MRI of Pulmonary and Paranasal Sinus Abnormalities in Children with Primary Ciliary Dyskinesia Compared to Children with Cystic Fibrosis

RATIONALE: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliary clearance leading to chronic progressive lung disease as well as chronic rhinosinusitis (CRS). Both diseases share morphological and functional commonalities on magnetic resonance imaging (MRI) of lungs and paranasal sinuses, but comparative MRI studies are lacking. OBJECTIVES: Does PCD show different associations of pulmonary with paranasal sinus abnormalities on MRI as well as with lung function tests in infants to adolescents in comparison to children with CF? METHODS: 18 children with PCD (median age 9.5 [3.4-12.7]y, range 0-18y) and 36 age-matched, cystic fibrosis transmembrane conductance regulator modulator (CFTRm)-naïve children with CF (median age 9.4 [3.4-13.2]y, range 0-18y) underwent same-session chest and paranasal sinus MRI, as well as spirometry (ppFEV1) and multiple breath washout (lung clearance index [LCI] z-score). Pulmonary and paranasal sinus abnormalities were assessed using previously validated chest MRI and CRS-MRI scoring systems. RESULTS: Mean chest MRI global score was similar in children with PCD and CF (15.0 [13.5-20.8] vs. 15.0 [9.0-15.0], P=0.601). Consolidations were more prevalent and severe in children with PCD (56% vs. 25% and 1.0 [0.0-2.8] vs. 0.0 [0.0-0.3], respectively; P<0.05). The chest MRI global score correlated moderately with ppFEV1 in both, children with PCD and with CF (r=-0.523 and -0.687, P<0.01), and with LCI in CF (r=0.650, P<0.001) but not in PCD (r=0.353, P=0.196). CRS-MRI sum score and mucopyocele subscore were lower in children with PCD than with CF (27.5 [26.3-32.0] vs. 37.0 [37.8-40.0] and 2.0 [0.0-2.0] vs. 7.5 [4.8-9.0], respectively; P<0.01). CRS-MRI sum score did not correlate with chest MRI scores in PCD (r=0.075 to 0.157, P=0.557-0.788), while it correlated moderately with MRI morphology score in CF (r=0.437, P<0.01). CONCLUSIONS: MRI detects differences in lung and paranasal sinus abnormalities between children with PCD and with CF. Lung disease does not correlate with CRS in PCD but in CF.

• Wucherpfennig, L.
• Wuennemann, F.
• Eichinger, M.
• Schmitt, N.
• Seitz, A.
• Baumann, I.
• Roehmel, J. F.
• Stahl, M.
• Hämmerling, S.
• Chung, J.
• Schenk, J. P.
• Alrajab, A.
• Kauczor, H. U.
• Mall, M. A.
• Wielpütz, M. O.
• Sommerburg, O.