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Hemoptysis

AS MY QUESTION FOR MY STUDENTS HOW DO WE MAKE THE DIFFERENCE BETWEEN HEMOPTYSIS AND BLOOD COMING FROM SOMEWHERE ELSE : ANSWER IS THE COUGH

coughing up of blood from the respiratory tract.

The blood can come from the nose, mouth, throat, the airway passages leading to the lungs, or the lungs themselves.

The word “hemoptysis” comes from the Greek "haima," meaning "blood," and "ptysis," which means "a spitting."

Blood-tinged mucus in a healthy nonsmoker usually indicates only a mild infection and is generally no cause for concern.

Indeed, the most common cause of hemoptysis is the least serious – a ruptured small blood vessel caused by coughing and/or a bronchitic infection.

In patients who have a history of smoking or are otherwise at risk for any underlying lung disease, however, hemoptysis is often a sign of serious illness, including cancer.

Other serious diseases that can cause hemoptysis [SEE BELOW DETAILS] include bronchiectasis (chronic dilation and infection of the bronchioles and bronchi); pulmonary embolus (a clogged artery in the lungs that can lead to tissue death);

pneumonia (a lung infection); and tuberculosis (see Tuberculosis).

Hemoptysis can also result from breathing in a foreign body, such as a particle of food that pokes and ruptures a blood vessel

Hemoptysis refers specifically to blood that comes from the respiratory tract.

Often when a person spits up blood,

however, they’re spitting up something that has come from somewhere else - the nose, the back of the throat, or some part of the gastrointestinal tract.

When blood originates outside of the respiratory tract, the spitting is commonly known as "pseudohemoptysis.

" Vomiting up blood, hematemesis, is one type of pseudohemoptysis.

Differentiating between hemoptysis and hematemesis is an integral part of diagnosis.

Since they involve entirely different parts of the human body, the prognoses (the prospect of recovery) and treatments are radically different.

Hemoptysis is defined as the spitting of blood or of blood-stained sputum.

While the definition is straightforward, verifying the presence of hemoptysis from the patient's history can be difficult.

Patients often have difficulty differentiating true hemoptysis from nonpulmonary sources of blood such as the oropharnx or gastrointestinal tract.

Some authors have designated the nonpulmonary expectoration of blood, pseudohemopysis, and list some helpful points to differentiate between the two

Clinical Features	Hemoptysis	Pseudohemoptysis
Origin of blood	Respiratory tract	Oral cavity, larynx, esophagus, stomach, factitious
Cough	More likely	Less likely
Respiratory symptoms	More likely	Less likely
Esophogastric symptoms	Less likely	More likely
Alcohol use, hepatic disease	Less likely	More likely
Vomiting, nausea	Less likely	More likely
Hematemesis and melena	Less likely	More likely
Color of expectorated blood	Bright red	Brown or black
Consistency of expectorant	Clotted or liquid	Coffee grounds appearance
Frothiness of expectorant	Usually	Never or seldom
pH of expectorant	Alkaline	Acidic
Alveolar Macrophages in sputum	Present	Absent
Food particles in expectorant	Absent	Present

Other pertinent elements of the history include:

smoking history,

prescence of "B" symptoms or other systemic complaints suggestive of cancer,

and the amount and duration of hemoptysis.

It can not be stressed enough that a thorough history and physical exam is imperative in the evaluation of hemoptysis.

A CBC, ABG, PT, PTT, electrolytes, serum creatinine, sputum for gram stain, culture, cytology, and an urinanlysis might be helpful. A recent CXR to evaluate for any obvious abnormalities is mandatory. Spirometry is usually not very helpful in the acute setting.

There is no consensus on when to admit patients with hemoptysis except in the case of life-threatening hemoptysis.

IN KIDS

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:

Mild	Less than 60 cc of blood lost for the whole episode.
Massive	More than 200 cc of blood lost in a 24 hour period.
Life-threatening	More 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.

Historical Investigation

A detailed history will often assist in deducing the etiology of hemoptysis in the pediatric patient and may be particularly valuable to differentiate true hemoptysis from non-pulmonary sources of blood, such as the mucosa of the oropharynx and gastro-intestinal tract. When obtaining a history consider the following details.

1. Timing and duration of the hemoptysis. It is important to differentiate acute from chronic onset of hemoptysis. A child with acute onset hemoptysis, may require immediate attention because of the potential for rapid deterioration if the problem is neglected. You must also determine the frequency at which the patient is bringing up blood in order to identify whether the hemoptysis is an isolated, episodic, or continuous event. This information may help you to gauge the amount of blood lost.

2. Triggers and pattern. It is not uncommon for children to have "swallowed" a foreign body, e.g. a piece of lego or a button, hence it is essential to ask the caregiver about this possibility. Focus on the time of day that the hemoptysis occurred and whether it was associated with particular activities such as eating, playing, exercise, or bouts of coughing.

3. Nature of the Sputum. The appearance of the blood/sputum can provide many important etiologic clues. If possible, have the patient or caregiver describe the expectorant. Focus particularly on the amount of blood in a 24 hour or 1 week period (teaspoon, tablespoon, or cupfull). Ask questions about the colour and appearance of the hemoptysis.

4. Differentiate between pulmonary and gastrointestinal sources of the blood. Hematemasis (vomited blood) can readily mimic hemoptysis and, as such, you should never overlook the gastrointestinal tract as a possible source of blood. If the blood has a "coffee ground" appearance consider this blood of GI origin.

5. Associated clinical findings. The classical picture of hemoptysis in association with chronic cough, sputum production, and fever strongly suggests an infectious cause. Other respiratory symptoms that may occur are shortness of breath, wheezing, and chest pain. Extra-pulmonary signs and symptoms that are sometimes associated with hemoptysis are palpitations, fever, bruising, nausea, vomiting, diarrhea, melena, or hematuria.

6. Past medical history. A number of topics may be addressed in this part of the history: everything from allergies and asthma to past surgery and endoscopy. You should inquire about whether the child has had any prior respiratory tract or systemic infections. Postinfectious inflammatory conditions of the respiratory tract are not uncommon sources of bleeding. Congenital abnormalities can also predispose to hemoptysis, especially in the younger pediatric patient. Assess the patient’s use of medications. Certain medications, particularly anticoagulants can predispose to secondary bleeding. This can be followed up during the physical exam portion of the investigation by assessing various organ systems for bleeding. It is also important for you to inquire about the child's family history, particularly with regard to bleeding disorders.

Physical Examination

Of particular importance is the measurement of blood pressure, heart rate, respiratory rate, temperature, and any signs of hypovolemia It may be helpful to continue with a ENT exam to look for bleeding from the nose, nasopharynx, tonsils, gums, and oropharynx. This will help rule-out non-pulmonary sources of bleeding.

In your examination of the respiratory system, identify any wheezing, airflow obstruction, localized or diffuse crackles (parenchymal bleeding), or pleural friction rubs. Remember not to forget the skin! Important diagnostic clues, such as petechiae (pinpoint capillary hemorrhages in skin or mucosa < 2mm), have often been missed when an examination of the skin was neglected.

_______________

Etiology HEMOPTYSIS

Cardiac	Pulmonary
Mitral stenosis	Bronchitis
Tricuspid endocarditis	Bronchiectasis
	Cystic fibrosis
Hematologic	Bullous emphysema
Coagulopathy
Platelet dysfunction	Iatrogenic
Thrombocytopenia	Bronchoscopy/ Lung Biopsy
Disseminated intravascular coagulation	Swan-Ganz catheterization
	Transtracheal aspirate
Infection	Lymphangiography
Lung abscess
Mycetoma	Vascular
Necrotizing pneumonia (Staph, Klebsiella, Legionella)	Pulmonary embolism
Parasitic (paragonimiasis, amebiasis, ascariasis, clonorchiasis, echonococciasis, hookworm, strongyloidiasis, trichinosis)	Pulmonary hypertension
Fungal (Aspergillus, Coccidiodes, Mucor, Madura, Histoplasma, Blastomyces)	Arteriovenous malformation
Viral (Influenza, varicella)	Aortic aneurysm
	Vascular prosthesis
Neoplastic
Bronchial adenoma	Drugs/Toxins
Lung cancer	Aspirin
Metastatic disease	Anticoagulation
	Penicillamine
Traumatic	Solvents
Aortic aneurysm	Trimellitic anhydride
Chest trauma	Crack cocaine
Ruptured bronchus
Fat embolism	Miscellaneous
Tracheal-innominate artery fistula	Amyloidosis
	Broncholithiasis
Systemic Disease	Bronchopleural fistula
Goodpasture's Syndrome	Endometriosis
Idiopathic Pulmonary hemosiderosis	Foreign body
Systemic lupus erythematosis	Cryptogenic hemoptysis
Vasculitis

IN AIDS PATIENTS

Diagnosis	Definite	Presumptive
Infectious	15	17
Pneumonia	13	15
Bacterial	3	13
M. tuberculosis	3	0
PCP	1	2
M. avium-intracellulare	3	0
Fungal	3	0
Bronchitis	0	1
Bronchiectasis	1	1
Endocarditis	1	0
Noninfectious	5	3
Kaposi's sarcoma	3	2
Pulmonary embolism	1	1
Lung Cancer	1	0
Total	20	20

Differential Diagnosis of Hemoptysis of Lower Respiratory Passages

Airways Disease

· Acute or chronic bronchitis

· Bronchiectasis

· Lung cancer

· Certain bronchial tumors (growths)

Airway disease is the most common cause with the bronchitis, bronchiectasis and bronchogenic carcinoma being the top causes of hemoptysis. Bronchial carcinoid is less common, but it also originates in the airways.

Parenchymal Disease

· Tuberculosis

· Lung abscess

· Pneumonia

· Mycetoma (fungus ball)

· Goodpasture’s syndrome

· Idiopathic pulmonary hemosiderosis

These causes of hemoptysis are frequently infections. Goodpasture’s and Idiopathic pulmonary hemosiderosis are rare.

Vascular Disease

· Pulmonary embolism

· Increased pressure in the blood vessels of the lungs

Left ventricle failure (Heart failure)

Mitral stenosis

· Vascular malformation

Hemoptysis related vascular lesions are problems with the circulation through the lungs. Pulmonary embolism (obstruction of a pulmonary artery or one of its branches) causes elevated pressures in the pulmonary arteries and can cause death of the lung tissue. Pulmonary edema can cause frothy pink or red-tinged sputum.

Pulmonary venous pressure can be elevated with mitral stenosis (narrowed (diseased) heart valve).

Diagnosing the cause of Hemoptysis

Patients should notify their doctor immediately if they are coughing up blood[believe me they will since the view of the blood is scarry meaning a big thing is going on].

The doctor will want to perform a thorough exam and review medications that the patient is on to determine if any may cause bleeding.

Obtain a chest x-ray and or a CAT Scan, and lab work.

A bronchoscopy may also be done to see where the bleeding is coming from.

The physician is able to view the airways of the lungs during this procedure and obtain biopsies and cultures.

Differences between hemoptysis & hematemesis - [clinic always clinic]

Hemoptysis -

Prodrome of irritation / discomfort in chest & urge to cough.

Blood is coughed out.

Blood is bright red & frothy.

Hematemesis -

Prodrome of nausea, urge to vomit out.

Blood is vomited out.

Blood is an altered red / brown colour - may contain food particles, not frothy as in hemoptysis

Conditions NOT associated with hemoptysis -

Miliary TB

Metastatic Ca to the lung

Viral / pneumococcal pneumonias - the hemoptysis if present is in form of scanty blood streaked sputum.

Clinical conditions with hemoptysis & the probable diagnosis -

Young, otherwise healthy female with chronic hemoptysis - Bronchial adenoma

Recurrent hemoptysis with marked sputum production - Bronchiectasis, chronic bronchitis.

Weight loss, anorexia, hemoptysis in a male smoker > 40 years age - Ca bronchus

H/O blunt trauma - lung contusion

Acute pleuritic chest pain with hemoptysis -

Pulmonary embolism (recurrent) with infarction.

Lung abscess

Fungal cavity

Vasculitis

Localisation of site of bleeding in patient with minimal other findings -

Patient tends to lie with affected side dependent - to prevent aspiration by other lung & blood pneumonitis. Blood pneumonitis may cause confusion on CXR - clears within 1 week.

H/O burning / deep pain - may localise the site of bleeding.

Bronchoscopy - helps to diagnose site of bleeding & prevents aspiration by the normal lung - prevents blood pneumonitis.

Indications for bronchography - very few -

Establish the presence of localised bronchiectasis (sequestered lobe) - surgery may be done.

Rule out generalised bronchiectasis in a patient with suspected localised disease who is being considered for surgery.

Treatment - the basic

Treat the basic cause.

The hemoptysis generally will stop spontaneously & no treatment apart from reassurance is needed.

General treatment -

Keep patient calm.

Complete bed rest

Suppress cough if it is troublesome & is aggravating the hemoptysis.

1st achieve stoppage of bleeding - then do the other investigations - especially the invasive respiratory investigations.

Life threatening hemoptysis -

General treatment as above.

Prevent aspiration of blood & loss of function of the normal lung by intubation which will isolate the normal lung - balloon / cuffed tube may be introduced through a bronchoscope.

Ensure adequate ventilation.

Ensure circulation by transfusion IV fluids, colloids, & blood if needed - monitor the BP & pulse.

Once patient stabilised - CXR - depending on this definitive therapy started.

Surgery indicated in -

Localised bronchiectasis - sequestered lobe

Cavitary disease - any cause

Lung abscess

Resectable neoplasm

Bronchoscopy done immediately in surgical patients. In patients to be treated conservatively - bronchoscopy deferred until after a week - itself can aggravate the hemoptysis.

Other modalities of treatment -

Arterial catheterization & selective embolisation of bleeding vessels.

Laser Nd - YAG coagulation through bronchoscope

These are useful especially in advanced lung Ca for palliation.

Anatomy

It is potentially important for the management of hemoptysis, especially life threatening hemoptysis,

to know the source of bleeding.

As different etiologies may have different sources of bleeding, a basic knowledge of the blood supply to the lungs is helpful.

The lungs are supplied by two independant circulations.

The low pressure pulmonary circulation interacts with the airway only at the level of the terminal bronchiole and actively participates in gas exchange.

The bronchial circulation, which usually arises from the aorta and is therefore under systemic pressure, supplies nutrients to the lung. It runs the entire length of the bronchial tree and forms an extensive submucosal bronchial plexus.

In many different disease states which cause airway inflammation, the bronchial arteries can become dilated and tortuous.

Any further insults, such as infection or inflammation, can cause bleeding form these abnormal vessels which may be massive.

This scenerio is probably most common in patients with bronchiectasis and massive hemoptysis.

Classifying hemoptysis

Massive hemoptysis

Hemoptysis is considered massive, or major, when there is so much blood that it interrupts a person’s breathing (generally more than about 200-240 ml, or about * cup, in 24 hours).

Massive hemoptysis is considered a medical emergency:

the mortality rate for patients with massive hemoptysis can be as high as 75%.

Most patients who die from hemoptysis suffer from asphyxiation due to too much blood in the airways.

Mild hemoptysis

If there is only a small amount of blood or whitish yellow sputum streaked with blood, the spitting is considered mild hemoptysis.

In 60% to 70% of all mild hemoptysis cases, the underlying disorder is fairly benign and disappears on its own without causing any serious problems or permanent damage.

But even in cases of mild hemoptysis, a patient may suffer critical breathing problems, depending on the underlying cause of the bleeding.

Additionally, hemoptysis tends to occur intermittently and recur sporadically, and there is no way to predict if patients with mild hemoptysis are at risk for a massive hemoptysis episode in the future.

So diagnosing mild episodes is just as important as diagnosing massive ones, so as to not overlook situations that could potentially erupt into something more serious in the future.

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.

PULMONARY HEMORRHAGE IN THE NEONATAL PERIOD

Frequently fatal, predominantly affects premature infants and associated with severe systemic illness.

In intubated neonates, airway trauma from tube itself or from suction catheters is often source of bleeding.

Usually this is mild and self limited, but cases of severe airway bleeding have been reported.

In a series of 12 cases of fatal massive pulmonary hemorrhage in neonates left ventricular failure, secondary to congenital lesions, severe RDS, sepsis or other causes, was the precipitating factor.

Blood actually represents severe pulmonary edema with extravasation of red cells into airspaces.

If hematocrit and protein content of blood within airspaces is compared with that seen in peripheral blood, composition is different.

Kernicteurs, severe intracranial hemorrhage and hypothermia have also been associated as leading to pulmonary hemorrhage.

Mechanism is felt to be secondary to severe hypoxia and increased left ventricular end-diastolic pressure.

Bacterial pneumonia and sepsis with DIC can also trigger massive pulmonary hemorrhage from direct tissue injury and leaking of blood into alveolar spaces.

Hyperammonemia can induce intrapulmonary bleeding that is felt to be related to direct toxicity on the pulmonary vascular endothelium.

Congenital malformations, including vascular lesions, will rarely present with pulmonary hemorrhage in neonatal period.

PULMONARY HEMORRHAGE IN INFANCY

Multiplicity of causes, most will represent bleeding from a primary lung problem.

Pulmonary hemosiderosis (PH): Probably the most common cause in this age group, however no good epidemiologic data available. A study in Sweden estimated incidence of pulmonary hemosiderosis at 0.24 cases per million population.

PH is a clinical syndrome and not a specific disease. Characteristic triad of iron deficiency anemia, chronic or recurrent respiratory symptoms, and patchy infiltrates on chest radiograms during symptomatic episodes. In infants Heiner's syndrome is the most common cause, idiopathic PH is seen less commonly. Other causes of PH are rarely the reason for PH in infants.

Heiner's Syndrome (PH with sensitivity to cow's milk):

Usual presentation is by age 6 months, but first episode has occurred in children as old as 9 years.

History of recurring respiratory symptoms, mostly cough and tachypnea, with or without fever.

Laboratory investigations will reveal iron deficiency anemia and radiographic abnormalities.

Chest radiograms are usually clear between episodes, and infiltrates clear fast (days to weeks).

Diagnosis based on presence of milk precipitins in serum and demonstration of hemosiderin laden macrophages, which are only seen after lung parenchymal bleed.

Since infants swallow respiratory secretions, macrophages of lung origin can be found in gastric aspirate.

Bronchoscopy with bronchoalveolar lavage is definitive diagnostic procedure, hemosiderin laden alveolar macrophages in lavage fluid can be easily demonstrated.

However, these children may be seen primarily because of anemia and will have stool guaiacs performed, which will be positive, and be wrongly diagnosed as having GI bleed.

High index of suspicion is crucial to make correct diagnosis.

After a diagnosis of Heiner's syndrome is established, treatment is based on milk products avoidance, oral corticosteroids and close follow up.

Relapses are usually related to inadvertent ingestion of foods with some content of milk protein, and this should be clearly established if there is a relapse.

Prognosis is usually good if relapses do not occur. In some cases disappearance of milk sensitivity may occurre as the child gets older, however any re-challenge should be performed under close supervision.

Idiopathic PH (IPH):

In those infants on whom milk precipitins in serum are not found and on whom no other cause for bleeding can be found, diagnosis should be that of Idiopathic PH.

Few cases have been reported where an IgA or IgG subclass 4 deficiency was detected,

however the possible etiologic role of this is not clear.

Since precipitins are neither specific nor sensitive, it is worth to have these patients go through a trial of milk products-free diet.

Treatment is similar to that followed by patients with Heiner's syndrome.

It is not unusual to see that relapses are related to viral infections, so the family must be instructed to watch closely the child during any respiratory infections.

Patients who fail to respond to corticosteroids can be tried on immunesuppresors such as cyclophosphamide and azathioprine.

IVIG has also been tried with variable success.

Prognosis is variable, usually good for those who show response to therapy.

Most patients will experience recurrence of pulmonary bleeds and some will die during severe episodes. In addition, patients with multiple bleeds will develop lung scarring and fibrosis which will impair their lung function.

Close follow-up and periodic reevaluation are important, since there have been cases of other autoimmune disorders that have presented early in life as IPH as their only manifestation for many years.

Congenital malformations:

Less common cause of pulmonary bleeding in infants.

Bronchogenic cysts, particularly when infected, can be the source of large bleeding.

Thoracic gastroenteric cysts have gastric mucosa and the ability to secerete acid, for this reason the cyst wall can be eroded and the erosion can extend to adjacent bronchial walls, vessels or lung parenchyma, resulting in massive bleeding.

Congenital airway hemangiomas:

Usually will become symptomatic by age 6 months.

However, the symptomatology is mostly related to airway obstruction, but cases of massive bleeding from an airway hemangioma have been reported.

Primary pulmonary malignancies or metastatic lesions: Rarely the reason for pulmonary bleeding in infants.

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.

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.

In the 20%-30% of all cases that don’t have an indentifiable underlying cause, treatment should be fairly conservative and the hemoptysis carefully monitored for at least 2 or 3 years after the initial diagnosis. In 90% of all patients who have a normal chest x-ray and bronchoscopy, the hemoptysis usually disappears within 6 months.

For chronic hemoptysis, the treatment is largely dependent on the symptoms and what is causing it. Sometimes all that’s necessary is switching antibiotics. In other instances, more aggressive treatment may be necessary.

Bronchial artery embolization involves injecting substances into the bloodstream that can stop blood flow. It is a proven technique for stopping life-threatening massive hemoptysis and can have some beneficial long-term effects as well, although it is not always successful and is not without complications.

Surgical resection is the surgical removal of the abnormal tissue responsible for the hemoptysis. It is often recommended as an early treatment for hemoptysis caused by aspergilloma (see Apergilloma).

Bronchoscopic laser therapy involves using laser therapy during a bronchoscopy to remove tumors and lesions or widen airways.

All of these techniques – bronchial artery embolization, surgical resection, and bronchoscopic laser therapy -- have proved beneficial in controlling or curing hemoptysis due to bronchiectasis or other inflammatory disorders.

The pros and cons of the various techniques depend on the skill of the doctor performing the procedure and the availability of the necessary equipment.

The patient should always discuss thoroughly the pros and cons of the various procedures with their primary physician, thoracic surgeon, and radiologist.

Treat the basic cause.

The hemoptysis generally will stop spontaneously & no treatment apart from reassurance is needed.

General treatment -

Keep patient calm.

Complete bed rest

Suppress cough if it is troublesome & is aggravating the hemoptysis.

1st achieve stoppage of bleeding - then do the other investigations - especially the invasive respiratory investigations.

Life threatening hemoptysis -

General treatment as above.

Prevent aspiration of blood & loss of function of the normal lung by intubation which will isolate the normal lung - balloon / cuffed tube may be introduced through a bronchoscope.

Ensure adequate ventilation.

Ensure circulation by transfusion IV fluids, colloids, & blood if needed - monitor the BP & pulse.

Once patient stabilised - CXR - depending on this definitive therapy started.

Surgery indicated in -

Localised bronchiectasis - sequestered lobe

Cavitary disease - any cause

Lung abscess

Resectable neoplasm

Bronchoscopy done immediately in surgical patients. In patients to be treated conservatively - bronchoscopy deferred until after a week - itself can aggravate the hemoptysis.

Other modalities of treatment -

Arterial catheterization & selective embolisation of bleeding vessels.

Laser Nd - YAG coagulation through bronchoscope

These are useful especially in advanced lung Ca for palliation.