Presentation
Haematuria and bilateral loin pain. Hyperaemic skin lesions of the face and extremities.
Patient Data
Subependymal calcified nodules are noted, suggestive of subependymal hamartomas.
Multiple bilateral variable-sized thin-walled cysts as well as bilateral scattered pulmonary nodules. These features in light of the patient’s history are highly suggestive of pulmonary lymphangioleiomyomatosis.
Mild right pleural effusion, with partial consolidation of the right lower lobe showing air bronchogram.
Enlarged heart.
Heterogenous retroperitoneal densities containing areas of fat densities and initially hyperdense areas as well as areas of soft tissue components, likely representative of bilaterally enlarged kidneys with giant angiomyolipomas.
Enlarged liver, dilated IVC and dilated renal veins bilaterally.
Mild free ascites.
Case Discussion
Angiomyolipomas (AML) are present in 80% of patients with tuberous sclerosis. Patients with a ruptured renal AML often present with pain or shock at acute onset. Renal AML in patients with tuberous sclerosis is one of the main causes of morbidity and mortality and a leading cause of death. Renal AML is a silent killer and rarely presents with manifestations until it’s too late. Early diagnosis and follow-up are key to prevent complications.
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