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.