Osteogenesis Imperfecta

Osteogenesis imperfecta (OI), also known as brittle bone disease, is a genetic disease involving primarily the skeleton but affecting all organ systems. It is an extremely multifarious condition causing frequent fractures and limb and spinal deformity, which can often be severe. While there are described surgical procedures to treat these patients, due to the variety of the deformities and the fragility of the bone, the orthopaedic surgeon is often forced to adapt or modify a plan during an operation to achieve success. Surgical experience with the disease worldwide is often limited and variable, and due to constraints on medical equipment and surgical implants in resource-constrained countries, alternate methods of achieving the similar surgical endpoints are possible.
This book will help guide decision-making in surgery using biologic and surgical principles to assist the planning and execution of surgery with available resources. It will then provide the surgeon with background knowledge of the genetic, medical and surgical principles necessary to formulate a comprehensive treatment plan, illustrated by varied and complex patient cases from experienced surgeons and clinicians worldwide. Beginning with an introduction to osteogenesis imperfecta and the general care of the patient, the book is divided into thematic sections covering general surgical considerations, principles of extremity surgery, and surgical cases on the pelvis, upper and lower extremities, and the spine, it will incorporate aspects of surgical decision-making, including cultural and geographic factors, to give a truly global perspective on the care of these complex patients.

Richard W. Kruse, DO, MBA, FAAOS, FAAP, FAOA, Clinical Professor, Department of orthopaedic Surgery, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA