Presentation
Acute abdominal pain.
Patient Data
Multiple giant bilateral renal angiomyolipomas complicated by rupture, large left-sided hematoma and ascites.
The CT images show a small myocardial fatty focus on the left side of the interventricular septum (blue and white arrows).
Visualization of the interventricular septum due to anemia.
Multiple small thin-walled cysts surrounded by normal lung parenchyma.
Scattered small random ill-defined lung nodules throughout the lung in a random distribution (Multifocal micronodular pneumocyte hyperplasia).
Moderate right pleural effusion (~20mm).
Multiples subependymal hamartomas associated with calcification.
Multiples cortical tubers in bilateral frontal, parietal and occipital lobes (blue arrow).
Some radial glial bands (orange arrow) and subependymal hamartomas (red arrow) are seen as well.
Case Discussion
This patient was admitted to the hospital because of abdominal pain with no history of tuberous sclerosis before.
Multifocal micronodular pneumocyte hyperplasia, LAM and bilateral renal AML, cortical tubers together are radiographic features of tuberous sclerosis complex. This patient meets criteria for a definite diagnosis of TSC.
Myocardial fatty foci are common in TSC but uncommon in the unaffected population and can be a useful pointer to the diagnosis in uncertain cases.