Purpose: To describe variations of paranasal sinus development in patients
with cystic fibrosis (CF) and in non-CF patients examined for inflammatory
sinonasal disease. We focused on anatomic variants that predispose to orbit
al and cerebral penetration during functional endoscopic sinus surgery (FES
S), e.g. hypoplasia of the maxillary sinus and low ethmoid roof.
Material and Methods: One hundred and sixteen CF patients (3-54 years, medi
an 18) and 136 control patients (7-51 years, median 31) were examined with
coronal CT of the paranasal sinuses. CF patients were grouped according to
number of confirmed mutations: CF-2 (n=70), CF-1 (n=32), CF-0 (n= 14). CT i
mages were evaluated with respect to paranasal sinus development, pneumatiz
ation variants and bony variants.
Results: Frontal sinus aplasia and maxillary, ethmoid, and sphenoid sinus h
ypoplasia were markedly more frequent in CF-2 than in control patients. No
CF-2 patient had pneumatization variants such as Haller cells or concha bul
losa. Low ethmoid roof was seen in 30% of CF-2 children, but in no control
children. CF-I and CF-0 groups had prevalences of aplasia and hypoplasia in
termediate to that of CF-2 and control patients.
Conclusion: Genetically verified CF patients had less developed sinuses, la
cked pneumatization variants, and more often had anatomic variants that pre
dispose to complications during FESS. Normally developed sinuses and pneuma
tization variants in some genetically unverified CF patients (CF-1, CF-0) s
uggest that these patients may be erroneously diagnosed.