[Osteo-neuro-meningeal-lumbosacral involvement in Marfan syndrome. Report of a case].
Marfan syndrome is an hereditary condition which primarily affects conjunctive tissue with predominant vascular lesions, aortic insufficiency and aortic dissection which condition vital prognosis. Until further progress is made in the genetic determination of the disease, the diagnosis is currently based on the association of clinical criteria, which enables multidisciplinary management. This approach should lead to specific medical and surgical treatment--which may reduce Marfan morbidity and mortality. We report the case of a 45 year-old patient with suspected Marfan syndrome during adolescence. The presence of a cardiovascular lesion and a recently reported abnormality i.e. a sacral erosion by a dural ectasia, enabled us to confirm the diagnosis. We reviewed the current criteria in Marfan diagnosis and their specific management.
Boyer
A
A
Département de Médecine Interne, CHU Rouen Boisguillaume.
Marie
I
I
Primard
E
E
Cailleux
N
N
Lévesque
H
H
Courtois
H
H
D001847:Bone Diseases / Q000175:diagnosis*; D002318:Cardiovascular Diseases / Q000175:diagnosis; D002493:Central Nervous System Diseases / Q000175:diagnosis*; D005128:Eye Diseases / Q000175:diagnosis; D006801:Humans; D008161:Lumbosacral Region; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000235:genetics; D008588:Meningocele / Q000175:diagnosis*; D008875:Middle Aged
Atteinte ostéo-neuro-méningée lombosacrée au cours du syndrome de Marfan. A propos d'une observation.