Materials and Methods:Twenty children with a clinical diagnosis of MS were recruited from the Centre for Rare Diseases for Marfan Syndrome and Related Disorders of Tor Vergata University Hospital and evaluated in the Departments of Orthodontics of the same University. All the subjects underwent standard nocturnal polygraphy testing. Inclusion criteria were: genetic assessment of MS, Caucasian ancestry, first permanent molars erupted and second deciduous molars present, prepubertal stage of cervical vertebral maturation (CS1, CS2), good quality of records, AHI > 1. Sixteen Marfan patients (7F,9M;mean age 8.8yy +/- 1.5yy) with AHI>1 were enrolled. Marfan Group (MG)was compared with a control group (CG) of 17 prepubertal subjects without Marfan syndrome(9F,8M;mean age 8.5yy+/-1.7yy)with no transverse skeletal discrepancies presenting with nose-breathing pattern.For each subject dental casts were taken before any treatment.Maxillary casts were scanned and a gingival plane and a distal plane were used as boundaries for measuring palatal area and volume on digital cast. Unpaired t-test was used to test significant differences between MG and CG for area and volume measurements.The Pearson correlation coefficient (PCC) was used to measure the linear correlation between the degree of OSA (AHI index) and the palatal volume and the palatal surface.
Results: 80% of Marfan children presented an AHI>1 and a diagnosis of OSA. The MG presented statistically significant lower values of the palatal surface area (662.68 mm2; P<0.0001) and palatal volume (2578.1 mm3; P<0.0001) with respect to the CG (923.0 mm2 and 3756.6 mm3, respectively). The results of the correlation analysis showed that the apnoea–hypopnoea index had no linear correlation with the palatal area (r = – 0,07) and with the palatal volume (r= – 0,11).
Conclusions: OSA is highly prevalent in children with Marfan’s syndrome (80%). Marfan children present a reduction of the area and the volume of the palate when compared to healthy subjects. OSA in Marfan children is not linear correlated to the palatal morphology and it shows a multifactorial aetiology.
Paoloni V, Cretella Lombardo E, Laganà G, Cozza P.
Department of Orthodontics, Department of Clinical Sciences and Translational Medicine, University of Rome Tor Vergata, Rome, Italy