Hemoptysis in children

INTRODUCTION — Hemoptysis is the expectoration of blood or the presence of blood in the sputum. Young children usually swallow their sputum; as a result, hemoptysis is rare in children unless the bleeding is substantial [1].

DEFINITIONS

●Hemoptysis – Hemoptysis refers to expectoration of blood originating from the lower respiratory tract. Blood from the upper respiratory tract, nasopharynx, and upper gastrointestinal tract can be expectorated and can mimic true hemoptysis. (See 'Mimics of hemoptysis' below.)

●Hemoptysis severity – There is no consensus for quantifying the degree of hemoptysis. Although management depends, in part, on an estimate of the volume of hemoptysis, it is also influenced by individual patient characteristics and clinical judgement [2,3].

In this topic review, we will use the following categories to categorize the severity of hemoptysis:

•Scant – Less than 5 mL (eg, streaks of blood).

•Mild to moderate – ≥5 to approximately 200 mL within 24 hours; no hemodynamic instability or impaired gas exchange.

•Life-threatening or massive – More than approximately 200 mL within 24 hours and/or evidence of hemodynamic instability (tachycardia, hypotension), abnormal gas exchange, difficulty maintaining a patent airway, or very brisk bleeding.

The volume estimates noted above are partly based on a consensus guideline developed for hemoptysis in patients with cystic fibrosis (CF), which has been one of the most common underlying etiologies of hemoptysis in pediatric patients [4]. Various thresholds have been used to define life-threatening or massive hemoptysis; we use a threshold of approximately 200 mL, which is slightly below the threshold proposed by the CF consensus guideline (240 mL) but within the wide range proposed for defining this category for other conditions in adults (see "Evaluation and management of life-threatening hemoptysis"). Volume estimates defining massive hemoptysis in smaller children are lacking. In any case, these thresholds are approximate and are only one component of the clinical assessment of the patient. It should be recognized that estimates of hemoptysis volume are often imprecise, do not account for the size of the patient, and are only weakly correlated with the cause of hemoptysis or with clinical outcomes.

PATHOGENESIS — Bleeding can arise from either of the lung's two separate blood supplies:

●Bronchial circulation – Hemoptysis most commonly arises from the bronchial circulation, including in hemoptysis due to bronchiectasis (eg, in cystic fibrosis [CF]), invasive infections (eg, tuberculosis, necrotizing pneumonia), pulmonary vascular disorders, and trauma. This circulation is at high (systemic) pressures, and, thus, the associated bleeding tends to be substantial. It carries a much smaller volume of oxygenated blood, an estimated 1 percent of the cardiac output in a normal individual at rest [5].

Normally, there are three recognizable bronchial arteries: two that supply the left lung and one that supplies the right, although 20 to 30 percent of individuals have two vessels on each side [6]. These arteries typically originate from the aorta or the intercostal arteries and perfuse conducting airways approximately to the level of the terminal bronchioles. More distally, the airway structures are nourished by the pulmonary artery circulation.

●Pulmonary artery circulation – Hemoptysis from the pulmonary artery circulation is uncommon, accounting for 10 percent or fewer of the cases of hemoptysis in children [7]. This is the circulation involved in hemoptysis in individuals with left-sided cardiac disease, severe pulmonary arterial hypertension, pulmonary capillaritis, or arteriovenous malformations. The pulmonary artery circulation is a low-pressure system; maximal pulmonary artery pressure normally does not exceed 40 mmHg [8]. Because of the low pressure, bleeding arising from this system tends to be relatively slow or insidious.

The branches of this system accompany the bronchi down to the level of the terminal bronchioles. Ultimately, pulmonary vessels branch to supply the capillary bed in the walls of the alveoli and, then, blood returns to the left atrium via the pulmonary veins.

CAUSES OF HEMOPTYSIS — The causes of hemoptysis in children are outlined in the table (table 1) [9-12]. In children, respiratory infection, aspirated foreign bodies, and bronchiectasis (eg, due to cystic fibrosis [CF]) are the most common etiologies.

Respiratory infection — Infection is the most common cause of hemoptysis in children without underlying pulmonary, cardiac, hematologic, or neoplastic disease.

Infections that are particularly likely to cause hemoptysis are tuberculosis, aspergilloma, and acute endemic mycoses. However, most children with these infections do not have hemoptysis, because cavitary disease is uncommon in younger age groups [13,14]. (See "Pulmonary tuberculosis: Clinical manifestations and complications" and "Epidemiology and clinical manifestations of invasive aspergillosis" and "Chronic pulmonary aspergillosis: Epidemiology, clinical manifestations and diagnosis".)

In addition, pulmonary infections with relatively common community-acquired bacteria such as Staphylococcus, Streptococcus, Klebsiella, and Pseudomonas can cause hemoptysis, particularly if associated with necrotizing pneumonia or lung abscess [15,16]. Influenza virus, including H1N1 influenza A, also may present with significant bleeding [17-19]. In addition, alveolar hemorrhage has been reported as a complication of pneumonia due to coronavirus disease 2019 (COVID-19), presumably related to the immunothrombosis pathology [20,21].

Finally, minor hemoptysis (blood-streaked sputum) can occur with any acute respiratory infection that causes forceful coughing, due to mechanical trauma to the airway. This type of hemoptysis is usually benign and self-limited.

Foreign body aspiration — Foreign body aspiration is common in children, especially in those under three years of age. In a substantial majority of cases, the aspiration is not initially recognized within the first 24 hours of the event and the child may present days or weeks after the aspiration event with wheezing (usually monophonic and perhaps unilateral), chronic cough, pneumonia, or hemoptysis [22]. A history of choking is highly suggestive of foreign body aspiration, but the event may not be recalled at the time of presentation. (See "Airway foreign bodies in children".)

Cystic fibrosis-related hemoptysis

●Bronchiectasis – CF-associated bronchiectasis has been a common cause of hemoptysis in children and adolescents. In a series of 228 patients admitted to a tertiary center with hemoptysis, 65 percent had CF [23]. Children and young adults with CF frequently experience small-volume hemoptysis with blood-streaked sputum, particularly during pulmonary exacerbations. Historically, up to 60 percent of patients older than 18 years of age with CF have blood-streaked sputum in intermittent episodes and 4.1 percent developed large-volume or massive hemoptysis during their lifetime [4]. The risk for hemoptysis will likely decrease in the future with the use of highly effective modulator therapies for CF [24]. (See "Cystic fibrosis: Management of advanced lung disease" and "Cystic fibrosis: Treatment with CFTR modulators".)

In patients with CF, chronic airway infection and inflammation damages the airway, stimulates neovascularization, and promotes growth of dilated and fragile bronchial arteries. These vessels lie close to the inflamed mucosal surface, increasing the risk of bleeding with cough or increased infection. (See "Clinical manifestations and diagnosis of bronchiectasis in adults" and "Bronchiectasis in children: Pathophysiology and causes", section on 'Pathophysiology'.)

●Other causes in CF – In patients with CF, coagulopathy secondary to vitamin K deficiency caused by fat malabsorption may exacerbate hemoptysis. Esophageal varices due to hepatic cirrhosis are also important causes of bleeding, which can mimic hemoptysis. (See "Cystic fibrosis: Overview of the treatment of lung disease", section on 'Hemoptysis'.)

Bronchiectasis unrelated to CF (non-CF bronchiectasis) — Bronchiectasis occasionally develops in children without CF. In resource-limited countries and some indigenous populations, chronic and/or undertreated pulmonary infection remains a common cause of bronchiectasis. Bronchiectasis also may be related to immunodeficiency, impaired mucociliary clearance, chronic aspiration, or a variety of congenital or acquired lung disorders. (See "Bronchiectasis in children: Pathophysiology and causes".)

The risk for hemoptysis in children with progressive and advanced non-CF bronchiectasis is similar to that in CF bronchiectasis. (See 'Cystic fibrosis-related hemoptysis' above.)

Cardiac disease

●Congenital heart disease (CHD) – Hemoptysis is a known complication in patients with CHD. In a report from a pediatric tertiary care center, CHD was responsible for 25 percent of cases of hemoptysis [23]. Underlying defects that lead to hemoptysis include pulmonary venous obstructive disease, pulmonary hypertension (specifically, postcapillary hypertension), and left-sided cardiac obstructive lesions such as mitral stenosis and left ventricular diastolic dysfunction.

Hemoptysis associated with CHD has become less common with the evolution of early corrective cardiac surgery. However, there are case reports of children presenting with massive hemoptysis many years after undergoing a Fontan procedure for hypoplastic left heart syndrome or tricuspid valve atresia; the hemorrhage is from collateral neovascularization (aortopulmonary collaterals [APCs]) [25,26]. (See "Management of complications in patients with Fontan circulation", section on 'Bleeding'.)

●Other – Other cardiac-related causes of hemoptysis include congestive heart failure with pulmonary edema and high-altitude pulmonary edema. Pulmonary edema may present with pink, frothy sputum and, occasionally, with frank blood. (See "High-altitude pulmonary edema".)

Pulmonary vascular disorders — In the following disorders, the primary pathology is intrinsic to the pulmonary vasculature or affects the pressure within these vessels.

●Arteriovenous malformations – Hemoptysis may be a consequence of pulmonary arteriovenous malformations (PAVMs), either with or without underlying hereditary hemorrhagic telangiectasia (also known as Osler-Weber-Rendu syndrome). In a small series of infants with hemoptysis, aortopulmonary anastomoses were identified during cardiac catheterization and embolization of these abnormal anastomoses stopped the hemoptysis [27]. (See "Pulmonary arteriovenous malformations: Epidemiology, etiology, and pathology in adults" and "Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)".)

●Pulmonary hypertension – Hemoptysis is uncommonly seen in patients with pulmonary hypertension and is a sign of advanced, life-threatening disease. It may be minor, episodic, or massive [28]. (See "Pulmonary hypertension in children: Management and prognosis".)

●Pulmonary embolism – Pulmonary embolism may present with dyspnea, pleuritic pain, cough, and (rarely) with hemoptysis. Pulmonary embolism is uncommon in children younger than 15 years of age (incidence 0.2 cases per 100,000 children/year). Most children with pulmonary embolism have known risk factors [29,30]. (See "Venous thrombosis and thromboembolism (VTE) in children: Risk factors, clinical manifestations, and diagnosis", section on 'Pulmonary embolism'.)

Trauma

●Endotracheal tube or tracheostomy – In children with endotracheal or tracheostomy tubes, suctioning beyond the tracheal opening of the tube can lead to mucosal injury of central airways, resulting in bloody sputum. This usually only causes scant bleeding and is not clinically important. Use of humidification, soft suction catheters, and suction to a measured length to ensure that it does not exceed the length of the tube may help prevent bleeding or allow the injured mucosa to heal. Similarly, children with a chronic tracheostomy may develop granulation tissue, which also can cause minor hemoptysis.

In approximately 10 percent of such children, the bleeding is more than scant; bronchoscopy findings may include mucosal abrasions or granulation tissue.

Rarely, massive hemoptysis can occur due to an airway tube eroding into a large vessel in the mediastinum or tracheal wall; several case reports describe presentations with episodes of non-massive “sentinel” bleeding [31-33]. For patients with a tracheostomy, brisk bleeding should be urgently evaluated by a surgical specialist, typically with fiberoptic endoscopy.

●Transbronchial biopsy – In patients undergoing transbronchial biopsy, bleeding occurs in between 0.26 and 5.0 percent of cases and seldom is massive, based on studies in adults [3].

●Chest trauma – Chest trauma can cause hemoptysis because of direct trauma to the airway and/or a lung contusion. (See "Overview of intrathoracic injuries in children" and "Chest wall injuries after blunt trauma in children".)

Causes of diffuse alveolar hemorrhage — Causes of bleeding originating from the pulmonary parenchyma fall into several major categories:

●Coagulopathy – Any coagulopathy can predispose to hemoptysis, such as von Willebrand disease [34], thrombocytopenia, or treatment with anticoagulants. Because these are not inflammatory disorders, this is sometimes termed "bland" pulmonary hemorrhage. (See "Approach to the child with bleeding symptoms".)

●Pulmonary capillaritis – Pulmonary capillaritis is characterized by neutrophilic invasion and fibrinoid necrosis of the alveolar capillary walls. It may be isolated or part of a systemic vasculitis such as microscopic polyangiitis, granulomatosis with polyangiitis, anti-glomerular basement membrane disease (Goodpasture), systemic lupus erythematosus, immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura) [35-37], IgA nephropathy, or antiphospholipid antibody syndrome. Capillaritis is also associated with certain medications, including phenytoin, retinoic acid, and propylthiouracil. (See "Childhood-onset systemic lupus erythematosus (SLE): Clinical manifestations and diagnosis", section on 'Pulmonary' and "Granulomatosis with polyangiitis and microscopic polyangiitis: Clinical manifestations and diagnosis" and "The diffuse alveolar hemorrhage syndromes".)

Pulmonary capillaritis is probably an underrecognized cause of diffuse alveolar hemorrhage in children [38,39]. Lung biopsy is required for diagnosis, and experienced and expert pathologic evaluation may be needed to identify the correct diagnosis.

●Pulmonary-renal syndrome – This is the syndrome of diffuse alveolar hemorrhage and glomerulonephritis that may occur with a variety of vasculitic diseases including anti-glomerular basement membrane disease, granulomatosis with polyangiitis, microscopic polyangiitis, and systemic lupus erythematosus. (See "Anti-GBM (Goodpasture) disease: Pathogenesis, clinical manifestations, and diagnosis" and "Vasculitis in children: Evaluation overview".)

●Down syndrome – Children with Down syndrome are at increased risk for diffuse alveolar hemorrhage due to underlying risk factors that may include associated congenital cardiopulmonary abnormalities, pulmonary artery hypertension, pulmonary capillaritis, infection and aspiration, and/or immune dysfunction [40].

●Idiopathic pulmonary hemosiderosis – Idiopathic pulmonary hemosiderosis is a rare disease found primarily in children. It typically presents with recurrent dyspnea, cough, and anemia. Hemoptysis usually develops later but occasionally is a presenting symptom, and the hemoptysis may not be evident in smaller children. Clinical characteristics include hemosiderin-laden macrophages in bronchoalveolar lavage fluid and no evidence of capillaritis or other cause of bleeding on lung biopsy [41]. (See "Idiopathic pulmonary hemosiderosis".)

●Acute idiopathic pulmonary hemorrhage of infancy (AIPHI) – AIPHI is defined as an onset of pulmonary hemorrhage in a previously healthy infant less than one year of age, in whom no other causes for bleeding can be found [42]. Affected infants present with hemoptysis or blood in the nose or upper airway with no evidence of upper respiratory or gastrointestinal bleeding [43]. In some reported cases, the infants required mechanical ventilation but recovered completely [44].

Among infants presenting with pulmonary hemorrhage, only a few will ultimately be diagnosed with AIPHI. In a case series of 157 infants presenting with acute pulmonary hemorrhage, an underlying cause could be identified in the vast majority after a thorough evaluation [45]. The most common causes were CHD or prematurity (each approximately 30 percent) and congenital or acquired lung disorders or coagulopathies (each approximately 15 percent); AIPHI was identified in less than 1 percent.

The cause(s) and risk factors for AIHPI have not been established. One report suggests that von Willebrand disease caused or contributed to pulmonary hemorrhage in several cases that were initially diagnosed as AIPHI [34]. A few case reports raised the possibility of an association with exposure to Stachybotrys chartarum (also known as Stachybotrys atra) toxin, but further epidemiologic data have not supported such an association [42,46,47].

●Miscellaneous – Miscellaneous causes of diffuse alveolar hemorrhage include:

•Hematopoietic stem cell transplant (presenting with hemoptysis during the first months after transplant). (See "Pulmonary complications after autologous hematopoietic cell transplantation", section on 'Diffuse alveolar hemorrhage'.)

•Pulmonary veno-occlusive disease.

•Catamenial hemoptysis (recurrent hemoptysis that coincides with menses due to intrathoracic endometriosis).

•Celiac disease associated with pulmonary hemosiderosis (this has been termed "Lane-Hamilton syndrome" and appears to be rare [48,49]).

•Medications (eg, phenytoin, retinoic acid, amiodarone, propylthiouracil, or penicillamine).

•E-cigarette or vaping product use-associated lung injury [50]. (See "E-cigarette or vaping product use-associated lung injury (EVALI)".)

•Cocaine-induced pulmonary hemorrhage. (See "Pulmonary complications of cocaine use".)

•Other inhalational injuries (nitrogen dioxide, pesticides). Case reports describe hemoptysis due to inhalation of nitrogen dioxide in ice arenas with malfunctioning ice resurfacing and ventilation equipment [51,52].

(See "Etiology of hemoptysis in adults", section on 'Miscellaneous'.)

Tumors — Occasionally, hemoptysis is caused by tracheobronchial tumors, such as papillomatosis or adenoma, carcinoid, or mucoepidermoid tumor. Parenchymal tumors are even more rare; they may be metastatic or primary pulmonary tumors.

MIMICS OF HEMOPTYSIS — Hemoptysis specifically refers to expectoration of blood originating from the lower respiratory tract. Bleeding from the upper respiratory tract, oropharynx or nasopharynx, or upper gastrointestinal tract (eg, from esophageal varices due to cirrhosis) can mimic true hemoptysis.

Factitious hemoptysis, where children have inflicted injuries mimicking pulmonary bleeding, has been described [53]. Munchausen syndrome (or Munchausen syndrome by proxy) should be considered in a patient who presents with hemoptysis, unusual symptoms, and a negative evaluation [54]. (See "Factitious disorder imposed on self (Munchausen syndrome)" and "Medical child abuse (Munchausen syndrome by proxy)".)

INITIAL ASSESSMENT AND CONTROL OF BLEEDING

Assess severity and source

●Severity – The quantity and appearance of the bloody sputum should be assessed by direct observation, if possible, as well as thorough questioning of the patient and caretakers. The severity is categorized (scant, mild/moderate, or life-threatening), based on a global clinical assessment that includes hemodynamic stability, estimated blood volume lost, and rapidity of bleeding, recognizing that any volume estimate is imprecise. (See 'Definitions' above.)

●Source of bleeding – The initial assessment should attempt to localize the bleeding source, based on the following characteristics:

•Lower respiratory tract – Blood from the lower respiratory tract (true hemoptysis) usually is bright red or rust-colored, and it can be frothy and mixed with sputum. The pH usually is alkaline. Symptoms include coughing or a gurgling noise perceived by the patient. Older children may report discomfort in one hemithorax that may help localize the bleeding site. Frothy sputum suggests an alveolar source of bleeding.

•Upper respiratory tract/nasopharyngeal – Bleeding from the nasopharynx, including epistaxis, can mimic hemoptysis if the blood is expectorated. Epistaxis is more likely to be occult if the source is in the posterior rather than anterior nares and/or if it occurs when the patient is supine. In all patients, the possibility of nasopharyngeal bleeding should be explored by a careful inspection of the nares and oropharynx during the physical examination; consultation with a specialist (eg, otorhinolaryngology) and/or nasopharyngoscopy also may be helpful.

•Gastrointestinal – Blood from a gastric source (hematemesis) usually is crimson or brown with a coffee-ground appearance and may contain food particles. The pH is acidic, and nausea or retching may precede the episode.

The above characteristics are helpful, but they are not reliable indicators of the source of the bleeding. In some patients, gastrointestinal endoscopy or nasopharyngeal fiberoptic endoscopy may be needed to exclude gastrointestinal or nasopharyngeal sources of bleeding. In complex cases, it may be prudent to arrange for bronchoscopy, esophagogastroduodenoscopy, and nasopharyngoscopy under a single anesthetic. As an example, patients with advanced cystic fibrosis (CF) may be at risk for bleeding from either bronchiectasis or esophageal varices. (See 'Mimics of hemoptysis' above.)

Control of life-threatening hemoptysis — For patients with life-threatening (massive) hemoptysis, the first steps are to stabilize the patient and prevent further bleeding, as outlined below and summarized in the algorithm (algorithm 1). In these patients, evaluation for the cause of the hemoptysis may be performed concurrently with stabilization or as a second step. (See 'Initial assessment and control of bleeding' above.)

General measures — Measures to stabilize a patient with life-threatening (massive) hemoptysis include:

●Mechanical ventilation – Intubate and initiate mechanical ventilation for all patients who are hypoxic or showing signs of respiratory distress. With massive hemoptysis, intubation can be very difficult as the airway landmarks are often obscured. Therefore, anesthesia expertise and availability of advanced airway equipment are important. Mechanical ventilation with relatively high positive end-expiratory pressure (PEEP), eg mean PEEP >10 cm H₂O, may improve oxygenation. In theory, it might also tamponade the site of hemorrhage for venous or capillary bleeding. We generally initiate ventilation with a moderate level of PEEP (eg, 6 to 8 cm H₂0), then titrate up if tolerated, based on the patient's oxygenation. Selective intubation to the unaffected lung may help decrease the risk of asphyxiation and assist in ventilation. When the site of bleeding is unknown, double-lumen tubes may be helpful, if available for the child's size [55].

Noninvasive ventilation such as bilevel positive airway pressure should not be initiated for patients with massive hemoptysis, due to the risk of further aspiration of blood, emesis from swallowing blood, and the need for a more stable airway and access to identify the source of the bleeding. This guidance is outlined in a consensus guideline for patients with CF [4].

●Circulatory support – Provide circulatory support as needed.

●Coagulation – For all patients, assess for and treat coagulopathy if present (eg, transfuse platelets for thrombocytopenia or fresh frozen plasma for coagulopathy) and/or reverse any anticoagulant. (See 'Interventions to improve hemostasis' below.)

●Positioning – If the hemithorax in which the bleeding originates can be identified (either by chest radiograph or physical findings), position the patient with the bleeding side down to prevent blood from spilling over into the unaffected lung.

●Medications – Discontinue medications that may affect clotting, including nonsteroidal antiinflammatory drugs [4]. Patients with CF should be treated with systemic antibiotics and treatment with inhaled hypertonic saline should be stopped or avoided (table 2), as recommended in a consensus guideline [4].

●Other – To prevent worsening of hemoptysis, discontinue chest physiotherapy and suppress cough with opiates.

Therapeutic interventions — If the large-volume bleeding does not stop with the above measures, there are two options:

●Bronchoscopy – In addition to identifying a site of bleeding, performing bronchoscopy early in the evaluation of hemoptysis may provide options for therapeutic benefit. (See 'Therapeutic bronchoscopy' below.)

●Bronchial artery embolization (BAE) – Pulmonary arteriography can be used to localize the bleeding site and potentially treat it by performing BAE. (See 'Bronchial artery embolization' below.)

The decision to perform either of these procedures and the choice between them is complex and depends on the underlying condition, clinical stability of the patient, and availability of appropriately trained proceduralists. The decision should be individualized and made collaboratively between the critical care team, pediatric pulmonologist, interventional radiologist, and other consulting services.

Some examples of considerations based on the presentation and underlying disease include:

●Patients with CF – BAE is an important tool for managing life-threatening hemoptysis and should be implemented promptly, as suggested by a consensus guideline [4]. Several case series suggest that BAE is successful for initial control of bleeding in 75 to 90 percent of cases, and up to 50 percent require additional BAE procedures [56-61]. For patients with CF who are clinically stable, bronchoscopy usually is performed first to evaluate and control active bleeding.

Management of hemoptysis in patients with CF is discussed in greater detail separately. (See "Cystic fibrosis: Overview of the treatment of lung disease", section on 'Hemoptysis'.)

●Patients with suspected foreign body aspiration – Rigid bronchoscopy is the intervention of choice in this setting, as discussed separately; the procedure is performed in an operating room for optimal safety and airway management. (See "Airway foreign bodies in children", section on 'Foreign body removal'.)

●Patients with an apparent focal source of bleeding:

•Pulmonary arteriovenous malformations (PAVMs) and aortopulmonary collaterals (APCs) – BAE is generally the preferred procedure for patients presenting with life-threatening hemoptysis due to known underlying PAVMs or APCs. In children, PAVMs most commonly occur in patients with hereditary hemorrhagic telangiectasia (also called Osler-Weber-Rendu syndrome). APCs can occur in certain forms of congenital heart disease (CHD). Management of PAVMs is discussed separately. (See 'Bronchial artery embolization' below and "Hereditary hemorrhagic telangiectasia (HHT): Evaluation and therapy for specific vascular lesions", section on 'Principles of PAVM management'.)

•Patients with localized bleeding on imaging – For patients without underlying pulmonary or cardiac disease who present with life-threatening hemoptysis and who appear to have a localized source on chest imaging, it may be appropriate to perform pulmonary arteriography with BAE if a source is identified [62]. (See 'Bronchial artery embolization' below.)

●Diffuse alveolar hemorrhage – For patients with radiographic evidence of a diffuse process, bronchoscopy is generally warranted for diagnostic purposes. (See 'Diagnostic bronchoscopy' below and "The diffuse alveolar hemorrhage syndromes", section on 'Diagnostic evaluation' and "Idiopathic pulmonary hemosiderosis", section on 'Bronchoscopy'.)

Control of mild or moderate hemoptysis — For patients with mild or moderate hemoptysis, management depends on the clinical presentation of the patient, presence or absence of a known underlying disease (eg, CF), and progression of symptoms (algorithm 2).

In most children and adolescents without a chronic underlying disease, hemoptysis is scant or mild (streaks or a few mL of blood). For such patients, a focused history, physical examination, and, in some cases, a chest radiograph usually are sufficient to determine a likely cause. (See 'History' below and 'Physical examination' below and 'Imaging' below.)

If a definite or likely cause of hemoptysis is identified, the main intervention is to optimize treatment for the underlying disease. We typically hospitalize such patients for observation and supportive care; the setting depends on the patient's condition and underlying disease.

Common scenarios are:

●Minor trauma – For patients with a history of minor trauma, such as forceful coughing in the setting of pneumonia or viral respiratory infection or irritation from deep suction in patients with an artificial airway in place. In these scenarios, the hemoptysis tends to resolve spontaneously and is unlikely to recur. No further intervention is necessary, other than avoidance of further trauma and observation for recurrence and appearance of other symptoms. (See 'Trauma' above.)

●Suspicion of foreign body – Particular attention should be paid to clinical characteristics that suggest the possibility of foreign body aspiration, especially in young children or those with swallowing dysfunction. These include a history of choking (days to weeks prior to hemoptysis, even if transient) and new respiratory symptoms such as wheezing. Children with foreign body aspiration may or may not have an abnormal chest radiograph and fever. If foreign body aspiration is suspected, rigid bronchoscopy should be performed for diagnosis and removal. (See 'Foreign body aspiration' above.)

●CF – For patients with CF and mild or moderate hemoptysis (≥5 mL blood), stop any nonsteroidal antiinflammatory drugs and treat with systemic antibiotics, as recommended in a consensus guideline (table 2) [4]. The guideline also suggests continuing inhaled antibiotics and bronchodilators in most cases of mild or moderate hemoptysis, but with some reservations, and suspending inhaled airway clearance therapies. At our institution, we often administer supplemental vitamin K just in case the bleeding is related to vitamin K deficiency (although this is rarely present). (See 'Cystic fibrosis-related hemoptysis' above and "Cystic fibrosis: Overview of the treatment of lung disease".)

DIAGNOSTIC EVALUATION — For most children presenting with hemoptysis, the first steps in the evaluation include key blood tests (complete blood count and coagulation profile) and chest radiograph. Additional evaluation is based on the history and physical examination.

History — If the cause of hemoptysis is not apparent, a thorough history should be sought once the patient is stable. Findings that provide clues to the cause of hemoptysis include:

●Signs of infection:

•Mucopurulent sputum streaked with blood often suggests tracheobronchitis, pneumonia, or bronchiectasis

•Fever or chills accompanying blood-streaked purulent sputum suggest pneumonia

•Putrid-smelling sputum raises the possibility of anaerobic lung abscess

●Choking – A history of a choking episode suggests the possibility of a tracheal or airway foreign body, even if it occurred several days or weeks prior to the hemoptysis. (See 'Foreign body aspiration' above.)

●Exposures – The following exposures are associated with hemoptysis:

•Illicit drugs, particularly cocaine smoking or other inhalational drugs. (See "Cocaine: Acute intoxication", section on 'Crack lung and other pulmonary complications'.)

•Vaping (nicotine or cannabis), with associated pneumonia-like respiratory symptoms [50]. (See "E-cigarette or vaping product use-associated lung injury (EVALI)".)

•Medications that can be associated with alveolar hemorrhage, such as phenytoin, retinoic acid, amiodarone, propylthiouracil, or penicillamine. (See 'Causes of diffuse alveolar hemorrhage' above.)

•Medications that can impair platelet function or produce thrombocytopenia (See "Drug-induced immune thrombocytopenia".)

•Fumes from malfunctioning ice resurfacing and ventilation equipment in an ice arena (nitrogen dioxide inhalation). (See "Etiology of hemoptysis in adults", section on 'Miscellaneous'.)

•Travel and exposure – Inquire about risk factors for tuberculosis, including history of living in areas with endemic tuberculosis, close contact with people experiencing homelessness, incarceration, institutionalized individuals, or migrant workers. Likewise, inquire about travel to areas associated with endemic mycoses or similar exposures (eg, archeological digs, spelunking, etc).

●Underlying medical problems – Known diagnosis or symptoms suggesting an underlying medical problem, including:

•Trauma

•Bleeding disorder (eg, von Willebrand disease) or suggestive history of bleeding from other sites (eg, easy bruising or menorrhagia)

•Chronic pulmonary or cardiac disease

•Systemic symptoms of collagen vascular disorders or vasculitis syndromes, which can be associated with bronchiectasis

•Hematuria, suggesting a pulmonary-renal syndrome (see "The diffuse alveolar hemorrhage syndromes")

Physical examination — Findings on the physical examination that provide clues to the cause of hemoptysis include:

●Bruises around the chest or neck (indicating trauma)

●Crepitus suggesting airway disruption

●Telangiectasia or hemangioma (suggesting arteriovenous malformations)

●Digital clubbing (suggesting chronic suppurative lung disease, pulmonary arteriovenous malformations [PAVMs], or congenital heart disease [CHD])

●Bleeding in the oral cavity or nasopharynx or a missing tooth that was not recovered raise the question of foreign body aspiration (a lost tooth can be aspirated, especially if the child is asleep)

●Focally abnormal breath sounds may be seen in infectious causes, foreign body aspiration, or localized airway or parenchymal bleeding

Imaging

●Conventional chest radiograph – Chest radiographs in at least two views should be obtained, if possible. Typical radiographic findings with hemorrhage include parenchymal and alveolar opacities that may be diffuse throughout both lungs (image 1) or may identify a focal site of the bleeding. Aspiration of blood from nonpulmonary sources may have a similar appearance.

Features suggesting a specific etiology of bleeding include:

•Foreign body (if radiopaque) or signs of related endobronchial obstruction (either hyperinflation, volume loss, or focal pneumonia). (See "Airway foreign bodies in children", section on 'Imaging'.)

•Cavitations (suggesting tuberculosis, fungal infection, or granulomatosis with polyangiitis).

•Bronchiectasis – Plain radiography has low sensitivity for detecting bronchiectasis unless it is severe. Severe bronchiectasis may have a "tram-track" appearance. (See "Bronchiectasis in children: Clinical manifestations and evaluation", section on 'Imaging'.)

However, up to one-third of children with hemoptysis will have normal radiographs [63,64].

●Advanced imaging – Patients with moderate or severe hemoptysis that is not explained after a focused history, examination, and chest radiograph should be further evaluated with computed tomography (CT) of the chest with contrast by multidetector CT angiography.

Since most pulmonary hemorrhages originate in the bronchial arteries, the CT angiography protocol should be optimized to visualize the bronchial arteries. This protocol should also suffice to evaluate the pulmonary arterial circulation for pulmonary embolism [65]. CT angiography will also allow assessment of the airways and lung parenchyma; alveolar hemorrhage typically appears as "ground-glass" opacities in the perivascular areas (image 2). In some cases, the CT may help identify anatomic abnormalities of both airways and vessels. (See "Clinical presentation, evaluation, and diagnosis of the nonpregnant adult with suspected acute pulmonary embolism", section on 'Computed tomography pulmonary angiography'.)

●Arteriography – Pulmonary arteriography should be considered if a high suspicion for vascular malformations exists despite a negative CT scan. In massive hemoptysis, bronchial arteriography may localize the area of bleeding and facilitate bronchial artery embolization (BAE) [66]. (See 'Bronchial artery embolization' below.)

Laboratory evaluation — The laboratory evaluation should be directed by the history and physical examination. For patients with hemoptysis that is at least mild (≥5 mL blood) and of unknown cause, consultation with a pediatric pulmonologist is recommended. Routine testing for such patients should include:

●For all children with unexplained hemoptysis:

•Complete blood count.

•Coagulation studies, including studies to exclude von Willebrand disease (plasma von Willebrand factor antigen, von Willebrand factor activity, and factor VIII activity) [34]. (See "Clinical presentation and diagnosis of von Willebrand disease".)

•If sputum can be obtained, culture for bacteria, fungus, and virus, with stains for acid-fast bacilli.

•Urine analysis to screen for hematuria. Patients with hematuria require further evaluation, as discussed below.

●Other tests depend on patient characteristics:

•For children with signs of diffuse alveolar hemorrhage on chest radiograph and/or systemic symptoms, evaluate for systemic vasculitides. The evaluation for these disorders is described separately. (See "Vasculitis in children: Evaluation overview".)

•For children with any risk factors for tuberculosis, screen with tuberculin skin test or interferon-gamma release assay. (See "Tuberculosis infection (latent tuberculosis) in children", section on 'Diagnosis'.)

•For children with features suggestive of endemic mycoses (eg, travel to an endemic area and with compatible radiographic findings), perform a comprehensive evaluation for endemic mycoses (histoplasmosis, blastomycosis, coccidiomycosis, and Cryptococcus gattii), regardless of the patient's immune function. (See "Diagnosis and treatment of pulmonary histoplasmosis" and "Clinical manifestations and diagnosis of blastomycosis" and "Primary pulmonary coccidioidal infection" and "Coccidioidomycosis: Laboratory diagnosis and screening" and "Cryptococcus gattii infection: Clinical features and diagnosis".)

•For patients with hematuria (gross or microscopic) or renal dysfunction, evaluate specifically for diseases that cause pulmonary-renal syndrome, which is the syndrome of diffuse alveolar hemorrhage and glomerulonephritis that may occur with a variety of diseases including anti-glomerular basement membrane disease, granulomatosis with polyangiitis, microscopic polyangiitis, and systemic lupus erythematosus. (See "Anti-GBM (Goodpasture) disease: Pathogenesis, clinical manifestations, and diagnosis" and "Vasculitis in children: Evaluation overview".)

•For children with bronchiectasis on imaging that is otherwise unexplained, evaluate for the cause of bronchiectasis, beginning with testing for cystic fibrosis (CF). (See "Bronchiectasis in children: Clinical manifestations and evaluation".)

•For patients with moderate or massive hemoptysis, send a specimen for blood type and crossmatch in case bleeding necessitates transfusion. The need for transfusion is uncommon unless the hemoptysis is massive.

Diagnostic bronchoscopy — For most patients with hemoptysis that is at least mild (≥5 mL blood) and otherwise unexplained, we suggest proceeding to diagnostic bronchoscopy once the bleeding is controlled (except for CF patients with massive hemoptysis, for whom BAE is the next step). (See 'Bronchial artery embolization' below.)

Flexible rather than rigid bronchoscopy usually is used for this purpose. Bronchoscopy permits direct inspection of the airways to identify the bleeding site and possible cause. Key observations on the fluid from the bronchoalveolar lavage fluid are:

●Inspection – The presence of blood confirms alveolar bleeding (picture 1).

●Cytology – The bronchoalveolar lavage fluid should be sent for cytology to evaluate for hemosiderin-laden macrophages. Hemosiderin-laden macrophages typically appear three days after the episode of bleeding and may persist for days or weeks; if present, they suggest that the bleeding is subacute or chronic, rather than acute [1,67,68].

●Tests for infection – The bronchoalveolar lavage fluid should also be sent for comprehensive infectious disease workup including stains and cultures for bacteria, fungi, acid-fast bacteria, and viruses. Molecular diagnostics by polymerase chain reaction allow for rapid assessment of many viruses as well as Chlamydia and Mycoplasma. Evaluation of fungal cell wall antigen (D-glucan and galactomannan) can help evaluate for fungal infection, particularly in immunocompromised patients.

Rigid bronchoscopy is the procedure of choice if a foreign body extraction is required or if the bleeding is profuse. (See 'Therapeutic bronchoscopy' below and "Airway foreign bodies in children".)

Lung biopsy — A lung biopsy is generally reserved for patients with suspected autoimmune systemic disorders, including pulmonary capillaritis and idiopathic pulmonary hemosiderosis (table 1). Thus, it is generally only indicated in children with:

●Persistent unexplained hemoptysis or imaging findings suggesting diffuse alveolar hemorrhage, and

●Negative results for a serologic evaluation for vasculitis (see 'Laboratory evaluation' above)

If the serologic testing is negative, a lung biopsy may still reveal vasculitis because there are reported cases of immune-mediated lung disease with negative serology [38,69]. Lung biopsy also may be useful for patients with a provisional diagnosis of vasculitis but poor response to treatment.

THERAPEUTIC INTERVENTIONS FOR SELECTED PATIENTS

Therapeutic bronchoscopy — In addition to identifying a site of bleeding, fiberoptic bronchoscopy early in the evaluation of hemoptysis may provide options for therapeutic benefit [70].

●Instrument – No clear consensus exists as to whether rigid or flexible bronchoscopy (also called fiberoptic bronchoscopy) is preferable. The information gained from these techniques is complementary, and the procedures may be performed sequentially under the same sedation.

•Rigid bronchoscopy is preferred by some clinicians in cases of ongoing profuse bleeding because it allows airway control and more rapid suctioning of large volumes of clots and debris. The larger lumen also permits interventions to provide tamponade. However, this technique must be performed using general anesthesia and, generally, it is performed by a surgeon in an operating room, so the patient may need to be moved. The visual range of rigid bronchoscopy is limited to the major bronchi.

•Flexible bronchoscopy also can be used to control bleeding. It is less useful than rigid bronchoscopy for managing profuse bleeding, but it can be performed at the bedside and permits visualization of the distal airways because of the smaller diameter of the flexible bronchoscope.

•Combined rigid and flexible bronchoscopy may be the best choice in certain patients.

●Hemostasis – If a fairly isolated bleeding site is identified during bronchoscopy, direct tamponade by the bronchoscope may help reduce the bleeding. Attempts at directed vasoconstriction with iced saline infusion or topical alpha agonists (epinephrine, norepinephrine) through the bronchoscope have met with limited success [71,72]. If this fails, a mixture of fibrinogen and thrombin has been used [73]. Other procedures that have been described include bronchoscopy with carbon dioxide or Nd-YAG laser, and balloon tamponade of a lobe or main bronchus using a balloon-tipped catheter (Fogarty catheter) placed during bronchoscopy [3,74]. (See "Evaluation and management of life-threatening hemoptysis".)

Bronchial artery embolization — Bronchial artery embolization (BAE) during bronchial angiography should be considered in cases of persistent bleeding. This technique is widely used in patients with cystic fibrosis (CF) and hemoptysis and has also been successfully utilized in other sources of bronchial artery bleeding [4,75]. The main contraindication to embolization is the presence of spinal arteries arising directly from the bronchial vessels; such vessels can be identified by CT angiography as well as during the bronchial angiography.

In some situations, imaging with multidetector CT prior to BAE helps to precisely locate the bleeding vessels [76]. Once the bleeding vessels are identified, the appropriate vessels are cannulated and then injected with an occlusive material. (See "Evaluation and management of life-threatening hemoptysis", section on 'Arteriography'.)

The outcome of a single BAE is favorable in approximately 80 percent of cases, but bleeding recurs in a significant percentage (eg, in approximately 20 percent of patients with CF within one year) [56,77,78]. Up to 40 percent of these recurrences may require a second embolization procedure.

Rare complications include transverse myelitis or bowel necrosis if spinal arteries or superior mesenteric artery are inadvertently embolized. In addition, fever, dysphagia, and back and chest pain can occur after the procedure.

Interventions to improve hemostasis — For all patients, assess for and treat coagulopathy if present (eg, transfuse platelets for thrombocytopenia or fresh frozen plasma for coagulopathy) and/or reverse any anticoagulant.

Tranexamic acid (TXA) is an antifibrinolytic medication used in a variety of settings, including to control bleeding in a perioperative setting or trauma. Accumulating reports of success with TXA have stimulated interest in its use for hemoptysis [79,80], but its efficacy for managing hemoptysis not been established [81]. At many institutions, inhaled TXA is increasingly used for management of hemoptysis due to a variety of causes because of the simplicity of use and relative safety. Administration of TXA to children for hemoptysis is an off-label use. (See "Perioperative blood management: Strategies to minimize transfusions", section on 'Antifibrinolytic agents' and "Etiology and diagnosis of coagulopathy in trauma patients".)

Use of TXA in children and adults with CF-associated hemoptysis, as well as for patients with other causes of hemoptysis that did not respond to embolization, has been described in case series and a small randomized trial [78,82-87]. TXA has been administered intravenously [87,88], inhaled [85], and orally [86] for adult patients with nonmassive hemoptysis. One study in adults with CF described successful use of intravenous and oral TXA, including in patients with moderate and massive hemoptysis [86]. Based on this limited data from adult studies, all of these routes of administration seem to have some effectiveness and have been well tolerated. It should not be used for patients at risk for thromboembolism. (See "Evaluation and management of life-threatening hemoptysis", section on 'Correct bleeding diathesis' and "Cystic fibrosis: Overview of the treatment of lung disease", section on 'Hemoptysis'.)

Management of diffuse alveolar hemorrhage after autologous hematopoietic cell transplantation is discussed separately. (See "Pulmonary complications after autologous hematopoietic cell transplantation", section on 'Diffuse alveolar hemorrhage'.)

Other measures

●Surgery – Surgical resection of the bleeding site (lobectomy or pneumonectomy) is a last resort for control of ongoing focal hemoptysis [4,89]. It is indicated only in patients who have massive bleeding or intractable hemorrhage, after all other measures to control bleeding have failed. Candidates for surgical resection ideally should have an adequate respiratory reserve (forced expiratory volume in one second [FEV₁] >40 percent predicted) to avoid extreme pulmonary disability postoperatively. Unfortunately, patients with CF who have significantly advanced disease and limited pulmonary reserve may tolerate this procedure poorly. Decisions regarding the surgical approach should take future lung transplant candidacy into account.

●Extracorporeal life support – Extracorporeal membrane oxygen (ECMO) support has also been described in the management of life-threatening hemoptysis [25]; however, it requires anticoagulation, which may worsen the bleeding. In patients with advanced pulmonary arterial hypertension and hemoptysis, urgent evaluation and listing for lung transplantation may be in order. These and other techniques for controlling hemoptysis in adults are discussed separately. (See "Evaluation and management of life-threatening hemoptysis".)

SUBSEQUENT MANAGEMENT — Once the hemoptysis has come under control and a specific etiology has been properly addressed, any previously needed therapy should be reviewed as to when it should be restarted.

●For patients with cystic fibrosis (CF), airway clearance therapy (chest physiotherapy) should be restarted cautiously a day or two after the hemoptysis has stopped. Inhaled medications such as hypertonic saline for CF patients can be cautiously restarted if the airway clearance is well tolerated without restarting the bleeding.

●For patients with hemoptysis from systemic vasculitis, the bleeding usually stops with initiation of appropriate therapy and only needs to be readdressed if there is a recurrence.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Hemoptysis".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Coughing up blood (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Definition and mimics of hemoptysis – Hemoptysis refers to expectoration of blood originating from the lower respiratory tract. Blood from the upper respiratory tract, oropharynx and nasopharynx, and upper gastrointestinal tract can be expectorated and can mimic true hemoptysis. A bright red color, alkaline pH, and, sometimes, thoracic pain can help distinguish hemoptysis from blood originating in the gastrointestinal tract. (See 'Mimics of hemoptysis' above and 'Assess severity and source' above.)

●Causes – The most common causes of hemoptysis in children are respiratory infection, aspirated foreign bodies, and bronchiectasis; bronchiectasis is most common in children with cystic fibrosis (CF). A variety of other processes can also cause hemoptysis (table 1). (See 'Causes of hemoptysis' above.)

Foreign body aspiration is especially common in children under three years of age. In a substantial proportion of cases, the aspiration is not initially recognized and the child may present days or weeks after the aspiration event with wheezing, chronic cough, and/or hemoptysis. (See 'Foreign body aspiration' above and "Airway foreign bodies in children".)

●Initial management of life-threatening or massive hemoptysis – Emergency management of a patient with massive hemoptysis who is hemodynamically unstable includes intubation and mechanical ventilation, circulatory support, and replacement of blood products (algorithm 1). Mechanical ventilation with high positive end-expiratory pressure (PEEP) may improve oxygenation. (See 'Control of life-threatening hemoptysis' above.)

Interventions to stop the bleeding include bronchoscopy with hemostasis interventions and/or bronchial artery embolization (BAE). (See 'Therapeutic bronchoscopy' above and 'Bronchial artery embolization' above.)

BAE is a particularly important tool for managing life-threatening hemoptysis in patients with CF, as discussed separately. (See "Cystic fibrosis: Overview of the treatment of lung disease", section on 'Hemoptysis'.)

●Control of mild or moderate hemoptysis – For patients with mild or moderate hemoptysis, management depends on the clinical presentation of the patient, presence or absence of a known underlying disease (eg, CF), and progression of symptoms (algorithm 2). If a definite or likely cause of hemoptysis is identified, the main intervention is to optimize treatment for the underlying disease. We typically hospitalize such patients for observation and supportive care. (See 'Control of mild or moderate hemoptysis' above.)

●Diagnostic evaluation:

•Imaging – A chest radiograph and, in some cases, CT angiography may help to determine the location of the bleeding and often provide clues to the type of lung disease. Up to one-third of children with hemoptysis will have normal radiographs. (See 'Imaging' above.)

•Laboratory tests – For patients with hemoptysis that is at least mild (≥5 mL blood) and of unknown cause, laboratory testing should include a complete blood count and coagulation studies, including studies to exclude von Willebrand disease. Additional testing that may help to determine the cause of hemoptysis in selected patients includes evaluation for systemic inflammatory or vasculitis conditions and sputum culture for bacteria, fungus, virus, and acid-fast bacilli. (See 'Laboratory evaluation' above.)

•Bronchoscopy – If the cause remains unclear following the laboratory evaluation and the hemoptysis continues, we usually proceed to flexible bronchoscopy to identify the bleeding site; in some cases, the procedure can be used therapeutically to control active bleeding. Rigid bronchoscopy is the procedure of choice if a foreign body extraction is required or if the bleeding is profuse. (See 'Diagnostic bronchoscopy' above and 'Therapeutic bronchoscopy' above.)

•Lung biopsy – Lung biopsy may be required if the cause of hemoptysis remains unknown after an extensive evaluation, particularly if the radiographic findings are consistent with diffuse alveolar hemorrhage. (See 'Imaging' above and 'Lung biopsy' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Diana R Quintero, MD, and Leland L Fan, MD, who contributed to earlier versions of this topic review.