Disclosure None Lymphatic Anatomy and Flow Lung Lymphatic System - PowerPoint PPT Presentation

3/10/2017 Disclosure None Lymphatic Anatomy and Flow Lung Lymphatic System (Riquet M, Thorac Surg Clin 17 (2007) 619–638) 1

3/10/2017 Lymphatic Imaging Lymph flow disorders LAG T2W MRI DCMRL Lymphatic Lymphatic Conduction Lymphedema leaks overproduction abnormalities Traumatic Idiopathic Perfusion abnormalities Heart Mesentery Lung Kidney Tissue Liver Paravertebral Chylopericardium Ascites Chyloptysis Chylurea Edema PLE Chylothorax Anasarca PB Pulm edema Lung disease NC, CLFD (Dori et al. AJR 2014, Radiology 2014, Pediatrics 2014) Pre and Post Natal Lung Lymphatic Dysfunction PLPS ♥ Lung function shortly after birth is dependent on: 1) Lung surfactant � � lung compliance 2) Lymphatic function to adsorb fluid � Surfactant deficiency � RDS � Lymph dysfunction � lung edema � � lung compliance � RDS ♥ CCBE1 = lymphangiogenic factor ♥ CCBE1 -/- mice lack a lymphatic system but have otherwise normal lung anatomy ♥ Prenatal: � Normal CV system � Tissue edema ♥ Post natal: � Death from RDS � Unable to inflate the lungs (Jakus et al. JEM 2014) 2

3/10/2017 Prenatal – Nutmeg Lung Post Natal DCMRL – Neonatal Chylothorax (Saul et al. Ped. Rad. 2016) Congenital Lymphatic Flow Disorder (CLFD) Neonatal Chylothorax Treatment with Lymphangiogram Patterns of central lymphatic flow in CLFD: ♥ Abnormal conduction �� PLPS ♥ TD outlet obstruction �� PLPS, � Dermal flow ♥ Functional TD outlet obstruction �� PLPS, � Dermal flow ♥ Secondary obstruction �� PLPS, � Dermal flow 3

3/10/2017 CLFD - External Lymphatic Drainage Congenital Lymphatic Flow Disorder (CLFD) Before After 24 hours TD Outlet Occlusion D-TGA s/p Switch with CLFD 4

3/10/2017 CLFD CLFD – Hybrid Surgical LVA CLFD - Noonans Cylothorax Post Stage II 5

3/10/2017 PLPS Anatomic Categories PLPS In Patients with SV Glenn with Fontan with Fontan with Type 1 Type 2 Type 3 chylothorax chylothorax PB Type 4 Type 5 (Dori et al. Circulation 2016) Plastic Bronchitis (PB) Blue Dye Injection in PB 6

3/10/2017 Pre and Post DCMRL Contrast Lymphangiogram and SLDE ♥ 6 yo HLHS s/p Fontan with severe PB (Dori et al. Pediatrics 2014) DCMRL Outcome PIP PEEP Sat 45 9 90 88 40 8 86 35 7 84 30 6 82 25 5 80 78 20 4 76 15 3 Procedure Procedure Procedure 74 10 2 Date Date 72 Date 5 1 70 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 4 / 1 / 1 / 1 / 1 / 1 / 1 / 1 / 1 / 1 / 1 1 / 1 / / 1 / 1 / 1 / 1 0 0 1 2 4 6 8 0 2 4 6 8 0 2 4 6 8 0 3 8 / 8 / 8 / / 8 1 1 1 1 1 2 2 2 2 2 3 7 / 8 / 8 / 8 / 8 / 8 / 8 / 8 / 8 / 8 / 8 / 8 / 7/31/14 8/1/14 8/2/14 8/3/14 8/4/14 8/5/14 8/6/14 8/7/14 8/8/14 8/9/14 7/31/14 8/1/14 8/2/14 8/3/14 8/4/14 8/5/14 8/6/14 8/7/14 8/8/14 8/9/14 PS FiO2 25 120 100 20 80 15 60 10 40 Procedure 20 Procedure 5 Date Date 0 7/31/14 8/2/14 8/4/14 8/6/14 8/8/14 8/10/14 8/12/14 8/14/14 8/16/14 8/18/14 8/20/14 8/22/14 8/24/14 8/26/14 8/28/14 8/30/14 0 7/31/14 8/1/14 8/2/14 8/3/14 8/4/14 8/5/14 8/6/14 8/7/14 8/8/14 8/9/14 ♥ Discharged home 2 weeks post procedure ♥ No complications 7

3/10/2017 Idiopathic PB Versus Neonatal Adults with Idiopathic PB Chylothorax ♥ 76 yo with severe idiopathic PB (Itkin et al. Ann. ATS 2016) Chyloptysis Chyloptysis ♥ 57 yo with chyloptysis 8

3/10/2017 Chyloptysis Overall Survival in Gorham Stout and GLA n 5 year overall survival ± SE (%) GSD patients complicated by effusion 20 94 ± 6 GLA patients complicated by effusion 77 84 ± 5 GSD/GLA patients without effusion 55 100 (N/A) ww.lymphaticregistry.org Slide courtesy of Dr. Cameron Trenor Chylothorax - GLA Chylothorax - GLA ♥ 11 yo with GLA and chylothorax 9

3/10/2017 Chylothorax - GLA Chylothorax - KLA Lung Disease Lymphatic Embolization ♥ 4 yo with KLA and persistent chylothorax 10

3/10/2017 Summary ♥ Lymphatic flow disorders are serious, sometimes life threatening diseases ♥ PLPS can lead to abnormal lung mechanics and disease ♥ Imaging is the key first step in diagnosis and to guide therapy ♥ New lymphatic treatments are now available 11