BACKGROUND Marfan syndrome is a rare autosomal dominant inherited disease of the connective tissue associated with various craniofacial abnormalities. Aim of the present study was to assess the variability of palatal shape in a sample of 31 Marfan patients compared to a control group of no syndromic subjects, in two stages of dentition, by using 3D geometric morphometric analysis. METHODS Thirty one growing subjects with Marfan syndrome were selected and divided into two subgroups: MG1 with mixed dentition (10 M, 6F, mean age 7+/- 0.7 years), MG2 with permanent dentition (8 M, 7F, mean age 13+/- 0,5 years). Each subgroup was compared to a control group (CG1 mixed dentition, 9 M, 7F, mean age 7.6+/- 0.5 years; CG2 permanent dentition, 9 M, 6F, mean age 12.8+/- 0.7 years) matched on age, sex distribution, stage of dentition and skeletal maturation. Then the two subgroups were compared one to each other. For each patient maxillary dental casts were taken, scanned and digitized. 3D geometric morphometric methods were applied. Procrustes analysis was used and principal component analysis was performed to reveal the main patterns of palatal shape variation. RESULTS Both Marfan subgroups showed important reductions in the transversal plane associated with a deep palatal vault when compared to the control groups (MG1 vs CG1 P = 0,003; MG2 vs CG2 P = 0,07). Moreover a statistically significant difference between the palatal shape of MG1 and MG2 was found (P = 0.017) showing a significant worsening of palatal depth and constriction from mixed to permanent dentition in Marfan subjects. CONCLUSION Marfan subjects showed a specific palatal morphology with maxillary constriction and deeper palatal vault when compared to a control group of healthy subjects. The constriction and the depth of the palatal vault in Marfan patients worsen from mixed dentition to permanent dentition more then in no syndromic subjects.

中文翻译：

---

马凡生长受试者上颌形态的 3D 评估：一项对照临床研究。

背景马凡综合征是一种罕见的与各种颅面畸形相关的结缔组织常染色体显性遗传病。本研究的目的是通过使用 3D 几何形态测量分析，在牙列的两个阶段，评估 31 名马凡患者样本与无综合征受试者的对照组相比，腭形状的变异性。方法 选择 31 名成长中的马凡综合征受试者并分为两个亚组：混合牙列的 MG1（10 M，6F，平均年龄 7+/- 0.7 岁），恒牙列的 MG2（8 M，7F，平均年龄 13+） /- 0,5 年）。每个亚组与年龄、性别匹配的对照组（CG1 混合牙列，9 M，7F，平均年龄 7.6+/- 0.5 岁；CG2 恒牙列，9 M，6F，平均年龄 12.8+/- 0.7 岁）进行比较分布、齿列阶段和骨骼成熟。然后将两个亚组相互比较。对每位患者的上颌牙模型进行拍摄、扫描和数字化处理。应用了 3D 几何形态测量方法。使用 Procrustes 分析并进行主成分分析以揭示腭形状变化的主要模式。结果 与对照组相比，两个马凡亚组均显示与深腭穹窿相关的横切面显着减少（MG1 vs CG1 P = 0,003；MG2 vs CG2 P = 0,07）。此外，发现 MG1 和 MG2 的腭形状之间存在统计学显着差异 (P = 0.017)，表明 Marfan 受试者的腭深度和从混合牙列到恒牙列的收缩显着恶化。结论与健康受试者的对照组相比，马凡受试者表现出特定的腭形态，具有上颌收缩和更深的腭穹窿。马凡患者的腭穹窿收缩和深度从混合牙列恶化到恒牙列比没有综合征的受试者更严重。