Emphysema is characterised by loss of elasticity of the lung tissue; destruction of structures supporting the alveoli; and destruction of capillaries feeding the alveoli. The result is that the small airways collapse during expiration, leading to an obstructive form of lung disease (air is trapped in the lungs in obstructive lung diseases). Features are: shortness of breath on exertion--particularly when climbing stairs or inclines (and later at rest), hyperventilation and an expanded chest. As emphysema progresses, clubbing of the fingers may be observed, a feature of longstanding hypoxia.

Emphysema patients are sometimes referred to as "pink puffers". This is because emphysema sufferers may hyperventilate to maintain adequate blood oxygen levels. Hyperventilation explains why emphysema patients do not appear cyanotic as chronic bronchitis (another COPD disorder) sufferers often do; hence they are "pink" puffers (adequate oxygen levels in the blood) and not "blue" bloaters (cyanosis; inadequate oxygen in the blood).

Diagnosis

Diagnosis is by spirometry (lung function testing), including diffusion testing. Other investigations might include X-rays, high resolution spiral chest CT-scan, bronchoscopy (when other lung disease is suspected, including malignancy), blood tests, pulse oximetry and arterial blood gas sampling.

Pathophysiology

The scientific definition of emphysema is:

"Permanent destructive enlargement of the airspaces distal to the terminal bronchioles without obvious fibrosis".

Hence, the definite diagnosis is made by a pathologist. However, we can easily ascertain clinical diagnosis by history, clinical examination, chest radiography and lung function tests.

In normal breathing, air is drawn in through the bronchial passages and down into the increasingly fine network of tubing in the lungs called the alveoli, which are many thousands of tiny sacs surrounded by capillaries. These absorb the oxygen and transfer it into the blood. When toxins such as smoke are breathed into the lungs, the particles are trapped by the hairs and cannot be exhaled, leading to a localised inflammatory response. Chemicals released during the inflammatory response (trypsin, elastase, etc.) are released and begin breaking down the walls of alveoli. This leads to fewer but larger alveoli, with a decreased surface area and a decreased ability to take up oxygen and lose carbon dioxide. The activity of another molecule called alpha 1-antitrypsin normally neutralizes the destructive action of one of these damaging molecules.

After a prolonged period, hyperventilation becomes inadequate to maintain high enough oxygen levels in the blood, and the body compensates by vasoconstricting appropriate vessels. This leads to pulmonary hypertension. This leads to enlargement and increased strain on the right side of the heart, which in turn leads to peripheral edema (swelling of the peripherals) as blood gets backed up in the systemic circulation, causing fluid to leave the circulatory system and accumulate in the tissues.

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Spontaneous retropharyngeal and cervical emphysema: a rare singer's injury
From Ear, Nose & Throat Journal, 11/1/05 by Neville P. Shine

Abstract

Spontaneous retropharyngeal and cervical emphysema is rare. We describe a case that was unusual in its etiology: the result of singing. Although this condition is usually benign, hospital admission for close observation and supportive therapy is prudent.

Introduction

Spontaneous retropharyngeal and cervical emphysema is a rare clinical entity. (1) It generally occurs in association with spontaneous pneumomediastinum. (2) A wide variety of precipitating factors has been reported. (3) Singing has not previously been described as a cause for this condition.

The most common symptoms of spontaneous retropharyngeal and cervical emphysema are chest pain and dyspnea, although neck symptoms of varying intensity are often present, as well. (4) A presentation involving only complaints referable to the neck with no chest symptoms is unusual and may delay a correct diagnosis. (5)

We describe a case of acute spontaneous pneumomediastinum with retropharyngeal and cervical emphysema that occurred in a singer while she was performing. We also discuss the presentation, pathophysiology, and management of this condition.

Case report

A 27-year-old woman presented to the Accident and Emergency Department at Adelaide Meath and National Children's Hospital in Dublin complaining of sudden-onset anterior neck pain, odynophagia, and dysphonia. Her symptoms had manifested while she was performing during a singing engagement. She denied any associated chest pain or dyspnea. Her medical history was negative for chronic or explosive cough, trauma, or underlying respiratory disease.

On initial examination, the patient's vital signs were stable and no stridor or respiratory distress was evident, but she was dysphonic and unable to swallow her saliva. Her neck was tender to palpation, and its range of motion was limited. No demonstrable crepitus was palpable. Findings on oropharyngeal inspection and flexible fiberoptic nasopharyngoscopy were normal, as was the remainder of the physical examination.

Neck and chest x-rays revealed the presence of retropharyngeal air, subcutaneous emphysema in the soft tissues of the neck, and pneumomediastinum (figure 1). The patient was admitted for observation, supplemental oxygen, and supportive treatment, including intravenous fluids and analgesia. She made an uneventful recovery and was able to tolerate oral intake after 4 days. Six days after admission, a follow-up lateral neck x-ray confirmed resolution of the retropharyngeal emphysema (figure 2). The patient was discharged home, and she resumed singing 1 month later. She reported no further episodes at the 6-month review.

[FIGURE 1 OMITTED]

Discussion

The term spontaneous is applied to diagnoses of pneumomediastinum and cervical emphysema that have no obvious cause, such as surgery or trauma. Some possible precipitating factors that have been reported include weight lifting, parturition, glassblowing, explosive cough, and vomiting. (5-8) Underlying pulmonary diseases--asthma, emphysema, or respiratory tract infection--may predispose some patients to develop spontaneous pneumomediastinum. (9)

The mechanism by which air enters the mediastinum was elucidated by Macklin in 1939. (10) He postulated that an increase in alveolar pressure secondary to an increase in intrathoracic pressure against a total or partial obstruction (as occurs during Valsalva's maneuver and explosive cough) leads to marginal alveolar rupture. When underlying parenchymal disease is present, the alveoli may be weakened; as a consequence, alveolar rupture can be caused by changes in pressure that are less dramatic. Following rupture of the alveoli, extraalveolar air enters the pulmonary interstitium and dissects along the perivascular sheaths to the mediastinum. Proximal extension along subfascial tissue planes can occur, resulting in cervical and retropharyngeal emphysema.

Patients with spontaneous pneumomediastinum usually complain of retrosternal chest pain and dyspnea. Neck symptoms--including pain, odynophagia, and dysphonia--have been reported in as many as 72% of patients. (4) It has been suggested that the onset of neck symptoms in cervical and retropharyngeal emphysema coincides with a decrease in chest symptoms. (5) Physical findings include Hamman's sign (precordial crepitus), subcutaneous emphysema, and decreased cardiac dullness to percussion. (4) Radiographic examination confirms the diagnosis. On chest x-ray, the most consistent finding is a thin radiolucent line along the left edge of the heart. (8) Lateral x-ray of the soft tissues of the neck will show air in the soft-tissue planes.

In general, this condition is self-limiting and runs a benign course. However, in fulminant cases, impaired venous return and decreased cardiac output might result in potentially fatal hypotension. Hospital admission for observation, bed rest, and analgesia is sufficient for most patients. (3) Intravenous fluids may be required for patients who have significant odynophagia. Supplemental oxygen may expedite recovery by creating a favorable pressure gradient for nitrogen, resulting in gas resorption. Prophylactic antibiotics appear to be unnecessary. Close observation and serial radiography should be carried out to confirm the patient's recovery.

Neville P. Shine, AFRCS; Peter Lacy, MD; Brendan Conlon, MD; Donald McShane, FRCS

Reprint requests: Neville P. Shine, 5 Shem Court, St. James St., Vredehoek, Cape Town 8001, Republic of South Africa. Phone: 27-21-462-1898; fax: 27-21-462-1898; e-mail: shinerl@eircom.net

References

(1.) Ralph-Edwards AC, Pearson FG. Atypical presentation of spontaneous pneumomediastinum. Ann Thorac Surg 1994;58:1758-60.

(2.) Stack BC Jr., Ridley MB. Spontaneous cervical emphysema in a child. Otolaryngol Head Neck Surg 1994; 110:318-23.

(3.) Abolnik I, Lossos IS, Breuer R. Spontaneous pneumomediastinum: A report of 25 cases. Chest 1991;100:93-5.

(4.) Munsell WP. Pneumomediastinum. A report of 28 cases and review of the literature. JAMA 1967;202:689-93.

(5.) Granich MS, Klotz RE, Lofgren RH, et al. Spontaneous retropharyngeal and cervical subcutaneous emphysema in adults. Arch Otolaryngol 1983;109:701-4.

(6.) Asplund CA, Howard TM, O'Connor FG. Spontaneous pneumomediastinum in a weightlifter. Curr Sports Med Rep 2003;2:63-4.

(7.) Brandfass RT, Martinez DM. Mediastinal and subcutaneous emphysema in labor. South Med J 1976;69:1554-5.

(8.) Maravelli A J, Skiendzielewski JJ, Snover W. Pneumomediastinum acquired by glass blowing. J Emerg Med 2000;19:145-7.

(9.) Harley EH. Spontaneous cervical and mediastinal emphysema in asthma. Arch Otolaryngol Head Neck Surg 1987; 113:1111-12.

(10.) Macklin CC. Transport of air along sheaths of pulmonic blood vessels from alveoli to mediastinum: Clinical implications. Arch Intern Med 1939;64:913-26.

From the Department of Otolaryngology, South Infirmary-Victoria Hospital, Cork, Ireland (Dr. Shine), the Department of Otolaryngology, Great Ormond Street Hospital, London (Dr. Lacy), and the Department of Otolaryngology-Head and Neck Surgery, Adelaide Meath and National Children's Hospital, Dublin (Dr. Conlon and Dr. McShane).

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