Medical Image of the Week: Spontaneous Pneumothorax in End Stage Fibrotic Lung Disease

Figure 1. Chest x-ray showing moderate-sized right pneumothorax with a pigtail chest tube in place, diffuse reticular interstitial opacities.

Figure 2. Chest CT showing extensive centrilobular emphysema, moderate right pneumothorax with pigtail chest drain on the right, subpleural reticular opacities with peripheral and basilar preponderance suggesting interstitial fibrotic lung disease, and diffuse lung cysts - heterogenous in size.

A 61-year-old nonsmoking man with chronic obstructive lung disease, pulmonary hypertension, pulmonary fibrosis, hypertension, coronary artery disease with congestive heart failure, presented with recurrent pneumothorax, pneumomediastinum, extensive subcutaneous emphysema and bronchopleural fistula.

The patient reported ongoing symptoms of exertional dyspnea, fatigue, and coughing for years. His environmental exposures were notable for exposure to birds since early childhood. He had 6 cockatiels and 2 doves living inside his home and is directly responsible for their care. Former occupational exposures include painting and sandblasting. Family history was notable for early onset non specified lung disease in his father, and rheumatoid arthritis in his mother.

Lung function testing performed prior to the bronchopleural fistula revealed moderate obstructive ventilatory defect with severely limited DLCO. Chest x-ray (Figure 1) revealed a moderate-sized right pneumothorax with a pigtail chest tube in place and diffuse reticular interstitial opacities. His CT chest (figure 2) revealed extensive subcutaneous emphysema, diffuse lung cysts that are heterogenous in size, and subpleural reticular opacities with peripheral and basilar preponderance. Bronchoalveolar lavage revealed no infection, with predominant monocyte/ macrophages. Alpha-1 antitrypsin (A1AT) was normal, as were autoimmune panels. A hypersensitivity pneumonitis panel revealed positive IgG to Aureobasidium pullulans. A presumptive diagnosis of chronic hypersensitivity pneumonitis was made.

Spontaneous pneumothorax (SP), a potentially life-threatening complication, is defined by the accumulation of air in the pleural space with secondary lung collapse, and can be categorized as primary (without apparent lung disease) or secondary pneumothorax. While chronic obstructive pulmonary disease and Pneumocystitis jirovecii pneumonia are the most common causes of secondary spontaneous pneumothorax, other structural lung diseases such as fibrotic lung diseases have also been linked to SP. Interstitial lung diseases distort lung architecture and trigger formation of subpleural blebs that are susceptible to rupture leading to extra-alveolar air collection and air leakage in the pleural space. Presence of persistent air leak, as in our case, mandates surgical consideration to accelerate recovery and prevent recurrence of secondary SP.

Roula Altisheh MD and Tara Carr MD

Division of Pulmonary, Allergy, Critical Care and Sleep Medicine

Banner-University Medical Center

Tucson, AZ USA

References

1. Sahn S, Heffiner J. Spontaneous Pneumothorax. N Engl J Med 2000; 342:868-74 [CrossRef] [PubMed]
2. Onuki T, Ueda S, Yamaoka M, Sek iya Y, Yamada H, Kawakami N, Araki Y, Wakai Y, Saito K, Inagaki M, Matsumiya N. Primary and secondary spontaneous pneumothorax: prevalence, clinical features, and in-hospital mortality. Can Respir J. 2017: 6014967.  [CrossRef] [PubMed]
3. Koschel D, Handzhiev S, Cardoso C, Rolle A, Holotiuk O, Höffken G. Pneumomediastinum as a primary manifestation of chronic hypersensitivity pneumonitis. Med Sci Monit. 2011 Dec;17(12):CS152-5. [PubMed]
4. Ichinose J, Nagayama K, Hino H, et al. Results of surgical treatment for secondary spontaneous pneumothorax according to underlying diseases. Eur J Cardiothorac Surg. 2016;49(4):1132–6. [CrossRef] [PubMed]

Cite as: Altisheh R, Carr T. Medical image of the week: spontaneous pneumothorax in end stage fibrotic lung disease. Southwest J Pulm Crit Care. 2017;14(6):308-10. doi: https://doi.org/10.13175/swjpcc065-17 PDF 

Medical Image of the Week: Spontaneous Pneumomediastinum

Figure 1. Upright chest radiograph showing pneumomediastinum tracking into neck and small right apical pneumothorax (arrows).

Figure 2. Coronal slice of CT chest showing extensive pneumomediastinum and subcutaneous emphysema (arrows).

Figure 3. CT scan of chest showing the Macklin effect with air tracking along the bronchovascular sheath in the left lower lobe.

A 24-year-old man with a past medical history significant for type I diabetes mellitus presented to the emergency department with complaints of nausea and vomiting for several days. He reported he had been on drinking alcohol heavily 4 days prior to presentation and subsequently had multiple episodes of vomiting. Initial laboratory evaluation was consistent with diabetic ketoacidosis (DKA). A routine chest x-ray was obtained to evaluate for an infectious etiology of his DKA and revealed pneumomediastinum and a small right apical pneumothorax (Figure 1). A CT scan of the chest was done and showed extensive pneumomediastinum as well as air tracking along the bronchovascular sheaths in the left lower lobe (Figure 2 and 3). It did not reveal evidence of esophageal injury.

Spontaneous pneumomediastinum (SPM) refers to pneumomediastinum that is not associated with noticeable cause such as esophageal rupture or trauma. It is typically a benign condition thought to be due to alveolar rupture and subsequent air tracking along the bronchial tree (1). It has been associated with a number of conditions including asthma, DKA, anorexia nervosa, and other conditions that lead to excessive coughing or vomiting. The radiographic appearance of air dissecting through the pulmonary intersitium along the bronchovascular sheath is known as the Macklin effect and can be seen in Figure 3.

Spontaneous pneumomediastinum typically resolves without complications but must be differentiated from the much more serious diagnosis of esophageal rupture, or Boerrhaave’s syndrome. Boerrhaave’s syndrome is more likely to present with fever, hemodynamic instability, and hydropneumothorax. All patients presenting with suspected SPM should be evaluated for esophageal perforation with a radiographic contrast swallow (2). In our case it was negative for evidence of esophageal disruption and the patient recovered completely.

Lucie Griffin DO and Erik Kraai MD

Division of Pulmonary, Critical Car, and Sleep Medicine

University of New Mexico Health Sciences Center

Albuquerque, NM USA

References

1. Murayama S, Gibo S. Spontaneous pneumomediastinum and Macklin effect: Overview and appearance on computed tomography. World J Radiol. 2014 Nov 28;6(11):850-4. [CrossRef] [PubMed]
2. Kelly S, Hughes S, Nixon S, Paterson-Brown S. Spontaneous pneumomediastinum (Hamman's syndrome). Surgeon. 2010 Apr;8(2):63-6. [CrossRef] [PubMed] 

Cite as: Griffin L, Kraai E. Medical image of the week: spontaneous pneumomediastinum. Southwest J Pulm Crit Care. 2016 Mar;12(3):115-6. doi: http://dx.doi.org/10.13175/swjpcc015-16 PDF