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J Thorac Cardiovasc Surg 2008;136:217-218
© 2008 The American Association for Thoracic Surgery

Brief Communication

Unmasked diabetes insipidus after pericardial drainage and biopsy for pericardial effusion in association with Erdheim–Chester disease

^a Cardiothoracic Section, Anesthesiology and Critical Care, University of Pennsylvania School of Medicine, Philadelphia, Pa
^b Department of Cardiothoracic Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pa

Received for publication October 10, 2007; revisions received February 21, 2008; accepted for publication March 2, 2008.

^_* Address for reprints: John G. T. Augoustides, MD, FASE, Cardiothoracic Section, Anesthesiology and Critical Care, Dulles 680, HUP, 3400 Spruce St, Philadelphia, PA 19104-4283. (Email: yiandoc@hotmail.com).

The first 20% of the full text of this article appears below.

Erdheim–Chester disease (ECD) is a rare form of histiocytosis that is characterized by the absence of Langerhans cells.¹ Although long bone involvement is almost universal, more than 50% of patients also have extraskeletal involvement, including the orbit (exophthalmos), pituitary gland (diabetes insipidus), and pericardium (effusion).^1,2 We present a case of symptomatic pericardial effusion in a young woman with established ECD. After a subxiphoid pericardial window and biopsy under general anesthesia, the patient presented with severe diabetes insipidus in the postanesthesia care unit. To the best of our knowledge, this is the first report of this presentation as a perioperative manifestation of extraskeletal ECD.

Clinical Summary

A 27-year-old woman with established ECD presented with a symptomatic moderate pericardial effusion with echocardiographic features of cardiac tamponade. Because of her widespread ECD, she had bilateral exophthalmos and chronic bilateral femorotibial pain. She had never been given a diagnosis of diabetes insipidus.

The patient was scheduled for pericardial drainage, biopsy, and window creation to drain the symptomatic effusion, to obtain tissue for histology, . . . [Full Text of this Article]