Late Diagnose �Scimitar Syndrome� Associated  with Multiple Disorders: Tracheal Stenosis,  Tracheomalacia, Chronic Obstructive Pulmonary  Disease, Vertebral Malformation

Roxana Maria Nemes; Doina Adina Todea; Alexandra Diana Comes; Andreea Codruta Coman; Edith Simona Ianosi

Volume : IV, Issue : III, March - 2014

Late Diagnose �Scimitar Syndrome� Associated with Multiple Disorders: Tracheal Stenosis, Tracheomalacia, Chronic Obstructive Pulmonary Disease, Vertebral Malformation

Roxana Maria Nemes, Doina Adina Todea, Alexandra Diana Comes, Andreea Codruta Coman, Edith Simona Ianosi

Abstract :

We report the case of a 60 years old male patient (ex–smoker), admitted in our clinic with pneumonia and respiratory failure. CT relevated a mat retracted right hemitorax, left lung hernia, dextro–concave scoliosis with verteal malformation. Bronchoscopy diagnosed a complex tracheal stenosis, severe tracheomalacia, upper and middle right lobe atresia and a suppuration in the lower right lobe. Echocardiography found a mitral failure. CT angiography confirmed right pulmonary artery atresia and the anomalous connection of the right pulmonary veins into inferior vena cava. We considered a „scimitar syndrome” with late clinical onset and multiples comorbidities. The patients followed treatment for pneumonia, COPD and respiratory failure. As for the tracheal stenosis and tracheomalacia we recommended evaluation in an interventional onchoscopy service in order to consider tracheal dilations or stent insertion to prevent further complications. Particularities of the case: late, surprising diagnosis of the congenital disease, association with smoking, COPD and other disorders causating respiratory failure (tracheal stenosis, traheomalacia, pneumonia), the decisive role of CT angiography and multidetector CT in elucidating the diagnosis.