From Mayo-Scottsdle: VATs for diagnosing Erdheim Chester disease

http://www.ncbi.nlm.nih.gov/pubmed/22288060

Int J Surg Case Rep. 2012;3(3):107-10. Epub 2011 Dec 8.
Erdheim-Chester disease: The role of video-assisted thoracoscopic surgery in diagnosing and treating cardiac involvement.
Egan A, Sorajja D, Jaroszewski D, Mookadam F.
Source
Division 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.