Paediatric Imaging

Proteus syndrome

hamartomatous dysplasia involving all 3 germ layers. There is asymmetrical overgrowth of the hands and/or feet, plantar hyperplasia, haemangiomas, lipomas, lymphangiomas, macrocephaly, hyperostosis of the skull, long bone overgrowth and dysplastic vertebrae. Features may be present at birth but become more obvious with time. Bony abnormalities are best seen on plain films (Fig.1). MRI best demonstrates the mesodermal manifestations.

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Fig.1

Radiograph of the foot demonstrating massive overgrowth of both soft tissues and bones involving the first three rays.
Proteus syndrome, Fig.1