Laryngotracheobronchitis
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Croup, also known as laryngotracheobronchitis, is a type of respiratory infection that is usually caused by a virus. The infection leads to swelling inside the trachea, which interferes with normal breathing and produces the classic symptoms of "barking/brassy" cough, inspiratory
stridor Stridor (Latin for "creaking or grating noise") is a high-pitched extra-thoracic breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor which is a noise originating in the pha ...
and a hoarse voice. Fever and runny nose may also be present. These symptoms may be mild, moderate, or severe. Often it starts or is worse at night and normally lasts one to two days. Croup can be caused by a number of viruses including parainfluenza and influenza virus. Rarely is it due to a bacterial infection. Croup is typically diagnosed based on signs and symptoms after potentially more severe causes, such as epiglottitis or an
airway foreign body A foreign body (FB) is any object originating outside the body of an organism. In machinery, it can mean any unwanted intruding object. Most references to foreign bodies involve propulsion through natural orifices into hollow organs. Foreign bo ...
, have been ruled out. Further investigations, such as blood tests, X-rays and cultures, are usually not needed. Many cases of croup are preventable by immunization for
influenza Influenza, commonly known as "the flu", is an infectious disease caused by influenza viruses. Symptoms range from mild to severe and often include fever, runny nose, sore throat, muscle pain, headache, coughing, and fatigue. These symptoms ...
and diphtheria. Most cases of croup are mild and the child can be treated at home with supportive care. Croup is usually treated with a single dose of
steroids A steroid is a biologically active organic compound with four rings arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter membrane fluidity; and a ...
by mouth. In more severe cases inhaled
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands and ...
may also be used. Hospitalization is required in one to five percent of cases. Croup is a relatively common condition that affects about 15% of children at some point. It most commonly occurs between six months and five years of age but may rarely be seen in children as old as fifteen. It is slightly more common in males than females. It occurs most often in autumn. Before vaccination, croup was frequently caused by diphtheria and was often fatal. This cause is now very rare in the Western world due to the success of the
diphtheria vaccine Diphtheria vaccine is a toxoid vaccine against diphtheria, an illness caused by ''Corynebacterium diphtheriae''. Its use has resulted in a more than 90% decrease in number of cases globally between 1980 and 2000. The first dose is recommended ...
.


Signs and symptoms

Croup is characterized by a "barking" cough,
stridor Stridor (Latin for "creaking or grating noise") is a high-pitched extra-thoracic breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor which is a noise originating in the pha ...
, hoarseness, and difficult breathing which usually worsens at night. The "barking" cough is often described as resembling the call of a sea lion. The stridor is worsened by agitation or crying, and if it can be heard at rest, it may indicate critical narrowing of the airways. As croup worsens, stridor may decrease considerably. Other symptoms include fever, coryza (symptoms typical of the
common cold The common cold or the cold is a viral infectious disease of the upper respiratory tract that primarily affects the respiratory mucosa of the nose, throat, sinuses, and larynx. Signs and symptoms may appear fewer than two days after exposu ...
), and indrawing of the
chest wall The thoracic wall or chest wall is the boundary of the thoracic cavity. Structure The bone, bony human skeleton, skeletal part of the thoracic wall is the rib cage, and the rest is made up of muscle, skin, and fasciae. The chest wall has 10 lay ...
–known as Hoover's sign. Drooling or a very sick appearance can indicate other medical conditions, such as epiglottitis or
tracheitis Tracheitis is an inflammation of the trachea. Although the trachea is usually considered part of the lower respiratory tract, in ICD-10 tracheitis is classified under "acute upper respiratory infections". Symptoms * Increasing deep or barking cr ...
.


Causes

Croup is usually deemed to be due to a viral infection. Others use the term more broadly, to include acute laryngotracheitis (
laryngitis Laryngitis is inflammation of the larynx (voice box). Symptoms often include a hoarse voice and may include fever, cough, pain in the front of the neck, and trouble swallowing. Typically, these last under two weeks. Laryngitis is categorised ...
and
tracheitis Tracheitis is an inflammation of the trachea. Although the trachea is usually considered part of the lower respiratory tract, in ICD-10 tracheitis is classified under "acute upper respiratory infections". Symptoms * Increasing deep or barking cr ...
together), spasmodic croup, laryngeal diphtheria, bacterial tracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis. The first two conditions involve a viral infection and are generally milder with respect to symptomatology; the last four are due to bacterial infection and are usually of greater severity.


Viral

Viral croup or acute laryngotracheitis is most commonly caused by
parainfluenza virus Human parainfluenza viruses (HPIVs) are the viruses that cause human parainfluenza. HPIVs are a paraphyletic group of four distinct single-stranded RNA viruses belonging to the ''Paramyxoviridae'' family. These viruses are closely associated wit ...
(a member of the paramyxovirus family), primarily types 1 and 2, in 75% of cases. Other viral causes include
influenza Influenza, commonly known as "the flu", is an infectious disease caused by influenza viruses. Symptoms range from mild to severe and often include fever, runny nose, sore throat, muscle pain, headache, coughing, and fatigue. These symptoms ...
A and B,
measles Measles is a highly contagious infectious disease caused by measles virus. Symptoms usually develop 10–12 days after exposure to an infected person and last 7–10 days. Initial symptoms typically include fever, often greater than , cough, ...
,
adenovirus Adenoviruses (members of the family ''Adenoviridae'') are medium-sized (90–100 nm), nonenveloped (without an outer lipid bilayer) viruses with an icosahedral nucleocapsid containing a double-stranded DNA genome. Their name derives from the ...
and respiratory syncytial virus (RSV). Spasmodic croup is caused by the same group of viruses as acute laryngotracheitis, but lacks the usual signs of infection (such as fever, sore throat, and increased white blood cell count). Treatment, and response to treatment, are also similar.


Bacteria and cocci

Croup caused by a bacterial infection is rare. Bacterial croup may be divided into laryngeal diphtheria, bacterial tracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis. Laryngeal diphtheria is due to ''
Corynebacterium diphtheriae ''Corynebacterium diphtheriae'' is the pathogenic bacterium that causes diphtheria. It is also known as the Klebs–Löffler bacillus, because it was discovered in 1884 by German bacteriologists Edwin Klebs (1834–1912) and Friedrich Löffl ...
'' while bacterial tracheitis, laryngotracheobronchitis, and laryngotracheobronchopneumonitis are usually due to a primary viral infection with secondary bacterial growth. The most common cocci implicated are ''
Staphylococcus aureus ''Staphylococcus aureus'' is a Gram-positive spherically shaped bacterium, a member of the Bacillota, and is a usual member of the microbiota of the body, frequently found in the upper respiratory tract and on the skin. It is often positive ...
'' and '' Streptococcus pneumoniae'', while the most common bacteria are '' Haemophilus influenzae'', and '' Moraxella catarrhalis''.


Pathophysiology

The viral infection that causes croup leads to swelling of the
larynx The larynx (), commonly called the voice box, is an organ in the top of the neck involved in breathing, producing sound and protecting the trachea against food aspiration. The opening of larynx into pharynx known as the laryngeal inlet is about ...
, trachea, and large bronchi due to infiltration of white blood cells (especially histiocytes,
lymphocytes A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic adap ...
, plasma cells, and
neutrophils Neutrophils (also known as neutrocytes or heterophils) are the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. They form an essential part of the innate immune system, with their functions varying in ...
). Swelling produces airway obstruction which, when significant, leads to dramatically increased work of breathing and the characteristic turbulent, noisy airflow known as
stridor Stridor (Latin for "creaking or grating noise") is a high-pitched extra-thoracic breath sound resulting from turbulent air flow in the larynx or lower in the bronchial tree. It is different from a stertor which is a noise originating in the pha ...
.


Diagnosis

Croup is typically diagnosed based on signs and symptoms. The first step is to exclude other obstructive conditions of the upper airway, especially epiglottitis, an airway
foreign body A foreign body (FB) is any object originating outside the body of an organism. In machinery, it can mean any unwanted intruding object. Most references to foreign bodies involve propulsion through natural orifices into hollow organs. Foreign bo ...
,
subglottic stenosis Subglottic stenosis is a congenital or acquired narrowing of the subglottic airway. It can be congenital, acquired, iatrogenic, or very rarely, idiopathic. It is defined as the narrowing of the portion of the airway that lies between the vocal co ...
, angioedema, retropharyngeal abscess, and bacterial tracheitis. A frontal X-ray of the neck is not routinely performed, but if it is done, it may show a characteristic narrowing of the trachea, called the steeple sign, because of the subglottic stenosis, which resembles a steeple in shape. The steeple sign is suggestive of the diagnosis, but is absent in half of cases. Other investigations (such as
blood test A blood test is a laboratory analysis performed on a blood sample that is usually extracted from a vein in the arm using a hypodermic needle, or via fingerprick. Multiple tests for specific blood components, such as a glucose test or a cholester ...
s and viral culture) are discouraged, as they may cause unnecessary agitation and thus worsen the stress on the compromised airway. While viral cultures, obtained via nasopharyngeal aspiration, can be used to confirm the exact cause, these are usually restricted to research settings. Bacterial infection should be considered if a person does not improve with standard treatment, at which point further investigations may be indicated.


Severity

The most commonly used system for classifying the severity of croup is the Westley score. It is primarily used for research purposes rather than in clinical practice. It is the sum of points assigned for five factors: level of consciousness, cyanosis, stridor, air entry, and retractions. The points given for each factor is listed in the adjacent table, and the final score ranges from 0 to 17. * A total score of ≤ 2 indicates ''mild'' croup. The characteristic barking cough and hoarseness may be present, but there is no stridor at rest. * A total score of 3–5 is classified as ''moderate'' croup. It presents with easily heard stridor, but with few other signs. * A total score of 6–11 is ''severe'' croup. It also presents with obvious stridor, but also features marked
chest wall The thoracic wall or chest wall is the boundary of the thoracic cavity. Structure The bone, bony human skeleton, skeletal part of the thoracic wall is the rib cage, and the rest is made up of muscle, skin, and fasciae. The chest wall has 10 lay ...
indrawing. * A total score of ≥ 12 indicates impending respiratory failure. The barking cough and stridor may no longer be prominent at this stage. 85% of children presenting to the emergency department have mild disease; severe croup is rare (<1%).


Prevention

Croup is contagious during the first few days of the infection. Basic hygiene including hand washing can prevent transmission. There are no vaccines that have been developed to prevent croup, however, many cases of croup have been prevented by immunization for
influenza Influenza, commonly known as "the flu", is an infectious disease caused by influenza viruses. Symptoms range from mild to severe and often include fever, runny nose, sore throat, muscle pain, headache, coughing, and fatigue. These symptoms ...
and diphtheria. At one time, croup referred to a diphtherial disease, but with vaccination, diphtheria is now rare in the developed world.


Treatment

Most children with croup have mild symptoms and supportive care at home is effective. For children with moderate to severe croup, treatment with corticosteroids and nebulized epinephrine may be suggested. Steroids are given routinely, with
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands and ...
used in severe cases. Children with oxygen saturation less than 92% should receive oxygen, and those with severe croup may be hospitalized for observation. In very rare severe cases of croup that result in respiratory failure, emergency intubation and ventilation may be required. With treatment, less than 0.2% of children require endotracheal intubation. Since croup is usually a viral disease, antibiotics are not used unless secondary bacterial infection is suspected. The use of cough medicines, which usually contain dextromethorphan or
guaifenesin Guaifenesin, also known as glyceryl guaiacolate, is an expectorant medication that aids in the elimination of sputum from the respiratory tract. Chemically it is an ether of guaiacol and glycerine. It is often used in combination with other me ...
, are also discouraged.


Supportive care

Supportive care for children with croup includes resting and keeping the child hydrated. Infections that are mild are suggested to be treated at home. Croup is contagious so washing hands is important. Children with croup should generally be kept as calm as possible. Over the counter medications for pain and fever may be helpful to keep the child comfortable. There is some evidence that cool or warm mist may be helpful, however, the effectiveness of this approach is not clear. If the child is showing signs is distress while breathing ( inspiratory stridor, working hard to breath, blue (or blue-ish) coloured lips, or decrease in the level of alertness), immediate medical evaluation by a doctor is required.


Steroids

Corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involve ...
, such as dexamethasone and budesonide, have been shown to improve outcomes in children with all severities of croup, however, the benefits may be delayed. Significant relief may be obtained as early as two hours after administration. While effective when given by injection, or by inhalation, giving the medication by mouth is preferred. A single dose is usually all that is required, and is generally considered to be quite safe. Dexamethasone at doses of 0.15, 0.3 and 0.6 mg/kg appear to be all equally effective.


Epinephrine

Moderate to severe croup (for example, in the case of severe stridor) may be improved temporarily with
nebulized In medicine, a nebulizer (American English) or nebuliser (British English) is a drug delivery device used to administer medication in the form of a mist inhaled into the lungs. Nebulizers are commonly used for the treatment of asthma, cystic fibro ...
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands and ...
. While epinephrine typically produces a reduction in croup severity within 10–30 minutes, the benefits are short-lived and last for only about 2 hours. If the condition remains improved for 2–4 hours after treatment and no other complications arise, the child is typically discharged from the hospital. Epinephrine treatment is associated with potential adverse effects (usually related to the dose of epinephrine) including tachycardia,
arrhythmia Arrhythmias, also known as cardiac arrhythmias, heart arrhythmias, or dysrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. A resting heart rate that is too fast – above 100 beats per minute in adults ...
s, and
hypertension Hypertension (HTN or HT), also known as high blood pressure (HBP), is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms. Long-term high bl ...
.


Oxygen

More severe cases of croup may require treatment with oxygen. If oxygen is needed, "blow-by" administration (holding an oxygen source near the child's face) is recommended, as it causes less agitation than use of a mask.


Other

While other treatments for croup have been studied, none has sufficient evidence to support its use. There is tentative evidence that breathing heliox (a mixture of helium and oxygen) to decrease the work of breathing is useful in those with severe disease, however, there is uncertainty in the effectiveness and the potential adverse effects and/or side effects are not well known. In cases of possible secondary bacterial infection, the antibiotics vancomycin and cefotaxime are recommended. In severe cases associated with influenza A or B infections, the antiviral neuraminidase inhibitors may be administered.


Prognosis

Viral croup is usually a self-limiting disease, with half of cases resolving in a day and 80% of cases in two days. It can very rarely result in death from respiratory failure and/or cardiac arrest. Symptoms usually improve within two days, but may last for up to seven days. Other uncommon complications include bacterial tracheitis, pneumonia, and pulmonary edema.


Epidemiology

Croup affects about 15% of children, and usually presents between the ages of 6 months and 5–6 years. It accounts for about 5% of hospital admissions in this population. In rare cases, it may occur in children as young as 3 months and as old as 15 years. Males are affected 50% more frequently than are females, and there is an increased prevalence in autumn.


History

The word ''croup'' comes from the Early Modern English verb ''croup'', meaning "to cry hoarsely." The noun describing the disease originated in southeastern Scotland and became widespread after Edinburgh physician
Francis Home Francis Home FRSE FRCPE (17 November 1719 in Eccles, Berwickshire – 15 February 1813) was a Scottish physician, and the first Professor of Materia Medica at the University of Edinburgh, known to make the first attempt to vaccinate against ...
published the 1765 treatise ''An Inquiry into the Nature, Cause, and Cure of the Croup''. Diphtheritic croup has been known since the time of Homer's ancient Greece, and it was not until 1826 that viral croup was differentiated from croup due to diphtheria by Bretonneau. Viral croup was then called "faux-croup" by the French and often called "false croup" in English, as "croup" or "true croup" then most often referred to the disease caused by the diphtheria bacterium. False croup has also been known as pseudo croup or spasmodic croup. Croup due to diphtheria has become nearly unknown in affluent countries in modern times due to the advent of effective immunization. One famous fatality of croup was
Napoleon Napoleon Bonaparte ; it, Napoleone Bonaparte, ; co, Napulione Buonaparte. (born Napoleone Buonaparte; 15 August 1769 – 5 May 1821), later known by his regnal name Napoleon I, was a French military commander and political leader who ...
's designated heir,
Napoléon Charles Bonaparte Napoleon Bonaparte ; it, Napoleone Bonaparte, ; co, Napulione Buonaparte. (born Napoleone Buonaparte; 15 August 1769 – 5 May 1821), later known by his regnal name Napoleon I, was a French military commander and political leader who ...
. His death in
1807 Events January–March * January 7 – The United Kingdom of Great Britain and Ireland issues an Order in Council prohibiting British ships from trading with France or its allies. * January 20 – The Sierra Leone Company, faced with b ...
left Napoleon without an heir and contributed to his decision to divorce from his wife, the Empress Josephine de Beauharnais.


References


External links

* {{Respiratory pathology Animal viral diseases Acute upper respiratory infections Pediatrics Wikipedia emergency medicine articles ready to translate Wikipedia medicine articles ready to translate (full) Cough de:Pseudokrupp