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

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.