Managing respiratory complications in infants and newborns with congenital diaphragmatic hernia

ABSTRACT

Introduction: Congenital diaphragmatic hernia (CDH) is one of the most challenging neonatal surgical conditions due to its high mortality and morbidity. A multidisciplinary approach is required regarding the management of affected infants, before birth, during their initial hospitalization and in their long-term follow-up.

Areas covered: This review discusses the monitoring and management strategies for infants with CDH including during the prenatal and postnatal periods. It emphasizes the long-term sequelae these patients can suffer throughout childhood and adulthood. This article highlights the progress that has been made and where future developments have the potential to improve the outcome of this vulnerable patient population. A literature search was performed using Google Scholar, Science Direct and PubMed.

Expert opinion: Advances in antenatal and neonatal care have improved the early survival of infants with CDH, but many infants are surviving with long-term morbidity. An increasing number of CDH survivors are now reaching adolescence and adulthood. New challenges arise regarding the prevention and management of their long-term problems.

KEYWORDS:

• Congenital diaphragmatic hernia
• respiratory complications
• pulmonary hypertension
• gastro-esophageal reflux
• long-term outcomes

Article highlights

• The severity of CDH can be estimated prenatally using observed-to-expected lung–head ratios (by ultrasound) and total fetal lung volumes (by magnetic resonance imaging), as well as the fetal liver position.

• Infants with CDH require intensive cardiopulmonary support after birth, including immediate endotracheal intubation and ‘gentle ventilation,’ as well as judicious use of fluid boluses and inotropic support.

• All infants should undergo echocardiography in the first 48 hours after birth to assess if there are cardiac abnormalities and the degree of pulmonary hypertension, which may require the use of pulmonary vasodilators and other medical adjuncts or, in severe cases, extracorporeal life support, if available.

• Surgical repair of the diaphragmatic defect should usually be delayed until physiologic stability has been achieved, but a failure to perform surgery within the first two weeks after birth should prompt a team discussion with the family about possible outcomes.

• Infants with CDH should undergo long-term, multidisciplinary surveillance.

This box summarizes key points contained in the article.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was supported by the NIHR Biomedical Research Centre based at Guy’s and St Thomas’ NHS Foundation Trusts and King’s College London.