SESSION TITLE: Interstitial Lung Disease Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 27, 2013 at 07:30 AM - 08:30 AM
INTRODUCTION: We present a case of a patient with ipsilateral pulmonary fibrosis after lung herniation.
CASE PRESENTATION: An 82 year old, Hispanic woman presented to the pulmonary clinic for evaluation of an abnormal chest-CT. She had a history of asthma since childhood and a sudden cardiac arrest in 1997 treated with cardiopulmonary resuscitation. After this event, chest imaging revealed a moderate-sized intercostal herniation of the right upper and middle lobes. She reported chronic cough and dyspnea on exertion. Her medications included albuterol, ipratropium, and theophylline as well as ambulatory oxygen. On physical exam, all vital signs including oxygen saturation were normal, and her lung exam revealed the presence of dry, “velcro” crackles isolated to the right lung along with a non-tender, palpable, 6 x 3 cm area of crepitance over her right anterior chest wall. A review of her chest imaging revealed evidence of diffuse right lung fibrotic changes since 2008. The fibrosis was characterized by stacked cystic spaces, traction bronchiectasis, interlobular septal thickening, and architectural distortion. The portion of herniated lung also contained these fibrotic changes. Her left lung appeared largely normal. Her pulmonary function testing was limited due to her inability to participate with the test. The only acceptable measurement was an FEV1 of 0.74 L (47% of predicted).
DISCUSSION: Lung herniation is a rare phenomenon with approximately 40 cases documented in the literature. It is defined by protrusion of lung parenchyma outside of the thoracic cavity, and usually follows a thoracic wall injury or increased intrathoracic pressure such as coughing. Curative strategies involve surgical correction of the thoracic wall defect. Since this patient had fibrosis solely in the hernia-containing lung, this suggests that her pulmonary fibrosis may have been caused by the herniation itself. Such focal injuries are theorized to give rise to a focal interstitial pulmonary fibrosis mediated by “fibroblast foci” possibly leading to an asymmetric interstitial fibrosis pattern. However, in none of the reported cases of lung herniation was an association with pulmonary fibrosis reported. If this association is instead a cause, then a possibility exists that earlier surgical correction may have prevented the progression of her fibrosis.
CONCLUSIONS: Lung herniation can possibly cause ipsilateral pulmonary fibrosis.
Reference #1: Sarkar D, Warta M, Solomon J. Case Report: Transdiaphragmatic Intercostal Herniation following Blunt Trauma. Case Reports in Radiology, Volume 2012: Article ID 502765
Reference #2: Matthew O’Shea M, Cleasby M. Lung Herniation after Cough-Induced Rupture of Intercostal Muscle. New England Journal of Medicine 366:1. January 2012
Reference #3: Gross TJ , Hunninghake GW. Idiopathic Pulmonary Fibrosis. New England Journal of Medicine 347: Volume 7. August 2007
DISCLOSURE: The following authors have nothing to disclose: Charles Peng, Nan Li, Elizabeth Awerbuch, Pierre Kory, Albert Miller
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