In:
Jornal Vascular Brasileiro, FapUNIFESP (SciELO), Vol. 14, No. 4 ( 2015-12), p. 346-350
Abstract:
Abstract 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.
Type of Medium:
Online Resource
ISSN:
1677-5449
DOI:
10.1590/1677-5449.005615
Language:
Unknown
Publisher:
FapUNIFESP (SciELO)
Publication Date:
2015
detail.hit.zdb_id:
2571403-X
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