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