Patient B
This patient was a 6-week old baby boy born at 37 weeks gestational
age, diagnosed in utero with low urinary tract obstruction and prune
belly syndrome s/p placement of vesico-amniotic shunts x3. At birth, he
was found to have bilateral pneumothoraces, pneumomediastinum and
pneumopericardium and bilateral chylous effusions s/p bilateral pigtail
placement. He also required hemodialysis 3-4 times/week. Due to
significant chylous effusion and inability to clamp or remove the chest
tubes, the vascular team was involved and treatment with sirolimus was
initiated at 6 weeks age. The initial dose of sirolimus was 0.6
mg/m2/day PO, comparatively lower due to poor renal function. He had two
chest tubes. Chest Tube #1 had initial output of 2 mL on day 5, 34 mL
on day 6, and 28 mL on day 8. Chest Tube #2 had drainage of 14 mL on
day 12, 14 mL on day 14 and minimal drainage on days 15-19. Chest Tube
#1 was removed on day 12 and Chest Tube #2 on day 19 (Fig 1). By day
20 sirolimus levels were therapeutic (longer time than usual due to
renal dosing limitations). Sirolimus was continued for five more weeks
without re-accumulation of pleural effusion or without need to replace
chest tubes.