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.