disease has basis in dysfunction of FREM1 bifid nose BNAR syndrome BNAR BNAR syndrome is a very rare multiple congenital anomaly syndrome characterized by a bifid nose (with bulbous nasal tip but not associated with hypertelorism) with or without the presence of anal defects (i.e. anteriorly placed anus, rectal stenosis or atresia) and renal dysplasia (unilateral or bilateral renal agenesis) and without intellectual disability. BNAR syndrome is phenotypically related to Fraser syndrome and oculotrichoanal syndrome. GARD:0010595 ICD10:Q87.8 MESH:C567672 MONDO:0012165 OMIM:608980 Orphanet:217266 SCTID:717940006 UMLS:C2750433 UMLS:C4303547 bifid NOSE with or without anorectal and renal anomalies bifid nose with or without anorectal and renal anomalies syndromic anorectal malformation syndromic urogenital tract malformation syndromic renal or urinary tract malformation genetic multiple congenital anomalies/dysmorphic syndrome without intellectual disability