A surgical approach for complex foot deformity in Proteus’ Syndrome

reporte de un caso

Abstract

The Proteus Syndrome is characterized by the excessive or segmentary growth of multiple tissues And organs derived from the three germinal layers, which is associated with de development of tumors. This syndrome is not common with an incidence of less than one case per million. A family association has not been seen, but its occurrence in monocygotic twins has been reported. It affects equally both genders and has been seen in all ethnic groups. This observation supports the hypothesis of a mosaic somatic mutation. Due to its clinical variability, its diagnosis usually is erroneous and delayed, even when it is based in the criteria proposed by Biesecker and cols. The treatment of this disease is based on the management of the symptoms. The different surgical techniques seek to address abnormal bone growth. Even though there are no specific proposals with respect to the surgical treatment, most of the case reports in literature refer to amputation or disarticulation of the compromised extremity. This paper aims to propose a different surgical reconstructive technique for this specific case.