Recurrent and rapidly occurring pericardial tamponade in Erdheim Chester disease

Abstract

A B S T R A C TErdheim Chester disease is a very rare histiocytic disorder characterised by tissue infiltration by lipid laden histiocytes. The most common presentation is bone pains typically involving the long bones. Over time almost 50% of the patients develop extraosseous involvement. The prognosis depends on the extent and distribution of the extraskeletal manifestations. Cardiovascular involvement is seen in up to 40% of the patients and the most common manifestations are periaortic fibrosis and pericardial involvement. Respiratory distress, extensive pulmonary fibrosis, and cardiac failure are the most common causes of death in these patients. Cardiac tamponade has also been documented to cause death in these patients. We describe a patient of Erdheim Chester disease who presented with recurrent and very rapidly occurring cardiac tamponade in a short duration of time and benefited from timely recognition and management.

Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

“…Thus the management of these is based on anecdotal case reports. Various approaches have been tried, these include repeated pericardiocentesis, failing which there is pericardial window and, finally, surgical pericardiectomy 1 18 19. Our patient, in spite of having multiple pericardiocentesis and pericardial window, continued to experience clinical deterioration.…”

“…Most common cardiovascular manifestations are periaortic fibrosis and pericardial diseases. Periaortic disease presents as fibrosis of the aortic adventitial layer and is called ‘coated aorta’ 1 3. Pericardial disease can be in the form of pericarditis, pericardial effusion or pericardial tamponade 3.…”

“…Myocardial infiltration, myocardial infarction secondary to involvement of the coronary arteries, renovascular hypertension secondary to involvement of the renal artery, involvement of the interatrial septum, valvular abnormality (aortic and mitral regurgitation) and conduction abnormalities are the uncommon cardiovascular manifestations reported in the literature 2 15 16. Cardiovascular involvement has been attributed as a common reason for morbidity and poor outcome 1 15…”

“…Most commonly, ECD presents with bone pain secondary to bony involvement. Cardiac involvement in the form of periaortic fibrosis and pericardial involvement is seen in up to 40% of patients 1. Pericardial involvement can be severe in the form of rapid, recurrent, pericardial tamponade, and can be a cause of increased morbidity and mortality.…”

Management of a patient with pericardial decompression syndrome and HOCM

“…Most of the cases encountered in the current scientific literature describe patients having malignant effusion 1–3. Cases of PDS associated with infectious,3 endocrine5 and traumatic6 effusions are less frequent. In our case, neoplasia could be ruled out (clinically, CT scan, and laboratory analysis) and the effusion fully resolved after non-steroidal anti-inflammatory drugs (NSAID) and adjuvant antibiotic therapy (intravenous antibiotics had been initially administered to the patient because of transient CRP and leucocyte count elevation); therefore, we hypothesised that the effusion had been inflammatory or parainfectious in origin.…”