Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, Connecticut, USA.
Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida, Jacksonville, Florida, USA.
Int J Oral Maxillofac Surg. 2021 Jul;50(7):924-932. doi: 10.1016/j.ijom.2020.11.023. Epub 2020 Dec 29.
The severity of obstructive respiratory difficulty varies among affected Crouzon syndrome patients. The aim of this study was to investigate the correlation between the restricted airway volume in Crouzon syndrome and the associated type of cranial vault suture synostosis. Computed tomography scans of 68 unoperated Crouzon syndrome patients and 89 control subjects were subgrouped into four types: type I, bilateral coronal synostosis; type II, sagittal synostosis; type III, pansynostosis; type IV, perpendicular combinations of synostoses. Measurements were made using Mimics software. Of type I Crouzon patients, 42% had a restricted nasal airway (P=0.002), while the pharyngeal airway volume was not significantly reduced. Type II Crouzon patients grew normal segmental airway volumes. Crouzon patients of type III developed simultaneously reduced nasal and pharyngeal airway volumes in infancy, by 38% (P=0.034) and 51% (P=0.014), respectively. However, the nasal airway achieved a normal volume by 2 years of age without any intervention, while the pharyngeal airway remained significantly reduced up to 6 years of age, by 42% (P=0.013), compared to controls. Type IV Crouzon patients developed a reduced nasal airway volume (32%, P=0.048) and a non-significant restricted pharyngeal airway (18%, P=0.325). Airway compromise in Crouzon syndrome is variable when associated with different craniosynostosis fusion patterns. Type II (sagittal synostosis) Crouzon patients grew a normal nasopharyngeal airway volume. Those with types I (bicoronal synostosis) and IV (perpendicular synostoses) had significantly restricted nasal airways and a tendency towards a reduced pharyngeal volume. Type III (pansynostosis) Crouzon infants had the worst restriction of both airways, although there was some improvement with age.
患有颅缝早闭综合征的患者，其呼吸阻塞的严重程度各不相同。本研究旨在探讨颅缝早闭综合征患者气道容积受限与颅盖缝融合类型之间的相关性。将 68 例未经手术治疗的颅缝早闭综合征患者和 89 例对照者的 CT 扫描结果进行亚组分析，分为 4 种类型：I 型，双侧冠状缝早闭；II 型，矢状缝早闭；III 型，颅缝全部早闭；IV 型，多种缝早闭。使用 Mimics 软件进行测量。I 型颅缝早闭综合征患者中，42%存在气道狭窄（P=0.002），但咽气道容积无明显减小。II 型颅缝早闭综合征患者的气道各段容积正常。III 型颅缝早闭综合征患者在婴儿期同时出现鼻气道和咽气道容积减小，分别减少 38%（P=0.034）和 51%（P=0.014）。然而，鼻气道在 2 岁时达到正常体积，无需任何干预，而咽气道在 6 岁时仍明显减小，减小 42%（P=0.013），与对照组相比。IV 型颅缝早闭综合征患者出现鼻气道容积减小（32%，P=0.048）和咽气道受限（18%，P=0.325）。当颅缝早闭综合征与不同的颅缝融合类型相关时，气道阻塞的程度不同。II 型（矢状缝早闭）颅缝早闭综合征患者的鼻咽气道容积正常。I 型（双冠状缝早闭）和 IV 型（垂直颅缝早闭）患者的鼻气道明显受限，咽气道容积有减小的趋势。III 型（颅缝全部早闭）婴儿的气道受限最严重，尽管随着年龄的增长有所改善。
