Congenital diaphragmatic hernia

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FETAL IMAGING

Congenital diaphragmatic hernia Amy R. Mehollin-Ray 1 Received: 14 January 2020 / Revised: 13 April 2020 / Accepted: 12 May 2020 / Published online: 19 November 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract 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. Keywords Congenital diaphragmatic hernia . Diaphragm . Fetus . Hernia . Lung volume . Magnetic resonance imaging . Ultrasound

Introduction

Imaging technique

Congenital diaphragmatic hernia (CDH) occurs in approximately 1 in every 4,000 live births. The true frequency is unknown because there are likely cases lost early in pregnancy that are not included [1]. Historically, survival in CDH has been very poor, but improvements in prenatal detection, antenatal care and neonatal medical and surgical management have led to improved survival in recent years [2]. The newest fetal therapy, fetal tracheal occlusion (FETO), has made a significant contribution to improved survival in the most severe cases [3]. The prenatal diagnostic imaging strategy includes the use of both US imaging and fetal MRI to identify specific anatomical features, such as hernia location and contents; to create volumetric measurements for risk stratification; and to recognize additional malformations or features suggestive of a genetic syndrome, for both prognosticating and determining eligibility for fetal and neonatal therapies. The fetal radiologist’s role is to provide these data accurately to inform the fetal care team and allow for the most informed parental counseling and management.

Fetal US imaging should be performed as a comprehensive scan to include biometric measurements, the fetal environment and detailed fetal anatomy. Specific to the diaphragmatic hernia, imaging in multiple orthogonal planes is helpful in determining the hernia location and contents. Utilization of multiple transducers, including linear and 3-D, as well as color and power Doppler techniques, allows for more detailed evaluation. Measurement of lung–head ratio should be standardized within each fetal center. Magnetic resonance imaging provides benefits in characterizing additional anomalies, such as those affecting the palate and brain, but its main advantage is in obtaining volumetric measurements of lung and liver.