More about Blebs, Bullae and Spontaneous Pneumothorax

This is part of an ongoing discussion at Cirugia de Torax, with periodic updating and additions.

Recommended Reading:

Haynes D, Baumann MH (2011).  Pleural controversy: aetiology of pneumothorax.  Respirology. 2011 May;16(4):604-10. doi: 10.1111/j.1440-1843.2011.01968.x   If you are only going to read one article about primary spontaneous pneumothorax (PSP), this article by researchers at the University of Mississippi is recommended.

Blebs and Bullae: part two

Since there has been a lot of interest in the initial posts on blebs, bullae and blebectomies from around the world, this post has been designed to provide readers with additional resources and information.

One of the most frequent inquiries has been related to pain after thoracic surgery.

Another frequently asked question has been about the etiology or causes of bleb disease, so part of this discussion includes a list of some of the lesser known/ discussed causes of bleb disease.

Pneumothorax without a cause?

Frustrating as it is for many patients, in primary spontaneous pneumothorax (PSP), there may be no known underlying condition or cause.  Much of what we do know, may be just speculation and hypothesis (Dejene, Ahmed, Jack, Anthony 2013).

In other cases, the cause of spontaneous pneumothorax may be detected by underlying lung tissue changes on CT scan.  More rare, or lesser known causes of spontaneous pneumothorax may be more insidious.

Who this affects/ who may need additional testing:  Individuals with ONE isolated spontaneous pneumothorax with no signs of lung disease on CT scan will not need additional testing.  However,  in patients with multiple, bilateral pneumothoraces or strong family history of such conditions, additional testing may be warranted.

However, I caution patients not to attempt to self-diagnose based on these articles, but to use this material to prompt more earnest discussions with their medical providers.

Diagnosis:

Many, if not all of these conditions will require additional testing such as CT scan, lung biopsy or genetic marker or serum testing.  For systemic conditions or conditions that also affect areas outside the lung (such as the skin lesions in Birt-Hogg-Dube syndrome), pathological and radiological examination of other areas of the body may be required to reach a diagnosis.

Less common causes of bleb disease and spontaneous pneumothorax:

Birt-Hogg-Dube syndrome:  this is a genetic condition, (thus often runs in families).  It is “characterised by fibrofolliculomas (skin tumors), renal tumours, pulmonary cysts and pneumothorax” (Furuya M, Nakatani Y. (2013).

This paper by Furuya & Nakatani, Japanese researchers describes more about the clinical and pathological features of this condition.

Furuya M, Nakatani Y. (2013). Birt-Hogg-Dube syndrome: clinicopathological features of the lung.  Clin Pathol. 2013 Mar;66(3):178-86. doi: 10.1136/jclinpath-2012-201200. Epub 2012 Dec 8. Review.

Pulmonary alveolar proteinosis (PAP): this group of disorders is characterized by the build-up of fatty proteins in the lung tissue.  Spontaneous pneumothorax is a rare complication of this rare disease.  (It’s actually rare enough to be categorized as an ‘orphan’ disorder).

Campo I, Mariani F, Rodi G, Paracchini E, Tsana E, Piloni D, Nobili I, Kadija Z, Corsico A, Cerveri I, Chalk C, Trapnell BC, Braschi A, Tinelli C, Luisetti M. (2013). Assessment and management of pulmonary alveolar proteinosis in a reference center.   Orphanet J Rare Dis. 2013 Mar 13;8:40. doi: 10.1186/1750-1172-8-40.  This Italian article discusses findings over twenty years of study, as well as the process of diagnosing / treating this disorder.

Treatment of PAP:

Stoica R, Macri A, Cordoş I, Bolca C. (2012).  Whole lung lavage for pulmonary alveolar proteinosis after surgery for spontaneous pneumothorax.  J Med Life. 2012 Sep 15;5(3):311-5. Epub 2012 Sep 25.  Article has several images of CT, pathology and radiographic findings in this case report.

Bullous lung disease:

Vanishing lung syndrome, (type I bullous disease): In this condition, the appearance of a large bullae on chest radiographs may mimic the appearance of a pneumothorax.  Placement of a chest tube can actually worsen the situation by unintentionally rupturing the intact bullae.  (On a chest X-ray it can be difficult to see a difference between a large intact air ‘bubble’ and a collapsed lung). If these bullae should rupture, the patient will have a pneumothorax.  This condition is usually diagnosed in young men with a smoking history.

Tsao YT, Lee SW. (2012). Vanishing lung syndrome.  CMAJ. 2012 Dec 11;184(18):E977. doi: 10.1503/cmaj.111507. Epub 2012 May 22.  Short Taiwanese case report with radiographic images.

Núñez Delgado Y, Eisman Hidalgo M, Valero González MA.  (2012).  Primary bullous disease of the lung in a young male marijuana smoker. Arch Bronconeumol. 2012 Nov;48(11):428-9. doi: 10.1016/j.arbres.2012.04.024. Epub 2012 Jun 15.  Article also available in Spanish, “Enfermedad primaria bullosa del pulmón en varón joven fumador de cannabis”.  Article discusses how smoking marijuana harms the lungs in additional ways in comparison to tobacco smoking, and leads to the formation of bullous lung disease.

References/ Additional Reading

Gunnarsson SI, Johannesson KB, Gudjonsdottir M, Magnusson B, Jonsson S, Gudbjartsson T. (2012).  Incidence and outcomes of surgical resection for giant pulmonary bullae–a population-based study.  Scand J Surg. 2012;101(3):166-9.  Small Icelandic study of 12 patients and their outcomes after bullectomy.

Hamilton N, Hills MA. (2012).  Medical Image: The Vanishing lung.  NZ Med J. 2012 Sep 21;125(1362):92-4. Case report of very large bullae with radiographs, CT images and discussion of diagnosis and treatment.

Is pneumothorax related to depletion of the Ozone layer?

Note:  As many readers are aware, I am a nurse in thoracic surgery, not pulmonology, so much of the diagnosis of the cause of blebs and bullae are outside of my area of expertise.  However, I hope that this post serves as a resource for patients in pursuing discussions with their clinicians after spontaneous pneumothorax, particular in cases of repeated pneumothorax or unknown etiology. 

Pulmonologists, pathologists and thoracic surgeons are invited to provide additional comments.