Wunderlich syndrome due to angiomyolipoma rupture in tuberous sclerosis complex

Case contributed by Dr Hien The Nguyen

Presentation

Acute right flank pain with a history of hypertension, anemia, and angiofibromas.

Patient Data

Age: 30 years
Gender: Male

Both kidneys contain innumerable cysts and fatty component lesions.

A 78x75mm heterogeneous mass composed of solid tissue and fatty component is located at the right kidney upper pole, showing a contrast enhancement. Its anterior border is ill-defined and uninterrupted with a solid density region in perirenal and pararenal spaces. This region demonstrates no contrast enhancement and a mass effect by pushing the liver anteriorly. No contrast extravasation suggesting an active bleeding is observed in arterial phase (not shown). These findings suggest a Wunderlich syndrome - a large angiomyolipoma (AML) complicated by a hemorrhage.

A 39x34mm homogeneous solid attenuation mass is seen at the right kidney lower pole and shows a contrast enhancement.

These macroscopic fat-containing lesions in bilateral kidneys are typical of AMLs. The mass in the right kidney that does not demonstrate macroscopic fat component is suggestive of a poor-fat AML.

Fluid is accumulated in retroperitoneal spaces and cul-de-sac. A minimal pleural effusion is found on the right side.

Postcontrast brain CT images demonstrate multiple calcified subependymal tubers on lateral ventricle wall.

Sclerotic lesions are found bilaterally in frontal bones and greater wings of sphenoid bones.

Numerous red papules were found on nasolabial folds, cheeks, and chin. These lesions are so-called adenoma sabeceum, which is a misname of angiofibroma.

Case Discussion

In this case, diagnosis of tuberous sclerosis (TS) with specific lesions in 4 systems: brain, kidneys, bone, and skin was almost certain. The multiple AMLs and renal cystic disease occur approximately in 80% and 50% of cases, respectively, making renal manifestations the second most common findings in TS. The condition in which a renal hemorrhage occurs spontaneously is described as Wunderlich syndrome, and AML rupture is one of the causal factors.

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Case information

rID: 57690
Published: 11th Jan 2018
Last edited: 11th Jan 2018
Inclusion in quiz mode: Excluded
Institution: Pham Ngoc Thach University of Medicine

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