Management of children with megafoot secondary to Proteus syndrome: a report of three cases with long-term follow-up

Abstract

Proteus syndrome is a rare disorder characterized by overgrowth of limbs and organs and neurocutaneous findings. Methods: We examined three Proteus syndrome cases with unilateral foot enlargement, megafoot. The patients had ambulatory and cosmetic difficulties. Debulking surgery was performed. Results: After the surgeries, patient mobility and functional scores were analyzed and found to be improved;only one patient had recurrence after 5 years. In the literature, there is no consensus about the treatment and the timing of treatment of Proteus syndrome. The literature suggests that early surgery for megafoot leads to better functional results. Debulking surgery and amputation are the two options mentioned. In the present cases, soft-tissue debulking was performed. Conclusions: In patients with megafoot caused by Proteus syndrome, the functional results were better for those with early debulking surgery compared with those who did not undergo surgery. Patients were able to fit into shoes and walk without difficulty.