Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=26090116
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis
#MMPMID26090116
Ellender CM
; Williams TJ
; Gooi J
; Snell GI
; Whitford HM
Respirol Case Rep
2015[Jun]; 3
(2
): 72-4
PMID26090116
show ga
This case reports the successful management of chylothorax in a non-transplanted
patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy
failed, including total parenteral nutrition, pleural drainage, surgical
pleurodesis, and pleurectomy. Commencement of sirolimus 2?mg daily (2?mg
alternating days had failed) led to resolution of chylothorax after 20 days.
Discontinuation of sirolimus for abdominal surgery led to recurrence of the
chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion,
stabilization of lung function, and there has been no recurrence in the ensuing 4
years. We conclude that sirolimus should be considered in the management of
pLAM-related chylothorax, perhaps before surgical intervention.