Bannayan-Riley-Ruvalcaba syndrome

ICD-10 Code E71.440

Bannayan-Riley-Ruvalcaba syndrome

Definition

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by hamartomatous intestinal polyposis, lipomas, macrocephaly and genital lentiginosis.

Also known as Bannayan syndrome, Bannayan-Riley-Ruvalcaba syndrome, Bannayan-Zonana syndrome, BRRS, macrocephaly with multiple lipomas and hemangiomas, Myhre-Riley-Smith syndrome, RILEY-SMITH syndrome, Ruvalcaba-MYHRE-SMITH syndrome — per MONDO

Also identified as