Patient D
This patient was a 4-week old infant born at 32 weeks at an outside hospital due to hydrops fetalis with Trisomy 21 and bilateral chylous pleural effusion secondary to lymphatic malformation. She was initially treated with octreotide at the outside facility but due to significant persistent chylous effusions even after two weeks, she was transferred to TCH and transitioned here to sirolimus at a starting dose of 0.8 mg/m2/day PO. There was large volume of chest tube output of 550 ml on day 0 which gradually decreased to 300 ml a week later, to less than 100 ml by day 15 and ultimately 10 ml on day 19, and chest tube removed on day 20 (Fig 1). Levels were supratherapeutic at the time of chest tube removal at 23 ng/ml, but no sirolimus-related toxicity noted. The patient was transitioned to comfort care measures only due to inability to control fluid overload, and passed away at 3.5 months of age.