Chest
CHEST PearlsA 34-Year-Old Man With a Chylothorax and Bony Pain
Section snippets
Physical Examination Findings
On physical examination his oxygen saturation was 91% on room air; respiratory rate, 28 breaths/min; and heart rate, 106 beats/min. There was no evidence of clubbing and no palpable lymphadenopathy. The chest was dull to percussion bilaterally and on auscultation there was decreased air entry at both lung bases. The remainder of his examination produced unremarkable results.
Diagnostic Studies
Chest radiography revealed bilateral pleural effusions (Fig 1). There was a slightly elevated WBC count (12.9 × 109 cells/L) with neutrophil predominance (11.27 × 109 cells/L), and Pao2 measured on arterial blood gas sampling was 68 mm Hg. CT pulmonary angiography revealed bilateral pleural effusions, with no evidence of thromboembolism (Fig 2). Thoracentesis yielded opaque milky-appearing pleural fluid characteristic of chylous material, and subsequent biochemical analysis revealed an exudate,
Discussion
Generalized lymphatic anomaly (GLA), also called pulmonary lymphangiomatosis or diffuse pulmonary lymphangiomatosis, is a rare idiopathic condition characterized by a nonneoplastic proliferation of lymphatic vessels. Patients frequently display lymphatic abnormalities in their skin, soft tissues, and abdominal and thoracic viscera. These lymphatic changes can cause organ dysfunction and pericardial, pleural, or peritoneal effusions, associated with significant morbidity. Up to 75% of patients
Clinical Pearls
- 1.
GLA is a rare but important cause of chylothorax, and there remains considerable scope for advancements in the diagnosis and treatment of this complex lymphatic disorder.
- 2.
GLA should be considered in any patient with bone lesions and a pleural effusion without evidence of malignancy.
- 3.
Histologic findings of significant lymphatic proliferation and positive AE1/3 and D2-40 staining are consistent with GLA.
- 4.
Sirolimus is a potential noninvasive treatment that may have significant benefit in this rare
Acknowledgments
Financial/nonfinancial disclosures: None declared.
Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.