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.