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Percutaneous intramedullary fixation of long bone deformity in severe osteogenesis imperfecta
K A McHale1, J J Tenuta, L L Tosi
1Orthopaedic Surgery Service, Walter Reed Army Medical Center, Washington, DC 20307-5001.
Insights
This study shows percutaneous intramedullary fixation is a safe method for treating severe osteogenesis imperfecta in young, fragile patients. It provides early stability, enabling mobility and supporting patients until definitive treatment is possible.
Area of Science:
- Orthopedic Surgery
- Pediatric Orthopedics
- Biomedical Engineering
Background:
- Severe osteogenesis imperfecta (OI) presents significant challenges in young, fragile patients.
- Unbraceable deformities and fragility often preclude early surgical intervention.
- Existing treatments may not be suitable for extremely ill or very young children with OI.
Purpose of the Study:
- To evaluate the safety and efficacy of percutaneous intramedullary fixation in severe pediatric osteogenesis imperfecta.
- To assess the method's utility in providing early skeletal stability for very young or medically compromised patients.
- To determine if this approach facilitates interim mobility and supports patients until definitive surgical treatment.
Main Methods:
- Seven pediatric patients with severe osteogenesis imperfecta (OI) underwent percutaneous intramedullary fixation of 25 long bones.
- Procedures included fixation of tibiae, femora, and ulnae, with some patients having all four lower extremity long bones operated on simultaneously.
- Follow-up ranged from 2 to 11 years.
Main Results:
- No neurologic, vascular complications, compartment syndromes, or growth plate issues were observed.
- All surgically treated bones achieved union.
- One patient experienced femoral pin migration into the knee joint.
- Post-bracing, all patients could sit, and five could stand and walk.
- Patients were supported until definitive fixation, averaging over 2 years post-procedure.
Conclusions:
- Percutaneous intramedullary fixation is a safe and reproducible technique for severe pediatric OI.
- This method offers early, stable fixation for patients too young or ill for extensive open surgery.
- The procedure does not compromise future treatment options and improves patient mobility.
Abstract:
Reported here are seven patients who had severe osteogenesis imperfecta, and who were extremely fragile, with three having cardiac disease. Their age ranged between 8 and 35 months. All seven patients had unbraceable deformities. Twenty five long bones, including 14 tibiae, 10 femora, and 1 ulna underwent percutaneous intramedullary fixation. Four patients had all four lower extremity long bones operated on simultaneously. Followup was 2 to 11 years. There were no neurologic or vascular complications, compartment syndromes, growth plate problems, or transfusion requirements. All bones healed. One patient had migration of a femoral pin into the knee joint. After bracing, all patients were able to sit; five were able to stand and eventually walk. All living patients were able to be sustained until successful definitive long bone fixation could be accomplished, approximately 2 years or longer after this initial procedure. With this safe, reproducible method, early stable fixation can be provided to patients too young and/or too sick for definitive or extensive open surgery. Future treatment regimens are not compromised.