Experience of balloon tracheal occlusion in secondary pulmonary hypoplasia in fetuses with congenital diaphragmatic hernia

2018 ◽  
Vol 18 (3) ◽  
pp. 52 ◽  
Author(s):  
M. V. Pavlichenko ◽  
N. V. Kosovtsova ◽  
T. V. Markova
2003 ◽  
Vol 6 (6) ◽  
pp. 536-546 ◽  
Author(s):  
Amy E. Heerema ◽  
Joseph T. Rabban ◽  
Roman M. Sydorak ◽  
Micheal R. Harrison ◽  
Kirk D. Jones

Fetal intervention for congenital diaphragmatic hernia was developed to lessen the high morbidity and mortality of pulmonary hypoplasia. Lung pathology and morphometry in patients treated with fetal intervention have not been described. We report clinical and autopsy findings, as well as basic lung morphometry in 16 cases of congenital diaphragmatic hernia with fetal intervention (12 cases tracheal occlusion; 4 cases hernia repair), and 19 cases of congenital diaphragmatic hernia without fetal intervention. All patients who underwent fetal intervention were born premature. Lung enlargement with increased lung-to-body weight ratio was observed with fetal tracheal occlusion, accompanied by lower than normal radial alveolar counts and increased alveolar size. Patients treated with tracheal occlusion also had early alveolar development (at 29.8, 30.6, and 30.9 wk postconceptual age) as well as mucous fluid pooling in airways and alveoli. All cases showed severe alveolar septal widening, more extensive in patients without fetal intervention. When grouped by postconceptual age, no statistically significant difference was found between patients with and without fetal intervention with respect to lung-to-body weight ratio, radial alveolar count, mean alveolar length, and relative arteriolar media thickness. Lung enlargement has been observed with fetal tracheal occlusion sonographically; our studies suggest that this is due in part to emphysema and mucous fluid pooling. The lung remains abnormal with low radial alveolar counts and increased alveolar size. Tracheal occlusion did not prevent development of lung pathology associated with pulmonary hypoplasia.


2016 ◽  
Vol 264 (6) ◽  
pp. 929-933 ◽  
Author(s):  
Jamila Al-Maary ◽  
Mary P. Eastwood ◽  
Francesca Maria Russo ◽  
Jan A. Deprest ◽  
Richard Keijzer

2017 ◽  
Vol 37 (10) ◽  
pp. 1046-1049 ◽  
Author(s):  
Viola Seravalli ◽  
Eric B. Jelin ◽  
Jena L. Miller ◽  
Aylin Tekes ◽  
Luca Vricella ◽  
...  

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.


Author(s):  
Felipe Fromm ◽  
Katharina Wenke ◽  
Thomas Krebs ◽  
Michael Boettcher ◽  
Georg Eschenburg ◽  
...  

Background Severe congenital diaphragmatic hernia (CDH) is prenatally managed by fetoscopic tracheal occlusion (FETO) to improve lung growth and maturation. As FETO is not able to reduce the pressure onto the developing lungs originating from the intestine growing into the thoracic cavity, fetal abdominal decompression may alleviate this pressure effect by directing the growing intestine into the amniotic cavity away from the lungs. Therefore, aim of this study was to establish an animal model for fetoscopic abdominal decompression in fetal sheep with CDH. Methods CDH was created surgically on day 75 of 145 day gestation in eight fetuses. 2-3 weeks later, an opening was created in the fetal abdomen by fetoscopic surgery. The fetuses were retrieved by cesarean section at the end of pregnancy and evaluated. Results Five fetuses with CDH were treated with fetoscopic abdominal decompression. Three fetuses with CDH were taken as controls. One fetus was lost after creation of the CDH and two other after creation of the abdominal defect. Preliminary sterological results showed that the septal thickness of the experimental group was smaller than in the CDH group. Conclusion This study demonstrates the general feasibility of fetoscopic abdominal decompression for diaphragmatic hernia in our new animal model. Although not statistically significant, the lungs of treated fetuses were larger and heavier than those of untreated controls. Our findings support the hypothesis of palliative fetal surgery for severe CDH compared to tracheal occlusion. More controlled animal trials are needed.


Sign in / Sign up

Export Citation Format

Share Document