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Musculoskeletal Imaging

Acrocephalopolysyndactyly

a group of several syndromes (Carpenter's syndrome, Noack's syndrome, Sakati's syndrome, Goodman's syndrome, Summit's syndrome) whose basic features are craniosynostosis and polysyndactyli.

Carpenters syndrome is of autosomal recessive inheritance and is characterized by radiographic findings of relatively late closure of the coronal suture and a variety of abnormalities in the hands and feet. Syndactyly frequently occurs between the third and fourth digits, and the tubular bones of the hands are shortened. Polydactyly of the toes is common.

In Noacks syndrome, patients have duplication of the great toes and enlargement of the thumbs. Patients with Sakatis syndrome have polysyndactyly, shortened lower extremity tubular bones, craniosynostosis, and bowing of the femora. Goodman's syndrome and Summit's syndromes are now considered variants of Carpenter's syndrome.

DR