Wunderlich syndrome - angiomyolipoma rupture in tuberous sclerosis

Case contributed by Dr Nguyen The Hien

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.

7.8 x 7.5 cm heterogeneous right upper renal pole mass of solid and fatty components of with 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 mass effect, pushing the liver anteriorly. No contrast extravasation suggesting active bleeding is observed in arterial phase (not shown). These findings suggest Wunderlich syndrome secondary to a large bleeding angiomyolipoma.

3.9 x 3.4 cm homogeneous solid right lower renal pole mass with contrast enhancement.

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

Fluid in retroperitoneal spaces and cul-de-sac. Very shallow right-sided pleural effusion.

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

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

Numerous red papules were found on nasolabial folds, cheeks, and chin. In tuberous sclerosis, these lesions are called "adenoma sebaceum", which is a misnaming of angiofibromas.

Case Discussion

In this case, a diagnosis of tuberous sclerosis (TS) with specific lesions in four systems: brain, kidneys, bone, and skin was almost certain. The multiple renal AMLs and 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 common causes.

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