PEDIATRIC EMERGENCIES 0889-8537/01 $15.00 + .

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GENERAL PEDIATRIC
EMERGENCIES
Acute Pulmonary Edema

Tetsu Uejima, MD

Acute pulmonary edema occurs less frequently in children because

of the absence of coronary artery disease. The pathophysiologic mecha-
nism behind any form of pulmonary edema, however, is based on the
variables of the Starling equation44:
Q = k[(Pcap- Pint) - u(mcap - mint)]

The resulting interactions of the pulmonary capillary hydrostatic

pressure (Pcap),the interstitial hydrostatic pressure (Pint),the plasma
oncotic pressure (rcap), the interstitial oncotic pressure (mint),the protein
reflection coefficient (a),and the fluid filtration coefficient (k) determine
net filtration (Q), which ultimately determines the nature of the pulmo-
nary edema. Under normal circumstances, Q is slightly positive, re-
sulting in a net positive fluid flux into the interstitium. This fluid is
drained into the vascular system as lymph. The lymphatic system can
accommodate a 300% increase in flow before fluid accumulates in the

Cardiogenic pulmonary edema occurs when Pcap,which can be

measured as the pulmonary capillary wedge pressure, is excessively
high, resulting in a large Q. The resulting edema fluid in this situation
is low in protein content.
Noncardiogenic pulmonary edema occurs in the presence of a nor-
mal pulmonary capillary wedge pressure with a high protein content in
the alveolar fluid signifying capillary leak. Clinically, this mathematic
view of pulmonary edema is simplistic, but allows a better understand-

From the Department of Anesthesia, Children’s Memorial Hospital, Northwestern Univer-

sity Medical School, Chicago, Illinois

ANESTHESIOLOGY CLINICS OF NORTH AMERICA

VOLUME 19 NUMBER 2 * TUNE 2001 383

384 UEJIMA

ing of the pathophysiology. This article focuses on three types of acute

pulmonary edema in children: negative-pressure pulmonary edema,
neurogenic pulmonary edema, and cardiogenic pulmonary edema.

NEGATIVE-PRESSURE PULMONARY EDEMA

Negative-pressure pulmonary edema, also known as postobstruc-

tive pulmonary edema, occurs secondary to upper airway obstruction,
and is a rare but well-recognized phenomenon. The true incidence is
unknown. Various types of airway obstruction, including croup, epiglot-
titis, laryngospasm after tonsillectomy, hanging, and tumors have been
reported to cause negative-pressure pulmonary edema.3,15, zo* 45, 47 Nega-
tive-pressure pulmonary edema also can occur with aspirated foreign
bodies, esophageal foreign bodies, and after biting of a laryngeal mask
airway.1z,17, 33 Unilateral pulmonary edema and negative-pressure-in-
duced pulmonary hemorrhage also have been reported.', 4, 38 One recent
paper reported negative-pressure pulmonary edema in a burn patient
with a patent airway secondary to pulmonary emb01i.l~
The exact mechanism by which negative-pressure pulmonary edema
occurs is unknown, although it is most likely multifactorial in 32

Large, negative intrapleural pressures are the pathophysiologic hallmark

of negative-pressure pulmonary edema. These pressures increase lymph
flow and interstitial edema, causing pulmonary edema.8,42 Hemody-
namic changes associated with severe intrapleural pressures include
increased venous return to the right circulation and pooling of blood
in the pulmonary venous circulation during inspiration.28In negative-
pressure pulmonary edema, the Starling equation states that increases in
fluid flux are related directly to differences in hydrostatic pressure.
Left ventricular end diastolic pressures increase with negative pleural
pressurezz,34 because of increased venous return to the right ventricle,
causing right ventricular distention, and a leftward shift of the interven-
tricular septum, causing decreased left ventricular compliance.z8,z9 HYP-
oxia and the CNS may play a role in negative-pressure pulmonary
edema. In high-altitude pulmonary edema, hypoxia increases pulmonary
vascular re~istance.~~ Hypoxia also can precipitate a massive CNS-in-
duced adrenergic response similar to that seen in neurogenic pulmonary
edema.18,z5
Clinically, negative-pressure pulmonary edema is characterized by
its rapid onset and short-lived course. Pulmonary edema often occurs
once the obstruction is relieved. Onset is usually within minutes, but
may occasionally be hours. Treatment is symptomatic because the course
is self-limited. Airway patency must be maintained. Most patients re-
quire endotracheal intubation with continuous positive airway pressure
or positive end-expiratory pressure. Mask continuous positive airway
pressure (CPAP) may be considered in patients requiring small amounts
of CPAP who are not in ventilatory failure. Inspired oxygen can be
titrated to a desired oxygen saturation, usually more than 90%. Inotropic
 ACUTE PULMONARY EDEMA 385

support, diuretics, and invasive hemodynamic monitoring usually are

not required if the diagnosis is clearly negative-pressure pulmonary
edema. Symptoms commonly resolve within 12 to 24 hours. If symptoms
persist, pulmonary aspiration and cardiogenic causes of pulmonary
edema should be considered.
Caution should be used regarding the use of intramuscular succinyl-
choline. Intramuscular succinylcholinehas been implicated in precipitat-
ing acute pulmonary edema and pulmonary hemorrhage in the absence
of obvious upper airway obstruction." The exact mechanism of this
phenomenon is unknown.

NEUROGENIC PULMONARY EDEMA

Shanahan first reported neurogenic pulmonary edema in 1908 in a

series of 11 patients who had epileptic seizures.39Battle-related head
injuries were associated with neurogenic pulmonary edema during
World War IZ6and during the Vietnam War.40Since then, neurogenic
pulmonary edema has been recognized as a form of pulmonary edema
that most commonly occurs after CNS injuries associated with a rise in
the intracranial pressure. Various CNS conditions, including open and
closed head 35, 40 subarachnoid 36 cerebral hemor-
rhage? meningiti~;~ postictal 39 and cervical spine injuries31have
been reported to cause neurogenic pulmonary edema.
The exact cause of neurogenic pulmonary edema is unknown. Ex-
perimental evidence in animals has isolated certain areas of the CNS
most likely to be responsible for neurogenic pulmonary edema. These
include the A, and A, areas of the medulla, the nuclei of solitary tract,
the area postrema, and the hypothalamus.1° Two avenues of speculation
have arisen regarding the cause of neurogenic pulmonary edema: in-
creased capillary hydrostatic pressure and increased capillary permeabil-
ity. Increased capillary hydrostatic pressure is the so-called blast theory.46
The blast theory hypothesizes that a CNS injury triggers a massive
adrenergic response, causing severe pulmonary and systemic hyperten-
sion accompanied by an increase in venous return. The increase in Pcap
causes an increase in net filtration and pulmonary edema.2,48 After the
blast, capillary permeability increases because of barotrauma, unknown
neurogenic mechanisms, or not yet identified mediators, allowing pro-
tein to leak, and more pulmonary edema despite normal pulmonary
pressures.
Neurogenic pulmonary edema also can occur with normal pulmo-
nary pressures, suggesting increased capillary permeability as a cause.6,23
In this theory, u increases because of neuronal influences or by damage
to the capillary endothelium, which causes leakage of protein into the
interstitial and alveolar spaces, causing pulmonary edema. Analysis of
alveolar fluid in neurogenic pulmonary edema reveals a high protein
content, suggesting capillary leak as the primary cause.14More careful
386 UEJIMA

analysis of edema fluid protein to plasma protein ratios, however, do

not support this theory consistently? l4
A diagnosis of neurogenic pulmonary edema should be considered
in any patient with a history of head trauma or intracranial pathology.
The incidence of neurogenic pulmonary edema is believed to be signifi-
cantly lower in children than in adults. Onset is usually with a few
hours, but some patients may not manifest symptoms until 12 hours to
a few days after the insult.10Initial signs and symptoms are indistin-
guishable from other forms of pulmonary edema. Chest radiography
may mimic adult respiratory distress syndrome.16
The treatment of neurogenic pulmonary edema is primarily sup-
portive. Primary therapy should be directed at treating the CNS prob-
lem. Supplemental oxygen should be administered. Airway patency and
adequacy of ventilation must be ensured because most patients have
underlying CNS pathology and increased intracranial pressure. Patients
with a Glasgow Coma Scale score of 8 or less should be intubated as
quickly as p0ssib1e.l~Positive-pressure ventilation with hypocarbia may
be required to decrease intracranial pressure. Positive end-expiratory
pressure (PEEP) may be required to maintain oxygenation. Although the
effect of high levels of PEEP on intracranial pressure in patients with
head injury is controversial, intracranial pressure monitoring is probably
appropriate if PEEP exceeds 10 cm H,O. Ventilatory settings should be
adjusted to minimize hypotension and increases in airway pressure
because they may be harmful to patients with CNS pathology. Neuro-
genic pulmonary edema usually resolves within 24 to 48 hours. Persis-
tent pulmonary edema should prompt an examination for other causes,
such as aspiration or cardiogenic pulmonary edema.

CARDIOGENIC PULMONARY EDEMA

Cardiogenic pulmonary edema develops when Pcapis excessively

high, overwhelming the ability of the lymphatic system to resorb fluid.
In the adult, anesthesiologists are familiar with the patient with heart
failure and cardiogenic pulmonary edema. In children, cardiogenic pul-
monary edema occurs most often with congenital heart disease. Cardio-
genic pulmonary edema can be iatrogenic and solely attributable to fluid
overload in caretakers unfamiliar with fluid administration in children.
More commonly, cardiogenic pulmonary edema can occur with large
left-to-right shunting lesions, such as a patent ductus arteriosus or a
ventricular septa1 defect. Cardiogenic pulmonary edema also can occur
when there is a problem with left ventricular filling or emptying and
with left ventricular function, as in congenital critical aortic stenosis and
endomyocardial fibroelastosis. Lesions that obstruct normal emptying of
the pulmonary veins, as in total anomalous pulmonary venous return,
can cause cardiogenic pulmonary edema.7
Cardiogenic pulmonary edema usually occurs within the first 6
months of life. It rarely occurs after then without a concomitant medical
 ACUTE PULMONARY EDEMA 387

problem, such as an arrhythmia, a cardiomyopathy, infective endocardi-

tis, pneumonia, or high output states, such as severe anemia.30Chrono-
logically, cardiogenic pulmonary edema presenting in the first week of
life is often coincident with closure of the ductus arteriosus, the so-
called ductal-dependent lesions. These infants often have critical obstruc-
tions to systemic arterial flow, as seen in preductal coarctation of the
aorta. Early presentation also is seen in lesions causing pulmonary
venous obstruction to ventricular filling, as in cor triatriatum. In the next
few weeks, pulmonary vascular resistance decreases, allowing left-to-
right shunting lesions to become more evident. This scenario most
commonly is seen with a ventricular septal defect.
Infants with cardiogenic pulmonary edema are in severe respiratory
distress, with tachycardia, tachypnea, grunting, pallor, and diaphoresis.
Parents often report poor feeding, irritability, and poor weight gain.
Some congenital lesions are associated with left-to-right shunting and
mixing of venous and arterial blood, as in complete atrioventricular
canal and transposition of the great vessels with a large ventricular
septal defect. These infants present with signs and symptoms of conges-
tive heart failure and cyanosis.
Treatment requires definitive or palliative treatment of the underly-
ing lesion. In ductal-dependent lesions, prostaglandin El administration
maintains ductal patency, and helps to relieve vascular congestion. Treat-
ment, otherwise, is directed at decreasing oxygen consumption and
improving oxygenation and cardiac function. As in adults with cardio-
genic pulmonary edema, medical management includes the use of sup-
plemental oxygen, fluid restriction, diuretics, digoxin, inotropes, and
systemic unloading agents.

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Address reprint requests to

Tetsu Uejima, MD
Department of Anesthesia
Children’s Memorial Hospital
Northwestern University Medical School
2300 Children’s Plaza
Chicago, 1L 60614