scholarly journals Clinical case of congenital diaphragmatic hernia

2021 ◽  
Vol 5 ◽  
pp. 56-60
Author(s):  
O.Ya. Slobodyanik ◽  
V.V. Bila ◽  
V.M. Tyshkevych ◽  
M.V. Protsyk ◽  
V.S. Shevchenko

Congenital diaphragmatic hernia is a rare congenital anomaly of diaphragmatic development with a frequency of approximately 1 in 2,500 live births. The structural defect is accompanied by pathophysiological disorders, in particular, pulmonary hypertension, as well as pulmonary hypoplasia of various degrees, which actually determine the severity of the defect. In 84% of cases, the diaphragmatic hernia is located on the left dome of the diaphragm, possible right-sided and left-sided localization of the malformation, which is 14 and 2%, respectively.The defect can be either isolated or combined with other congenital anomalies, such as congenital heart disease or chromosomal abnormalities. Violation of the closure of the diaphragm during the 4-10th week of fetal development leads to the formation of the diaphragmatic grid. During the same period (5th week of gestation) is the formation of lungs and bronchs. Three-dimensional ultrasound diagnosis allows to detect prenatal defect in almost 60% of fetuses. Fetal MRI is a method of choosing the anatomical assessment of the lungs, determining their volume allows you to more accurately predict the development of complications and the required amount of care after birth. The article is devoted to the description of clinical observation of ENT with a description of the features of perinatal diagnosis and organization of postnatal care. The defect was diagnosed at 31 weeks, vaginal delivery occurred during full-term pregnancy. The complex of resuscitation measures, preoperative preparation, the course of the postoperative period is described. Timely prenatal diagnosis of EDC and its anatomical variant makes it possible to correctly develop the patient’s route, starting with the tactics of pregnancy, method of delivery, as well as to predict the algorithm of staff actions at birth and subsequent treatment of the newborn.

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.


2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Parkash Mandhan ◽  
Amer Al Saied ◽  
Mansour J. Ali

Congenital diaphragmatic hernia is a common developmental anomaly encountered by paediatric surgeons. It is known to be associated with extradiaphragmatic malformations, which include cardiac, renal, genital, and chromosomal abnormalities. Herein, we report a newborn born with concurrent congenital diaphragmatic hernia, Meckel’s diverticulum, and heterotopic pancreatic tissue. This is the first case report of such a triad with description of possible mechanisms of the development.


1970 ◽  
Vol 7 (1) ◽  
pp. 28-30 ◽  
Author(s):  
SE Khan ◽  
AKMZ Siddiq ◽  
M Nessa

Introduction: Congenital diaphragmatic hernia (CDH) is one of the major surgical causes of respiratory distress in neonates. Reported survival averages 60% but may be significantly lower. Pulmonary hypertension and pulmonary hypoplasia are recognised as two corner stones of the pathophysiology of CDH. Objective: Objective of the study was to evaluate the outcome of this birth defect in Bangladesh situation. Method: This retrospective study was carried out at the Department of Paediatric Surgery of Combined Military Hospital, Dhaka over a period of five years. During this period a total of 8 neonates of CDH were admitted in this hospital. All the data were collected from record sheet and were compiled. Result: Age of patients ranged from 1 day to 20 days. Out of 8 neonates 5 (62.50%) were male and 3 (37.50%) were female. All patients were diagnosed postnatally. All the 8 neonates had Bochdalek type of CDH. Seven patients (87.50%) had left sided hernia. Two patients (25%) died before operation in the stabilization phase while on ventilator and 6 (75%) were operated. Out of these 6 patients, 5 (left sided) were operated through abdominal route and 1 (right sided) was approached through thorax. Overall outcome was satisfactory in 5 neonates and one died. Conclusion: Early intervention can result good prognosis in CDH. Key words: Congenital diaphragmatic hernia; neonate; Bochdalek type DOI: http://dx.doi.org/10.3329/jafmc.v7i1.8623 JAFMC Bangladesh. Vol 7, No 1 (June) 2011; 28-30  


2022 ◽  
Author(s):  
Kasra Khalaj ◽  
Rebeca Lopes Figueira ◽  
Lina Antounians ◽  
Sree Gandhi ◽  
Matthew Wales ◽  
...  

Pulmonary hypoplasia secondary to congenital diaphragmatic hernia (CDH) is characterized by impaired branching morphogenesis and differentiation. We have previously demonstrated that administration of extracellular vesicles derived from rat amniotic fluid stem cells (AFSC-EVs) rescues development of hypoplastic lungs at the pseudoglandular and alveolar stages in rodent models of CDH. Herein, we tested whether AFSC-EVs exert their regenerative effects at the canalicular and saccular stages, as these are translationally relevant for clinical intervention. To induce fetal pulmonary hypoplasia, we gavaged rat dams with nitrofen at embryonic day 9.5 and demonstrated that nitrofen-exposed lungs had impaired branching morphogenesis, dysregulated signaling pathways relevant to lung development (FGF10/FGFR2, ROBO/SLIT, Ephrin, Neuropilin 1, beta-catenin) and impaired epithelial and mesenchymal cell marker expression at both stages. AFSC-EVs administered to nitrofen-exposed lung explants rescued airspace density and increased the expression levels of key factors responsible for branching morphogenesis. Moreover, AFSC-EVs rescued the expression of alveolar type 1 and 2 cell markers at both canalicular and saccular stages, and restored markers of club, ciliated epithelial, and pulmonary neuroendocrine cells at the saccular stage. AFSC-EV treated lungs also had restored markers of lipofibroblasts and PDGFRA+ cells to control levels at both stages. EV tracking showed uptake of AFSC-EV RNA cargo throughout the fetal lung and an mRNA-miRNA network analysis identified that several miRNAs responsible for regulating lung development processes were contained in the AFSC-EV cargo. These findings suggest that AFSC-EV based therapies hold potential for restoring fetal lung growth and maturation in babies with pulmonary hypoplasia secondary to CDH.


2019 ◽  
Author(s):  
Karen M. Davidson

The normal and abnormal development of the organs lying within the fetal thorax is discussed.  The abnormalities reviewed include more common findings of pulmonary hypoplasia, congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, as well as the rarer conditions of congenital fetal hydrothorax, congenital high airway obstruction syndrome, bronchogenic cysts, neurenteric cysts, and lung agenesis.  With each abnormality, the clinical implications for the fetus, best methods for prenatal diagnosis, as well as possible additional anomalies, syndromes, and aneuploidies are described.  In utero and postnatal treatments are also reviewed.   This review contains 10 figures, and 37 references. Key Words: Pulmonary hypoplasia, lung-head ratio, congenital diaphragmatic hernia, congenital pulmonary airway malformation, congenital fetal hydrothorax, bronchopulmonary sequestration, congenital high airway obstruction syndrome, bronchogenic cyst, neurenteric cyst, lung agenesis


2019 ◽  
Author(s):  
Karen M. Davidson

The normal and abnormal development of the organs lying within the fetal thorax is discussed.  The abnormalities reviewed include more common findings of pulmonary hypoplasia, congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, as well as the rarer conditions of congenital fetal hydrothorax, congenital high airway obstruction syndrome, bronchogenic cysts, neurenteric cysts, and lung agenesis.  With each abnormality, the clinical implications for the fetus, best methods for prenatal diagnosis, as well as possible additional anomalies, syndromes, and aneuploidies are described.  In utero and postnatal treatments are also reviewed.   This review contains 10 figures, and 37 references. Key Words: Pulmonary hypoplasia, lung-head ratio, congenital diaphragmatic hernia, congenital pulmonary airway malformation, congenital fetal hydrothorax, bronchopulmonary sequestration, congenital high airway obstruction syndrome, bronchogenic cyst, neurenteric cyst, lung agenesis


2008 ◽  
Vol 36 (5) ◽  
pp. 273-278 ◽  
Author(s):  
Rodrigo Ruano ◽  
Marie-Cécile Aubry ◽  
Bruno Barthe ◽  
Yves Dumez ◽  
Marcelo Zugaib ◽  
...  

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