Aortic stenosis with large arterial occlusive process

Case contributed by Dr Hidayatullah Hamidi

Presentation

Not presented

Patient Data

Age: 15 years
Gender: Male

Heart: Well opacification of cardiac chambers.
Aorta: Normally arising from the left ventricle. The normal ascending aorta is seen. Left side aortic arch.
Normal brachiocephalic trunk and normal left common carotid artery.
Thrombosis of the left subclavian artery from its origin to the level of origin of the left vertebral artery (3.2 cm in length).
Wall calcifications of the descending aorta are seen just starting from the level of the left subclavian artery.
There is marked irregular gradual narrowing of the mid-thoracic descending aorta (from D5 to D8) with irregular wall thickening:
- The length of the involved aortic segment is about 6.3 cm.
- The lumen of the involved part of descending thoracic aorta at the narrowest point is about 0.7cm in diameter. Proximal normal caliber descending aorta measures 2.1cm and distally at the thoracoabdominal junction measuring 1.4cm in diameter.
No discrete narrowing of the abdominal aorta is noted.
Variation is noted in the origin of the celiac trunk and the SMA (celiacomesenteric trunk): The superior mesenteric artery and the celiac trunk share a common origin from the aorta.
Narrowing of the origin of the celiacomesenteric trunk.
Both renal arteries appear normal.
The inferior mesenteric artery is dilated.
There are multiple collaterals in the right hemi abdomen.
Normal aortic bifurcation to common iliac arteries.
Normal internal and external iliac arteries bilaterally.

Coronary arteries: Right and left coronary arteries are normally originating from the right and left coronary cusps respectively.
Pulmonary outflow: Normal pulmonary trunk, pulmonary arteries to lobar and segmental branches without any filling defect to suggest pulmonary thrombo-embolism.
Pulmonary veins are normally draining into the left atrium.
IVC and SVC are normally draining to the right atrium.

Lungs and pleura:

Mosaic attenuating of both lungs is identified.
Prominent hypo attenuating mediastinal and hilar lymph nodes are seen.
Bilateral mild to moderate pleural effusion with underlying lung atelectatic changes.
Air space consolidation in the right lung middle lobe.

Abdominal organs:

Multifocal peripheral small hypodense areas in both kidneys.
Normal aspect of liver, gall bladder, spleen, adrenals, GI tract and pancreas is noted.
Bones: Multi-level Schmorl's nodes along the lumbar spine.

Case Discussion

Mid thoracic descending aortic wall thickening and luminal narrowing with aortic wall calcifications (? sequela of any long-standing inflammatory process). 
Thrombosis of the left subclavian artery from its origin to the level of origin of the left vertebral artery.

Congenital variation of origin of the celiac trunk and SMA is seen (the so-called celiacomesenteric trunk) with narrowing of the origin of the celiacomesenteric trunk (and resultant inferior mesenteric artery dilatation, and multiple collaterals in the right hemi abdomen).

Mosaic attenuating of both lungs due to abnormal flow pathern.

Prominent mediastinal and hilar lymph nodes, bilateral mild to moderate pleural effusion and consolidation in the right lung middle lobe (? infectious process)
Multifocal peripheral small hypodense areas in both kidneys (? focal ischemic changes/infarcts).

Tuberculosis can be considered as one possible cause for the entire process, however further workup is needed to look for other causes of major arterial occlusive/inflammatory process.

Unfortunately no other investigations available. 

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