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Disclosure

None

Lymphatic Anatomy and Flow Lung Lymphatic System

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

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Lymphatic Imaging

LAG

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

T2W MRI DCMRL

Lymphatic leaks

Lymph flow disorders

Conduction abnormalities

LymphedemaLymphatic overproduction

Traumatic Idiopathic Perfusion abnormalities

MesenteryLiver

AscitesPLE

LungParavertebral

ChyloptysisChylothorax

PBPulm edemaLung diseaseNC, CLFD

Kidney

Chylurea

Heart

Chylopericardium

Tissue

EdemaAnasarca

PLPS Pre and Post Natal Lung Lymphatic Dysfunction

♥ Lung function shortly after birth is dependent on:1) Lung surfactant ��lung compliance2) 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)

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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

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Congenital Lymphatic Flow Disorder (CLFD)

CLFD - External Lymphatic Drainage

Before After 24 hours

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

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CLFD – Hybrid Surgical LVA CLFD

CLFD - Noonans Cylothorax Post Stage II

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PLPS In Patients with SV

Glenn withchylothorax

Fontan withchylothorax

Fontan withPB

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

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Contrast Lymphangiogram and SLDE

(Dori et al. Pediatrics 2014)

♥ 6 yo HLHS s/p Fontan with severe PB

Pre and Post DCMRL

DCMRL Outcome

0

5

10

15

20

25

30

35

40

45

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

PIP

0

1

2

3

4

5

6

7

8

9

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

PEEP

ProcedureDate

ProcedureDate

0

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

0

20

40

60

80

100

120

7 / 31 / 1

4 8 / 2

/ 1 4

8 / 4/ 1 4

8 / 6

/ 1 4

8 / 8/ 1 4

8 / 1

0 / 14

8 / 12 / 1

4 8 / 1

4 / 14

8 / 16 / 1

4 8 / 1

8 / 14

8 / 20 / 1

4 8 / 2

2 / 14

8 / 24 / 1

4 8 / 2

6 / 14

8 / 28 / 1

4 8 / 3

0 / 14

FiO2

ProcedureDate

ProcedureDate

♥ Discharged home 2 weeks post procedure

♥ No complications

70

72

74

76

78

80

82

84

86

88

90

7 / 31 / 1

4 8 / 2

/ 1 4

8 / 4/ 1 4

8 / 6

/ 1 4

8 / 8/ 1 4

8 / 1

0 / 14

8 / 12 / 1

4 8 / 1

4 / 14

8 / 16 / 1

4 8 / 1

8 / 14

8 / 20 / 1

4 8 / 2

2 / 14

8 / 24 / 1

4 8 / 2

6 / 14

8 / 28 / 1

4 8 / 3

0 / 14

Sat

ProcedureDate

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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

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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)

www.lymphaticregistry.org Slide courtesy of Dr. Cameron Trenor

Chylothorax - GLA♥ 11 yo with GLA and chylothorax

Chylothorax - GLA

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Chylothorax - GLA Chylothorax - KLA

Lymphatic Embolization♥ 4 yo with KLA and persistent chylothorax

Lung Disease

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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