Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings
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FETAL IMAGING
Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings Christian A. Barrera 1 & Teresa Victoria 1 & Fernando A. Escobar 2 & Ganesh Krishnamurthy 2 & Christopher L. Smith 3 & Julie S. Moldenhauer 4,5 & David M. Biko 1 Received: 13 December 2019 / Revised: 25 February 2020 / Accepted: 31 March 2020 / Published online: 19 November 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Lymphangiectasias are lymphatic malformations characterized by the abnormal dilation and morphology of the lymphatic channels. The classification and treatment of these disorders can be challenging given the limited amount of literature available in children. Various imaging modalities are used to confirm suspected diagnosis, plan the most appropriate treatment, and estimate a prognosis. Prenatal evaluation is performed using both prenatal US imaging and fetal MRI. These modalities are paramount for appropriate parental counseling and planning of perinatal care. During the neonatal period, chest US imaging is a useful modality to evaluate pulmonary lymphangiectasia because other modalities such as conventional radiography and CT display nonspecific findings. Finally, the recent breakthroughs in lymphatic imaging with MRI have allowed us to better classify lymphatic disorders. Dynamic contrast-enhanced lymphangiography, conventional lymphangiography and percutaneous lymphatic procedures offer static and dynamic evaluation of the central conducting lymphatics in children, with excellent spatial resolution and the possibility to provide treatment. The purpose of this review is to discuss the normal and abnormal development of the fetal lymphatic system and how to best depict it by imaging during the prenatal and postnatal life. Keywords Fetus . Lymphangiectasia . Lymphangiography . Lymphatic diseases . Lymphatic system . Magnetic resonance imaging . Ultrasonography
Introduction Lymphangiectasia denotes lymphatic malformations characterized by the abnormal dilation and morphology of the Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00247-020-04673-6) contains supplementary material, which is available to authorized users. * Christian A. Barrera [email protected] 1
Department of Radiology, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA 19104, USA
2
Department of Radiology, Section of Interventional Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
3
Division of Cardiology, Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
4
Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
5
Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
lymphatic channels [1]. The incidence of these anomalies is still unknown, but autopsy reports in the perinatal period have estimated that 0.5–1% of feto-neonatal deaths can be attr
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