scholarly journals Anatomical variants of congenital diaphragmatic hernia, their clinical significance and feasibility of prenatal differentiation

Author(s):  
G.O. Grebinichenko ◽  
◽  
I.Y. Gordienko ◽  
O.K. Sliepov ◽  
A.O. Zhuravel ◽  
...  

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.

2018 ◽  
pp. 40-44
Author(s):  
G.O. Grebinichenko ◽  
◽  
I.Yu. Gordienko ◽  

The objective: to compare lung indices in fetuses with different anatomical variants of left-sided congenital diaphragmatic hernia, determined by two-dimensional ultrasound examination. Materials and methods. Data from 157 ultrasound examinations in 136 fetuses as patients with the most typical anatomical variants of left-sided diaphragmatic hernia were analyzed. The area of the lungs and the herniated liver were measured in the standard cross section of fetal thorax, at the level of a four-chamber view, with following calculation of prognostic indices. Comparison of indices’ means in groups depending on the abdominal organs herniated into fetal chest was performed using Student’s t-test. Results. The most numerous group in our study was one with herniation of the liver, stomach and intestines (62.5%). Significantly lower values of all lung indices were found in this group (p≤0.0001). In particular, observed-to-expected area ratio equaled to 35.59±8.8 and 31.73±10.51 in these fetuses in II and III trimesters respectively. In fetuses with intestines herniation, this index was 60.67±10.93 and 57.37±7.98, in fetuses with stomach and intestines herniation – 53.20±7.02 and 51.77±9.57 in II and III trimesters, respectively. Differential analysis of lung indices depending on the degree of liver herniation showed the presence of statistically highly significant difference in both second and third trimesters. In the group with mild liver herniation, the largest lung sizes and indices were determined, which were similar to values registered in groups without hepatic herniation. The lowest values of all lung indices were registered in the group with severe liver herniation. Conclusions. The anatomical variant of left diaphragmatic hernia with herniation of liver, stomach and intestines into thorax was the most common among our study population; in this group the lowest values of term-independent lung indices were observed. However, this group turned out to be heterogeneous: with different degrees of liver herniation, different degrees of pulmonary hypoplasia were found. Significant differences in lung indices found in II trimester can become a basis for early assessment of the severity of pathology and prediction of postnatal outcome. Keywords: congenital diaphragmatic hernia, liver herniation, pulmonary hypoplasia, lung size, prenatal diagnosis.


2020 ◽  
pp. 47-53
Author(s):  
G.O. Grebinichenko ◽  
◽  
I.Yu. Gordienko ◽  
O.K. Sliepov ◽  
◽  
...  

The objective: to characterize pregnancy course and clinical outcomes for newborns in cases of congenital diaphragmatic hernia, and to compare these data with the results of prenatal examinations Materials and methods. Results of complex prenatal examination of 259 fetuses as patients with diaphragmatic hernia, which were referred to the department of Fetal medicine in 2007–2020, were analyzed. Data on pregnancy course and clinical outcome for infants were collected in 144 cases: from medical records of the Institute (n=77), and by direct survey, if delivery and specialized care took place in other institutions (n=67). The results were compared depending on the clinical outcome and the place of care. Results. Among 144 cases with known outcome, 140 (97.2%) were singleton and 4 (2.8%) were twin pregnancies with only one affected fetus. Termination of pregnancy for fetal anomalies before to 22 weeks was performed in 16.7% (44/144), 83.3% of cases (120/144) ended in childbirth. Before 22 weeks for prenatal examination were referred 30.6% (44/144) of women, of them 54.5% (24/44) have chosen termination in presence of unfavorable prognosis, and in the remaining 45.5% (20/44) cases pregnancy was prolonged. Of the 120 labors, 92.5% were term and 7.5% preterm. Antenatal death after 22 weeks occurred in 4.2% (5/120) cases. Among liveborn infants before surgical correction in the clinics of the Institute have died 25% (18/72), and in other institutions 37.2% (16/43), р>0.05. Postoperative mortality was 13% (7/54) in the Institute and 42.3% (11/26) in other institutions, р=0.0032. The rate of associated pathology was significantly higher among children treated in other institutions (25.6% vs. 5.6%), but the proportion of operated patients did not differ significantly (60.5% in other institutions and 75% in the Institute). Comparison of data of prenatal examination showed significantly higher rate of associated pathology (26.3% vs. 3.2%), polyhydramnios (50.9% vs. 20.6%), right-sided hernia (21.2% vs. 6.3%) and liver herniation in left-sided hernia (81.8% vs. 28.8%) in the group with negative outcome. Conclusions. The majority of pregnancies with diaphragmatic hernia in the fetus ended in childbirth, with high mortality rate. Strategy of complex prenatal examination in cases of congenital diaphragmatic hernia, prognosis evaluation by multidisciplinary council with individualized pregnancy/labor management planning, and delayed surgical correction allow to optimize specialized care and to avoid ineffective surgical interventions in extremely severe clinical and anatomical variants of pathology. Keywords: congenital diaphragmatic hernia, prenatal diagnosis, chromosomal anomalies, congenital malformations, pregnancy, labor.


Author(s):  
G. Grebinichenko ◽  
I. Gordienko ◽  
O. Tarapurova

Purpose – to present detailed description for typical variants of the stomach location in left-sided congenital diaphragmatic hernia in the fetus in relation to the main topographic landmarks of the chest and abdomen, and to determine the relationship between the position of the stomach and anatomical variants of pathology. Material and methods. Analysis of the data from 235 ultrasound examinations of 157 fetuses in patients with left-sided diaphragmatic hernia, which were referred in 2007-2020. Results. In 3 cases (1.91 %) identification of the stomach and its location was impossible. In the remaining 154 cases, the following typical variants of stomach position were identified: intra-abdominal (22.73 %), intra-thoracic medial (12.99 %) and medial oblique (4.55 %), mid-ventral (21.43%), transverse ( 16.23 %), mid-dorsal (5.19 %), retrocardiac (5.84 %), paravertebral (2.6 %), left paracostal (1.3 %), and left pseudoanatomical (7.14 %) . With the abdominal localization of the stomach there was a shift of its pyloric part down to the bladder in 74.3 %, and in 17.1 % of cases stomach position was changed for intra-thoracic. Liver herniation was registered in 14.3% of cases with intra-abdominal stomach position, in 87.5% of mid-dorsal, and in 100% of cases with central-ventral, transverse, retrocardiac, paravertebral and left paracostal position. The liver was not detected in the thorax in cases with medial stomach position, and its identification was complicated in cases with oblique medial position. Conclusions. Typical anatomical variants of the stomach and other herniated abdominal organs location in the thorax of fetuses with left-sided diaphragmatic hernia were determined. The proposed classification system of anatomical variants of left diaphragmatic hernia with an emphasis on the stomach position will allow to improve the accuracy of prenatal ultrasound diagnosis, as well as individual prediction of postnatal outcome. Key words: congenital diaphragmatic hernia, congenital malformations, liver herniation, stomach position.


2018 ◽  
Vol 53 (1) ◽  
pp. 113-117 ◽  
Author(s):  
Carmen Mesas Burgos ◽  
Björn Frenckner ◽  
Matias Luco ◽  
Matthew T. Harting ◽  
Pamela A. Lally ◽  
...  

2015 ◽  
Vol 46 (2) ◽  
pp. 150-154 ◽  
Author(s):  
I. S. Werneck Britto ◽  
O. O. Olutoye ◽  
D. L. Cass ◽  
I. J. Zamora ◽  
T. C. Lee ◽  
...  

2019 ◽  
Vol 30 (01) ◽  
pp. 051-058
Author(s):  
Keita Terui ◽  
Kouji Nagata ◽  
Masahiro Hayakawa ◽  
Hiroomi Okuyama ◽  
Shoichirou Amari ◽  
...  

Abstract Introduction We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings. Material and Methods Derivation (2011–2016, n = 350) and validation (2006–2010, n = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we created a prediction model and weighted scoring system from the derivation dataset and calculated the odds ratio of an unsatisfactory prognosis (death within 90 days of life or hospitalization duration exceeding 180 days). Five adverse prognostic factors obtained using prenatal ultrasound, including an observed/expected lung area-to-head circumference ratio (o/eLHR) <25%, liver herniation occupying more than one-third of the thoracic space, thoracic stomach, right-side CDH, and severe malformations, were used as predictors. The obtained model was validated using the validation cohort. Results The unsatisfactory prognosis prediction model was obtained based on the adjusted odds ratios. The C statistics of the model were 0.83 and 0.80 in the derivation and validation datasets, respectively. The five variables were weighted proportionally to their adjusted odds ratios for an unsatisfactory prognosis (o/eLHR <25%, 1 point; liver herniation occupying more than one-third of the thoracic space, 1 point; thoracic stomach, 1 point; right-side CDH, 2 points; and severe malformations, 3 points). Unsatisfactory prognosis rates for the low- (0–2 points), intermediate- (3–5 points), and high-risk (6–8 points) groups were 17, 46, and 100%, respectively (p < 0.001), in the validation cohort. Conclusion Our simple risk score effectively predicted the prognosis of fetuses with CDH.


2000 ◽  
Vol 183 (5) ◽  
pp. 1067-1069 ◽  
Author(s):  
Danielle S. Walsh ◽  
Anne M. Hubbard ◽  
Oluyinka O. Olutoye ◽  
Lori J. Howell ◽  
Timothy M. Crombleholme ◽  
...  

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