Hypoplasia of the sphenoid sinuses as a diagnostic tool in cystic fibrosis.
OBJECTIVE
To measure and compare the size of the sphenoid sinuses in patients with cystic fibrosis (CF) to patients with inflammatory sinonasal disease, and to correlate the size with number of CF mutations in each patient.


METHODS
Ninety-six CF patients aged 5-47 years (median 19 years) and 130 control patients aged 7-51 years (median 32 years) were examined using coronal CT of the paranasal sinuses. In each patient, the CT image with the largest coronal area of the sphenoid sinuses was scanned into a Macintosh computer with image processing and analysis software. Largest coronal area and largest circumference of the right and left sphenoid sinuses were automatically measured. Additionally, antero-posterior extension of the sphenoid sinuses was calculated from the lateral scanograms. CF patients were grouped according to number of confirmed mutations (CF-0, CF-1, or CF-2).


RESULTS
CF patients generally had small sphenoid sinuses. The largest differences for all parameters were observed between the CF-2 and the control groups (p<0.0001). No CF-2 patient had pneumatization beyond the presphenoid. The CF-0 and CF-1 groups consisted of two populations, one overlapping the CF-2 group and another overlapping the control group.


CONCLUSIONS
Hypoplasia of the sphenoid sinuses is a characteristic finding in CF patients. When pneumatization of the basisphenoid is present, the existing CF diagnosis should be questioned.
Eggesbø
H B
HB
Department of Radiology, Aker Hospital, University of Oslo, Norway.
Eken
T
T
Eiklid
K
K
Kolmannskog
F
F
D000293:Adolescent; D000328:Adult; D001798:Blood Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010254:Paranasal Sinus Diseases / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics; D013101:Sphenoid Sinus / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed
D001798:Blood Proteins; C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator