Genetic and Developmental Disorders: Fellow Case Report Poster - Genetic and Developmental Disorders |

Pulmonary Sequestration: A Congenital Abnormality Seen in the Adult Pulmonologist’s Office FREE TO VIEW

Laurie Manka, MD
Author and Funding Information

National Jewish Health, Denver, CO

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

Chest. 2016;150(4_S):641A. doi:10.1016/j.chest.2016.08.734
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SESSION TITLE: Fellow Case Report Poster - Genetic and Developmental Disorders

SESSION TYPE: Affiliate Case Report Poster

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

INTRODUCTION: Pulmonary sequestration is a rare congenital malformation. Diagnosis is often delayed until the 2nd or 3rd decade. Common symptoms including recurrent infections and hemoptysis. Surgical resection is usually recommended due to the risk of massive hemoptysis. The following is a case of a 42 year old male presenting with his sentinel event of sequestration, which underscores the importance of adult pulmonologists recognizing congenital conditions.

CASE PRESENTATION: A 42 year old male, with minimal past medical history, presented with small volume hemoptysis. In the Emergency Department, he was hemodynamically stable without hypoxia. CT of the chest with IV contrast was obtained due to the concern of pulmonary embolus. The CT showed ground glass infiltrates in the medial right lower lobe with an aberrant feeding vessel arising from the thoracic aorta. He was discharged in stable condition with pulmonary follow up.

DISCUSSION: Pulmonary sequestration is a rare finding accounting for about only 5% of congenital pulmonary malformations. It is characterized by dysplastic lung tissue fed by an aberrant artery arising directly from the aorta. In extralobar sequestration, there is a distinct visceral pleura surrounding the dysplastic parenchyma. It is usually diagnosed in vitro, but often does not cause symptoms until adulthood. On imaging, it is commonly seen as a mass because it is rarely aerated. Intralobar sequestration, on the other hand, is more common and is not surrounded by an independent visceral pleura. It is more likely to result in recurrent infection, and therefore, earlier presentation in childhood. Intralobar sequestration imaging shows aerated but abnormal lung tissue. Both forms of sequestration usually require surgical resection due to the risk of massive, life-threatening hemoptysis. Standard thoracotomy or video assisted thoracic surgery (VATS) can be performed. There is limited data but increasing interest in endovascular intervention in adults.

CONCLUSIONS: Pulmonary sequestration is a congenital malformation, but often presents in the adult pulmonologist’s office. Recognition is imperative because intervention can be life-saving.

Reference #1: Qian X, Sun Y, Liu D, Wu X, Wang Z, and Tang Y. 2015. Pulmonary sequestration: a case report and literature review. Int J Clin Exp Med. 8(11):21822-21825.

Reference #2: Leonici G, Rossi UG, Ferro C, and Chessa L. 2011. Endovascular treatment of pulmonary sequestration in adults using Amplatzer® vascular plugs. Interact Cardiovasc Thorac Surg. 12:98-100.

DISCLOSURE: The following authors have nothing to disclose: Laurie Manka

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