Obstructive Lung Diseases: Fellow Case Report Poster - Obstructive Lung Disease |

Swyer James Syndrome: An Unusual Masquerader of Pulmonary Embolism FREE TO VIEW

Anil Singh, MBBS; Tina Dudney, MD; Michael McCormack, MD; JF Turner, MD
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UTMCK, Knoxville, TN

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):905A. doi:10.1016/j.chest.2016.08.1005
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SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Swyer James syndrome (SJS) is a unilateral acquired hypoplastic lung disease due to childhood infectious obliterative bronchiolitis which can remain undiagnosed until adulthood and may mimic pulmonary embolism. We report a case of SJS fasely diagnosed as pulmonary embolism.

CASE PRESENTATION: A 61-year old female with past history of hypogammaglobinemia, recurrent bronchitis, obstructive sleep apnea and unprovoked left lower lobe pulmonary embolism five prior to admission. She presented to Emergency Department with acute onset chest pain. Work up for acute coronary syndrome was negative. Subsequently she underwent ventilation perfusion lung scan which revealed left lower lobe mismatched perfusion defect suggestive of acute pulmonary embolism. Due to lack of risk factors and negative hypercoagulable work up, patient underwent CT angiogram of chest which showed no evidence of pulmonary arterial filling defects but a diminutive left lower lobe pulmonary artery with decreased overall size of left hemithorax, peripheral scarring, atelectasis consistent with SJS. Further history revealed that patient had history of recurrent respiratory tract infection as childhood. We hypothesized that her history of left lower lobe pulmonary embolism was mostly likely due to underlying hypoplastic left pulmonary artery and not true pulmonary thromboembolic disease. She was treated symptomatically for musculoskeletal pain and discharged to follow up with pulmonary clinic.

DISCUSSION: Swyer James syndrome was first described by Swyer and James in 19531. This is a rare condition characterized by unilateral hyperlucency of partial or complete lung secondary to infectious bronchiolitis acquired as childhood leading to arrested lung growth with resulting regional post obstructive hyperinflation and pulmonary artery hypoplasia. This disparity may explain mismatched perfusion defects seen in V/Q scan in our patient similar to another reported case2.

CONCLUSIONS: Although usually diagnosed in early childhood, SJS may remain undiagnosed until later in life and continue to be a source of confusion and misdiagnoses due to its unique regional ventilation and perfusion characteristics.

Reference #1: Sawyer PR, James GC. A case of unilateral pulmonary emphysema. Thorax. 1953;8:133-6.

Reference #2: Misdiagnosis of pulmonary embolism. Q J Med 2007; 100:245.

DISCLOSURE: The following authors have nothing to disclose: Anil Singh, Tina Dudney, Michael McCormack, JF Turner

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