Abstract

Osteogenesis imperfecta (OI) often results in recurrent fractures and/or progressive bowing of the long bones, including the arms. Upper extremity deformity has been shown to negatively impact function. The objective of this retrospective case series is to assess the ability to correct deformity, improve function and evaluate the complications and revision rates in our patients with OI who have undergone forearm deformity correction. A retrospective study, approved by The University of Nebraska Medical Center Institutional Review Board, was conducted with OI patients who underwent forearm osteotomy and fixation of one or both forearm bones between December 2011 and August 2018. There were no exclusion criteria. The electronic medical records were reviewed for patient demographics, surgical details, revisions and complications. A total of 48 procedures on 27 forearms in 18 patients were performed during the study. Surgery was performed in children with forearm deformity and recurrent fractures that were interfering with function. Half of the patients had surgery on one forearm and half had surgery on both forearms. The majority of the patients have Type III OI. There were multiple complications, the most common being wire migration which required either replacement or advancement of the wire. In conclusion, forearm deformity in OI is possible, with good healing of osteotomies and fractures, although many patients may require multiple surgical interventions.

Highlights

  • Osteogenesis imperfecta (OI) is a heterogeneous genetic disorder most commonly causing Type I collagen abnormalities

  • Surgery was performed in children with forearm deformity and recurrent fractures that were interfering with function

  • Surgical treatment of femoral and tibial bowing with osteotomies and intramedullary fixation is well accepted in patients with moderate to severe OI and has been shown to improve function and decrease fracture rates [2]

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Summary

Introduction

Osteogenesis imperfecta (OI) is a heterogeneous genetic disorder most commonly causing Type I collagen abnormalities. Abnormal Type I collagen results in frequent fractures often from minimal trauma, bowing deformities of long bones, short stature and scoliosis. Two recent publications from high volume OI centers reviewed patients who underwent forearm deformity correction and showed improved function and decreased fracture burden [5,7]. The objectives of this retrospective case series are to assess the ability to correct forearm deformity and function with variations of surgical intervention practiced in our clinic and to evaluate the rates of complication and revision surgery

Materials and Methods
Results
Unilateral
Bilateral Forearm Surgery Patients
Single versus Multiple Forearm Surgery
Ulnar Fixation Only
Discussion
Full Text
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