A Case of Holt - Oram Syndrome

SUMMARY Holt Oram Syndrome consisting of upper limb defcts, cardiac anomalies and narrow shoulder, was first described by Holt and Oram in 1960. The inheritance pattern is autosomal dominant and most commonly encoutered cardiac anomalies being ASD, although all variaties of C.H.D are reported. Upper limb anomalies are not specific, although scaphoid bone deformity is almost characteristic in this syndrome. We reported a 7 mounths old infant with full-flaged constellations of Holt-Oram Syndrome. It is however of some interest to note that hypoplastic humerous, bilaterally, is rarely reported in literature.