Scimitar syndrome (congenital pulmonary venolobar syndrome)

Q.Scimitar syndrome is characterized by all except

a. small ipsilateral lung

b. mediastinal shift away from lesion side

c. an abnormal vessel usually drainig down and enlarging towards         diaphragm

d. the lung is normally connected to bronchial tree

 ANSWER====b

Facts about  Scimitar syndrome

• Scimitar syndrome is a unique form of lobar agenesis or aplasia, associated with other abnormalities of pulmonary vessels and the thorax. 

• The lung is normally connected to the bronchial tree.
•  The common feature in all cases of pulmonary venolobar syndrome is hypoplasiaor aplasia of one or more lobes of the right lung. 

• The variable components including partial anomalous pulmonary venous returnfrom the abnormal lung (often seen as a scimitar-shaped vein; absent or small pulmonary artery perfusing the abnormal lung; arterial supplyto the abnormal segment of lung partly or wholly from the thoracic aorta, abdominal aorta or coeliac axis; ipsilateral hemidiaphragm anomalies; absent IVC and anomalies of the bony thorax with excessiveextrapleural areolar tissue). 

• Appearance on chest radiography           
•           1.a small ipsilateral lung 

                2.mediastinal shift towards the affected side.

                3.An abnormal vessel usually seen draining down and                          enlarging towards the diaphragm in the shape of a                              Turkish‘scimitar’ sword.

REF:

1.TEXT BOOK OF RADIOLOGY  AND IMAGING: DAVID SUTTON
2.radiologyupdate.blogspot.com
3.www.doctorshangout.com