Osteogenesis Imperfecta (OI) and Spine Deformity

Trial Name

Osteogenesis Imperfecta (OI) and Spine Deformity

What is the trial about?

The first aim of this study is to maintain a registry of patients who were treated surgically for a spinal deformity and associated OI.

To measure quality of life outcomes, the following questionnaires will be administered:

• SRS-22  (Scoliosis Research Society)
• PROMIS (Patient Report)

Who can participate?

• Patients age >5 years and <21 years
• Male or female
• Osteogenesis Imperfecta (OI) Diagnosis
• Diagnosis of spinal deformity such as but not limited to: scoliosis, spondylolysis, spondylolisthesis craniocervical junction abnormalities for which surgery is recommended to prevent progression of the curvature or to correct deformity.

What is involved?

All study related evaluations will be performed during your routine care visits with your Doctor. Study-related evaluations will require an additional 20-30 minutes to complete during your routine care visits. You are expected to come back for follow-up visits with your surgeon at approximately 1 month after surgery, 6 months, 1 year, 2 years, 5 years, and 10 years. We may follow you for up to 25 years. These are the normal clinical visits for this condition. We may have to contact you by telemedicine, phone, email, or other communication methods for follow-up as needed if you do not have the means or need to travel back to Nemours for a clinical visit.

The researchers will be collecting the following information about your surgery:

• Information from surgical, anesthesia and neuromonitoring reports
• Complications
• Hospital Course
• Overall spine curvature correction seen from X-rays
• A tracing of the shape of the rods will be taken on a piece of paper in the operating room before the rods are implanted

The information from the routine tests listed below will be used for the study. The SRS questionnaire and PROMIS questionnaires are not specifically study related and are part of your clinical care. Scoliometer and trunk flexibility measurements, are study-related and are not a part of your routine care. Questionnaires can be completed on a paper form, responding to investigator by phone/telemedicine or electoronically, using the REDCap database system. The REDCap questionnaires system is a secure, password protected Nemours system that can be completed using a tablet at the clinical visit, by opening a link sent by email from the investigators or responding by phone/telemedicine.

There are no study required X-rays and blood draws.