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非综合征性矢状缝早闭和综合征性影响对眼眶和眶周畸形的影响。

Influence of Nonsyndromic Bicoronal Synostosis and Syndromic Influences on Orbit and Periorbital Malformation.

机构信息

From the Division of Plastic and Reconstructive Surgery, Yale School of Medicine; Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; and Department of Plastic Surgery, University of São Paulo.

出版信息

Plast Reconstr Surg. 2022 May 1;149(5):930e-942e. doi: 10.1097/PRS.0000000000009051. Epub 2022 Mar 14.

Abstract

BACKGROUND

Oculoorbital disproportion in patients with craniosynostosis has similarities and dissimilarities between syndromic and nonsyndromic cases. The authors hypothesized that these two conditions have specific individual influences as they relate to development of the orbital and periorbital skeletons.

METHODS

A total of 133 preoperative computed tomography scans (nonsyndromic bicoronal synostosis, n = 38; Apert syndrome bicoronal synostosis subtype, n = 33; Crouzon syndrome bicoronal synostosis subtype, n = 10; controls, n = 52) were included. Craniometric and volumetric analyses related to the orbit and periorbital anatomy were performed.

RESULTS

Orbital cavity volume was mildly restricted in nonsyndromic bicoronal synostosis (7 percent, p = 0.147), but more so in Apert and Crouzon syndromes [17 percent (p = 0.002) and 21 percent (p = 0.005), respectively]. The sphenoid side angle in Apert syndrome was wider than when compared to Crouzon syndrome (p = 0.043). The ethmoid side angle in Apert patients, however, was narrower (p = 0.066) than that in Crouzon patients. Maxilla anteroposterior length was more restricted in Apert syndrome than Crouzon syndrome (21 percent, p = 0.003) and nonsyndromic cases (26 percent, p < 0.001). The posterior nasal spine position was retruded in Crouzon syndrome (39 percent, p < 0.001), yet the anterior nasal spine position was similar in Apert and Crouzon syndromes.

CONCLUSIONS

Orbit and periorbital malformation in syndromic craniosynostosis is likely the combined influence of syndromic influences and premature suture fusion. Apert syndrome expanded the anteriorly contoured lateral orbital wall associated with bicoronal synostosis, whereas Crouzon syndrome had more infraorbital rim retrusion, resulting in more severe exorbitism. Apert syndrome developed maxillary hypoplasia, in addition to the maxillary retrusion, observed in Crouzon syndrome and nonsyndromic bicoronal synostosis patients.

CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

摘要

背景

颅缝早闭患者的眶距异常在综合征型和非综合征型病例之间存在相似性和差异性。作者推测,这两种情况具有特定的个体影响,因为它们与眼眶和眶周骨骼的发育有关。

方法

共纳入 133 例术前计算机断层扫描(非综合征性冠状缝早闭,n = 38;Apert 综合征冠状缝早闭亚型,n = 33;Crouzon 综合征冠状缝早闭亚型,n = 10;对照组,n = 52)。对与眼眶和眶周解剖结构相关的颅计量和容积分析进行了研究。

结果

非综合征性冠状缝早闭患者的眶腔容积轻度受限(7%,p = 0.147),但 Apert 和 Crouzon 综合征患者的眶腔容积受限更明显[17%(p = 0.002)和 21%(p = 0.005)]。Apert 综合征患者的蝶骨侧角比 Crouzon 综合征患者的更宽(p = 0.043)。然而,Apert 患者的筛骨侧角更窄(p = 0.066)。Apert 综合征患者的上颌前后长度比 Crouzon 综合征患者更受限(21%,p = 0.003)和非综合征性病例(26%,p < 0.001)。Crouzon 综合征患者的后鼻棘位置后移(39%,p < 0.001),而 Apert 和 Crouzon 综合征患者的前鼻棘位置相似。

结论

综合征型颅缝早闭患者的眶和眶周畸形可能是综合征型影响和过早融合的综合影响。Apert 综合征扩大了与冠状缝早闭相关的前侧轮廓的外侧眶壁,而 Crouzon 综合征则有更多的眶下缘后缩,导致更严重的眼球突出。Apert 综合征除了 Crouzon 综合征和非综合征性冠状缝早闭患者的上颌后缩外,还出现了上颌发育不良。

临床问题/证据水平:风险,II。

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