Obstructive sleep apnea in children with Marfan syndrome: Relationships between three-dimensional palatal morphology and apnea-hypopnea index.

Objective:To evaluate the relationship between the severity degree of OSA (apnea/hypopnea index AHI > 1)and palatal area and volume, measured by 3D analysis of digital casts in Marfan children.Methods:Twenty children with a clinical diagnosis of MS were recruited from a tertiary medical center. All thesubjects underwent standard nocturnal polygraphy testing. Sixteen Marfan patients (7F,9 M; mean age8.8yy ± 1.5yy) with AHI > 1 were enrolled. Marfan Group (MG) was compared with a control group (CG) of 17children without Marfan syndrome (9F,8 M; mean age 8.5yy ± 1.7yy) presenting with nose-breathing pattern.For each subject maxillary digital casts were taken and palatal area and volume were measured. Unpairedt-testwas used to test significant differences between MG and CG for area and volume measurements. Pearson cor-relation coefficient (PCC) was used to measure the linear correlation between the degree of OSA (AHI index) andpalatal volume and palatal area.Results:80% of Marfan children presented an AHI > 1 and a diagnosis of OSA. MG presented statistically sig-nificant lower values of palatal surface area (662.68 mm2; P < 0.0001) and palatal volume (2578.1 mm3;P < 0.0001) with respect to CG (923.0 mm2and 3756.6 mm3, respectively). Correlation analysis showed thatAHI index had no linear correlation with palatal area (r = - 0,07) and with palatal volume (r =−0,11).Conclusion:OSA is highly prevalent in children with Marfan's syndrome (80%). Marfan children present a re-duction of palatal area and volume when compared to healthy subjects. OSA in Marfan children is not linearcorrelated to the palatal morphology and it shows a multifactorial aetiology