Tuberous sclerosis

This code represents tuberous sclerosis complex (TSC), a rare, multisystem genetic disorder characterized by the growth of benign tumors in various organs, most commonly the brain, skin, kidneys, heart, and lungs. Manifestations can include epilepsy, intellectual disability, autism spectrum disorder, and distinctive skin lesions.

Use this code when documentation confirms a diagnosis of tuberous sclerosis complex, whether genetically confirmed or based on established clinical diagnostic criteria. This code is appropriate for initial diagnosis, ongoing management, and reporting of the underlying genetic condition. Supporting documentation should clearly state the diagnosis of tuberous sclerosis.

• Do not confuse with neurofibromatosis (Q85.0-), which is a distinct neurocutaneous syndrome.
• Code all associated manifestations (e.g., epilepsy, renal angiomyolipomas, cardiac rhabdomyomas) in addition to the primary diagnosis of tuberous sclerosis.
• Ensure documentation specifies "tuberous sclerosis" and not a generic term like "neurocutaneous disorder" for accurate coding.