CHYLOTHORAX. Why you don't want to see it nor do we know how to treat it. Vijay Anand, MD FRCPC

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1 CHYLOTHORAX Why you don't want to see it nor do we know how to treat it Vijay Anand, MD FRCPC

2 DISCLOSURES None

3 THANK YOU Jason Buckley and the PC4

4 CHYLOTHORAX WHY WE DON T WANT TO SEE IT

5 CHYOTHORAX You ve just finished managing a Norwood, complex hetertoaxy, (etc) after a rocky few days You ve started feeding as extubation is still a few days away You re ready to handover to the daytime intensivist, when you get called to the bedside and get handed

6 CHYOTHORAX Why is this a big deal?

7 OUTCOMES ICU LOS Chylothorax No Chylothorax McCulloch et al. (2008) 38.7 ± ± 6.7 Bauman et al. (2013) 6 (1, 61) 2.5* Ismail et al. (2014) 12 ± ± 9 White et al. (2014) 40.5 (26, 64.5) 7 (4, 15) 18 (7-42) 3 (2-7) LENGTH OF STAY IN DAYS Buckley et al. Manuscript in Submission

8 OUTCOMES HOSPITAL LOS PHIS DATABASE Chylothorax No Chylothorax Neonates 47 (30-74) 20 (13-36) Infants 17 (9-36) 7 (5-13) Young Children 17 (10-30) 5 (3-9) Older Children 14 (6-27) 4 (3-6) Teenagers 10 (7-41) 5 (4-7) 30 (15-57) 7 (4-13) LENGTH OF STAY IN DAYS Buckley et al. Manuscript in Submission Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

9 CHYOTHORAX What causes the increased length of stay Total Protein Albumin Globulin Fibrinogen Fat Electrolytes AMOUNT g/l g/l g/l mg/l g/l [Plasma] Mallick and Bodenham. British Journal of Anaesthesia. 2003: 91(2):

10 OUTCOMES MORBIDITY Chylothora x No Chylothorax p value Pneumonia 3% 1% Urinary Tract Infection 6% 1% < Bloodstream Infection 5% 1% < Surgical Site Infection 3% 1% < Surgical Site Infection (Deep) 1% Buckley et al. Manuscript in Submission

11 OUTCOMES COST PHIS DATABASE Chylothorax No Chylothorax Neonates 210 ( ) 99 (63-165) Infants 82 (48-160) 42 (30-69) Young Children 74 (47-127) 34 (24-53) Older Children 59 (26-162) 35 (25-53) Teenagers 50 (32-198) 41 (30-62) P= < COST (1000US) Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

12 OUTCOMES MORTALITY PHIS DATABASE Chylothorax No Chylothorax Neonates 11.3% 4.8% Infants 4.3% 1% Young Children 1.1% 0.5% Older Children 1.7% 0.5% Teenagers 0% 0.45% OVERALL MORTALITY FOR PATIENTS WITH CHYLOTHORAX 5.9% 10% Buckley et al. Manuscript in Submission Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

13 CHYLOTHORAX Longer hospital length of stay Longer ICU length of stay Higher mortality Higher costs More infections We must be getting better though right?

14 INCIDENCE OVER TIME INCIDENCE OF CHYLOTHORAX PHIS DATABASE 3.8% Buckley et al. Manuscript in Submission Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

15 CHYLOTHORAX Hospital length of stay increasing ICU length of stay higher Mortality increasing Costs increasing More infections And we are seeing it more

16 THAT S WHY WE DON T T TO SEE IT EVEN THOUGH WE A

17 BUT HOW DO WE TREAT IT?

18 TREATMENT PROTOCOLS OF TREATMENT Several published Variation between protocols Those studied showed decrease in hospital resource utilization Wide variation in timing So what are we actually doing?

19

20 TREATMENT PROTOCOLS OF TREATMENT Informal Survey of CICUs Themes Lots of frustration Inconsistency Protocol inconsistency Variations on published protocols

21 DEFINITION Cardiology in the Young Sep 29: 1-10

22 DEFINITION Milky appearance WBC >1000 cells/μl Lymphocytes >80% Pleural:Serum Triglyceride Level >1 Triglyceride level >1.1mmol/L Presence of lymphatic fluid in the pleural space, commonly secondary to leakage from the thoracic duct or one of its main tributaries. Thoracocentesis is the gold standard for diagnosis and generally reveals a predominance of lymphocytes and/or a triglyceride level greater than 110 mg/dl Brown et al. Cardiology in the Young Sep 29: 1-10

23 DEFINITION BROWN ET AL (2016) PROTOCOLS Milky appearance Little comment WBC >1000 cells/μl (universally agreed) Universally agreed Lymphocytes >80% 75-90% Pleural:Serum >1 Universally agreed Triglyceride level >1.1mmol/L >1.0 Brown et al. Cardiology in the Young Sep 29: 1-10

24 PROTOCOLS CHYLOTHORAX CONFIRMED LOW HIGH 3, 5, 10, 20, 50 ml/kg/day

25 PROTOCOLS CHYLOTHORAX CONFIRMED LOW HIGH 3, 5, 10, 20, 50 ml/kg/day

26 PROTOCOLS CHYLOTHORAX CONFIRMED LOW HIGH

27 PROTOCOLS LOW : FIRST STEP Diet Change Medium Chain Triglyceride Formula Defatted Milk (DFM) Length of Diet Change 3 12 weeks Mostly 4-6 weeks LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

28 PROTOCOLS LOW : SECOND (?) STEP If at any point in time the output passed the threshold for low output into high output, switched arms to the high volume arm LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

29 PROTOCOLS HIGH : FIRST STEP Diet Change Medium Chain Triglyceride Formula Defatted Milk (DFM) Length of Diet Change 24 hours to 21 days LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

30 PROTOCOLS HIGH : SECOND STEP Total Parenteral Nutrition NPO LOW CHYLOTHORAX CONFIRMED HIGH Length in this phase 5 days to 3 weeks TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

31 PROTOCOLS HIGH : THIRD STEP Total Parenteral Nutrition + Octreotide Other Options: Steroids (not consistent) Milrinone (not consistent) Sildenafil (not consistent) Anticoagulation (not consistent) LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

32 OCTREOTIDE OCTREOTIDE Dose range of mcg/kg/hr Side effects: Abdominal distension Diarrhea Necrotizing enterocolitis Transient hypoglycemia/transient hypothyroidism No practice recommendation was made LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON Das and Shah. Cochrane Database of Systematic Reviews 2010, Issue 9

33 OCTREOTIDE OCTREOTIDE Since 2010 Multiple single institution case series published with success rate of 50-80% Decreasing use in PHIS database (24% in 2004 to 13% in 2011, p=0.034) 1 The only treatment strategy to change over time LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON 1 Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

34 PROTOCOLS HIGH : THIRD STEP Total Parenteral Nutrition + Octreotide Dose Range 1-12 mcg/kg/hr Escalating doses? Length in this phase 3 days to 17 days Wean? LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

35 PROTOCOLS HIGH : FOURTH STEP Intervention Thoracic Duct Ligation Pleurodesis Catheter Intervention LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

36 INTERVENTION THORACIC DUCT LIGATION CHYLOTHORAX CONFIRMED LOW HIGH Ann Thorac Surg Jul;88(1): TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

37 INTERVENTION THORACIC DUCT LIGATION 20 patients 18 patients had resolution Range of time to intervention: days No pre-ligation protocol Has become the mainstay surgical option for chylothorax But there is hesitancy LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON Nath et al. Annals of Thoracic Surgery 2009 Jul;88(1):246-51

38 THORACIC DUCT LIGATION CHYLOTHORAX CONFIRMED LOW HIGH Variation in path of the thoracic duct Unnecessary surgery Need for reoperation TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

39 RATES IN BENCHMARK OPS RATE (%) FONTAN 9.38 NORWOOD 8.51 ASO/VSD 8.38 AVC 8.02 GLENN 7.08 ASO 7.02 RATE (%) TRUNCUS 4.91 TOF 4.35 COARCTATION 3.55 REPAIR BT SHUNT 2.92 VSD 1.31 Mavroudis et al. Annals of Thoracic Surgery May; 97(5):

40 INTERVENTION THE THORACIC DUCT If not surgery then what? LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON 1 Mery et al. (2014) The Journal of Thoracic and Cardiovascular Surgery. 147(2)

41 INTERVENTION CHYLOTHORAX CONFIRMED LOW HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

42 PROTOCOLS Itkin et al JTCVS (3):

43 PROTOCOLS CHYLOTHORAX CONFIRMED LOW HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

44 PROTOCOLS DORI ET AL (2016) Demonstrated successful cannulation of cysterna chyli Successful embolization Same method for traumatic chylous leaks

45 3 YO HLHS S/P TCPC 3 MO IN HOSPITAL RESPIRATORY DISTRESS AND PERSISTENT LEAK.

46 INTERVENTION CATHETERIZATION Offers non-surgical approach Complications Transient abdominal pain (10/18) Transient hypotension (14/18) Bleeding? Stay tuned

47 BACK TO PROTOCOLS WHAT ABOUT IMAGING? ECHO Assess RVSP SVC Assessment Upper extremity ultrasound Potential clots LOW CHYLOTHORAX CONFIRMED HIGH TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

48 PROTOCOLS POST-SUCCESSFUL INTERVENTION MCT/Low Fat Diet? TPN + NPO? LOW CHYLOTHORAX CONFIRMED HIGH Most protocols opt for MCT/Low Fat Diet TPN + NPO TPN + NPO + OCTREOTIDE SURGICAL INTERVENTI ON

49 CHYLOTHORAX CONFIRMED VARIATION OF 9 DAYS 59 DAYS LOW 3, 5, 10, 20, 50 ml/kg/day HIGH DAYS 1-21 DAYS TPN + NPO 5-21 DAYS TPN + NPO + OCTREOTIDE 3-17 DAYS SURGICAL INTERVENTION DUCT LIGATION PLEURODESIS CATHETER INTERVENTION Summary of feedback from over 20 protocols and 50+ CICUs

50 OTHER QUESTIONS Low output persistent chylothorax

51 CHYLOTHORAX CONFIRMED VARIATION OF 9 DAYS 59 DAYS LOW 3, 5, 10, 20, 50 ml/kg/day HIGH DAYS 1-21 DAYS TPN + NPO 5-21 DAYS TPN + NPO + OCTREOTIDE 3-17 DAYS SURGICAL INTERVENTION DUCT LIGATION PLEURODESIS CATHETER INTERVENTION Summary of feedback from over 20 protocols and 50+ CICUs

52 WHAT S BEING STUDIED NOW? CURRENT RESEARCH Search of trials registry 10 studies 4 recruiting Most studies are about formula type

53 WHAT S NEXT WHAT DO WE DO NEXT? Clinical Trial with Protocol Melissa.Winder@imail.org David.bailly@hsc.utah.edu Randomized Controlled Trial in the OR Role of the PCICS

54 SUMMARY Why we don t want to see chylothorax is clear We kind of sort of know what we are doing Wide degree of variation in practice Some innovative treatment options on the horizon

55 THANK YOU

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