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J Thorac Cardiovasc Surg 2009;138:497-498
© 2009 The American Association for Thoracic Surgery

Brief Clinical Report

Treatment of refractory lymphangioleiomyomatosis–associated chylous effusion with a pleuroperitoneal window and omental flap

^a Division of Thoracic Surgery, Department of Surgery, Brigham & Women's Hospital, Boston, Mass
^b Division of Translational Medicine, Department of Medicine, Brigham & Women's Hospital, Boston, Mass

Received for publication February 18, 2008; revisions received February 25, 2008; accepted for publication March 2, 2008.

^_* Address for reprints: Raphael Bueno, MD, Brigham & Women's Hospital, Division of Thoracic Surgery, 75 Francis St, Boston, MA 02115. (Email: rbueno@partners.org).

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

	Introduction

 
Lymphangioleiomyomatosis (LAM) is a rare progressive cystic disease of the lung that predominantly affects women. Clinical features of the disease include spontaneous pneumothoracices, dyspnea, cough, as well as the formation of chylous effusions. These effusions are often refractory to treatment. We describe a case of a 74-year-old woman with LAM associated chylous effusions that was refractory to standard treatments including bowel rest with total parental nutrition (TPN), thoracic duct ligation, and pleurectomy but was successfully managed with a novel combination of oral progesterone, rapamycin, and doxycycline combined with the creation of a pleuroperitoneal shunt with omental flap placement in the pleural cavity.

	Clinical Summary

 
A 74-year-old woman presented with recurrent chylopericardium and chylothorax. Thirty years earlier, she had 3 left pneumothoraces treated by means of pleurodesis, with findings of "blisters" on her lungs. At age 71 years, she experienced abdominal discomfort, abdominal computed tomographic scanning showed a complex retroperitoneal infiltrative mass, and biopsy showed lymphangioma. Three years later, she presented with dyspnea and was found to have bilateral pleural effusions, a pericardial effusion, and diffuse cystic parenchymal changes on chest computed tomographic scanning (Figure 1, A). Echocardiographic analysis revealed pericardial tamponade, which was drained for 600 mL of chyle. . . . [Full Text of this Article]