Prenatal and Postnatal Evaluation of Lymphatic Disorders

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1 Prenatal and Postnatal Evaluation of Lymphatic Disorders David M Biko, MD Director, Section of Cardiovascular and Lymphatic Imaging Children s Hospital of Philadelphia Assistant Professor of Radiology University of Pennsylvania Perelman School of Medicine

2 Background Neonatal lymphatic flow disorders Group of rare disorders Presents as chylous pleural effusion, chylous ascites and/or soft tissue edema Confusing varying terminology in literature congenital pulmonary lymphangiectasia congenital chylothorax non-immune neonatal hydrops fetalis congenital lymphatic dysplasia primary lymphatic dysplasia

3 Lymphatic Flow 3 distinct systems which all communicate via the cisterna chyli 1) Soft tissue lymphatic system Collect residual interstitial fluid 2) Intestinal lymphatic system Absorption of dietary fat 3) Liver lymphatic system Deliver hepatic proteins to circulation Continue to thorax as the thoracic duct and into venous system at junction of left subclavian and IJ Illustration courtesy of Yoav Dori, MD, Phd and Max Itkin MD Joint HUP/CHOP Center for Lymphatic Imaging and Interventions

4 Neonatal Lymphatic Flow Disorders Pulmonary Lymphatic Perfusion Syndrome (PLPS) -> Neonatal Chylothorax Central Lymphatic Flow Disorder Congenital Chylous Ascites

5 Prenatal Lymphatic Imaging Effusions, ascites, hydrops Lung Described prenatally as lymphangiectasia Nutmeg lung on prenatal imaging T2 hyperintense branching structures from pulmonary hila to lung periphery Association with HLHS 1 Higher mortality of patients with HLHS and nutmeg lung Nutmeg 1 Saul D et al. Pediatric radiology 46.4 (2016):

6 Prenatal Lymphatic Imaging Images Courtesy of Teresa Victoria, MD, PhD

7 Neonatal Lymphatic Imaging Lung Surface Ultrasound Described by Lam et al. Supine imaging with linear transducer of 26 children with/without 2 o lymphangiectasia due to CHD Transverse intercostal approach Lam CZ., et al. Pediatric Radiology (2017):

8 Neonatal Lymphatic Imaging Lung Surface Ultrasound Findings in Lymphangiectasia Irregular lung surface (LR 6.8) Subpleural cystic appearing structures (LR 3.6) Combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9) 64% of pts with lymphangiectasia died Lam CZ., et al. Pediatric Radiology (2017):

9 Neonatal Lymphatic Flow Imaging - MRI Dynamic contrast enhanced lymphangiography (DCMRL) 0.1 mmol/kg contrast into groin nodes at 0.5 to 1 ml/min Time resolved MR angiography sequence 1 minute after injection (TWIST) Scan every seconds Scan time about 15 minutes Navigated 3D spoiled gradient echo 1.2 mm isotropic

10 Pulmonary Lymphatic Perfusion Syndrome (PLPS) Supply of the lungs via dilated lymphatic ducts from the TD Thoracic duct (TD) may be narrowed or occluded with tortuous proximal TD Neonates -> Neonatal Chylothorax Illustration courtesy of Yoav Dori, MD, Phd and Max Itkin MD Joint HUP/CHOP Center for Lymphatic Imaging and Interventions

11 Neonatal Chylothorax

12 Central Lymphatic Flow Disorder (CLFD) Also called Congenital Lymphatic Dysplasia Abnormal or reduced central lymphatic flow Effusions in more than 1 compartment Dermal backflow Usually only see in infants less than 1 year of age Poorer prognosis Illustration courtesy of Yoav Dori, MD, Phd and Max Itkin MD Joint HUP/CHOP Center for Lymphatic Imaging and Interventions

13 Central Lymphatic Flow Disorder (CLFD)

14 Trying to Tie it All Together Retrospective review over 11 year period of diagnosed with NLD All subjects with prenatal fetal MRI and US and postnatal dynamic contrast MR lymphangiography (DCMRL) within 6 months of birth 5 subjects met inclusion criteria HLHS in 1 patient Biko DM et al. Academic radiology (2018).

15 Trying to Tie it All Together- Imaging Prenatal Imaging Nutmeg lung present in all 5 subjects Pleural effusions (n=5) Body wall edema (n=1) Postnatal Imaging - DCMRL Perfusion to the lungs only (n=3) Absent in subject with HLHS Dermal backflow and perfusion to lungs (n=1) CLFD NC (PLPS) CLFD Biko DM et al. Academic radiology (2018).

16 Trying to Tie It All Together - Outcomes Neonatal Chylothorax (n=3) Effusions resolved with lymphatic intervention (n=2) or conservative therapy (n=1) Central Lymphatic Flow Disorder (n=1) Expired due to respiratory distress HLHS (Secondary Nutmeg Lung) Expired due to respiratory distress Biko DM et al. Academic radiology (2018).

17 Conclusion Prenatal imaging with nutmeg lung If Primary Lymphatic Disorder Body Wall Edema No Body Wall Edema?CLFD?Poor Prognosis?NC?Better Prognosis

18 Conclusion Postnatal imaging of nutmeg lung Consider lung surface ultrasound as screening tool Look for irregular lung surface and subpleural cystic change

19 Postnatal imaging of nutmeg lung Conclusion Consider evaluation with DCMRL if persistent symptoms of NLD Chylous effusion, ascites, body wall edema NC CLFD

20 Thank you! Questions?