Анатомічні варіанти вродженої діафрагмальної кили, їх клінічне значення та можливості пренатальної диференціації

Abstract

Purpose — to present verified typical anatomical variants of isolated congenital diaphragmatic hernia and clinical outcomes in newborns depending on the type of pathology, to compare with data of prenatal examination, and to assess feasibility of prenatal differentiation of congenital diaphragmatic hernia. Materials and methods. The data of operation protocols and autopsy results of newborn patients with isolated congenital diaphragmatic hernia for the period 2007–2020 were analyzed, and then compared with prenatal exam data and clinical outcomes. Data from different anatomical variants of congenital diaphragmatic hernia were analyzed using descriptive statistics methods. Results. Anatomical data were evaluated in 67 cases with the following typical variants: left-sided non-communicating defect (20.9%), left-sided communicating with herniation of intestine (19.4%), intestine and stomach (26,9%), intestine, stomach and liver (19.4%, 13/67), right- sided communicating with intestine and liver herniation (10.4%), right- sided non-communicating (1.5%), bilateral communicating defects (1.5%). Mortality at the stage of stabilization in these variants was 0%, 0%, 11.1%, 30.8%, 71.4%, 0% and 100%, postoperative mortality, respectively, 7.1%, 0%, 12.5%, 44.4%, 0%, 0% (excluding bilateral hernia), total mortality 7.1%, 0%, 22.2%, 61.5%, 71.4%, 0%, 100%. Comparison of lung indices in patients with left-sided hernias showed their similarity in groups with non-communicating defects and communicating with herniation of intestine. Significant differences were found in the groups with herniation of the intestine and stomach, and intestines, stomach and liver. The mean liver-to-lung ratio in right-sided communicating defects was 3.7±1.9, in left-sided communicating defects 1.7±0.8 and in non-communicating 0.44±0.25, the difference between all groups was highly significant. Patterns of stomach position in different variants of pathology were determined. Conclusions. Analysis of postnatally verified cases of diaphragmatic hernia showed marked anatomical variability. The highest mortality and the lowest rate of surgical correction registered was in communicating right-sided defects, and in communicating left-sided with simultaneous herniation of the intestine, stomach and liver. The best outcomes were found in non-communicating defects, or in communicating with herniation of intestine. Prenatal evaluation of stomach position may be the basis to differentiation between clinico-anatomical variants of the pathology. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the Institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: congenital diaphragmatic hernia, congenital malformations, prenatal diagnosis.