Rosai-Dorfman Disease as Constrictive Pericarditis: An Unusual Childhood Presentation.

Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.

Idiopathic Chylous Pericardial Effusion Due to Lymphatic Malformation of Mediastinum.

Chylopericardium can be due to a variety of secondary causes like trauma, radiation, tumors, following cardiac surgery, etc., or may be idiopathic due to abnormal lymphatic system and mediastinal lymphangiectasia, which is a rare entity. Here, we present a case of a 34-year-old previously healthy male presenting with idiopathic chylopericardium. 2D echocardiography revealed massive pericardial effusion without features of cardiac tamponade. Following pericardiocentesis, a CT scan of the thorax and MR lymphangiogram were done to arrive at a diagnosis of idiopathic chylopericardium. In addition to medical management, surgical treatment included partial pericardiectomy and sclerotherapy of the mediastinal lymphatic sac. The patient had an uneventful post-operative period.