Bipartite scaphoid with proximal pole osteonecrosis in a patient with Holt-Oram syndrome

The Holt-Oram syndrome features a spectrum of upper limb abnormalities, inc luding an abnormal shape of the scaphoid, Our report concerns a 19-year-old man with Holt-Oram syndrome and flattening and sclerosis of the proximal p ole of a bipartite scaphoid, The initial ossification centre in the scaphoi d was located distally at 8 Sears of age. By age 13, a pair of ossific cent res were present. After an automobile accident at age 18, the proximal pole became sclerotic and then began to fragment over a 22-month period. Comput ed tomography confirmed the presence of two poles of the scaphoid and scler osis and fragmentation of the proximal pole.