RATIONALE: Chronic rhinosinusitis (CRS) contributes to morbidity in patients with cystic fibrosis (CF). However, longitudinal data on CRS onset and progression is lacking. OBJECTIVES: To longitudinally evaluate CRS in CF from infancy to school age with paranasal sinus magnetic resonance imaging (MRI). METHODS: 64 children with CF (mean age at baseline 1.1±1.6y, range 0-5y) underwent a mean of 5.8±2.2 (range 3-11) subsequent annual MRI examinations. Additional 24 children (9.2±4.4y, range 3-17y) homozygous for the F508del mutation underwent MRI before and at least two months after starting lumacaftor/ivacaftor. MRI was assessed using the previously evaluated CRS-MRI score. RESULTS: In infancy, 65-87% of paranasal sinuses were opacified, and mucosal swelling was the dominant abnormality (58-97%). At preschool age (1-5y), 79-94% of sinuses were opacified (P<0.05 vs. infancy), and mucosal swelling was the most dominant abnormality (79-94%, P<0.05). At school age (≥6y), almost all sinuses were opacified (71-99%, P<0.001-0.357 vs. preschool age), and mucopyoceles were the dominant abnormality in maxillary and frontal sinuses (53-56%, P<0.05-0.808). The CRS-MRI sum score increased from 22.4±9.6 in infancy to 34.2±9.6 in preschool age (P<0.001) and was 34.0±5.7 in school age (P=0.052). In children under lumacaftor/ivacaftor therapy the CRS-MRI sum score (-0.5±3.3, P<0.05) and maxillary sinus subscore (-0.5±1.5, P<0.05) improved. CONCLUSIONS: Longitudinal paranasal sinus MRI detects an early onset and progression of severity of CRS from infancy to school age, and response to lumacaftor/ivacaftor therapy in children with CF. Our data support its role for comprehensive non-invasive monitoring of CRS in children with CF. Clinical trial registered with ClinicalTrials.gov (NCT02270476).
- Wucherpfennig, L.
- Wuennemann, F.
- Eichinger, M.
- Schmitt, N.
- Seitz, A.
- Baumann, I.
- Stahl, M.
- Graeber, S. Y.
- Chung, J.
- Schenk, J. P.
- Alrajab, A.
- Kauczor, H. U.
- Mall, M. A.
- Sommerburg, O.
- Wielpütz, M. O.
