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Minerva Ortopedica e Traumatologica 2018 December;69(4):124-31

DOI: 10.23736/S0394-3410.18.03892-4

Copyright © 2018 EDIZIONI MINERVA MEDICA

lingua: Inglese

The orthopedic management in Proteus syndrome

Raffaele D. D’AMATO, Andrea VOTO , Federica FORMICONI, Antonio MEMEO

Unit of Orthopedics and Pediatric Traumatology, ASST Gaetano Pini-CTO, Milan, Italy



Proteus syndrome is a complex and very rare disorder characterized by asymmetric overgrowth of different body parts: the etiology is unknown but a postzygotic mutation of AKT1 gene is thought to be the responsible for the clinical manifestations. The diagnosis is based on clinical criteria proposed by Biesecker in 1999. Between 1990 and 2017 we treated 4 patients affected by Proteus syndrome with different manifestations, mainly involving lower limbs. We described our cases and we did a review of literature concerning the orthopedic manifestations of the Proteus syndrome to delineate the indications for the orthopedic treatment in these patients. Recurrence, vascular complications and delay in healing were the main problems encountered. The role of the orthopedic surgeon in Proteus syndrome, because of the potential for rapid overgrowth that requires urgent surgical correction, is to follow the patient during his/her all life to reduce to the minimum the number and entity of complications related to the syndrome.


KEY WORDS: Proteus syndrome - Amputation - Orthopedic surgeons

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