BRANCHIO-OTO-RENAL SYNDROME-CASE REPORT

Branchio-oto-renal syndrome (MelnickFraser syndrome) is a rare autosomal dominant disorder characterized by syndromic association of branchial cysts or fistulae along with external, middle, inner ear malformations and renal anomalies. We are reporting an 8 yrs old male child born to second degree consanguinous parents who presented with oliguria, stunted growth and right anotia. Ultrasonogram abdomen revealed a cyst (2X1.5mm) in left renal area, right hydronephrosis. He was diagnosed as a case of Branchio-oto-renal syndrome (Melnick-Fraser syndrome) and his parents and sibling were normal.