探讨先天性膈肌发育异常疾病患儿手术治疗效果。

选择2011年1月1日至2023年9月30日于本中心小儿外科采取手术治疗的41例先天性膈肌发育异常疾病患儿为研究对象。将其中先天性膈疝(CDH)患儿，纳入CDH组(n＝27)；将先天性膈膨升(CDE)患儿，纳入CDE组(n＝14)。①对2组患儿的出生体重、出生胎龄、手术时年龄、诊断时肺头比，手术时间、术中出血量、术后住院时间等临床资料进行统计学分析。②对2组患儿术后总体生存(OS)率、复发率，上呼吸道症状、消化道症状、肺功能低下发生率，患儿身高、体重、活动能力、学习能力等临床资料进行统计学分析。本研究符合2013年修订的《世界医学协会赫尔辛基宣言》相关要求。患儿监护人对诊疗均知情同意，并签署临床研究知情同意书。本研究对2组患儿的随访时间定义为，除外术后住院期间死亡的4例CDH患儿，将术后继续参加随访，并且随访时间<5年者，分别定义为CDH短期随访患儿(n＝23)及CDE短期随访患儿(n＝14)，将随访时间≥5年者，分别定义为CDH中、长期随访患儿(n＝16)及CDE中、长期随访患儿(n＝8)。

①2组患儿的出生体重、出生胎龄，手术时间、术中出血量及术后住院时间分别比较，差异均无统计学意义(P>0.05)；而CDH组患儿的肺头比及手术时日龄均较CDE组小，差异均有统计学意义(P<0.05)。②CDH组与CED组患儿术后OS率分别为77.8%(21/27)、92.9%(13/14)，差异无统计学意义(P>0.05)。Kaplan-Meier生存曲线分析结果显示，术后2组出院患儿OS率比较，差异亦无统计学意义(P>0.05)。③术后CDH组完成短期随访患儿的消化道症状及肺功能低下发生率，分别为65.2%(15/23)与60.9%(14/23)，均较CDE组的28.6%(4/14)与21.4%(3/14)高，并且差异均有统计学意义(P<0.05)，而术后2组完成中、长期随访患儿的消化道症状及肺功能低下发生率分别比较，差异均无统计学意义(P>0.05)。术后2组患儿于短期及中、长期随访中，均未出现疾病复发及学习能力低于同龄儿表现。术后，2组短期及中、长期随访患儿的上呼吸道症状发生率与身高、体重低于同性别同龄儿及活动能力受限占比比较，差异均无统计学意义(P>0.05)。④术后，CDH组完成中、长期随访患儿的上呼吸道症状、消化道症状、肺功能低下发生率，分别为25.0%(4/16)、18.8%(3/16)与12.5%(2/16)，均较术后完成短期随访患儿的82.6%(19/23)、65.2%(15/23)与60.9%(14/23)低，并且差异均有统计学意义(P<0.05)，而其术后完成短期与中、长期随访患儿的身高、体重低于同性别同龄儿及活动能力受限占比比较，差异均无统计学意义(P>0.05)。⑤CDE组完成中、长期随访患儿的上呼吸道症状发生率为12.5%(1/8)，低于术后短期的68.8%(11/16)，并且差异有统计学意义(P<0.05)，而其术后完成短期与中、长期随访患儿的消化道症状、肺功能低下发生率与身高、体重低于同性别同龄儿及活动能力受限占比比较，差异均无统计学意义(P>0.05)。⑥41例患儿中，总体死亡率为17.1%(7/41)，其中左侧CDH患儿死亡为4例，右侧CDH为1例，双侧CDH为1例，右侧CDE为1例。

CDE患儿术后总体预后均较CDH患儿更好，CDE与CDH患儿术后中、长期预后均较短期预后好。但是，CDH患儿术后短期更容易出现消化道症状及肺功能低下症状，重症CDH患儿的预后仍然不是十分理想。

To investigate effectiveness of surgical treatment in children with congenital diaphragmatic dysplasia diseases.

A total of 41 cases of children with congenital diaphragmatic anomalies admitted to Department of Pediatric Surgery, Children′s Medical Center, Sichuan Provincial People′s Hospital, University of Electronic Science & Technology of China from January 1, 2011 to September 30, 2023 and treated surgically were selected for this study. Children with congenital diaphragmatic hernia (CDH) were included into CDH group (n=27), and children with congenital diaphragmatic eventration (CDE) were enrolled into CDE group (n=14). ①Clinical data such as birth weight, gestational age at birth, age at the time of surgery, lung-head ratio at the time of diagnosis, duration of surgery, intraoperative bleeding, and postoperative hospital stay were statistically analyzed in 2 groups. ②The postoperative overall survival(OS) rate and recurrence rate, incidences of upper respiratory tract symptoms, gastrointestinal tract symptoms, pulmonary hypofrontality, and children′s height, weight, mobility, learning ability and other clinical data of 2 groups were statistically analyzed. The study protocol was performed in accordance with the Helsinki Declaration of the World Medical Association revised in 2013, and informed consents were obtained from guardians of all participants. Excluding the 4 children with CDH who died during postoperative hospitalization, those who continued to participate follow-up after surgery in the above 2 groups with a follow-up period of <5 years were defined as children with short-term follow-up in CDH (n=23) and children with short-term follow-up in CDE (n=14), respectively. Those with a follow-up period of ≥5 years were defined as children with medium- and long-term follow-up in CDH (n=16) and children with medium- and long-term follow-up in CDE (n=8), respectively.

①There were no statistical differences between two groups in birth weight, gestational age at birth, duration of surgery, intraoperative bleeding and postoperative hospital stay (P>0.05); whereas the lung-head ratio and age at the time of surgery in CDH group were smaller than those in CDE group, and the differences were statistically significant (P<0.05). ②The postoperative OS rates of children in CDH group and CDE group were 77.8% (21/27) and 92.9% (13/14), respectively, with no statistically significant difference (P>0.05). Kaplan-Meier survival analyses indicated that there was no statistical difference in OS rate of children discharged from hospitals after surgery in both groups (P>0.05). ③The incidences of short-term postoperative gastrointestinal symptoms and pulmonary hypofrontality in CDH group was 65.2% (15/23) and 60.9% (14/23), respectively, which was higher than those of 28.6% (4/14) and 21.4% (3/14) in CDE group, and the differences were statistically significant (P<0.05), while there were no statistical differences in the incidences of medium- and long-term postoperative gastrointestinal symptoms and pulmonary hypofrontality between 2 groups (P>0.05). In the short-term, medium- and long-term postoperative follow-up period, there was no recurrence of CDH or CDE and the learning ability was lower than that of children of the same age in both groups, and differences in the incidence of upper respiratory symptoms, proportions of height and weight lower than that of children with the same gender and age, and activity limitation in the short-term, and medium- and long-term postoperative period of two groups were not statistically significant (P>0.05). ④The incidences of medium- and long-term postoperative upper respiratory symptoms, gastrointestinal symptoms, and pulmonary hypofrontality in CDH group were 25.0% (4/16), 18.8% (3/16), and 12.5% (2/16), respectively, which were lower than those of 82.6% (19/23), 65.2% (15/23), and 60.9% (14/23) in the short-term follow-ups, and all the differences were statistically different (P<0.05). While there were no statistical differences in the proportions of height and weight lower than those of children with the same gender and age, and mobility limitation in the short-term follow-up and medium- and long-term follow-up (P>0.05). ⑤The incidence of medium- and long-term postoperative upper respiratory symptoms in CDE group was 12.5% (1/8), which was lower than that 68.8% (11/16) in short-term follow-up (P<0.05), while the incidences of short-term, and medium- and long-term postoperative gastrointestinal symptoms, pulmonary hypofrontality, proportions of height and weight lower than children of the same gender and age, and mobility limitation in CDE group were not statistically significant (P>0.05). ⑥Of the 41 children, the overall mortality rate was 17.1% (7/41), with four deaths in children with left-sided CDH, one in right-sided CDH, one in bilateral CDH, and one in right-sided CDE.

Overall postoperative prognosis of children with CDE is better than that of children with CDH, and children with CDE and CDH all have a better medium- and long-term prognosis than short-term prognosis. However, children with CDH are more likely to develop gastrointestinal symptoms and pulmonary hypoplasia in short term, and prognosis of children with severe CDH is still not very satisfactory.