PULMONARY HEMORRHAGE AND HEMOPTYSIS

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PULMONARY HEMORRHAGE AND HEMOPTYSIS

Milla, MD

Last updated 7/96 for content, 9/97 for format

PULMONARY HEMORRHAGE AND HEMOPTYSIS

1. OVERVIEW AND DEFINITIONS

Relatively uncommon but potentially serious and life threatening problem

in children. Not a disease in itself but presenting symptom of an

underlying condition, of which bleeding is usually the presenting, if not

the only, symptom.

Severity of the bleeding not necessarily correlates with the severity or

seriousness of the underlying problem. Multiple unrelated problems can

have bleeding as part of their symptom complex, thus differential

diagnosis based solely on the presence and severity of bleeding can be

quite difficult.

When bleeding results in death it is almost always because of

asphyxiation and not from exsanguination. Therefore, maintaining adequate

airway and ventilation is crucial.

Source of bleeding usually difficult to pinpoint. Eroded airway vessel,

diffuse parenchymal bleed, congenital malformation (not necessarily

vascular), traumatized airway or lung parenchyma can be potential sources

of bleeding.

Data on incidence in children is lacking. In neonates occurrence of

pulmonary hemorrhage has been estimated at 0.7 to 3.8 events per 1,000

live births.

Pulmonary Hemorrhage: Bleeding that occurs within the lungs and that has

a parenchymal or bonchial source. May or may not lead to hemoptysis.

Depending on source, children will not always be able to cough up all the

blood, even more if source is parenchymal.

Pulmonary Hemosiderosis: Accumulation of iron, in the form of

hemosiderin, within alveolar macrophages. Always the result of bleeding

into the lungs, and more likely after bleeding at the alveolar level than

at the large airway level.

Hemoptysis: Expectoration of fresh blood derived from the lungs. Blood is

coughed out in variable amounts, usually the patient will mention a

'mouthful' or 1 to 2 ounces of bright red, foamy blood being spit out.

Assessment of severity of hemoptysis can be based on amount of blood lost

during episode:

MildLess than 60 cc of blood lost for the whole episode.

MassiveMore than 200 cc of blood lost in a 24 hour period.

Life-threateningMore than 120 cc of blood lost in an hour.

These criteria applies mostly to older children and adolescents. At any

age, bleeding that results in respiratory distress and altered gas

exchange is life-threatening, regardless of amount of blood (remember,

amount of blood expectorated not necessarily represents the total amount

lost into the airspaces).

Hemoptoic expectoration: Coughing out of sputum with streaks of blood

mixed with it. This is not hemoptysis.

Click here to go to section 2: Where is it from?

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are strictly those of the page author. The contents of this page have not

been reviewed or approved by the University of Minnesota.

HEMORRHAGE AND HEMOPTYSIS

2. WHERE IS IT FROM?

Not always the spitting of blood will mean that bleeding has occurred in

the lungs. Because of the seriuosness that pulmonary hemorrhage implies,

the lungs as the source of the bleed has to be well established.

By history, try to establish whether the blood was vomited or coughed.

Have the parents and the patient describe the blood. Blood coming from

the GI tract will have different characteristics than that coming from

the lungs, and different symptoms may be related to episode:

GI Respiratory tract

Dark red or brown Bright red

In clumps Foamy, runny

Mixed with food Mixed with mucus

acidic pH alkaline pH

Stomachache, abdominal discomfort Chest pain, localized warmth or

gurgling over chest

Nausea, retching before/after episode Persistent cough

Determine if there is any pre-existing medical condition. Currently the

most common underlying condition associated with hemoptysis in children

is CF, but also children with congenital heart disease, sickle-cell

anemia and autoimmune disorders can present with hemoptysis.

Physical exam can also be revealing. Start with a good HEENT exam,

bleeding from the nose, nasopharynx, tonsils, tongue, gums, or oropharynx

can be easily identified. Most importantly, do a quick assessment of

vital signs, respiratory status (is distress present or not?, is

oxygenation adequate?) and hemodynamic status. Lung exam may be

non-contributory, but decreased breath sounds, crackles, ronchi or

wheezes can be heard diffusely or localized.

At presentation, just a complete blood count and a chest radiogram will

be enough to help establish the seriousness of the problem (along with

the history and physical exam findings) and the need for further

intervention and investigations.

Click here to go to section 3: Pulmonary Hemorrhage in the Neonatal

Period

Back to table of contentsThe views and opinions expressed in this page

are strictly those of the page author. The contents of this page have not

been reviewed or approved by the University of Minnesota.

PULMONARY HEMORRHAGE AND HEMOPTYSIS

5. PULMONARY HEMORRHAGE IN CHILDREN AND ADOLESCENTS

Airway Inflammation: Mild bleeding can be seen during episodes of acute

tracheobronchitis or bacterial tracheitis. It is usually self-limited and

due to friability of the inflamed airway mucosa. Bloody sputum can also

be seen with pneumococcal pneumonia, however this is also mild and

self-limited bleeding, rarely requiring any intervention.

Bronchiectasis: Dilated bronchi, with weakened walls and presence of

acute and chronic inflammatory changes. These are common in children with

CF and bleeding can be seen during acute exacerbations of the chronic

infection. Because of this, CF is probably the most common cause of

significant bleeding in this age group. Bleeding occurs at the level of

tortous bronchial vessels that feed these airways and become engorged as

a result of the inflammation of the airway walls.

Congenital malformations: Pulmonary sequestrations and bronchogenic cysts

can be incidentally discovered in children after a superimposed infection

triggers bleeding in them and prompts for medical attention.

Arteriovenous malformations can also manifest themselves during childhood

with bleeding, although the most common symptom related to these is

shunting. Approximately 50% of these patients will be diagnosed with

familial hemorrhagic telangiectasia (Osler-Weber-Rendu disease), so the

presence of skin telangiectases in a child with pulmonary bleed is very

suggestive of this diagnosis. The finding on auscultation of a bruit

(which can be acentuated by having the patient attempt inspiration with

the glotis closed), although not always present, is also suggestive of an

arteriovenous malformation.

Cardiovascular problems: Any anomalies that result in pulmonary arterial

flow obstruction, increased bronchial circulation or pulmonary venous

congestion can lead to pulmonary hemorrhage, and this is more likely to

occur during adolescence. Eisenmenger complex, corrected pulmonic

stenosis or tetralogy of Fallot, obstruction of pulmonary veins or

arteries, and mitral valve stenosis are associated with pulmonary

hemorrhage. Pulmonary embolism and acute chest crises in children with

Sickle cell disease can also induce pulmonary bleeding, most commonly in

adolescents.

Immunologic disorders: Pulmonary hemorrhage can be the initial

manifestation of a variety of immunologically mediated diseases.

Goodpasture syndrome: Predominantly affects older adolescents and young

adult males. Massive hemoptysis with a concurrent proliferative

glomerulonephritis. Represents a Gell-Coombs type II reaction.

Circulating antibasement membrane antibody is diagnostic, however some

10% of the patients do not have circulating antibodies, but it can be

demonstrated in lung or kidney biopsy specimens.

Immune-complex-mediated glomerulonephritis with pulmonary hemorrhage: An

entity clinically undistinguishable from Goodpasture's, but with

different pathophysiology (Gell-Coombs type III reaction). It has been

described only in children and diagnosis is made by lung or kidney biopsy

findings.

Henoch-Schonlein purpura: Diffuse vasculitis presumably precipitated by

an immunologic reaction. Only few cases have been reported were pulmonary

hemorrhage was part of the presenting symptoms.

Miscellaneous: Systemic lupus erythematosus, polyarteritis nodosa,

Behcet's disease and Wegener's granulomatosis have been reported as

presenting during adolescence with pulmonary hemorrhage.

Infections: Lung abscesses, Fungal cavitary infections, allergic

bronchopulmonary aspergillosis, lung parasitic infections

(Paragonimiasis, hydatidosis), can all produce massive pulmonary bleeding

from erosion of airway and vessel walls.

Retained intrabronchial foreign body: A retained foreign body, especially

if it is organic material, can elicit an intense local inflammatory

response with hyperplasia of the blood vessels and weakening of the

airway wall. Considerable time may elapse between the aspiration of the

foreign body and the onset of bleeding, so history is usually negative

and this possibility is usually overlooked. Bleeding can be massive,

particularly in the presence of bronchiectatic changes in the affected

airway. Radiologic studies may not necessarily be compatible with foreign

body aspiration, but may reveal presence of bronchiectasis. Diagnosis is

usually made on pathologic analysis of resected bronchopulmonary

segement.

Pulmonary compression injury: Direct trauma to the chest from an

accelerating or decelerating force when the glottis is closed induces

compression of the lung parenchyma. Because of the pliability of the

chest wall in children, rib fractures not necessarily occur. The abrupt

increase in intraalveolar pressure results in tissue disruption.

Extensive hemorrhage, edema and atelecatsis quickly develop in the

affected segments. On presentation the child will show variable degrees

of respiratory distress, hypoxia and a presistent cough. Chest exam will

reveal decreased breath sounds in the affected lobes and a combination of

wheezing and coarse crackles. Chest radiograms will demonstrate

atelectatic areas, which can be uni- or bilateral.

Inhalational injury: Exposure to toxins such as nitrogen dioxide, carbon

monoxide, cocaine or crack cocaine may induce disruption of the integrity

of the alveolo-capillary membrane and result in hemorrhage. Peripheral

eosinophilia in previously healthy adolescent with pulmonary hemorrhage

is highly suggestive of a toxic inhalation.

Click here to go to section 6: Guidelines for Management

Back to table of contentsThe views and opinions expressed in this page

are strictly those of the page author. The contents of this page have not

been reviewed or approved by the University of Minnesota.

PULMONARY HEMORRHAGE AND HEMOPTYSIS

6. GUIDELINES FOR MANAGEMENT

3 important steps (to be followed in order):

Assess and ensure adequate ventilation and oxygenation: Remember, what

kills these patients is asphyxia. Do not hesitate to intubate a child

with respiratory distress, even if the bleeding seems to be mild. In

infants most of the blood will remain in the lung parenchyma and filling

the airspaces. If the bleeding site (or at best, lung) is identified,

keep the ipsilateral side dependent so the unafected lung can sustain

ventilation. Selective intubation and ventilation of the unaffected lung

can also be attempted, particularly if the bleeding site is in the right

lung. If initial assessment is good, monitor oxymetry. Monitor blood

gases if there is evidence of mild hypoventilation. Ensure that the

patient adequately clears secretions, this is of particular importance in

patients with CF, were secretions clearance is already a problem. Except

for patients with pulmonary trauma, there is no contraindication for

respiratory therapy in patients with hemoptysis. This will help clear the

airways from clots and avoid the loss of functional lung units and

significant V/Q mismatch.

Assess and maintain intavascular volume: Even though death from

exsaguination is rare in children, development of hypovolemia or

significant anemia (which children with PH usually already have) will

complicate management. Check vital signs, capillary refill and look for

orthostatic changes. Start a good IV line (the largest bore possible),

have blood typed and ready for transfusion if the need arises. Check

platelet count and obtain a coagulation profile. Ensure that the patient

is not on any drugs that will impair coagulation. DDAVP (IV or

intranasal, depending on the patient's condition) can be tried. Monitor

hematocrit, expect it to drop in the first few hours if the patient has

had an acute onset of massive bleeding and is evaluated shortly after it

started.

Determine the cause and site (if possible) of the bleeding: Good history,

physical exam, chest X ray and CBC to start. A chest CT (with contrast)

may add valuable information in the child with an abnormal X ray,

particularly when bronchiectasis or congenital malformations are

suspected. In children with underlying conditions (like CF) cause not an

issue. For previously healthy children, keep in mind possible causes

mentioned on previous sections. Bronchoscopy has been advocated as

indicated in any child with acute bleeding or without a clearcut cause

for the bleeding. Usually possible to look for upper airway lesions or

identify lung segment from which blood is coming from (rarely bleeding

site will actually be identified). Through bronchoalveolar lavage

adequate specimens for hemosiderin stains can be obtained. Bronchoscopy

has also a therapeutic potential. Different procedures have been

described to help stop the bleed: segmental lavage with ice-cold saline,

bronchial blockage with balloon catheter, local instillation of

vasoactive agents, &c. Angiography can be attempted in the patient with

persistent massive bleeding. It will not always identify bleeding vessel,

but it may identify tortous vessels that are possible sources.

Embolization of these vessels with gelfoam pieces, metal coils or

thrombotic agents can be performed at same time and is usually succesful

in stopping bleeding.

Further management is dictated by the etiology of the bleeding. If

bleeding persists in spite of aggresive intervention, lobectomy should be

considered.

Becki

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