Pneumothoraces due to tuberous sclerosis

Case contributed by Stefan Tigges
Diagnosis certain

Presentation

Shortness of breath.

Patient Data

Age: 30 years
Gender: Female

Bilateral pneumothoraces wiith diffusely increased lung markings.

Left pleural pigtail catheter, trace left pneumothorax. Multiple thin-walled cysts both lungs, fairly uniform in size and distribution. Bibasilar atelectasis, no pleural effusions

Bilateral fat-containing renal tumors, aneurysm inferior pole right kidney with surrounding hematoma and possible active extravasation. Fat-containing liver masses.

Tortuous vessels with multiple small aneurysms. Interval embolization of the right inferior pole aneurysm that was present on the abdominal CT scan.

Multiple bilateral calcified subependymal nodules.

Case Discussion

Characteristic findings of tuberous sclerosis involving the lungs (cysts), liver (hepatic angiomyolipomas), both kidneys (renal angiomyolipomas) and the brain (calcified subependymal hamartomas). All of these abnormalities are associated with complications: 1) lung cysts may rupture and cause a pneumothorax, 2) hepatic angiomyolipomas may bleed, 3) renal angiomyolipomas are hypervascular and form aneurysms and may bleed and 4) subependymal hamartomas may become subependymal giant cell astrocytomas and obstruct the ventricular system, leading to hydrocephalus.

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