Congenital Diaphragmatic Hernia: Core Review and Novel Updates

2021 ◽  
Vol 40 (5) ◽  
pp. 305-312
Author(s):  
Susannah Reed-McCullough ◽  
Amy J. Jnah
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Pulmonary Hypoplasia ◽  
Surgical Repair ◽  
Disease Process ◽  
Standard Of Care ◽  
Developmental Defect ◽  
Thoracic Cavity ◽  
Care Providers ◽  
Repair Techniques

Congenital diaphragmatic hernia (CDH) is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the thoracic cavity. Pulmonary hypoplasia and pulmonary hypertension are consequences of this disease process. The incidence is approximately 2.4–4.1/10,000 births, and survival rate is estimated at 70–90 percent. To avoid potentially devastating delays in care, it is crucial that neonatal nurses and care providers in both tertiary and nontertiary care centers be familiar with the pathogenesis of CDH and the standard of care for initial stabilization of the neonate. Novel fetal and postnatal surgical repair techniques are also described here.

Care of the fetus/newborn with congenital diaphragmatic hernia

1994 ◽  
Vol 6 (2) ◽  
pp. 81-94 ◽  
Author(s):  
Duncan T Wilcox ◽  
Hratch L Karamanoukian ◽  
Philip L Glick
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Lung Development ◽  
Pulmonary Hypoplasia ◽  
Thoracic Cavity ◽  
Live Births ◽  
Surfactant System ◽  
Vascular Branching

Congenital diaphragmatic hernia (CDH) is a simple anatomical defect. The reported incidence is between 1:2000 and 1:5000 live births, with the majority affecting the left side. A defect in the diaphragm allows abdominal viscera to herniate into and stay within the thoracic cavity during crucial stages of lung development. This results in pulmonary hypoplasia, a reduction in pulmonary vascular branching and an alteration in the surfactant system.

Anesthesia for Congenital Diaphragmatic Hernia Repair in Infants

2021 ◽  
pp. 221-228
Author(s):  
Chirag Shah ◽  
Anna Clebone ◽  
Caitlin Aveyard
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Abnormal Development ◽  
Pulmonary Vasculature ◽  
Lung Hypoplasia ◽  
Developmental Defect ◽  
Thoracic Cavity ◽  
Bochdalek Hernia ◽  
Congenital Condition ◽  
Diaphragmatic Hernia Repair

A congenital diaphragmatic hernia is a congenital condition in which a developmental defect in the diaphragm allows abdominal contents to migrate into the thoracic cavity. It occurs in about 1 in 2,000 newborns. This leads to lung underdevelopment and lung hypoplasia in utero. A defect in the diaphragm is usually on the left side, most commonly in the posterolateral region, also known as a Bochdalek hernia. A defect in the anterior region is known as a Morgagni hernia. Lung hypoplasia and the abnormal development of pulmonary vasculature resulting in pulmonary hypertension contribute to the morbidity and mortality associated with a congenital diaphragmatic hernia.

Diaphragmatic Hernia

1983 ◽  
Vol 4 (8) ◽  
pp. 244-266
Keyword(s):  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Diaphragmatic Hernia ◽  
Neonatal Intensive Care ◽  
Pulmonary Hypoplasia ◽  
Thoracic Cavity ◽  
Hemodynamic Changes ◽  
Life Threatening ◽  
Pulmonary Arterial ◽  
Immediate Surgery

In spite of the availability of almost immediate surgery and neonatal intensive care, congenital diaphragmatic hernia is a life-threatening anomaly in the newborn. It is the result of early embryologic malformation or failure of fusion of the components of the diaphragm allowing for the displacement of the abdominal contents into the thoracic cavity. There is consequent compression of the lung which may result in pulmonary hypoplasia or compression of the cardiovascular structures resulting in deleterious hemodynamic changes. Hypoxia and acidosis result in the presentation of respiratory distress and cyanosis. This is frequently associated with pulmonary arterial hypertension with right to left shunting through fetal circuits.

CONGENITAL DIAPHRAGMATIC HERNIA: SURVIVAL TREATED WITH VERY DELAYED SURGERY, SPONTANEOUS RESPIRATION, AND NO CHEST TUBE

PEDIATRICS ◽  
1996 ◽  
Vol 97 (6) ◽  
pp. 810-810
Keyword(s):  
Extracorporeal Membrane Oxygenation ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Retrospective Review ◽  
Chest Tube ◽  
Surgical Repair ◽  
Spontaneous Respiration ◽  
Respiratory Care ◽  
Delayed Surgery ◽  
Membrane Oxygenation

This report suggests that stabilization of the intrauterine to extrauterine transitional circulation, combined with a respiratory care strategy that avoids pulmonary overdistension, takes advantage of inherent biological cardiorespiratory mechanics, and very delayed surgery for congenital diaphragmatic hernia results in improved survival and decreases the need for extracorporeal membrane oxygenation (ECMO). This retrospective review of a 10-year experience in which the respiratory care strategy, ECMO availability, and technique of surgical repair remained essentially contant describes the evolution of this method of management of congenital diaphragmatic hernia.

scholarly journals Intestinal Perforation during the Stabilization Period in a Preterm Infant with Congenital Diaphragmatic Hernia

2017 ◽  
Vol 05 (01) ◽  
pp. e51-e54
Author(s):  
Zbyněk Straňák ◽  
Karel Pýcha ◽  
Simona Feyereislova ◽  
Jaroslav Feyereisl ◽  
Michal Rygl
Keyword(s):  
Birth Weight ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Intestinal Perforation ◽  
Surgical Repair ◽  
Very Low Birth Weight ◽  
Surgical Intervention ◽  
Multiple Organ ◽  
Stabilization Period ◽  
Early Surgical Intervention

Background Delayed surgery after stabilization of infants with congenital diaphragmatic hernia (CDH) is an accepted strategy. However, the evidence favoring delayed versus immediate surgical repair is limited. We present an extremely rare case of a very low-birth-weight infant with prenatally diagnosed left-sided CDH and unexpected transmural bowel perforations developing within the postnatal stabilization period. Case Report A neonate born at 31st week of gestation with a birth weight of 1,470 g with antenatally diagnosed left-sided CDH presented with bowel dilation leading to transmural bowel perforations on the 2nd day of life. Meconium pleuroperitonitis resulted in severe systemic inflammatory response syndrome, pulmonary hypertension, multiple organ failure, and death. Conclusion In neonates with CDH deteriorating under standard postnatal management, intestinal perforation, and early surgical intervention should be considered.

Effect of surgical repair on respiratory mechanics in congenital diaphragmatic hernia

1987 ◽  
Vol 111 (3) ◽  
pp. 432-438 ◽  
Author(s):  
Hirokazu Sakai ◽  
Masanori Tamura ◽  
Yuhei Hosokawa ◽  
A. Charles Bryan ◽  
Geoffrey A. Barker ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Respiratory Mechanics ◽  
Surgical Repair

scholarly journals Congenital Diaphragmatic Hernia in Neonate: Experience in a Teaching Hospital

1970 ◽  
Vol 7 (1) ◽  
pp. 28-30 ◽  
Author(s):  
SE Khan ◽  
AKMZ Siddiq ◽  
M Nessa
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Birth Defect ◽  
Pulmonary Hypoplasia ◽  
Good Prognosis ◽  
Military Hospital ◽  
Paediatric Surgery ◽  
Stabilization Phase ◽  
Abdominal Route

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  

scholarly journals Amniotic fluid stem cell extracellular vesicles promote fetal lung branching and cell differentiation in experimental congenital diaphragmatic hernia

2022 ◽  
Author(s):  
Kasra Khalaj ◽  
Rebeca Lopes Figueira ◽  
Lina Antounians ◽  
Sree Gandhi ◽  
Matthew Wales ◽  
...  
Keyword(s):  
Amniotic Fluid ◽  
Congenital Diaphragmatic Hernia ◽  
Extracellular Vesicles ◽  
Diaphragmatic Hernia ◽  
Lung Development ◽  
Pulmonary Hypoplasia ◽  
Fetal Lung ◽  
Branching Morphogenesis ◽  
Neuroendocrine Cells ◽  
Beta Catenin

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.

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