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目的:探讨在小婴儿Pierre Robin序列征中腭及舌骨的发育情况及其相关性。方法:回顾性分析2017年1月至2018年12月在南京医科大学附属儿童医院烧伤整形科行双侧下颌骨牵张成骨术的年龄小于3个月龄的Pierre Robin序列征患儿的资料,根据患儿术前头面部三维螺旋CT结果,评估腭和舌骨的发育情况,并对患儿进行分组。根据腭形态将患儿分为腭裂组和高腭弓组,根据舌骨形态分为正常组、轻度组、重度组。分析各组患儿术前年龄、性别、体质量、术前及延长器置入术后呼吸支持(气管插管)、喂养支持(鼻饲)情况、术前及术后3个月(延长器取出术前)时的营养(血清白蛋白浓度)情况。正态分布数据以n ±n s表示,非正态分布数据以n M(n P25,n P75)表示。患儿年龄、体质量及血清白蛋白指标的组间比较采用独立样本n t检验,各组间患儿术后插管及鼻饲时间比较采用Kruskal-Wallis秩和检验,术前及术后血清白蛋白指标比较采用配对样本n t检验,各组间术前患儿性别、气管插管、鼻饲喂养情况采用Fisher确切概率法分析,n P0.05)。喂养支持方面,高腭弓组(45.7%,21/46)比腭裂组(28.7%,35/122)术前鼻饲率更高(n P=0.044),且术后高腭弓组鼻饲时间明显长于腭裂组(n H=11.565,n P=0.001),差异均具有统计学意义。但2组患儿气管插管时间差异无统计学意义(n P>0.05)。术后3个月2组患儿呼吸、喂养困难等情况均明显改善,术后均不需气管插管及鼻饲喂养,血清白蛋白均明显增加,与术前比较差异均具有统计学意义(n P 0.05);重度组的患儿年龄明显小于正常组和轻度组,术前血清白蛋白也较另2组低,差异具有统计学意义(n P值均0.05). The breathing and feeding difficulties of the two groups of children were significantly improved three months after operation, and there was no need for endotracheal intubation and nasal feeding after the operation, and the serum albumin was significantly increased compared with those before the operation (n P0.05); the age of the severe group was significantly younger than the normal and mild group, and the preoperative serum albumin level was also lower than the other two groups, and the difference was statistically significant (n P<0.05). In terms of preoperative respiratory support, the endotracheal intubation rate was higher in the severe group (34.3%) compared with the normal group (14.5%) or the mild group (14.0%), the difference was statistically significant (n P0.05). In terms of preoperative nasal feeding, the difference in nasal feeding rate between the mild group (40.4%), the severe group (45.7%) and the normal group (22.4%) was statistically significant (n P0.05). Compared with the normal and mild group, the severe group had significantly longer postoperative endotracheal intubation time and nasal feeding time, and the difference was statistically significant (n P0.05). Serum albumin level of the three groups increased significantly at 3 months after operation, and the differences were statistically significant compared with those before operation (n P<0.001).n Conclusions:Infants with Pierre Robin sequence exist different degrees of abnormal morphology of the upper palate and hyoid bone. Patients with HAP have more severe swallowing and feeding problems, and babies with severe hyoid bone deformities are more serious, with severe respiratory and feeding problem. Therefore, early evaluation of the morphology of the palate and hyoid bone may conduce to assess postoperative prognosis, respiratory and feeding monitoring, and related prevention and treatment as soon as possible.