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Int J Surg Case Rep. 2012;3(3):107-10. doi: 10.1016/j.ijscr.2011.12.001. Epub 2011 Dec 8.

Erdheim-Chester disease: The role of video-assisted thoracoscopic surgery in diagnosing and treating cardiac involvement.

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  • 1Division of cardiovascular Diseases Mayo Clinic Scottsdale, United States.

Abstract

INTRODUCTION:

Erdheim-Chester disease is a rare, non-Langerhans histiocytosis in which pericardial involvement is diagnosed with increasing frequency and is associated with high mortality rates.

PRESENTATION OF CASE:

A 53-year-old woman presented with progressive exertional dyspnea and pericardial effusion was discovered. Further investigations revealed the presence of a diffuse, infiltrating process and a diagnosis of Erdheim-Chester disease was made. An emergent pericardiocentesis by subxiphoid approach was completed but recurrent drainage obviated removal of the pigtail catheter. A pleuro-pericardial window was placed using video-assisted thoracoscopic surgery (VATS) and analysis of the resected specimen confirmed pericardial involvement.

DISCUSSION:

In this case, high pericardial fluid output demanded definitive treatment of the pericardial effusion. Traditionally this would be completed via thoracotomy. VATS is a minimally invasive alternative which permits exploration of the thoracic cavity and the creation of a pleuropericardial window.

CONCLUSION:

We describe, for the first time, the successful use of VATS for both diagnostic confirmation and therapeutic relief of recurrent pericardial fluid drainage due to pericardial involvement by Erdheim-Chester disease.

KEYWORDS:

Erdheim–Chester; Histiocytosis; Pericardial effusion; VATS

PMID:
22288060
[PubMed]
PMCID:
PMC3267285
Free PMC Article
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