Abstract
Spinal deformity in patients with cystic fibrosis (CF) is usually mild requiring no treatment. These patients are rarely considered as surgical candidates for scoliosis correction, as the pulmonary condition and other comorbidities increase the risk of general anaesthesia and recovery. This paper reviews all the literature up to date with regard to scoliosis in patients with CF and reports this unique case of a 14-year-old Caucasian girl with progressive scoliosis, who was treated surgically at the age of 17. She underwent a posterior spinal fusion T2-L3 with the use of unilateral segmental instrumentation. Preoperative workup included respiratory, cardiac, anaesthetic, endocrine, and dietician reviews, as well as bone density optimisation with zoledronic acid and prophylactic antibiotics. Surgical time was 150 minutes and intraoperative blood loss was 47% of total blood volume. Postoperative intensive care included noninvasive ventilation, antibiotic cover, pain management, chest physiotherapy, pancreatic enzyme supplementation, and nutritional support. She was discharged on day 9. At follow-up she had a good cosmetic outcome, no complaints of her back, and stable respiratory function. Multidisciplinary perioperative care and meticulous surgical technique may reduce the associated risks of major surgery in CF patients, while achieving adequate deformity correction and a good functional outcome.