Case 6

This is a contrast enhanced CT study of the abdomen and pelvis, demonstrating grossly enlarged kidneys - the renal parenchyma is almost completely replaced by cysts and fatty lesions interspersed with soft tissue density, and a few punctate focal calcifications.

The enlarged kidneys are exerting mass effect and compressing the abdominal contents centrally. There is a moderate amount of free fluid in the pelvis.

In the right kidney there is a large component of high density superiorly, which does not show post-contrast enhancement; there is also a small area in the left kidney superiorly with the same characteristics. These represent haemorrhage into the cysts. I cannot appreciate any active contrast extravasation to signify ongoing bleeding.

The liver is enlarged and also contains a few small fatty lesions. There is a small cyst in the pancreas.

There are diffuse, small, round lung cysts in the lung bases of similar size.

The findings are pathognomonic for a multisystem congenital disorder, resulting in hamartomatous growths in different organ systems- the kidneys contain innumerable cysts and angiomyolipomas; there are a few angiomyolipomas in the liver as well.

The lung involvement takes the form of lymphangioleiomyomatosis, due to the proliferation of smooth muscle cells which obstruct airways and lymphatics.

This patient has tuberous sclerosis. There is an acute event in this case, which is bleeding into the cysts in both kidneys - but to a greater extent on the right side.

I would review any past imaging if available; I would contact the referring physician and ask if the patient is a known case of tuberous sclerosis. I would then inform them of the bleeding complication. I would suggest that the case be discussed at the relevant MDT.