小婴儿Pierre Robin序列征中腭与舌骨发育情况的观察

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±s表示,非正态分布数据以M(P25,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,P<0.05认为差异有统计学意义。结果168例患儿被纳入研究,其中男79例,女89例,就诊年龄(36±22)d,入院时体质量(3.6±0.5)kg,术前插管率18.5%(31/168),术前鼻饲率33.3%(56/168)。观察腭形态发现122例(72.6%)患儿伴有腭裂(腭裂组),46例(27.4%)表现为高腭弓(高腭弓组)。腭裂组及高腭弓组患儿在入院年龄、体质量、性别、术前气管插管呼吸支持、血清白蛋白方面差异无统计学意义(P>0.05)。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(P=0.044),且术后高腭弓组鼻饲时间明显长于腭裂组(H=11.565,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(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(P<0.001)。舌骨形态发育情况显示76例(45.2%)患儿舌骨形态发育正常(正常组),57例(33.9%)舌骨体或舌骨支结构完整,但形态较正常小(轻度组),35例(20.8%)部分或全部舌骨结构缺如(重度组)。正常组和轻度组在入院年龄和术前血清白蛋白方面比较差异无统计学意义(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(P值均<0.05)。术前呼吸支持方面,重度组气管插管率更高(34.3%),与正常组(14.5%)或轻度组(14.0%)之间比较,差异具有统计学意义(P<0.05),正常组与轻度组之间比较,差异无统计学意义(P>0.05)。术前鼻饲喂养方面,轻度组(40.4%)、重度组(45.7%)与正常组(22.4%)鼻饲率差异均具有统计学意义(P<0.05),而轻度组与重度组之间差异无统计学意义(P>0.05)。术后情况比较,重度组与正常组和轻度组相比,气管插管时间及鼻饲时间明显延长,差异均具有统计学意义(P<0.05),而轻度组与正常组间比较,差异无统计学意义(P>0.05)。3组患儿术后3个月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(P<0.001)。结论Pierre Robin序列征患儿存在不同程度的腭及舌骨形态异常。伴高腭弓患儿有较重的吞咽及喂养困难;严重舌骨发育畸形的患儿病情更重,有严重的呼吸和喂养困难。因此,早期评价腭及舌骨形态可能有助于评估术后预后情况,可尽快进行呼吸和喂养监测及相关的预防和治疗。

The development of palate and hyoid bone in infants with Pierre Robin sequence

Objective To prospectively evaluate the development of palate and hyoid bone in infants with Pierre Robin sequence patients.Methods A retrospective study was performed on 168 patients younger than 3 months ago who underwent bilateral mandibular distraction osteogenesis in the Department of Burn and Plastic Surgery,Children’s Hospital of Nanjing Medical University from January 2017 to December 2018.Using three dimensional-computed tomography(3D-CT)scan to reconstruct and survey the pattern of palate and hyoid bones.According to the palate morphology,the patients were divided into cleft palate group(CP group)and high palate arch group(HAP group).And according to the severity,they were divided into normal group,mild group and severe group based on the morphology of hyoid bone.Data were analyzed including the preoperative age,gender,body weight,respiratory(endotracheal intubation)and feeding support(nasal feeding)before and after distractors placement,and nutrition(serum albumin level)before and 3 months after the operation(before the distractors removal).Normal distribution datais represented by Mean±SD,and non-normal distribution data is represented to M(P25,P75).Independent sample t-test was used to compare the age,body weight and serum albumin level.Kruskal-Wallis rank sum test was used to compare the postoperative intubation and nasal feeding time in each group.Paired sample t-test was used to compare the serum albumin before and after the operation.The preoperative gender,tracheal intubation,and nasal feeding of the children between each group were analyzed by Fisher’s precise test.P<0.05 considered the difference to be statistically significant.Results 168 infants(79 boys and 89 girls)with Pierre Robin sequence were included.The age was(36±22)d,the body weight at admission was(3.6±0.5)kg,and the preoperative intubation rate was 18.5%(31/168),the preoperative nasal feeding rate was 33.3%(56/168).122(72.6%)have cleft palate,and 46(27.4%)have highly arched palate.There was no significant difference in admission age,gender,preoperative respiratory support or serum albumin between the two groups(P>0.05).In terms of feeding support,the HAP group(45.7%,21/46)had a higher preoperative nasal feeding rate than the CP group(28.7%,35/122)(P=0.044),and had significantly longer postoperative nasal feeding time than CP group(H=11.565,P=0.001),the differences were statistically significant.However,there was no significant difference in the time of endotracheal intubation between the two groups(P>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(P<0.001).Further observation of hyoid bone morphology showed that normal hyoid bone morphological development in 76 cases(45.2%,normal group),complete hyoid body or hyoid ramus structure with smaller shape than normal in 57 cases(33.9%,mild group),and partial or complete hyoid bone structure absence in 35 cases(20.8%,severe group).There was no significant difference in the age of admission and preoperative serum albumin between the normal and mild group(P>0.05);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(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(P<0.05).There was no statistically significant difference between the normal group and the mild group(P>0.05).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(P<0.05),while there was no statistically significant difference between the mild and severe group(P>0.05).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(P<0.05),while the mild group and the normal group had no significant difference Significance(P>0.05).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(P<0.001).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.