Onset and development of chronic rhinosinusitis from infancy through adolescence in primary ciliary dyskinesia detected by longitudinal magnetic resonance imaging

RATIONALE: A cross-sectional study employing magnetic resonance imaging (MRI) of paranasal sinuses recently showed a high prevalence and extent of chronic rhinosinusitis (CRS) in children with primary ciliary dyskinesia (PCD). However, longitudinal imaging data on CRS onset and progression are lacking. OBJECTIVE: To longitudinally evaluate CRS in PCD from infancy through adolescence with MRI. METHODS: 22 children with PCD (median baseline age 8.5 year, range 0.0-18.0 year) underwent a median of 3 (range 1-9) annual standardized paranasal sinus MRI examinations. MRI were assessed by 2 independent readers using the previously evaluated CRS-MRI score, including assessment of the prevalence and dominance (defined as the most prominent abnormality within a sinus) of each abnormality. RESULTS: At infancy (0 year), 83%-100% of paranasal sinuses and mastoid cells were opacified. Mucosal swelling was the most prevalent (83%-100%) and in most sinuses the dominant abnormality (50%-100%). In maxillary sinuses, also polyps (75%) and mucopyoceles (13%) were prevalent. At preschool age (1-5 year), the prevalence of opacified sphenoid sinus increased (83% vs. 100%, P < .01), while the prevalence of opacification was stable for the other sinuses as well as for mastoid cells (P = .567-0.999 vs. infancy). Mucosal swelling remained the most prevalent (100%) and dominant abnormality (47%-100%). In maxillary sinuses, the prevalence and subscore of mucopyoceles increased (81% vs. 13% and 2.0 ± 1.6 vs. 4.1 ± 2.1, respectively; P < .05), while polyps were stable (70% vs. 75%, P > .999 vs. infancy). At school age (≥6 year), almost all maxillary, sphenoid and ethmoid sinuses (91%-100%), and 87% of mastoid cells were opacified (P = .122-.999 vs. preschool age). Especially in maxillary sinuses, the prevalence of mucopyoceles, polyps and sinus deformation decreased (60% vs. 81%, 40% vs. 70%, and 51% vs. 78%, respectively; P < .05 vs. preschool age). The CRS-MRI sum score averaged 27.8 ± 4.9 at infancy, was stable at preschool age (31.9 ± 5.6, P > .999) and decreased at school age to 25.6 ± 8.3 (P < .01). CONCLUSIONS: Longitudinal paranasal sinus MRI detects high prevalence and extent of paranasal sinuses abnormalities from infancy. Our data support its role for comprehensive non-invasive monitoring of CRS in children with PCD.