Tracheotomy in infancy helps patients with Pfeiffer syndrome to survive by preventing respiratory crisis, but difficulty in decannulation may consequently be a challenge. This study has investigated the regional abnormalities of the nasopharyngeal airway in children with Pfeiffer syndrome to provide an anatomical basis for the surgical treatment and decannulation of the upper airway. Seventy-two preoperative computed tomograms (CT) (Pfeiffer syndrome n=30; control n=42) were included. The airway volume, cross-sectional area, and cephalometrics were measured using Materialise software. Patients with Pfeiffer syndrome developed a 50% (p<0.001) reduction of nasal airway volume, and a 44% (p=0.003) restriction in pharyngeal airway volume. In patients with Pfeiffer syndrome the cross-sectional area at the choana was only half that of the controls (p<0.001). The posterior width of the nasal airway in patients with Pfeiffer syndrome was shortened by 13% (p=0.003), and the height reduced by 21% (p<0.001). The cross-sectional areas at the condylion and gonion levels, which indicate the calibre of the pharyngeal airway at the entrance and midsection, were reduced by 67% (p<0.001) and 47% (p<0.001), respectively, when compared with the controls. The volume of the nasal airway in patients with Pfeiffer syndrome was significantly restricted in length, height, and width, and by choanal stenosis in all cases in this cohort. The reduced anteroposterior length of the nasal airway contributed to the shortened maxilla more than the anteroposterior position. The limited height and width of the nasal pathway was the result of a hypoplastic sphenoid. Restricted mediolateral and anteroposterior dimensions were evident across the entire course of the pharyngeal airway. Mediolateral maxillary expansion in addition to maxillomandibular advancement is therefore likely to benefit these patients.

婴儿期气管切开术可通过预防呼吸道疾病帮助Pfeiffer综合征患者生存，但因此难以进行无环切术可能是一个挑战。本研究调查了菲佛综合症患儿鼻咽气道的局部异常，为上呼吸道的手术治疗和脱管提供了解剖学基础。包括七十二个术前计算机体层摄影（CT）（普发综合征30例，对照组42例）。使用Materialize软件测量气道容积，横截面积和脑电图。患有Pfeiffer综合征的患者的鼻气道容积减少了50％（p <0.001），并且咽气道容积受到了44％（p = 0.003）的限制。在患有Pfeiffer综合征的患者中，胸膜的横截面积仅为对照组的一半（p <0.001）。Pfeiffer综合征患者的鼻气道后宽度缩短了13％（p = 0.003），高度降低了21％（p <0.001）。相比于入口和中段，咽和气孔水平的横截面积分别减少了67％（p <0.001）和47％（p <0.001），这表明咽部入口和中段的口径。控件。在该队列中的所有病例中，Pfeiffer综合征患者的鼻道容积在长度，高度和宽度上均受到显着限制，并且由于狭窄性狭窄而受到限制。鼻气管前后长度的减少比上颌后位置的作用更大。鼻通路的高度和宽度受到限制是蝶骨发育不良的结果。在咽气道的整个过程中，明显限制了内侧和后方的尺寸。因此，除了上颌下颌前移以外，内侧外侧上颌骨扩张也可能使这些患者受益。