Demographics
The preliminary database consisted of twelve charts identified as eligible patients. Of those patients, seven met the inclusion criteria and were included in the final analysis. The remaining patients did not have chest tube output data available post sirolimus initiation and therefore were excluded. Age at diagnosis ranged from 3 days to 8 weeks old. There were five males (71.4%) and two females (28.6%). Patient A had a central conducting lymphatic anomaly (CCLA). Patient C was suspected RASopathy syndrome, in particular concern for Costello syndrome (but genetic studies were not completed). The other five patients had clinical diagnosis of generalized lymphatic anomalies, but due to their critical state, no lymphangiogram was performed to fully investigate the integrity of the lymphatic central system. The cohort of patients described have a very high morbidity index, are newborns (some premature) and the prognosis was poor from diagnosis. Three patients (two, three and four weeks old at sirolimus initiation) have since died at 2 months, 8 months and 3.5 months of age respectively due to severe co-morbidities. Other four patients are alive at the time of writing this manuscript.