Anatomy & Physiology Chapter 12 The Lymphatic System and Body Defenses.
Lymphatic Anatomy and Flow Lung Lymphatic System Anatomy and Flow Lung Lymphatic System (Riquet M,...
Transcript of Lymphatic Anatomy and Flow Lung Lymphatic System Anatomy and Flow Lung Lymphatic System (Riquet M,...
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
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)
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
6
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
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
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
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)
www.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