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Jornal Vascular Brasileiro

Print version ISSN 1677-5449On-line version ISSN 1677-7301


BERTANHA, Matheus et al. Clinical and psychosocial challenges in the treatment of a patient with Proteus syndrome. J. vasc. bras. [online]. 2015, vol.14, n.4, pp.346-350. ISSN 1677-5449.

Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life.

Keywords : amputation; congenital abnormalities; musculoskeletal abnormalities.

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