Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT, USA.
Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida, Jacksonville, FL, USA.
Br J Oral Maxillofac Surg. 2021 Jun;59(5):592-598. doi: 10.1016/j.bjoms.2020.10.008. Epub 2020 Dec 15.
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.
婴儿期行气管切开术可预防呼吸危机，帮助 Pfeiffer 综合征患者存活，但随后的拔管困难可能是一个挑战。本研究旨在探讨 Pfeiffer 综合征患儿咽鼻气道的区域性异常，为上气道的外科治疗和拔管提供解剖学基础。共纳入 72 例术前计算机断层扫描（CT）（Pfeiffer 综合征组 n=30；对照组 n=42）。使用 Materialise 软件测量气道容积、横截面积和头影测量值。Pfeiffer 综合征患儿的鼻腔气道容积减少了 50%（p<0.001），咽气道容积减少了 44%（p=0.003）。Pfeiffer 综合征患儿的后鼻孔横截面积仅为对照组的一半（p<0.001）。Pfeiffer 综合征患儿的鼻腔气道后段宽度缩短了 13%（p=0.003），高度降低了 21%（p<0.001）。提示咽气道入口和中段直径的髁突和下颌角水平的横截面积分别减少了 67%（p<0.001）和 47%（p<0.001）。与对照组相比，本组所有患者的鼻腔气道容积均明显受到限制，长度、高度和宽度均受到限制，后鼻孔狭窄。Pfeiffer 综合征患儿的鼻腔气道前后向长度缩短对上颌骨的影响大于其前后向位置。鼻腔气道的高度和宽度受限是由于蝶骨发育不良所致。咽气道的整个行程都存在限制的左右径和前后径。因此，除了上颌骨和下颌骨的前后向推进外，上颌骨的左右向扩张可能对这些患者有益。
