Brittle Bone Disease Clinical Trial
Official title:
Preventive Fixation of Lower Limbs in Osteogenesis Imperfecta (Brittle Bone Disease) With the Highlight of the Fassier-Duval
Osteogenesis imperfecta (or brittle bone disease) is a rare genetic disease characterized by
fragile bone and a low mass ossue, secondary to abnormal collagen synthesis. This is a real
congenital osteoporosis. The prevalence of the disease is not known precisely, but it is 1
in 10000 at 20000. There are many forms of osteogenesis imperfecta to classify patients with
symptomatology minor to the patients with lethal form during the neonatal period. The main
symptoms are dominated by fractures and bone deformities, particularly in the lower limbs.
Bisphosphonate medication is used for over 10 years to reduce the number of fractures.
However the long-term effects are not known to date, not allowing even to establish proof of
the benefit risk. Thus unable to process all of these patients and over a long time, these
drug treatment leaves much therapeutic solutions to surgery. The goal of surgery is to treat
fractures, treat bone deformities and prevent fractures future. In the long bones of the
limbs, the only effective techniques are intramedullary nailing. The majority of realized
nailing nailing are either sliding or telescopic enabling having a reinforced bone of an
intramedullary osteosynthesis material over its entire length during the period of bone
growth. It has been shown that the technique of the sliding nailing was inexpensive but
reliable especially before the age of 5 years. After that age, all are telescopic nailing
nailing. The first telescopic nail described is the Bailey-Dubow nail still widely used in
France. However, the number of complications relating to its use is important. Thus, there
are 8% of disunity equipment and 33 to 72% of the nail migration forcing him to change one
or more times during growth. A new nail presented at the French orthopedic company in 2005
and Fassier Duval reported a much lower complication rate because the rate of nail migration
is only 9%, without opening the knee joint, which is not possible with the highlight of
Bailey-Dubow.
It is proposed to conduct a prospective series of 10 nailing the lower extremities with this
nail Fassier-Duval in patients with osteogenesis imperfecta and compare the results with a
series of patients already treated with Bailey-Dubow nails in order to highlight the
advantages and disadvantages of using of such a nail.
n/a
Endpoint Classification: Safety/Efficacy Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment