Malformations associated with congenital diaphragmatic hernia: Impact on survival
Katarina BojanićEna PritišanacTomislav LuetićJurica VukovićJuraj ŠprungToby N. WeingartenDarrell R. SchroederRuža Grizelj
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Pulmonary Hypoplasia
As one kind of severe congenital malformation, congenital diaphragmatic hernia (CDH) initiates a series of pathological changes including diaphragmatic defect, pulmonary hypoplasia and pulmonary arterial hypertension. Among these, pulmonary hypoplasia is the dominant factor impacting its prognosis. Currently, postnatal surgical repair of diaphragm, ventilation and medical treatment are major therapeutic options for CDH in China. With the conceptual advances in treatment and techniques, the survival rate continues rising. However, postnatal surgical repair cannot change the pathological basis of pulmonary hypoplasia. Severe CDH children are prone to persistent pulmonary hypertension and postnatal respiratory failure so that the mortality of severe CDH stays at a high level. Therefore prenatal interventions for improving pulmonary development and achieving a higher survival have come into focus these years. At present, fetal endoscopic tracheal occlusion (FETO) is the most widely recognized intervention during fetal period. It has been applied to clinical practice in European countries, United States and some other countries. In recent decades, clinical researches and animal model experiments suggested that FETO might enable an accumulation of bronchial fluid and force lung to expand so as to improve the development of lung and boost survival rate in severe CDH cases. On the contrary, FETO increased the risk of premature rupture of membrane (PROM) and premature delivery and caused a higher mortality. Thus whether or not FETO is beneficial has remained controversial. Our research focused on research advances of FETO and evaluation of its prognosis in CDH cases.
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Fetoscopy; Congenital diaphragmatic hernia; Pulmonaryhypoplasia; Prognosis
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In previous papers, we described a new experimental model of congenital diaphragmatic hernia in rabbits, and we also reported noninvasive therapeutic strategies for prevention of the functional and structural immaturity of the lungs associated with this defect. In addition to lung hypoplasia, pulmonary hypertension, biochemical, and structural immaturity of the lungs, the hemodynamics of infants and animals with congenital diaphragmatic hernia are markedly altered. Hence, cardiac hypoplasia has been implicated as a possible cause of death in patients with congenital diaphragmatic hernia, and it is hypothesized to be a probable consequence of fetal mediastinal compression by the herniated viscera. Cardiac hypoplasia has also been reported in lamb and rat models of congenital diaphragmatic hernia. The purpose of the present experiment was to verify the occurrence of heart hypoplasia in our new model of surgically produced congenital diaphragmatic hernia in fetal rabbits.Twelve pregnant New Zealand rabbits underwent surgery on gestational day 24 or 25 (normal full gestational time - 31 to 32 days) to create left-sided diaphragmatic hernias in 1 or 2 fetuses per each doe. On gestational day 30, all does again underwent surgery, and the delivered fetuses were weighed and divided into 2 groups: control (non-surgically treated fetuses) (n = 12) and congenital diaphragmatic hernia (n = 9). The hearts were collected, weighed, and submitted for histologic and histomorphometric studies.During necropsy, it was noted that in all congenital diaphragmatic hernia fetuses, the left lobe of the liver herniated throughout the surgically created defect and occupied the left side of the thorax, with the deviation of the heart to the right side, compressing the left lung; consequently, this lung was smaller than the right one. The body weights of the animals were not altered by congenital diaphragmatic hernia, but heart weights were decreased in comparison to control fetuses. The histomorphometric analysis demonstrated that congenital diaphragmatic hernia promoted a significant decrease in the ventricular wall thickness and an increase in the interventricular septum thickness.Heart hypoplasia occurs in a rabbit experimental model of congenital diaphragmatic hernia. This model may be utilized for investigations in therapeutic strategies that aim towards the prevention or the treatment of heart hypoplasia caused by congenital diaphragmatic hernia.
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Pulmonary hypoplasia is one of the major risk factors for the high morbidity and mortality in fetuses with congenital diaphragmatic hernia (CDH). The lung-to-head ratio (LHR) was introduced in the last years to evaluate the lung size and estimate the risk for pulmonary hypoplasia. Aim of the study was to evaluate the LHR as a predictor for the neonatal outcome. We examined 28 fetuses with isolated CDH during pregnancy. Following biometric parameter were measured and compared with the outcome (survival or death) and different neonatal ventilation parameter (pO2, pCO2, FiO2, inspiratoric pressure, Oxygenation index, occurrence of pulmonary hypertension): anterior—posterior and transverse diameter of the contra lateral lung; LHR. The overall survival rate was 69%. 10 fetuses had a LHR below 1.0. 7 fetuses of them survived (70%) and 3 died (30%). 6 fetuses had a LHR above 1.4. Three of them died (50%) and 3 of them survived (50%). In 13 cases the diagnosis was made before 26 gw (survival rate 77%). 8 fetuses had a LHR below 1.0. Six of them survived (75%) and 2 died (25%). There was no fetus with a LHR above 1, 4 in this subgroup. Postnatal ventilation parameters shows typical changes depending on the severity of the pulmonary hypoplasia. But we could not found a significant correlation between the LHR (parameter for lung size) and the postnatal ventilation parameters or for the occurrence of pulmonary hypertension. The LHR is not a reliable biometric parameter to evaluate the risk for developing lethal pulmonary hypoplasia in fetuses with CDH. This must lead to the conclusion, that the fetal lung is a too complex organ as judge by only one diagnostic parameter.
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Predicting Neonatal Deaths and Pulmonary Hypoplasia in Isolated Congenital Diaphragmatic Hernia Using the Sonographic Fetal Lung Volume–Body Weight RatioRodrigo Ruano1 2, Marie-Cecile Aubry2, Yves Dumez2, Marcelo Zugaib1 and Alexandra Benachi2Audio Available | Share
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1) To define the best outcome of severe Congenital Diaphragmatic Hernia (CDH); 2) to critically evaluate deaths in order to identify possible criteria of exclusion from ECMO; and 3) to identify CDHs which could benefit from ECMO.
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Abstract Background : Congenital diaphragmatic hernia (CDH) is a simple diaphragmatic defect that is seen frequently in antenatal scans. Though the surgical repair is relatively easy in the neonate, the mortality is high due to pulmonary hypoplasia and pulmonary vascular changes. Materials and Methods : The goals of prenatal imaging are to establish the diagnosis. Assessment in a tertiary scanning center would identify prognostic factors which assist in counselling and planning antenatal management. However, the pulmonary hypoplasia associated with the CDH in moderate and severe cases makes the prognosis poor. Conclusions : Fetal intervention such as, fetal tracheal occlusion, is being trialled and may change the outcomes in the future.
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Background. Fetuses with congenital diaphragmatic hernia (CDH) are at risk of death from pulmonary hypoplasia at birth.
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