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Anesthesia and Analgesia Sep 2020Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by herniation of abdominal contents into the chest that results in... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by herniation of abdominal contents into the chest that results in varying degrees of pulmonary hypoplasia and pulmonary hypertension (PH). Significant advances in the prenatal diagnosis and identification of prognostic factors have resulted in the continued refinement of the approach to fetal therapies for CDH. Postnatally, protocolized approaches to lung-protective ventilation, nutrition, prevention of infection, and early aggressive management of PH have led to improved outcomes in infants with CDH. Advances in our understanding of the associated left ventricular (LV) hypoplasia and myocardial dysfunction in infants with severe CDH have allowed for the optimization of hemodynamics and management of PH. This article provides a comprehensive review of CDH for the anesthesiologist, focusing on the complex pathophysiology, advances in prenatal diagnosis, fetal interventions, and optimal postnatal management of CDH.
Topics: Hemodynamics; Hernias, Diaphragmatic, Congenital; Humans; Infant; Infant, Newborn; Predictive Value of Tests; Prenatal Diagnosis; Risk Factors; Treatment Outcome
PubMed: 31335403
DOI: 10.1213/ANE.0000000000004324 -
Seminars in Perinatology Feb 2020Antenatal ultrasound screening identifies more than 60% of Congenital Diaphragmatic Hernia (CDH) cases and provides the opportunity for in utero referral to a tertiary... (Review)
Review
Antenatal ultrasound screening identifies more than 60% of Congenital Diaphragmatic Hernia (CDH) cases and provides the opportunity for in utero referral to a tertiary care center for expert assessment and perinatal management. Prenatal assessment of fetuses with CDH has tremendously improved over the past ten years. The outcome may be predicted prenatally by medical imaging and advanced genetic testing. The combination of lung size and liver position determination by ultrasound measurements and MRI are widely accepted methods to stratify fetuses into groups that correlate not only with neonatal mortality but also with morbidity. Notwithstanding this, prediction of persistent pulmonary hypertension of the newborn still needs to be improved.
Topics: Abortion, Induced; Amniocentesis; Comparative Genomic Hybridization; Female; Fetal Therapies; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Imaging, Three-Dimensional; Liver; Lung; Magnetic Resonance Imaging; Organ Size; Pregnancy; Prenatal Diagnosis; Prognosis; Severity of Illness Index; Ultrasonography, Prenatal
PubMed: 31439324
DOI: 10.1053/j.semperi.2019.07.002 -
Pediatric Surgery International Apr 2020Congenital diaphragmatic hernia (CDH) is a developmental birth defect consisting of a diaphragmatic defect and abnormal lung development. CDH complicates 2.3-2.8 per... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a developmental birth defect consisting of a diaphragmatic defect and abnormal lung development. CDH complicates 2.3-2.8 per 10,000 live births. Despite efforts to standardize clinical practice, management of CDH remains challenging. Frequent re-evaluation of clinical practices in CDH reveals that management of CDH is evolving from one of postnatal stabilization to prenatal optimization. Translational research reveals promising avenues for in utero therapeutic intervention, including fetoscopic endoluminal tracheal occlusion. These remain highly experimental and demand improved antenatal diagnostics. Timely diagnosis of CDH and identification of severely affected fetuses allow time for delivery planning or in utero therapeutics. Optimal perinatal care and surgical treatment strategies are highly debated. Improved CDH mortality rates have placed increased emphasis on identifying and monitoring the long-term sequelae of disease throughout childhood and into adulthood. We review the current management strategies for CDH, highlighting where progress has been made, and where future developments have the potential to revolutionize care in this vulnerable patient population.
Topics: Disease Management; Female; Fetoscopy; Fetus; Follow-Up Studies; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Practice Guidelines as Topic; Pregnancy; Prenatal Diagnosis; Time Factors
PubMed: 32072236
DOI: 10.1007/s00383-020-04625-z -
Archives of Disease in Childhood. Fetal... Apr 2024The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a 'living document'.
OBJECTIVE
The Canadian Congenital Diaphragmatic Hernia (CDH) Collaborative sought to make its existing clinical practice guideline, published in 2018, into a 'living document'.
DESIGN AND MAIN OUTCOME MEASURES
Critical appraisal of CDH literature adhering to Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. Evidence accumulated between 1 January 2017 and 30 August 2022 was analysed to inform changes to existing or the development of new CDH care recommendations. Strength of consensus was also determined using a modified Delphi process among national experts in the field.
RESULTS
Of the 3868 articles retrieved in our search that covered the 15 areas of CDH care, 459 underwent full-text review. Ultimately, 103 articles were used to inform 20 changes to existing recommendations, which included aspects related to prenatal diagnosis, echocardiographic evaluation, pulmonary hypertension management, surgical readiness criteria, the type of surgical repair and long-term health surveillance. Fifteen new CDH care recommendations were also created using this evidence, with most related to the management of pain and the provision of analgesia and neuromuscular blockade for patients with CDH.
CONCLUSIONS
The 2023 Canadian CDH Collaborative's clinical practice guideline update provides a management framework for infants and children with CDH based on the best available evidence and expert consensus.
Topics: Infant; Child; Pregnancy; Female; Humans; Hernias, Diaphragmatic, Congenital; Canada; Prenatal Diagnosis; Hypertension, Pulmonary; Hypertension
PubMed: 37879884
DOI: 10.1136/archdischild-2023-325865 -
Pediatric Pulmonology Sep 2023A fetus was found to have a right diaphragmatic hernia during a prenatal ultrasonography examination late in the second trimester. A "green channel" with multi...
A fetus was found to have a right diaphragmatic hernia during a prenatal ultrasonography examination late in the second trimester. A "green channel" with multi department dynamic monitoring was instituted, at 40 + 4 weeks, with the infant under general anesthesia, hernia repair was later successfully performed. After the operation, the infant's vital signs were stable and their condition remained good during follow-up.
Topics: Pregnancy; Infant; Female; Humans; Hernias, Diaphragmatic, Congenital; Ultrasonography, Prenatal; Ultrasonography; Pregnancy Trimester, Second; Fetus
PubMed: 37314159
DOI: 10.1002/ppul.26542 -
The Annals of Thoracic Surgery Sep 2019Concurrence of a congenital Morgagni hernia and paraesophageal hernia is rare and can occasionally present as a medical emergency. Here, we report a unique case of a...
Concurrence of a congenital Morgagni hernia and paraesophageal hernia is rare and can occasionally present as a medical emergency. Here, we report a unique case of a patient with paroxysms of cough-induced syncope secondary to cardiac compression by a simultaneously occurring anterior Morgagni hernia and posterior paraesophageal hernia.
Topics: Aged; Cardiac Tamponade; Cough; Endoscopy, Digestive System; Follow-Up Studies; Hernia, Hiatal; Hernias, Diaphragmatic, Congenital; Herniorrhaphy; Humans; Laparoscopy; Male; Recovery of Function; Risk Assessment; Syncope; Treatment Outcome
PubMed: 30831110
DOI: 10.1016/j.athoracsur.2019.01.048 -
Nature Reviews. Disease Primers Jun 2022
Topics: Hernias, Diaphragmatic, Congenital; Humans
PubMed: 35650278
DOI: 10.1038/s41572-022-00371-9 -
Cirugia Espanola Jun 2022
Topics: Hernia, Hiatal; Hernias, Diaphragmatic, Congenital; Humans
PubMed: 35483585
DOI: 10.1016/j.cireng.2022.04.013 -
Pediatric Radiology Dec 2020Congenital diaphragmatic hernia (CDH) is a potentially severe anomaly that should be referred to a fetal care center with expertise in multidisciplinary evaluation and... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a potentially severe anomaly that should be referred to a fetal care center with expertise in multidisciplinary evaluation and management. The pediatric radiologist plays an important role in the evaluation of CDH, both in terms of anatomical description of the anomaly and in providing detailed prognostic information for use in caring for the fetus and pregnant mother as well as planning for delivery and postnatal care. This article reviews the types of hernias, including distinguishing features and imaging clues. The most common methods of predicting severity are covered, and current fetal and postnatal therapies are explained. The author of this paper provides a handy reference for pediatric radiologists presented with a case of CDH as part of their daily practice.
Topics: Child; Female; Fetus; Hernia; Hernias, Diaphragmatic, Congenital; Humans; Lung; Magnetic Resonance Imaging; Pregnancy; Prognosis
PubMed: 33252754
DOI: 10.1007/s00247-020-04718-w -
European Journal of Human Genetics :... Dec 2021Congenital diaphragmatic hernia (CDH) is a life-threatening malformation characterised by failure of diaphragmatic development with lung hypoplasia and persistent... (Review)
Review
Congenital diaphragmatic hernia (CDH) is a life-threatening malformation characterised by failure of diaphragmatic development with lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). The incidence is 1:2000 corresponding to 8% of all major congenital malformations. Morbidity and mortality in affected newborns are very high and at present, there is no precise prenatal or early postnatal prognostication parameter to predict clinical outcome in CDH patients. Most cases occur sporadically, however, genetic causes have long been discussed to explain a proportion of cases. These range from aneuploidy to complex chromosomal aberrations and specific mutations often causing a complex phenotype exhibiting multiple malformations along with CDH. This review summarises the genetic variations which have been observed in syndromic and isolated cases of congenital diaphragmatic hernia.
Topics: Genetic Testing; Hernia, Diaphragmatic; Humans; Mutation; Vitamin A
PubMed: 34621023
DOI: 10.1038/s41431-021-00972-0