Osteogenesis imperfecta: Treatment and results of a case series

OBJECTIVES:
To describe our experience in the management of patients with osteogenesis imperfect (OI).

MATERIAL AND METHODS:
We conducted a retrospective study of a series of cases affected with OI treated in the Clínica Univesidad de Navarra from 1980 to 2007, with a mean follow up of 17.3 years (7-27 years). We collected descriptive data of the sample, the fractures and the deformities, and the treatments given. The complications presented and the functional outcomes at the end of follow-up were also reviewed.

RESULTS:
The sample included ten patients. Approximately two-thirds (65%) of fractures were sustained in the lower limbs. One patient received medical treatment only. Three patients had combined medical and surgical treatment. Some type of surgical treatment was performed on 6 patients. The most common surgery was the Sofield-Millar performed on 37 occasions, with a third of them requiring revision surgery due to migration of the nails. There were 17 episodes of re-fracture. Complications such as non-union, iatrogenic fractures, and infections, were also observed. The functional outcome, according to the Hoffer-Bullock scale, at the end of follow-up was grade I/II in 7 patients.

CONCLUSIONS:
Despite the need for multiple interventions and complications presented during follow up, the appropriate treatment of patients with OI can provide acceptable functional outcomes.

CITA DEL ARTÍCULO  Rev Esp Cir Ortop Traumatol. 2014 Mar-Apr;58(2):114-9. doi: 10.1016/j.recot.2013.11.007.