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

3/10/2017 1 Disclosure None Lymphatic Anatomy and Flow Lung Lymphatic System

(Riquet M, Thorac Surg Clin 17 (2007) 619–638)

3/10/2017 2 Lymphatic Imaging

LAG

(Dori et al. AJR 2014, Radiology 2014, Pediatrics 2014)

T2W MRI DCMRL

Lymphatic leaks

Lymph flow disorders

Conduction abnormalities Lymphedema Lymphatic

• verproduction

Traumatic Idiopathic Perfusion abnormalities Mesentery Liver Ascites PLE Lung Paravertebral Chyloptysis Chylothorax PB Pulm edema Lung disease NC, CLFD Kidney Chylurea Heart Chylopericardium Tissue Edema Anasarca

PLPS Pre and Post Natal Lung Lymphatic Dysfunction

♥ 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)

3/10/2017 3 Prenatal – Nutmeg Lung

(Saul et al. Ped. Rad. 2016)

Post Natal DCMRL – Neonatal Chylothorax Neonatal Chylothorax Treatment with Lymphangiogram Congenital Lymphatic Flow Disorder (CLFD)

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

3/10/2017 4 Congenital Lymphatic Flow Disorder (CLFD) CLFD - External Lymphatic Drainage

Before After 24 hours

D-TGA s/p Switch with CLFD TD Outlet Occlusion

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

3/10/2017 6 PLPS In Patients with SV

Glenn with chylothorax Fontan with chylothorax Fontan with PB

PLPS Anatomic Categories

Type 1 Type 2 Type 3 Type 4 Type 5

(Dori et al. Circulation 2016)

Plastic Bronchitis (PB) Blue Dye Injection in PB

3/10/2017 7 Contrast Lymphangiogram and SLDE

(Dori et al. Pediatrics 2014)

♥ 6 yo HLHS s/p Fontan with severe PB

Pre and Post DCMRL DCMRL Outcome

PIP

PEEP

Procedure Date Procedure Date

5 10 15 20 25 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

20 40 60 80 100 120 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

FiO2

Procedure Date Procedure Date

♥ Discharged home 2 weeks post procedure ♥ No complications

Sat

Procedure Date

3/10/2017 8 Adults with Idiopathic PB

(Itkin et al. Ann. ATS 2016)

♥ 76 yo with severe idiopathic PB

Idiopathic PB Versus Neonatal Chylothorax Chyloptysis

♥ 57 yo with chyloptysis

Chyloptysis

3/10/2017 9 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

♥ 11 yo with GLA and chylothorax

Chylothorax - GLA

3/10/2017 10 Chylothorax - GLA Chylothorax - KLA Lymphatic Embolization

♥ 4 yo with KLA and persistent chylothorax

Lung Disease

3/10/2017 11 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