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贝-威二氏综合征患者行或不行舌切除术治疗后下颌生长的长期纵向评估

Long-term longitudinal evalutation of mandibular growth in patients with Beckwith-Wiedemann Syndrome treated and not treated with glossectomy.

机构信息

University of Milan, Smile House, Regional Center for CLP, Department of Maxillo-Facial Surgery, Santi Paolo and Carlo Hospital, Via di Rudinì 8, 20142, Milan, Italy; San Gerardo Hospital, Monza, Italy.

University of Milan, Smile House, Regional Center for CLP, Department of Maxillo-Facial Surgery, Santi Paolo and Carlo Hospital, Via di Rudinì 8, 20142, Milan, Italy.

出版信息

J Craniomaxillofac Surg. 2020 Dec;48(12):1126-1131. doi: 10.1016/j.jcms.2020.09.004. Epub 2020 Sep 12.

Abstract

AIM

This study compares long-term mandibular growth between a group of Beckwith-Wiedemann Syndrome (BWS) patients who underwent glossectomy at an early age and a group of patients not operated.

METHODS

Cephalometric measurements were performed in BWS patients comparing the data obtained between a group of patients operated at an early age and a group of non-operated patients who declined surgery. Statistics included independent sample T-test.

RESULTS

Twenty-four out of 78 BWS patients followed since birth completed longitudinal cephalometric x-rays at age 5, 10 and 15. Eighteen patients needed early surgery. Eleven families accepted glossectomy at 2.3 ± 1.3 years of age; seven declined surgery. No differences in mandibular growth were found between the two groups. Inclination of maxillary incisors results were statistically greater in the non-operated group (operated compared to the non-operated group: 103.58 ± 11.30 Vs 108.98 ± 12.47; p-value 0.0168 at 5; 107.06 ± 7.98 Vs 115.14 ± 7.05; p-value 0.0206 at 10; 109.80 ± 4.68 Vs 116.75 ± 5.28; p-value 0.0233 at 15).

CONCLUSION

Macroglossia has no role in the post-natal mandibular overgrowth in BWS and mandibular overgrowth is part of the syndrome. Therefore, early glossectomy does not change mandibular growth and does not prevent the development of class III skeletal malocclusion in these patients.

摘要

目的

本研究比较了一组早龄行舌切除术的 Beckwith-Wiedemann 综合征(BWS)患者与一组未手术患者的长期下颌生长情况。

方法

对 BWS 患者进行头影测量,比较早龄手术组和拒绝手术组患者的数据。统计方法包括独立样本 T 检验。

结果

自出生以来,78 例 BWS 患者中有 24 例完成了 5 岁、10 岁和 15 岁的纵向头颅侧位 X 线片。18 例患者需要早期手术。11 个家庭在 2.3±1.3 岁时接受了舌切除术;7 个家庭拒绝手术。两组患者的下颌生长无差异。上颌切牙倾斜结果在未手术组中统计学上更大(手术组与未手术组相比:103.58±11.30 Vs 108.98±12.47;p 值 0.0168 在 5 岁时;107.06±7.98 Vs 115.14±7.05;p 值 0.0206 在 10 岁时;109.80±4.68 Vs 116.75±5.28;p 值 0.0233 在 15 岁时)。

结论

巨舌症在 BWS 患者的下颌生长中没有作用,下颌生长是该综合征的一部分。因此,早期舌切除术不会改变下颌生长,也不能预防这些患者发生 III 类骨骼错颌畸形。

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