[Morpho-functional iterative surgery in a XXXX with von Recklinghausen disease].

@entity1 @entity851 @entity101 @entity5 @entity3292 @entity1635 @entity5150 @entity74 @entity4786 @entity1265
@entity4786 1 is an @entity74 with an estimated incidence 1:2500 to 1:3000 live newborns. The disease presents with multiple cutaneous and non cutaneous lesions. @entity1265 occurs with equal frequency in males and females and has been identified in all ethnic group. The morbidity and the mortality caused by @entity1265 are the result of complications that may involve any of the body systems. This disease has been linked with mutations of the @entity1265 gene which encodes @entity5 suppressor neurofibromin. At least half of @entity1 with @entity1265 will have only cutaneous involvement that is not considered to be a major medical problem, even though it can be a source of psychologic burden as a result of cosmetic disfigurement. The cardinal features of the disorder are caf  -au-lait spots, axillary freckling, cutaneous @entity3292 and Lisch nodules, but there are a lot of wide variety of complications affecting almost every system of the body, including the eyes ( @entity5150 ), the nervous system ( @entity5 ), the skeleton ( @entity851 , @entity1635 ), the endocrine and cardiovascular system ( @entity101 ). Manifestations of @entity1265 vary at different times in an individual's life. Substantial variability exists among affected members of a single family. This variability confounds clinical management and the severity of the disease cannot be predicted. We present a case in young @entity1 24 years-old treated by reiterative plastic surgery.
