Lymphangioleiomyomatosis manifesting as refractory chylothorax and chyloperitoneum

Yee Shiuan Chen, Pauras Memon

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Article numbere229958
JournalBMJ Case Reports
Volume12
Issue number7
DOIs
StatePublished - Jul 1 2019
Externally publishedYes

Keywords

  • medical management
  • respiratory medicine

ASJC Scopus subject areas

  • Medicine(all)

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