Presentamos un caso de síndrome de EhIers-Danlos en su forma clásica que debido a la hiperlaxitud articular y ligamentosa se acompaña de complicaciones ortopédicas y deformidades esqueléticas de diverso tipo. Pectus carinatum con escoliosis secundaria pocas veces descrito, displasia de cadera, genu recurvatum, pie plano valgo. Además presentaba osteoprosis generalizada y dedos supernumerarios que no hemos encontrado escritos en la literatura.
Entre los antecedentes familiares se han encontrado alteraciones torácicas del mismo tipo que no hemos podido estudiar.
Por último, la indicación quirúrgica ha sido hecha desde el punto de vista estético a pesar de no presentar ninguna sintomatología cardiorespiratoria según la técnica de Ravit.
Los resultados inmediatos han sido satisfactorios (fig. 9) habiéndose reducido la deformidad, sin embargo, la cicatriz a pesar del injerto es anfractuosa, atrófica, como ocurre habitualmente en este síndrome.
The authors present a case of Ehlers-Danlos’ syndrome in its classical form that due to articular and ligamentous hyperlaxity was accompanied by orthopedic complications and skeletal deformities of various types, pectus carinatum with secondary scoliosis seldom described, dysplasia of the hip, genu recurvatum, pes planovalgus. There was also generalized osteoporosis and supernumerary fingers that the authors have not found described in medical literature.
In the familial antecedents there were thoracic alterarions of the same type, but they could not be studied.
Finally, the surgical indication was made from the esthetic point of view in spite of not presenting any cardiorespiratory symptomatology according to Ravit's technique.
The immediate results have been satisfactory. The deformity was reduced, how ever, in spite of the graft, the scar in anfractuous, astrophic as habitually. occurs in this syndrome.
