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Bannayan-Riley-Ruvalcaba Syndrome (MONDO:0007924)

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

Overview

Category

syndromic disease

cardiovascular disorder

Ontology ID

MONDO:0007924

Internal:9257

Synonyms

BRRS

Bannayan syndrome

Bannayan-Riley-Ruvalcaba syndrome

Bannayan-Zonana syndrome

Myhre-Riley-Smith syndrome

RILEY-SMITH syndrome

Ruvalcaba-MYHRE-SMITH syndrome

macrocephaly with multiple lipomas and hemangiomas

BZS

RMSS

Riley-Smith syndrome

Ruvalcaba -Myhre-Smith syndrome

Ruvalcaba-Myhre-Smith syndrome

macrocephaly multiple lipomas and hemangiomata

macrocephaly pseudopapilledema and multiple hemangiomas

macrocephaly, multiple lipomas, and hemangiomata

macrocephaly, pseudopapilledema, and multiple hemangiomata

Mapped terms

DOID:0050657

GARD:0005887

ICD10CM:E71.440

ICD9:759.6

MEDGEN:78554

NCIT:C3939

NORD:1684

OMIM:153480

Orphanet:109

SCTID:21984008

UMLS:C0265326

icd11.foundation:357383447

Child terms

Unknown children.

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