METHOD: A 6-year-old male patient (case 1) presented to the Service with lipomatosis and asymmetry, as the primary findings. In this study we describe two patients who were treated at the Plastic and Reconstructive Surgery Service of the Federal University of Paraná. Proteus syndrome is difficult to diagnose, and is often confused with Klippel-Trenaunay-Weber syndrome, neurofibromatosis, or Sturge-Weber syndrome. It was first described in two patients in 1979, by Cohen and Hayden. INTRODUCTION: Proteus syndrome is a complex and rare disorder classified as a hamartomatous disease. ![]() ![]() Ivan Maluf Junior Flávia Thaiana Bonato Carolina Borralho Gobbato Luiza Lopes Wagner Allan Bertolotte Isis Scomação Marlon Augusto Camara Lopes Adriana Sayuri Kurogi Ruth Maria Graf Gilvani Azor de Oliveira e Cruz Renato da Silva Freitas
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