What will the Successes be Like as Adults?

Congenital Diaphragmatic
Hernia: What will the Successes be
Like as Adults?
Allan L Coates, MDCM, B Eng(elect)
With a big thanks to
Dr Priscilla Chiu
Dr Desmond Bohn
Dr Daniel Trachsel
No conflict of Interest
Introduction
¾ Relatively common with estimated incidence
of 1 in 3000 live births
¾ 85% are left sided, the classic posterolateral
Bochdalek hernia
¾ Antenatal diagnosis is common but does not
appear to affect outcome dramatically
¾ Associated abnormalities, (40-50%)
particularly congenital heart disease are affect
mortality
Early Respiratory Challenges
¾ Pulmonary hypoplasia
ƒ Bilateral but almost always worse on side of
hernia
¾ Pulmonary hypertension
ƒ One of the main reasons for NO and/or ECMO
¾ Reflux and aspiration
ƒ Usually a problem as soon as feeding begins
and often a problem for many years
Historical Milestone in Treatment
¾ ExtraCorporeal Membrane Oxygenation
(circa 70’s and 80’s)
¾ Stabilization before surgery (circa late 80’s)
¾ High Frequency Oscillation Ventilation
(circa 80’s)
¾ “Gentle” Ventilation or passive hypercapnea
(circa mid 90”s)
¾ Inhaled Nitric Oxide (90’s)
The CDH ECMO Controversy
¾ Sickkids vs Boston Children’s Hospital 19811994 > 400 cases
¾ Boston used ECMO as primary method of
resuscitation with 50% receiving ECMO and a
53% survival vs 1% at Sickkids and a 55%
survival
¾ Retrospective Study
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Bohn Am J Respir Crit Care Med 2002
Azarow et al J Pediatr Surgery 1997
Wilson et al J Pediatr Surgery 1997
Changing Mortality at Sickkids
¾ Conventional Ventilation (CV) n=77 1985-1989
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Survival
51%
HFOV
15%
ECMO
0%
Need for gastrostomy feeding 8%
¾ Gentle Ventilation (GV) n=66 1996-2000
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Survival
80% (p<0.05 Chi squared)
HFOV
36%
ECMO
4%
Need for gastrostomy feeding 34% (p<0.05)
¾ No mention is made of associate abnormalities such as
congenital heart disease
Chui et al J Pediatric Surgery 2006
Late Respiratory Challenges
¾ Airway Hyperractivity with asthma like
symptoms
ƒ In previous study 50% of “Gentle Ventilation”
group were prescribed bronchodilators
¾ Musculoskeletal abnormalities (about 25%)
ƒ Scoliosis, pectus excavatum, chest wall asymmetry
¾ Recurrence of hernia, especially with patch
repairs
¾ Long term gastrostomy feeding and GERD
AT BIRTH and 8 YEARS OLD
V/Q Scan
Right Lung 63%
Left Lung 37%
Pulmonary Function at Seven
¾ TLC
131%
RV/TLC 45%
¾ FEV1
63%
FVC
¾ FEV1/FVC
60%
¾ No response to bronchodilators
90%
Long Term Follow Up
(Sickkids)
¾ 1985–1991 Conventional Ventilation n=23
with age and gender matched controls
¾ Echocardiography
¾ Pulmonary Function Testing
¾ Cycle Ergometer Exercise Testing (progressive
2 min intervals) with Cardiac Output at each
end of each interval (CO2) exponential – Lands
et al)
Trachsel et al Pediatric Pulmonology 2006
Long Term Follow Up
(Sickkids)
¾ Echocardiography
ƒ Good myocardial function but smaller pulmonary
artery on affected side (p<0.01)
¾ Pulmonary function Cases vs Controls
ƒ FEV1 (% pred) 83±15% vs 98±10% (p<0.02)
ƒ RV/TLC 31±10% vs 22±6% (p<0.001)
¾ Exercise
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Wmax (% pred) 77±12% vs 91±16% (p<0.01)
Wmax correlated with FEV1 and RV/TLC in cases
All cardiac indices were normal
Long Term Follow Up
(Netherlands)
¾ Survivors from 1987-1999 ECMO excluded
n=53
¾ Case Control Study matching sex, age and
height
¾ Pulmonary Function Measurements
ƒ Spirometry
ƒ Lung volume (FRC by washout technique)
¾ Exercise Testing
ƒ Treadmill, Bruce Protocol, 3 minute stages
Peetsold et al Eur J Respir 2009
Results PFTs
¾ Pre bronchodilatation, the FEV1 was < Lower
Limit of Normal (LLN defined as Z score <
1.64) in 46% CDH but in no controls. Mean 1.63±1.78 vs 0.08±0.90. p<0.001
¾ Positive response to BD in 28% in CDH vs 6%
in controls (p=0.007)
¾ RV/TLC > ULN in 52% CDH vs 0 controls
(p<0.001)
¾ Abnormal PFTs associated with GERD and
length of ventilation
Peetsold et al Eur J Respir 2009
Results
¾ Exercise performance did not differ from
control subjects
¾ Changes with age
ƒ The CDH survivors ranged from 6-17 years
ƒ FEV1 Z-scores showed a trend to be lower in
the older subjects, p=0.06 but not controls
ƒ It is unclear whether this was due to improved
therapy eg less ventilator induced injury or
other factors
Issues Not Addressed In Both Studies
¾ Effects of type of surgery
ƒ Patch repairs have a higher incidence of recurrence
ƒ At Sickkids, VATS has high much higher
incidence of recurrence
¾ Effects of recurrence
¾ Effects of musculoskeletal abnormalities
¾ Mentioned but not addressed is that there was a
subset of the population that has neurological
sequellae
Common Follow Up Themes
¾ There is a significant degree of obstructive
findings
¾ There is a high incidence of asthma like
symptoms and findings
¾ Exercise abnormalities tend to be mild
¾ Despite early pulmonary hypertension and
decreased size of the affected pulmonary
artery, cardiac function becomes normal
Conclusions
¾ Despite increasing survival of those who would not
have survived 25 years ago, this new group of
survivors does not appear to have much greater
sequellae
¾ Some degree of obstructive disease, usually
responsive to brochodilators is common and likely
lasting well into adulthood
¾ CDH is another neonatal disease like pre term birth
with bronchopulmonary dysplasia that will give rise
to airway obstruction in young adults