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Bronchopulmonary dysplasia & Wilson-Mikity syndrome : Is
there any difference?
September 6, 2002
11th Congress of ASEAN Association of Radiology
Symposia IX
Paediatric Radiology
Mutsuhisa Fujioka, M.D.
Dokkyo University School of Medicine
What is this?
80 day-old-baby
Bronchopulmonary dysplasia(BPD) or chronic lung disease(CLD)
Why is it so important ?
Griscom NT: Caldwell Lecture. Respiratory problems of early life now allowing
survival into adult hood: concepts for radiologists. AJR 1993 Sep; 161(3) : 574-5
Survivors of bronchopulmonary dysplasia have decreased exercise
capacity, wheezing, and recurrent pneumonia, although their chest
radiographs may become normal or almost normal.
Aquino Sl, Schechter MS, Chiles C, Ablin DS et al :
High-resolution inspiratory and expiratory CT in older
children and adults with bronchopulmonary dysplasia.
AJR 1999 0ct. 173(4):963-7
Massachusetts General Hospital, USA
Abnormal findings on inspiratory and expiratory high-resolution
CT of older children with bronchopulmonary dysplasia include
scarring and air trapping with architectural distortion. The
correlation between these findings and physiologic evidence of air
trapping and obstructive lung disease was statistically significant.
What is BPD(bronchopulmonary dysplasia)?
Originally(1967): a new chronic pulmonary syndrome that is
associated with the use of intermittent positive pressure
respirators(IPPR) and high oxygen for longer than 150 hours.
Currently(1992): chronic pulmonary syndrome occurring in
prematurely born infants treated with positive pressure
ventilation and oxygen supplementation for respiratory
insufficiency.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
BPD or CLD ???
In 1989, the Bureau of Maternal and Child Health and Resources
Development put forward the following diagnostic criteria for
BPD
(1 ) Positive pressure ventilation during the first 2 weeks of life for
a minimum of 3 days
(2) Clinical signs of respiratory compromise persisting longer than
28 days of age
(3) Requirement for supplemental oxygen longer than 28 days of
age to maintain a PaO2 above 500mmHg
(4) Chest radiograph with findings characteristic of BPD
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Day 1
Day 2
Day 3
Day 4
Day 5
Day 6
Day 14
Day 20
Day 25
Day 30
Day 70
3 months
4.5 months
Radiographic findings of BPD
Chest radiograph: Original four-stage radiographic
progression to the severly hyperinflated cystic appearance
of the lungs in chronic BPD is still seen in severe cases.
More commonly, the radiographic changes are more subtle
and the progression may be more prolonged.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Radiographic findings of BPD
Complete opacification of the lungs, originally described as
stage II, is now uncommon.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Radiographic findings of BPD
The characteristic spectrum of chest radiographic findings of
chronic BPD are bilateral diffuse interstitial thickening of mild
to very severe degree with normal to increased expansion of the
lungs gradual onset with little change over time.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
The broadening of the spectrum of radiologic findings
characteristic of BPD has resulted in the use of terms such as
chronic lung disease.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Definition of CLD
(2) Clinical signs of respiratory compromise persisting longer than
28 days of age
(3) Requirement for supplemental oxygen longer than 28 days of
age to maintain a PaO2 above 500mmHg
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Is it preventative ?
Prevention
1 technologic approarch: decreasing pulmonary oxygen toxicity
and barotrauma, high –frequency ventilation, extracorporeal
membrane oxygenation
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Prevention
2 molecular-biologic approarch: exogenous surfactants(bovine,
porcine, human, synthetic); prenatal induction of pulmonary
surfactant and antioxidant enzyme activity by maternal
corticosteroid treatment and use of exogenous antioxidants as well
as steroid and nonsteroid inhibitors of the pulmonary inflammatory
cascade.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Prevention
3 socioeconomic-political approarch: Decreasing incidence of
premature birth by socioeconomic-political action
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Pathogenesis and Risk factors
1 respiratory distress or failure,
2 premature birth,
3 oxygen supplementation, and
4 intermittent positive pressure ventilation
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Pathogenesis and Risk factors
Causes of respiratory distress are now recognized to include not
only RDS but also meconium aspiration pneumonia, neonatal
pneumonia, congestive heart failure, Wilson-Mikity syndrome,
and prematurity. Pulmonary air leaks(pulmonary interstitial
emphysema, pneumomediastinu, and pneumothorax), pulmonary
edema and pulmonary infection, all of which prolong the need for
mechanical ventilation and supplemental oxygen therapy,.
Increase the incidence of BPD.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Day 1
Day 2
Day 3
Positive IgM : Wilson-Mikity Syndrome(WMS)
A cystic or reticular chest radiographic change is
occasionally seen in the first week of life in low birth weight
prematurely born infants being mechanically ventilated with
low concentrations of supplemental oxygen or room air. This
radiographic change has been suggested by some to represent
a new form of BPD but more likely represents a
reappearance of the Wilson-Mikity syndrome.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Chest radiographic changes of this type have been described
as early as the first day of life in the Wilson-Mikity
syndrome. Since infants with the Wilson-Mikity syndrome
generally do not die, pathologic confirmation of this clinical
diagnosis is usually not possible. The pathology of the
Wilson-Mikity syndrome and that of BPD are quite different.
Northway WH. Bronchopulmonary dysplasia: Twenty-five Years Later. Pediatrics (1992) 89:969-973
Fujimura M, Takeuchi T, Kitajima H, Nakayama M : Elevated serum IgM of the neonate and
chronic inflammation of the placenta with subsequent development of Wilson-Mikity
syndrome. Biol Neonate., 47 : 251-251, 1985
大出集、勝又大助、小幡一夫、名越廉、猪谷泰史、大野勉、新津直樹、藤岡睦久：
慢性肺疾患３２例の臨床的検討新生児学会誌 (1988) 24(2):464-472
Ohide S et al: Clinical evaluation in 32 cases with chronic lung disease. Acta Neonatologica Japonica (1988) 24(2):464-472
Fujimura M et al: Increased leukocyte elastase of the tracheal
as pirate at birth and neonatal pulmonary emphysema.
Pediatrics. 92(4):564-569, 1993 Oct.
The level of PMN elastase-alpha 1-Pl (polymorphonuclear
leukocyte elastase-alpha 1-proteinase inhibitor complex ) was
increased in the tracheal aspirates of newborns in whom
pulmonary emphysema developed. Intrauterine inflammation
may increase the level of PMN elastase in the fetal respiratory
tract. This increase in PMN elastase-alpha 1-Pl in fetal lung
tissue may cause lung injury in utero, resulting in postnatal
pulmonary empysema consisitent with the Wilson-Mikity
syndrome following ventiration
Ogawa Y:Chronic lung disease of the very low birth weight infant – is it preventable?
Turkish Jorunal of Pediatrics. 40(1):33-44, 1998 Jan-Mar
CLD(chronic lung disease)
An oxygen requirement greater than that obtainable in room air at 28 days of age, with
symptoms of persistent respirator distress and a hazy or emphysematous appearance
upon chest x-ray
4964 infants weighing less than 1500g at birth and born in 1990 admitted to and cared
for at level II and III neonatal care centers in Japan. A total 4293(86.3%) survived at 28
days after birth.
Six types classification according to their preceding illnesses
and chest x ray findings
Type I and II: CLD following the acute stage respiratory distress syndrome(RDS).
Type I with typical radiographic findings for classical BPD.
Type II with atypical radiographic findings, only diffuse haziness without
typical emphysema and fibrosis.
Type III has a history of intrauterine inflammation with typical bubling and cystic
appearance identical to those described with Wilson-Mikity syndrome(neonatal
pulmonary emphysema in the very low birth weight infant).
Type IV does not have a history of either intrauterine inflammation or RDS but
shows typical emphysematous and fibrous appearance upon chest x-ray.
Type V includes those with atypical chest x-ray appearance similar to Type II but
without history of RDS and intrauterine inflammation.
The prevention of Type I and II CLD or CLD following RDS,
should be accomplished by successful prophylactic surfactant
replacement therapy with additional application of high
frequency oscillatory ventilation(HFOV) in the acute stage of
RDS or at the time of stabilization right after birth.
Type III and III’ CLD present the most difficult challenge for
prevention strategy because the disease process already started
before birth. At the moment there are no effective measures
for prevention.
The strategy for the prevention of Type IV and V CLD may
reside in the early detection and treatment of patent ductus
arteriosus, sepsis and airway infection including pneumonia.
Management of premature infant with respiratory distress
Prenatal steroid: inducing pulmonary maturity, reduction in the incidence of
intraventricular hemorrhage and necrotizing enterocolitis
Surfactant: no longer confined to premature babies with RDS. Conditions such as
infection, meconium aspiration and diaphragmatic hernia
Postnatal steroid : short time therapy, long term treatment
Nitric Oxide:inhalated nitric oxide providing effective, non toxic, local vasodilatation
to treat secondary persistent pulmonary hypertension(PPHN)
High frequency ventilation: delivering small tidal volumes at very rapid rate with lower
proximal airway pressures, most useful in situations with air leak or as an option or a
bridge to ECMO when more conventional ventilation methods fail.
ECMO : severe reversible respiratory failure and pulmonary hypertension most often
related to sepsis, meconium aspiration or congenital diaphragmatic hernia. Because of the
need for anticoagulation, ECMO is contraindicated in infants with prexsisting intraventricular
hemorrhage(IVH) greater than grade 1-2 or premature infants with a high risk of IVH.
Conclusion
Wilson-Mikity syndrome is a specific entity in
premature infants caused by prenatal infection
from chorioamnionitis and may result in
bronchopulmonary dysplasia or chronic lung
disease.
Conclusion
Bronchopulmonary dysplasia is a condition of
infants with chronic lung disease showing
characteristic radiographic findings.
Thank you for your attention!
Department of RADIOLOGY

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