Q85.1

Billable

Tuberous sclerosis

HCC Category Mapping

V28HCC 23 — Melanoma and Other Skin Cancers

0.251

V24HCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.150

ESRDHCC 12 — Breast, Prostate, and Other Cancers and Tumors

0.000

RxHCCHCC 22 — Cancer, Other Specified Sites

0.000

What This Code Means

Tuberous sclerosis is an inherited genetic disorder causing benign tumors to grow in multiple organs including the brain, skin, heart, and kidneys.

Coding Tips

•Document affected organ systems (brain, skin, heart, kidneys) and associated complications separately
•Link with codes for seizures, developmental delay, or renal disease if present

Clinical Significance

Tuberous sclerosis complex is a multisystem genetic disorder with high morbidity affecting brain, skin, kidneys, heart, and lungs, requiring lifelong multidisciplinary management and surveillance for malignant transformation. The condition has significant neurological implications including seizures and intellectual disability, making early diagnosis crucial for developmental outcomes.

Documentation Requirements

✓Two major features or one major plus two minor TSC features
✓Brain manifestations (cortical dysplasias, subependymal nodules)
✓Skin manifestations (hypomelanotic macules, facial angiofibromas)
✓Renal involvement (angiomyolipomas, cysts)
✓Cardiac rhabdomyomas
✓Pulmonary involvement (lymphangioleiomyomatosis)
✓Genetic testing results (TSC1 or TSC2 mutations)
✓Seizure history and neurological assessments

Commonly Confused Codes

Q85.00 — Neurofibromatosis (different tumor syndrome)G40.909 — Epilepsy (symptom, not underlying syndrome)Q87.89 — Other congenital malformation syndromes D30.01 — Renal angiomyolipoma (individual manifestation)