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 the subjects underwent standard nocturnal polygraphy testing. 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 children 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. Unpaired t-test was 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) and palatal 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 mm2 and 3756.6 mm3, respectively). Correlation analysis showed that AHI 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 linear correlated to the palatal morphology and it shows a multifactorial aetiology.