Chart Review Protocol
Data were collected from the patients’ electronic medical records.
Charts were reviewed for demographic information, including age and
gender, volume of chest tube output, sirolimus blood level, duration of
chest tube placement, other medical and dietary interventions, lymphatic
anomaly diagnosis, and co-morbidities. Day 0/Hour 0 was the time of
first sirolimus dose. The data from the patients’ charts were recorded
in an Excel spreadsheet with all patient identifiers removed.
Chylous effusion was diagnosed by pleural fluid analysis findings
consistent with lymphocyte content of >80%, pleural fluid
triglyceride level >110 mg/dL and ratio of pleural fluid to
serum cholesterol <1.0. The vascular anomalies team was
involved in the care of all these patients. Our institutional guidelines
for use of Sirolimus are as follows. The starting dose of sirolimus is
based on age (0.4mg/m2/dose for patients younger than 6 months and
0.8mg/m2/dose PO or NG/G-tube q12h). Sirolimus trough levels of 8-12
ng/mL are considered therapeutic. A trough level is checked after 72
hours to evaluate for toxicity as some newborns can reach the upper
level of the range or even toxic troughs very early. In this case, the
sirolimus is held and levels checked until they are back in range and
dose adjusted/decreased as needed until reaching goal. If the level is
low or within range, it is checked again at 1 week and adjusted at that
point. During hospitalization, all patients had sirolimus levels at
least weekly. Administration was interrupted during proven or suspected
infections and restarted immediately afterwards.