree and vascular structure. CONCLUSIONS: CBA is a relatively rare, generally oligosymptomatic malformation. Knowledge of the signs present at chest X-ray can prompt the radiologist to perform a CT scan. MDCT with multiplanar reconstruction is fundamental for the characterisation, localisation and study of the distribution of the lesion, helping to make a precise diagnosis. Surgery is seldom required and at any rate only performed in case of complications.

Adolescent↗