Treating patients with rapidly deteriorating respiratory compromise in the emergency room is difficult and stressful. The patient in front of you is rapidly progressing towards total cardiorespiratory collapse and you may have no idea why. A case is reported of an adult presenting with impending cardiorespiratory collapse attributed to asthma who actually had upper airway obstruction caused by laryngeal papillomata. This case report reinforces the importance of airway assessment, gives an overview of respiratory papillomatosis, and reiterates both the non-surgical and surgical approach to the difficult airway.
- respiratory papillomatosis
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A 21 year old woman was brought to the emergency department by paramedic ambulance, having collapsed at a bus stop. The only history available was that of a sudden onset of respiratory difficulty with an associated “wheeze”. Initial treatment was oxygen and nebulised salbutamol. On arrival at the emergency department her condition had deteriorated. She was unresponsive, hypoventilating and cyanosed, pulse oximetry reading 60%, and a heart rate of 100 beats per minute.
Having excluded contamination of the oropharynx, bag and mask ventilation was attempted, however inflation of the lungs proved unsuccessful. Venous access was established.
An initial working diagnosis of bronchospasm was made. Anaphylaxis was considered, however hypotension, urticaria, and angio-oedema were not present.
Because of the inability to ventilate the patient's lungs, the decision was made to intubate the patient. Etomidate 10 mg followed by 100 mg suxamethonium was given intravenously.
The larynx was easily identified, with a view of the vocal cords and arytenoids (Cormack and Lehane Grade 1). However, located above the right vocal cord was a polypoid mass extending over the laryngeal inlet. This inhibited the passage of a size 8 cuffed orotracheal tube into the trachea.
One final attempt at oral endotracheal intubation was attempted while surgical airway equipment was being prepared. A gum elastic bougie was passed through the laryngeal inlet, passing the mass easily and a cuffed size 6 endotracheal tube was then passed over the bougie into the trachea. (The smallest endotracheal tube readily available in the department) (fig 1).
Ventilation of the lungs with 100% oxygen was now possible and the patient's oxygen saturation improved quickly despite the presence of pulmonary oedema fluid in the endotracheal tube.
On auscultation of the chest, air entry was present in both lungs with slight wheeze and fine crepitations. Lung compliance was good and tidal volumes of 600 ml were achieved with peak airway pressures of 25 cm water
The diagnosis of airway occlusion secondary to supraglottic tumour was made, with pulmonary oedema resulting from excessive negative intra pulmonary pressure.
The patient was sedated, paralysed, and transferred to intensive care.
Collateral history revealed that the patient suffered from respiratory papillomatosis and that she had an appointment for treatment of respiratory papillomata the following day.
The patient was taken to theatre and an extensive mass of supraglottic papillomatous tissue was excised. She was returned to the ICU where she was ventilated until the following morning, and was extubated without incident. She had no neurological deficit, and was discharged home four days later.
There are numerous reports of upper airway obstruction misdiagnosed as asthma (see table 1). It is understandable why such misdiagnoses can be made. In many circumstances the patient may present late (as in this case) therefore the classic features of the “obstructed upper airway” may no longer be apparent.
The airway can be divided in the upper and lower regions. The upper region includes the mouth to carina, and this subdivided into extrathoracic and intrathoracic regions.
An ideal assessment of a partially obstructed airway in the emergency setting includes a detailed history to assess symptoms of dysphonia, hoarseness, dysphagia, and drooling. Information should be sought relating to the speed of onset of problems, the progression of the symptoms and signs, any precipitant factors, for example, inhaled foreign body, upper airway burn, neck trauma or surgery, exposure to specific allergens (drugs, latex, or food). Symptoms related to infection should also be sought (epiglottitis, laryngitis, tracheitis, quinsy, tonsillitis, abscesses). Trauma in the anterior region of the neck may result in external tracheal compression; laryngeal fracture should also be considered especially if the classic triad of hoarseness of voice, palpable laryngeal damage, and subcutaneous emphysema are present.
Signs of upper airway obstruction are initially those of noisy respiration with stridor.
The stridor can originate from any site in the upper airway. In laryngeal stridor the sound differs from the wheeze of asthma in that it is inspiratory and most prominent in the neck. The wheeze of asthma is predominantly expiratory and louder in the chest.1 Stridor originating from trachea is a lower pitch than laryngeal noise. To illicit the sign the patient should be asked to cough then take a breath in. The noise is best heard at the mouth in the first breaths after this manoeuvre.1 Some authors suggest that intrathoracic obstruction may result in expiratory stridor.2
With worsening obstruction to airflow, work of respiration is increased because of the necessity to produce greater swings in inspiratory/expiratory pressure. This results in the use of accessory muscles of respiration and associated tracheal tug, supraclavicular and intercostal indrawing, and paradoxical see-saw respiration. The respiratory rate will also be increased. Powerful inspiratory effort may result in the development of dermal ecchymoses and even subcutaneous emphysema.
Progression of the obstruction results in inadequate respiration, resulting in hypercarbia and hypoxia. The patient will become agitated, distressed, and if the obstruction is not relieved progressive respiratory failure will result in coma. Bradycardia, bradypnoeia, hypotension, and cyanosis herald imminent cardiorespiratory arrest.3
In the less acute setting plain radiographs of neck and chest may show foreign body or soft tissue swelling, however caution must be exercised when requesting such investigations in the unstable patient. Spirometry and flow-volume loops may also give information as to the site of the upper airway obstruction.
When confronted with a patient who is in the pre-arrest phase of upper airway obstruction, and when the clinician has little or no history as a guide, it may be impossible to decide clinically the cause of the respiratory problem. Therefore, when considering tracheal intubation in the obtunded patient, upper airway obstruction should be considered, and therefore the appropriate staff and equipment present.
Recurrent respiratory papillomatosis (RRP) is an uncommon condition with an incidence of 3–4 per 100 000. In the United States an estimated 9000 adults and 6000 children are known to suffer from the disease, and in excess of 16 000 surgical procedures are conducted annually for this condition.4 However, this is not a condition commonly dealt with in the emergency department. To date, only one documented case of out of hospital respiratory arrest attributable to RRP has resulted in a non-fatal outcome.5 There are numerous recorded deaths as a direct result of total airway obstruction.6–8 There are no published cases of patients surviving acute respiratory occlusion from RRP in young adults (see table 2).
Papillomata are benign lesions associated with infection by human papilloma virus (HPV) types 6 or 11.9,10 They most commonly affect the larynx but can occur throughout the aerodigestive tract, at squamociliary epithelial junctions, and at sites of mucosal damage11 including tracheotomy wounds.12 The clinical course of the condition varies widely between people and can be difficult to predict. Growth of the lesions can be prolific, and there is a risk of malignant transformation.12
RRP may present either in childhood or in adult life. Upper airway papillomata generally present with hoarseness of voice whereas papillomata of the distal bronchopulmonary tree present after distal airway occlusion as atelectasis and after obstructive infection.12 There is a significant risk of subglottic stenosis in patients who have undergone repeated excision of tracheal lesions.13
Papillomata have a tendency to progress down the tracheobronchial tree,14 and this may complicate needle cricothyroidotomy as the presence of tracheal lesions may hinder location and cannulation of the tracheal lumen.
Formal tracheostomy should be avoided if at all possible in these patients, as it may predispose to distal dissemination of papillomata previously confined to the larynx and a consequent worsening of long term prognosis.9 It is therefore important that this condition is recognised if at all possible at the time of attempted intubation. The papillomata appear as raised wart-like lesions (fig 1), which may coalesce into a large fleshy mass of extremely friable tissue, sometimes significantly distorting or obscuring normal anatomy. There may also be scarring present from multiple excisions or laser ablations of the papillomata. The main differential diagnosis is squamous carcinoma, but papillomatosis should be especially suspected in those aged less than 40 years, in whom malignancy of this sort is less common. In this case, the ideal management would have been attempted intubation with a small diameter uncuffed endotracheal tube. If this had been unsuccessful a temporary airway may have been achieved with an airway exchange catheter, (Cook (UK Ltd), Monroe House, Letchworth SG6 1LN); such devices have been described for maintaining the airway in patients with RRP in the elective and emergency setting.15
If endotracheal intubation had not been possible then an emergency surgical airway would have been the next course of action. Needle cricothyroidotomy (as described in the ATLS manual) would provide a means of oxygenation before performing a tracheostomy.
The management of the airway in an emergency situation is often more difficult than in the elective setting.16,17 Patients may be unfasted, hypoxic, hypercarbic, shocked, and combative. The cause of the respiratory problem, as in this case, may be unexpected or require specialist airway skills and equipment.
This case shows the potential difficulties when treating airway problems in the emergency setting and emphasises the need for experienced practitioners when dealing with these problems.
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