The Coronal and Sagittal Vertebral Balance is Affected by the Severity of the Disease in Pediatric Patients with Cystic Fibrosis: A Pulmonary Function Test-Based Cross-Sectional Study.

Abstract

Background: Although cystic fibrosis (CF) mainly affects the respiratory and gastrointestinal systems, it may frequently present with musculoskeletal manifestations including bone fractures, low bone mineral density, and spinal pathologies. Assessment of spinal pathologies in CF patients is of vital importance because the effects on lung capacity and spinal posture are clearly defined.

Questions/purposes: The frequency of vertebral pathologies in CF patients has yet to be determined. The aim of this study was to investigate the frequency of scoliosis and hyperkyphosis and the relationship of coronal, sagittal, and spinopelvic parameters with disease severity in CF patients.

Methods: Patients were tested with forced expiratory volume in 1 second (FEV1), dual-energy x-ray absorptiometry (DEXA), and full spine radiographs. Measurements were taken of the major coronal curve in the coronal plane, cervical and lumber lordosis, thoracic kyphosis, and C7 plumb line values. Patients were categorized into 3 groups based on the FEV1 values (severity) from respiratory function tests (severe: group 1 FEV 1≤40, moderate: group 2 FEV1 40 to 80, mild: group).

Results: This cross-sectional study included 208 CF patients aged 5 to 21 years. The rates of scoliosis and thoracic hyperkyphosis were 31% (n=64) and 24% (n=50), respectively. The highest rates of scoliosis (63%) and thoracic hyperkyphosis (56%) were found in the severe CF group (P=0.016 and P=0.006, respectively). FEV1 and thoracic kyphosis were weakly and inversely but significantly correlated (rho: -0.200 and P=0.004). There was no difference in BMD between patients with and without scoliosis and between patients with and without hyperkyphosis. There was no significant difference in DEXA Z-score between patients with and without hyperkyphosis. The L1-L4 DEXA Z-score of patients without scoliosis was significantly higher (P=0.017).

Conclusions: Scoliosis and hyperkyphosis were more prevalent in the severe CF patients group, although the proportion of patients requiring treatment was relatively low. Understanding the relationship between disease severity and coronal and sagittal spinal balance, and spinopelvic parameters is crucial, as it guides the early detection and management of scoliosis in CF patients.

Level of evidence: Level II.