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CASE REPORT
Lymphangioleiomyomatosis manifesting as refractory chylothorax and chyloperitoneum
  1. Yee-Shiuan Chen and
  2. Pauras Memon
  1. Division of Medical Education, Department of Medicine, Barnes-Jewish Hospital, Saint Louis, Missouri, USA
  1. Correspondence to Dr Yee-Shiuan Chen, yee-shiuan{at}wustl.edu

Abstract

This is a rare case of sporadic lymphangioleiomyomatosis (S-LAM) manifesting as refractory chylothorax and chyloperitoneum. A middle-aged woman with unremarkable medical history presented with respiratory failure, abdominal distension and anasarca. She was found to have high-output chylous effusion that required chest tube drainage, as well as chylous ascites. Notably initial chest and abdominal CT did not reveal characteristic pulmonary cysts or the presence of angiomyolipomas suggestive of LAM. An extensive oncologic and infectious work-up was undertaken with negative findings. The chylous effusion was persistent and refractory to thoracic duct embolization, total parenteral nutrition with octreotide, and talc pleurodesis. Diagnosis of S-LAM was confirmed after repeat chest CT showed subtle pulmonary cystic changes, and serum vascular endothelial growth factor-D level was found to be elevated at 834 pg/mL. Patient was started on sirolimus therapy, but lost to follow-up after hospital discharge. Patient died approximately 1 year later.

  • respiratory medicine
  • medical management
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Footnotes

  • Contributors Y-SC and PM wrote the manuscript. Y-SC obtained consent and prepared the figure.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Next of kin consent obtained.

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