Thoracic aortic dissection and redissection

Case contributed by Dr Ian Bickle

Presentation

Marfans. Chest pain.

Patient Data

Age: 20 years
Gender: Male

Abnormal contour of the right mediastinum due to a dilated aortic root.

Heart size normal.

Lungs clear.

Dissection flap extending from the aortic root down to the level of the upper abdominal aorta.  The dissection flap begins just above the level of sinotubular junction down to the level of the upper abdominal aorta to just above the level of the origin of renal arteries.  The aortic root at the mid aortic sinus is 5.4 cm.  The coronary arteries are normally opacified.

The great vessels of the neck, celiac axis and SMA are supplied from the true lumen of the dissection.  The maximum diameter of the descending aorta is 2.1 cm and the aorta at the level of the arch is 2.5 cm.  Normal opacification of the pulmonary arteries.

Right-sided diaphragmatic hernia containing left lobe of the liver. Most of the left lobe of the liver is in the anterior mediastinum in the retrosternal region. 

Lungs clear.   No pleural effusions or mediastinal hematoma.  

Two years later. Asymptomatic.

x_ray

Median sternotomy.

Even allowing for the AP projection, the aortic arch and descending aortic are markedly dilated.

Lungs clear.

3 years later. Acute intrascapular pain and dyspnea

x_ray

Median sternotomy.

Even allowing for the AP projection, the aortic arch and descending aortic are markedly dilated.

Lungs clear.

Extensive aortic dissection extending from the mid ascending aorta to the upper abdominal aorta, just above the origin of the superior mesenteric artery - this is unchanged compared to the previous study. New findings of marked dilatation of the distal aortic arch and descending thoracic aorta. The descending portion now measures 6.6 cm in transverse diameter and the mid aortic arch measures 7.2 cm. Extensive mural hematoma in the arch and descending portion, and there is evidence of acute intramural hemorrhage, arising from the superolateral aspect of the aortic arch.

No gross mediastinal hemorrhage, pleural or pericardial effusion. 

No significant findings within the solid upper abdominal organs. Normal bilateral renal enhancement. New significant increase in size of the upper abdominal aorta which measures 4.7 cm in AP caliber compared to around 2.6 cm previously. No significant intramural hematoma within the abdominal aorta.

No free abdominal or pelvic fluid.

Additional radiographs from peripheral trauma attendance.

x_ray

No acute bony injury. 

Elongated appearance of the feet.

Case Discussion

Marfans syndrome patients are prone to aortic dissection.

On the 2nd CT, in addition to the initial findings of a type A aortic dissection which has been surgically corrected is an acute intramural hemorrhage in the descending thoracic aorta and marked new aneurysmal dilatation of the thoracic and upper abdominal aorta.  This indicates the second incidence of acute aortic syndrome.

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