Influence of maxillary morphology on nasal airway resistance in Marfan's syndrome

High nasal airway resistance (NAR) has been reported in Marfan's syndrome, and this appears to contribute to the development of obstructive sleep apno ea in these patients. The cause of high NAR in Marfan's syndrome is unknown , but these patients characteristically have a narrow maxilla, which could have an influence on nasal dimensions. The aim of this study was to define the mechanism(s) mediating high NAR in Marfan's syndrome. Five patients wit h Marfan's syndrome (mean age 29 +/- 4 (SEM) years) were compared with an e quivalent number of normal control subjects (31 +/- 1 years). NAR was measu red by posterior rhinomanometry, before and after topical decongestant, nas al stenting, or both. Dental impressions were taken to evaluate maxillary a rch morphology, allowing measurement of the following lateral distances: in ter-canine (ICD), inter-premolar (IPD), and inter-molar (IMD). NAR (at a fl ow of 500 ccm/s) was considerably higher in patients compared with controls at baseline (0.93 +/- 0.08 vs 0.35 +/- 0.08 Pa/ccm/s, p < 0.001), and foll owing decongestant and/or stenting. The maxillary arch was considerably nar rower in patients. There were strong inverse correlations between the later al maxillary dimensions and NAR after nasal decongestant, with or without s tenting. These results indicate a strong association between maxillary widt h and NAR, and suggest that maxillary constriction is the dominant mechanis m for the high NAR in Marfan's syndrome. The therapeutic implications of th is finding warrant further investigation.