SPINAL DEFORMITIES IN YOUNG PATIENT WITH CYSTIC FIBROSIS (CF): PROPOSAL OF A SCREENING AND FOLLOW UP PROTOCOL

Background: Increased survival in patients (pts) with CF showed the importance of assessing extrapulmonary complications, as scoliosis (S); the prevalence of S in North America was 20 times greater in CF children than in the general population. S is an evolutive spinal deformity which causes functional disorders on the spinal structures and leads to a restrictive pulmonary disease. Skeletal CF abnormalities (malnutrition, delayed bone mineralization and pubertal development, muscular dysfunctions, osteoporosis) may increase the risk of S. The conservative treatment with brace is often difficult in CF pts because of ventilatory problems. Aims: 1) create a screening protocol to determine prevalence of S in a group of CF pts; 2) assess the reliability of S screening by CF respiratory therapist (RT); 3) create a flow-chart to monitor S. Materials and Methods: Pts with CF diagnosis (confirmed by sweat test or genotyping), 6-19 years old (yrs), regular follow-up in Treviso CF Centre and ability to perform the evaluation maneuver were included. The study is divided into 4 parts: 1) Creation of a protocol to detect spinal deformities: evaluation of posture and measurement of hump by quantification of the angle of trunk rotation (ATR) using Bunnell’s scoliometer in the Adams forward bending test position; 2) RT evaluation of all pts; 3) Blind specialist (sp) evaluation of all pts with ATR≥4° and some with ATR 0°-3°; 4) Definition of a flow chart to monitor S evolution in all pts. Results: In all, 55 pts (26 males) with mean age 12.91 yrs (6.17–19.22), mean BMI 17.86 kg/cm2, mean FEV1 91.42%pred and mean FVC 96.36%pred performed the RT’s evaluation. Male and female groups are homogeneous for age, BMI and indexes of respiratory function. Based on ATR values, pts are divided in two groups: 1) negative group (N) with ATR 0°-3°; 2) positive group (P) with ATR>4°. Between N and P there was a statistical difference for FVC (p>0.001). Twenty thoracic (74.07%), 5 lumbar (18.52%) and 2 thoracolumbar (7.41%) curves were found; leg dysmetria >1 cm was evidenced in 66.66% of P. All pts of P and some of N were also blindly evaluated by a sp physician and measured ATR was compared to RT’s values. There was a significant correlation between the 2 evaluations (R2=0.857; p<0.0001). Seven spine radiographs were indicated by specialist’s assessment; S (>10° Cobb) was diagnosed in 3 pts (5.45%), 2 males, with the indications of brace. Conclusions: The higher prevalence of S in CF pts (5.45%) showed the importance of assessing this aspect. This simple screening protocol with ATR measurement is a feasible and reliable way to assess spinal deformities in CF pts and to identify the need for sp evaluation. Considering the frequency of reevaluation in the healthy population and the high prevalence of risk factors in young CF pts, a flow chart was created: all pts have to be reassessed by the rt every 6 months (pts with fast statural growth should be evaluated more frequently) and pts with ATR>4° should be sent to the sp. Further studies will be necessary to establish S prevalence in adult CF pts. Reassessment of these pts and follow-up of pts with brace prescription will be necessary.