[Acetabular protrusion in Marfan's disease]

The etiologic assessment that had been made on the person of a young girl revealed a protrusio acetabuli which was painful and stiff and the evolution of which was quick, was allowed us to reveal a Marfan's syndrome. On this occasion, we have studied the links between the Marfan's syndrome and the protrusio acetabuli. After having resumed the history of this illness, the author remind us of its main characteristics while insisting upon the orthopedic abnormalities. They define the three steps of a protrusio acetabuli according to the Ruelle and Dubois criteria. For the child, the protrusio acetabuli is said to be primary without any well determined etiology. On the contrary, numerous studies deal with the idiopathic chondrolysis of the hips and describe surprising associations such as scoliosis, kyphosis, deformations of the hands and hereditary characteristics. Twelve observations of the Marfan's syndrome are presented, eleven of which concerning children and one concerning an adult. Among these twenty-four hips, the authors find nineteen protrude hips (80%) which belong to one of the three steps described by Ruelle and Dubois. There is an absence of clinical symptoms in nine cases among the twelve observed and the protrusion is systematically found. In three cases, there existed a functional symptomatology which in two cases, has led us to reveal a Marfan's syndrome. There were great pains of the mechanical type, linked to a limitation of the articular mobility; the radiography revealed an idiopathic chondrolysis of the hips. Moreover, the authors underline the primary importance of the skin-biopsy and of the metacarpal index measure in order to confirm the diagnostic of the Marfan's syndrome. Thus, the protrusio acetabuli appear to be a quasiconstant sign of Marfan's syndrome and has to be included in the long check of the orthopedic abnormalities of such a syndrome. The verification of a deep hip needs to research of the Marfan's syndrome. The primary protrusio acetabuli has to be closely studied, since we have just showed that the Marfan's syndrome was one its rare etiologies known up to now.