[Osteogenesis imperfecta: Treatment and results of a case series].
OBJECTIVE
To describe our experience in the management of patients with osteogenesis imperfect (OI).


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.
Departamento de Cirugía Ortopédica y Traumatología, Clínica San Miguel, Pamplona, Navarra, España.
R J
RJ
Escribano-Rey
Departamento de Cirugía Ortopédica y Traumatología, Complejo Hospitalario de Navarra, Pamplona, Navarra, España. Electronic address: duart.julio@gmail.com.
J
J
Duart-Clemente
Servicio de Cirugía Ortopédica y Traumatología, Hospital de Cruces, Barakaldo, Vizcaya, España.
O
O
Martínez de la Llana
Departamento de Cirugía Ortopédica y Traumatología, Clínica Universidad de Navarra, Pamplona, Navarra, España.
J L
JL
Beguiristáin-Gúrpide
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D050723:Fractures, Bone / Q000209:etiology; D006801:Humans; D010013:Osteogenesis Imperfecta / Q000150:complications / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
Enclavamiento; Nailing; Osteogenesis imperfecta; Osteogénesis imperfecta; Osteotomy; Osteotomía; Outcomes; Resultados funcionales; Tratamiento; Treatment