Bifid nose with or without anorectal and renal anomalies
OMIM number: 608980
Comments closing date: 18/12/2025
Bifid nose with or without anorectal and renal anomalies (BNAR) is a rare genetic condition caused by mutations in the FREM1 gene. It follows an autosomal recessive inheritance pattern; both parents must carry the gene change for a child to be affected. The condition can range from a mild split at the nasal tip to a more pronounced bifid nose. Some individuals also have anorectal malformations (such as a misplaced or narrowed anus) and kidney anomalies (such as one or both kidneys missing or underdeveloped). Growth and learning are usually normal. BNAR is a very rare, and its severity varies widely, even within the same family. Treatment focuses on surgical correction of structural issues and monitoring kidney and bowel health.
