congenital diaphragmatic hernia antenatal diagnosis diagnosis van mieghem… · 06/10/2013 1 tim...
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06/10/2013
1
Tim Van Mieghem
Congenital Diaphragmatic Hernia
Antenatal Diagnosis
Overview
• introduction
• diagnosing CDH – tips and tricks
• outcome prediction
ultrasound
MRI
functional tests
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incidence 1/2000-4000
85% left sided
13% right sided
2% bilateral
25% anatomical anomalies
20% chromosomal anomalies
Epidemiology
Survival isolated CDH
61 %
n=146
78 %
n=167
72 %
n=105 79 %
n=117
71 %
n=1222
70%
n=63
70 %
n=86
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Prenatal statistics
19%
4%
30%
47%
TOP
IUFD
NND
live at discharge
Gallot et al, UOG 2007; Central France Birth Registry 1986-2003
Overview
• introduction
• diagnosing CDH – tips and tricks
• outcome prediction
ultrasound
MRI
functional tests
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Prenatal Diagnosis
Diaphragm
hypoechogenic line between lungs – viscera
13 wk 21 wk
Prenatal Diagnosis
prenatal diagnosis = 60-80%
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Paradoxical breathing
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Liver herniation
Right sided CDH
Conturso UOG 2012
‘Horizontal stomach’
13 wks 24 wks
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Bilateral CDH
Hiasa et al. Clin Imaging 2012
Overview
• introduction
• diagnosing CDH – tips and tricks
• outcome prediction
ultrasound
MRI
functional tests
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In ISOLATED CDH
Pathology
pulmonary
hypoplasia
pulmonary
hypertension
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Outcome prediction: LHR
Learning curve
N=77
Cruz-Martinez, UOG 2010
LHR @ 22-29 weeks
0
10
20
30
40
50
60
70
80
90
100
<0.7 0.8-0.9 1.0-1.1 1.2-1.3 1.4-1.5 >1.6
Lung to Head Ratio (mm)
Surv
ival
rat
e (
%)
Outcome prediction: LHR
Jani et al 2006
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Prediction: O/E LHR
OBSERVED Fetal Lung Area
(fetus at risk for lung hypoplasia)
area measured
0.5
EXPECTED Fetal Lung Area
(matched normal fetus)
matched fetus
based on
gestational age
area predicted
2.0
O/E = 25%
Liver herniation Antenatal CDH registry 2006
Survival: 75% → 53%
Prediction: liver
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< 15 15-25 26-35 36-45
O/E LHR (%)
0
10
20
30
40
50
60
70
80
90
100
Surv
ival
rat
e (
%)
46 and higher
extreme
liver in abdomen (“down”) liver in thorax (“up”)
severe moderate mild
Clinical algorithm
Deprest J et al, Sem Fetal Neonat Med, 2008 - n= 329.
Isolated CDH expectantly managed (n=100)
Jani et al UOG 2008
≤ 25 26-45 >45
Patch rate (%)
0
10
20
30
40
50
60
70
80
90
100
O/E LHR (%)
0
10
20
30
40
50
60
Conventional ventilation
day
s
≤ 25 26-45 >45
O/E LHR (%)
0
10
20
30
40
50
60
Enteral feeding
day
s
<25 26-45 >45
O/E LHR (%)
0
10
20
30
40
50
60
NICU**
day
s
<25 26-45 >45
O/E LHR (%)
0
10
20
30
40
50
60
70
80
90
≤ 25 26-45 >45
O/E LHR (%)
Oxygen O2 at 28d (%)
100
Morbidity
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Problems with 2D-US
Single plane
Single lung
3D volume
Both lungs
Solutions
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3D ultrasound
Ruano JUM 2013
3D volume
Both lungs
Solutions
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≤ 25 26-35 36-45 > 45
Observed to expected total FLV (%)
0
10
20
30
40
50
60
70
80
90
Surv
ival
rat
e (%
)
≤ 25 26-35 36-45 > 45
Observed to expected total FLV (%)
0
10
20
30
40
50
60
70
80
90
Surv
ival
rat
e (%
)
Liver down
Liver up: Survival 50% - volume linear relation to survival Liver down: Survival 75% - volume is not predictive of outcome
Cannie et al UOG 2008
20% survival rate
~ 33%
MRI volumetry
Liver up
Lee et al. J Ped Surg 2011
Morbidity prediction MRI
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Liver-thorax ratio
Cannie et al, UOG2008 – Lazar J Pediatr Surg 2012
0 10 20 30 40 50
LiTR (%)
0
10
20
30
40
50
60
70
80
o/e
TF
LV
(%
)
Cannie et al. Radiology 2008 Rypens et al. Radiology 2001
MRI - nomograms
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Isolated CDH born alive >30 wks, paired observations (n=76)
False positive rate (%)
Sen
siti
vity
(%
)
100 75 50 25 0
100
75
50
25
0
2D-US
MRI
Cannie et al UOG 2008
2D-US vs. MRI
Kehl et al. JUM 2013
3D-US vs. MRI
Survival Need ECMO
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Overview
• introduction
• diagnosing CDH – tips and tricks
• outcome prediction
ultrasound
MRI
functional tests
Current problems
pulmonary hypoplasia
pulmonary
hypertension
pulmonary hypoplasia
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Functional tests
Pulmonary artery Doppler
Hyperoxygenation test
3D lung vascularity
Fractional moving blood volumes
Pulmonary artery Doppler
Cruz-Martinez UOG 2013
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UOG 2011
PAH
No PAH
DEATH PAH
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Fractional moving blood volume
Cruz-Martinez FD&T 2010
Conclusions
• CDH is diagnosed in (only) 60-80%
• outcome prediction isolated cases:
ultrasound (O/E LHR – liver)
MRI (lung volume – LiTR)
• functional tests experimental
