ÌÇÐÄ´«Ã½

Skip to main content
 
Notice: Users may be experiencing issues with displaying some pages on stanfordhealthcare.org. We are working closely with our technical teams to resolve the issue as quickly as possible. Thank you for your patience.
ÌÇÐÄ´«Ã½

THE IMMOTILE CILIA SYNDROME - RADIOLOGICAL MANIFESTATIONS

ÌÇÐÄ´«Ã½

THE IMMOTILE CILIA SYNDROME - RADIOLOGICAL MANIFESTATIONS RADIOLOGY NADEL, H. R., STRINGER, D. A., LEVISON, H., TURNER, J. A., STURGESS, J. M. 1985; 154 (3): 651–55

Abstract

The immotile cilia syndrome (ICS) is an uncommon disorder characterized by specific and genetically determined defects of cilia that cause upper and lower respiratory disease. We reviewed the radiographic patterns in 30 patients who had ICS (15 females, 15 males) and ranged in age from newborn to 26 years. Except for two neonates, sinusitis and otitis were present in all patients. Chest radiographic abnormalities, universally present, included bronchial wall thickening, hyper-inflation, segmental atelectasis or consolidation, and segmental bronchiectasis. Situs inversus, present in 50% (7 females, 8 males), was not an essential part of this disorder. Radiologically, the disease progresses from bronchial wall thickening with or without hyperinflation, to increasing hyperinflation plus parenchymal changes including segmental atelectasis, consolidation, and bronchiectasis. There is also a predilection for anatomic middle lobe abnormalities. The radiological appearance and clinical state have similarities to cystic fibrosis, although they are less severe and less progressive. ICS should be considered in the differential diagnosis of slowly progressive chronic lung disease, sinusitis, and otitis.

View details for

View details for

View details for