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

The Holt-Oram syndrome features a spectrum of upper limb abnormalities, including 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 pole of a bipartite scaphoid. The initial ossification centre in the scaphoid was located distally at 8 years of age. By age 13, a pair of ossific centres were present. After an automobile accident at age 18, the proximal pole became sclerotic and then began to fragment over a 22-month period. Computed tomography confirmed the presence of two poles of the scaphoid and sclerosis and fragmentation of the proximal pole.