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Surgical outcomes for spinal deformity in osteogenesis imperfecta
Susan Sienko1, Carol Tucker1,2, Michelle Cameron Welborn3
1Shriners Children's Portland, 3101 SW Sam Jackson Park Rd, Portland, OR, 97239, USA.
Spine Deformity
|November 12, 2022
Summary
Scoliosis affects 18.1% of pediatric patients with osteogenesis imperfecta (OI), with only 17.2% requiring surgery. Revision surgery rates were 8%, highlighting the need for careful management in this fragile population.
Area of Science:
- Pediatric Orthopedics
- Genetics
- Spine Surgery
Background:
- Osteogenesis imperfecta (OI) is a prevalent genetic bone disorder in children.
- Scoliosis is frequently reported in OI patients, but perioperative outcomes are poorly understood.
- This study investigates scoliosis prevalence and surgical outcomes in OI patients.
Purpose of the Study:
- To determine the prevalence of scoliosis in pediatric patients with OI.
- To analyze surgical progression, complication rates, and revision surgery in this population.
- To explore associations between complications and surgical intervention factors.
Main Methods:
- Retrospective review of the Shriners Health Outcomes Network (SHOnet) database (2011-2017).
- Inclusion criteria: ICD codes for OI and scoliosis.
- Data collected: demographics, fractures, complications, bisphosphonate use, surgical details.
Main Results:
- 18.1% of 2372 OI patients had scoliosis; 17.2% of these underwent surgery.
- Average pre-operative Cobb angles were 58.18° (thoracic) and 59.83° (thoracolumbar).
- 8% of surgically treated patients required revision surgery at an average of 3.88 years post-op.
Conclusions:
- Scoliosis incidence in OI may be lower than previously reported (18.1% in this study).
- A small proportion of OI patients with scoliosis require surgical intervention.
- Bisphosphonate use did not show a clear association with progression to surgery in this cohort.
