Abstract
Individuals with severe osteogenesis imperfecta who require surgery often present a difficult management problem due to limb deformity and shortening, kyphoscoliosis, and deformity of the rib cage. All of these features may be associated with respiratory problems and impaired cardiovascular reserve. Surgical procedures and anaesthetic management represent a substantial challenge in these individuals. Here, we describe the clinical outcome of laparoscopic surgery to remove multiple gallstones in an individual with severe osteogenesis imperfecta. Meticulous pre-operative planning, combined with careful anaesthetic management resulted in a favourable outcome with a beneficial effect on quality of life. We provide a detailed account of the challenges faced and how these were surmounted in the hope that this may be of benefit to other clinicians faced with similar problems. Our experience demonstrates that laparoscopic surgery can be successfully performed in people with severe osteogenesis imperfecta with a favourable outcome.