Spontaneous surgical emphysema in children

Manuscript type : Case Report | Article Date : 2015/07/26

  • Autors

    1. Karthikeyan Padmanabhan
    2. Pulimoottil Davis Thomas
  • Abstract

    A fourteen year old girl presented to the outpatient ENT department of a tertiary care centre with complaints of sudden onset of swelling over the neck and upper chest associated with pain and mild breathlessness, following an episode of violent coughing. Examination revealed surgical emphysema over the entire neck and upper chest with absent laryngeal crepitus. Patient was also found to have signs suggestive of chronic pharyngotonsillitis. Auscultation of the chest revealed bilateral wheeze. Plain X-ray soft tissue neck (anteroposterior and lateral views) showed evidence of air in the pre-sternal region, pre-vertebral region and soft tissues of neck, suggestive of subcutaneous emphysema. As the patient’s condition was stable and the patient was maintaining oxygen saturation at room air, the patient was managed conservatively with intravenous broad spectrum antibiotics, oral corticosteroids, bed rest and other supportive therapy. The patient improved within a week, with complete relief of all symptoms and full clinical resolution of the surgical emphysema. A review of the possible mechanism is presented. This case is being reported due to its rarity, as spontaneous tracheal rupture is extremely uncommon and has only very rarely been reported in the pediatric population.
  • Description

    INTRODUCTION

    Spontaneous subcutaneous emphysema is defined as subcutaneous emphysema without an obvious cause, but is unusual and very rare. Subcutaneous emphyse-ma may develop from gas-forming infections, trauma or a break in the skin, airway or gastrointestinal tract.1 Tracheal rupture is a rare, potentially life-threatening condition that is iatrogenic or traumatic in the vast majority of cases, but may be spontaneous in a few cases.2,3 Spontaneous tracheal ruptures are extremely uncommon, and few cases have been reported in the English literature. Almost all reported cases occurred in adults, but one pediatric case was also reported.1,4-6

    CASE REPORT

    A 14-year-old girl presented to the outpatient ENT department of a tertiary care centre with complaints of swelling over the neck and upper chest, neck pain, throat pain, odynophagia and mild dysphagia. The patient gave a history of low grade fever and produc-tive cough for 5 days. The patient reported that the swelling over the neck and upper chest, the neck pain and the pain and difficulty in swallowing all appeared following a bout of severe coughing the day before the visit to the clinic. The patient had no history of trauma to the neck, foreign-body ingestion or aspira-tion, bronchial asthma or abnormalities of the central airway or neck. On examination, the patient was neither dyspnoeic nor cyanotic. The patient’s vital signs were all within normal limits and oxygen saturation was maintained at normal limits with room air. Examination of the throat revealed bilateral congested anterior pillars, bilateral grade I tonsillar enlargement and a granu-lar posterior pharyngeal wall. Indirect laryngoscopy showed a normal study. On palpation, crepitus was present over the anterior and bilateral lateral aspects of the neck and over bilateral supraclavicular fossae and over the mammary areas of the chest. Ausculta-tion of the chest revealed bilateral normal vesicular breath sounds with bilateral wheeze. Radiograph soft tissue neck (anteroposterior and lat-eral views) revealed evidence of air collections in the pre-sternal region, pre-vertebral region and the soft tissues of the neck, suggestive of subcutaneous emphysema (Fig. 1). Chest radiograph showed clear lung fields with no evidence of pneumothorax or pneumomediastinum (Fig. 2). Videolaryngoscopic examination was performed, which revealed a normal study of the laryngeal inlet, with no signs of inflam-mation or blood clots (Fig. 3). The patient was admitted and treated conservative-ly with intravenous antibiotics, oral corticosteroids, analgesics and cough suppressants. The child re-ceived oxygen at 2 L/minute via nasal prongs. Daily physical examination revealed gradual absorption of the subcutaneous emphysema with no additional aggrevation. A repeat radiograph confirmed that the air collections had disappeared and the child was dis-charged from the hospital one week after admission. The patient was followed up upto 3 months, and re-mained asymptomatic with no recurrence of the sur-gical emphysema.

    DISCUSSION

    Knott reported the first case of spontaneous asth-ma-associated subcutaneous emphysema of the neck and mediastinum.2 Several various causes have been reported in literature to cause spontaneous cervical and mediastinal surgical emphysema. Most cases were found to be the result of increased intrathorac-ic pressure and were seen in postoperative patients with positive pressure ventilation, following SCUBA diving, during labour, following Valsalva-type ma-neuver, following excessive phonation, following ex-cessive blowing and following lung function tests.7-13 Cases of spontaneous surgical emphysema have also been reported following coughing.3,16 Other cases of spontaneous surgical emphysema were seen in pa-tients with diseases of the lungs, including bronchiol-itis, pneumonia and bronchial asthma.13-15 Most cases however, are idiopathic and no particular cause can be found in them. In this patient, the clinical signs and symptoms were seen following a bout of repeat-ed coughing and we postulate that the cause of the surgical emphysema in this patient was the result of a spontaneous tracheal rupture, most probably in the posterior membranous wall of the trachea. The pathophysiology of spontaneous tracheal rup-ture is poorly understood. In cases following severe coughing, it has been postulated to be caused by high intratracheal pressure, combined with weakening of tracheobronchial tissue by tracheal inflammation such as in tracheobronchitis. Weakening of the tra-cheobronchial tissue by tracheal inflammation, ste-roid medication or age-related regressive changes were factors predisposing to spontaneous tracheal rupture in the reported adult cases.4,6,7,16 The laceration usually occurs longitudinally in the posterior membranous wall of the trachea or at the junction be-tween the membranous wall and cartilaginous ring. Most tracheal ruptures occur in the lower third of the trachea. Another common area of rupture is the crico-thyroid membrane. The development of the clinical signs and symptoms of tracheal rupture depends on the dissection of air through the tissue planes from the initial wound. The air leaking from a laryngotracheal rupture spreads through the soft tissue planes of the neck. This results in subcutaneous emphysema and some respiratory distress from increased compression of the extended airway. The air then dissects downwards to collect in the retropharynx and mediastinum to form a pneu-momediastinum. The degree of airway distress varies with the size of the air leak, the progression of the dissection, and haemodynamic disturbances.17 The mechanism of air leakage from the respirato-ry tract was first described by Macklin on the basis of laboratory animal experiments and is called the “Macklin effect”. An increased pressure gradient between the intra-alveolar and interstitial spaces enhances air leakage from small alveolar openings and ruptured alveoli into the perivascular adventitia causing interstitial emphysema. The pressure gradi-ent favours air dissection along the vascular sheaths towards the hilum. Pneumothorax may occur if the mediastinal pressure rises abruptly. However, be-cause the visceral layers of the deep cervical fascia are continuous with the mediastinum, air usually de-compresses into the neck, preventing physiological tamponade and pneumothorax.17 Tracheal rupture may potentially be life-threatening without early detection and adequate intervention. Tracheal rupture is very difficult to diagnose during a first examination. The initial symptoms may be mild and variable, leading to delays in diagnosis. Dekel et al. showed that the main symptoms of subcutaneous cervical and mediastinal emphysema were neck pain and swelling, chest pain and dyspnoea.10 Pulmonary interstitial emphysema may be involved in pneu-momediastinum and result in an unusual precordial crunching sound known as “Hamman’s sign”.18 Soft tissue neck and chest radiographs are useful for initial diagnosis, but computed tomography is excel-lent to disclose the extension of air and helps to reveal hidden pathologic conditions.19 Chest radiographs may illustrate multiple thin, lucent streaks outlining mediastinal structures, elevating the mediastinal pleu-ra, and often extending into the neck or chest wall. The lateral view of soft tissue radiographs of neck is more sensitive and can visualize air in the retroster-nal space.20 Computed tomography is very sensitive for detecting tracheal rupture or related complications such as subcutaneous emphysema, pneumomediasti-num and pneumothorax. CT scan with the appropriate window settings allows direct visualization of the tracheal injury in 70% of cases. A definitive diagnosis may be made by using fiberoptic endoscopy and it must be performed early if there is any question of an airway rupture, because tracheal rupture is potentially lethal and is sometimes not detected on CT or radiographs that reveal only localized air collections. Oesophagoscopy may be a dangerous procedure, as it may convert a contained oesophageal perforation into a non-contained one. Bronchoscopy facilitates cough and passage of air from the ruptured alveoli to the mediastinum, thus enhancing the pneumomediastinum; hence, some re-ports argue that the procedure should be contraindi-cated. Serial plain radiographs may be sufficient for evaluating the resolution of the air collections, but a follow-up CT can be performed to determine if it is safe to discharge the patient from the hospital after safely excluding the potentially lethal complications of tracheal rupture. In most cases of mediastinal and soft tissue emphy-sema, conservative management is indicated as spon-taneous resolution occurs. Management consists of treating the underlying cause (if identified), rest, anal-gesia and simple clinical monitoring. When no cause is identified, it is reasonable to treat the patient em-pirically with bronchodilators, steroids and oxygen. Antibiotic therapy is not necessary unless the poten-tial for infection exists, in which case broad-spec-trum antibiotic therapy is recommended in an effort to prevent deep neck infection or mediastinitis from the infected respiratory tract. The patient must be cautioned against any form of coughing or straining, which would increase intrathoracic pressure.1 Although most instances of pneumomediastinum and subcutaneous emphysema are physiologically unim-portant, these forms of extra-alveolar air can occa-sionally pose a threat to life. Massive subcutaneous emphysema can be life-threatening if it involves the deeper tissues of the thoracic outlet, chest and abdom-inal wall.When potentially fatal complications result from subcutaneous emphysema, surgical intervention seems indicated. Previous case reports have described successful treatment with tracheostomy, infraclavic-ular “blow holes”, skin incisions and subcutaneous drains.7 In acute tracheal rupture, early surgical repair in healthy tissues appears to be the preferred treatment by most authors, although conservative management is an alternative for patients judged unsuitable for sur-gery and for small tears, with a reasonable chance of spontaneous sealing and uneventful recovery. Non-surgical treatment is advisable in small, uncomplicat-ed tears

    CONCLUSION

    Spontaneous pneumomediastinum and subcutane-ous emphysema is probably a more frequent disorder than usually reported because it is under-diagnosed or mis-diagnosed. It is self-limited and resolves spon-taneously with rest. Spontaneous tracheal rupture can occur in children with respiratory tract infection following a bout of severe coughing. A high degree of clinical suspicion is required and initial soft tissue neck and chest radiographs along with CT examina-tion will help clinch the diagnosis. The treatment is mainly supportive with bed rest, analgesia and oxy-gen administration. A small uncomplicated rupture might resolve without surgical intervention.
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