Patient Management. March 26, 2013 รศ.พญ. นฤมล เด นทร พย ส นทร

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1 Patient Management March 26, 2013 พญ.น ชชนาฏ นชชนาฏ ธรรมเน ยมด ธรรมเนยมด รศ.พญ. นฤมล เด นทร พย ส นทร

2 Case scenario เด กหญ งไทยอาย 2 ป โรคประจ าตว : Tetralogy of Fallot การผ าต ด การผาตด : Total repair Incision : median sternotomy

3 Case scenario Day 10 after an operation : developed clinical dyspnea and decreased breath sound at the right lung

4 CXR : pre operation

5 CXR : post operation operation CXR day 10

6 CXR : post operation operation day 10

7 Content from the right chest drain

8 What is your provisional diagnosis y p g and further investigation?

9 Investigations Content from right chest drain Triglyceride (TG) 1,381 mg/dl (serum TG 102) Protein 6 g/dl cell count: 14,400 cell/mm 3, lymphocyte 93% Culture: no growth

10 Investigations Content from right chest drain Triglyceride 1,381 mg/dl (serum TG 102 mg/dl) Protein 6 g/dl Diagnosis of chylothorax cell count: 14,400 cell/mm 3, lymphocyte 93% Culture: no growth milky appearance - TG > 110 mg/dl - lymphocyte predominated d - sterile in culture

11 Characteristics of chylous effusion Appearance: milky appearance Odor: odorless Cell count: lymphocyte predominate Culture: sterile Triglyceride > 110 mg/dl (more than plasma TG) < 50 mg/dl can rule out chyle unless no fat ingestion Valetine VG. Chest. 1992;102:

12 Characteristics of chylous effusion Total protein g/dl albumin g/dl globulin g/dl Electrolytes l t Na meq/l K meq/l Cl meq/l Ca meq/l Valetine VG. Chest. 1992;102:

13 Characteristics of chylous effusion

14 Diagnosis: Chylothorax

15 Chylothorax : causes Traumatic Post-intrathoracic operation: most common cause of chylothorax in children Direct penetrating injury of thoracic duct Non-traumatic Malignancy: lymphoma Infection Congenital malformation of thoracic lymphatic system

16 Anatomic pathway of thoracic duct (2) continues cephalad through the aortic hiatus into the posterior mediastinum Right of the midline between the aorta and the azygos vein crosses the midline at the T4-T6 levels behind the esophagus enters the left posterior mediastinum lie behind the aortic arch just left to the esophagus more superiorly, behind the left subclavian artery arches into the superior mediastinum anterolaterally drains to the systemic blood circulation at the left brachiocephalic h vein

17 Thoracic duct ดเส นขวางส แดงเบ ยว ด เสนขวางสแดงเบยว collects most of the lymph in the body, except right arm right side of the chest, neck, and head drains to the systemic blood circulation at the left brachiocephalic vein, right between where the left subclavian vein and left internal jugular connect.

18 LIPID Triglyceride = Triacylglycerol Fatty acid : = 3 Fatty acid + glycerol CH 3 -(CH 2 ) n -COOH 3 2 n C 2-6 : SCFA (small chain fatty acid) C 8-10 : MCFA (medium chain fatty acid) C : LCFA (long chain fatty acid) C 20 : VLCFA (very long chain fatty acid)

19 LIPID LIPID Glycerol Triglyceride HO - CH CH 2 -OH CH 2 - OH CH 2 -OR 1 R 2 O - CH 2 CH 2 -OR 3

20 LIPID ABSORPTION LCT intestinal epithelial cells bile salt phospholipid o p micelle e lipase FFAs, 2-monoglycerol diffusion intestinal epithelial cell

21 LIPID ABSORPTION INTESTINAL CELL FFAs, 2-monoglyceride TG cholesterol apolipoprotein chylomicrons lymphatic vessels Venous circulation thoracic duct cisterna chyli

22 LIPID ABSORPTION The serum concentration of chylomicrons peak is 3-5 hr. after meal, giving a milky-white appearance to the serum MCT intestinal epithelial cells portal vein liver

23 Chylothorax : no contraindication for enteral feeding MCT diet 1 week Improvement Drain < 50% of initial pleural fluid volume MCT diet 3 weeks Cure Normal diet J Pediatr. 2000;136:653-8 Improvement and cure 2 weeks MCT diet 3 weeks cure Failure Drain > 50% of initial iti pleural fluid volume TPN 1 week No improvement in 1 week

24 No improvement in 1 week Octreotide tid + TPN Surgery for 2 weeks Improvement No improvement MCT diet 2 weeks cure J Pediatr. 2000;136:653-8 surgery cure Other surgical option Failure

25 Medium chain triglyceride (MCT) rich diet MCT-rich diet: liquid form Add 4 ml MCT to nonfat milk 100 ml Energy: (8.4*4)+(10/30*100) = 67 kcal/dl Protein: 3.3 g/dl Minerals: Na, Fe Vitamins: water- and fat-soluble Inappropriate to infant

26 MCT rich diet: solid foods เน อส ตว : เน อไก ส วนอก ไม เอาหน ง, ไข ขาว ใช น าม น ใ MCT oil ในการประกอบอาหารแทนน าม นจาก เมล ดพ ช ผ กใบเข ยว, ผลไม ท กชน ด ยกเว น ยกเวน ถ ว, (almond, cashew, macadamia, peanut, pumpkin, soybean, sunflower) ผลไม หร อผ กท ม เมล ด ผลไมหรอผกทมเมลด

27 MCT rich diet : non fat milk

28 MCT rich diet : non fat milk

29 MCT rich diet : chicken breast, no skin

30 MCT rich diet : egg white

31 MCT oil

32 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

33 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

34 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

35 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

36 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

37 การสอนโภชนาการ/ท าอาหารให ผ ป วยเม อกล บบ าน การสอนโภชนาการ/ทาอาหารใหผ ปวยเมอกลบบาน

38 Current Practice in chylothorax case Chylothorax แพทย order MCT-rich diet สอนอาหารท ก นได - ท มพยาบาล ไม ได แก ผ ป วยและ ผ ปกครอง สอนอาหารท ก นได -ไม ได ไ ท มพยาบาล และการ ตรวจสอบอาหาร โรงคร ว กล มงาน ท าอาหารใน ก อนให ผ ป วย โภชนาการ รายท จ าหน าย ก อน 28 ว น

39 Management and clinical course in this case Start treatment as algorithm. intercostal drainage at the right chest MCT rich diet Record volume of chylous fluid from a chest drain everyday If not respond, plan to assess CBC, serum albumin, globulin, electrolyte once a week

40 Management and clinical course in this case Off a chest drain at Day 14 of MCT- rich diet treatment MCT- rich diet 28 days Resolve No recurrence after switch to a normal diet

41 Management and clinical course in this case

42 Management and clinical course in this case CXR

43 หล ง off ICD no recurrence of chylothorax

44 Monitoring Effusion Volume and turbidity of effusion TG and protein content of effusion Blood albumin, lymphocyte count, electrolytes, Ca, P (Immunoglobulin) (Fat-soluble vitamins) i (Essential fatty acids) Clinical: BW Edema xerophthalmia Infection (intercostal t catheter, central venous catheter) Compliance of food intake, incorrect foods

45 Current progression problems ท มพยาบาล Care of Intercostal drainage tube การส งอาหารพ เศษเฉพาะโรค การสงอาหารพเศษเฉพาะโรค ไปย งฝ ายโภชนาการของ โรงพยาบาล การตรวจสอบอาหารก อนให ผ ปวยรบประทานจรง ป ป ท มน กโภชนาการ การท าอาหารพ เศษเฉพาะ การทาอาหารพเศษเฉพาะ โรค (MCT diet)

46 Chylothorax : Principle of management

47 Siriraj data 16 patients (10 male, 6 female) Age: range from 11 days-14 years 14 cases had congenital heart diseases treated by cardiac surgery 2 cases diagnosed with thymolipoma and neuroblastoma had undergone tumor removal 14 cases (87.5%) resolved after conservative treatment 1 case resolved after surgery 1 case died with septicemia Densupsoontorn N. Asia Pac J Clin Nutr. 2005;14:182-7

48 Indication for surgery imminent nutritional deterioration during conservative management reaccumulation of chyle after the reinstitution of dietary fat daily chyle loss > 1500 ml in adult or 100 ml/year of age in children for a five-day period or no diminution by 2 wk of conservative management Ann Surg. 1973;177:245-9

49 Thank you for your attention

50 Lipoprotein classification TC lipid concentration (%) TG Phospholipid & protein Chylomicrons VLDL IDL LDL HDL

51 Chylothorax is a condition characterised by the presence of a thick milk-liked fluid in the pleura cavity, most typically on the left side (Figure 1). This usually occurs as a result of a tear or disruption of the thoracic duct resulting in leakage of chyle into the pleura space. The amount can be huge since the thoracic duct transport up to 4L/day of chyle in a normal adult.

52 Common causes can be categorised into malignant and nonmalignant aetiologies. Most common malignant aetiology is an anterior mediastinal malignancies (50% of cases) such as lymphoma as in this case, which accounts for 60% of cases (Figure 2: arrow heads). Other malignant causes include pancoast tumour of the lung. Non-malignant causes include tuberculosis, sarcoidosis, cirrhosis, amyloidosis, filariasis and trauma.

53 The incidence of chylothorax after cardiothoracic surgeries is about 0.2-1% due to the traumatic disruption of the thoracic duct during dissection of the mediastinum. In children, it can accounts for as much as 69-85% of all cases following cardiac surgeries. Congenital chylothorax accounts for the leading cause of pleural effusion in

54 Patients usually present with the classical symptoms of a large pleural effusion (Figure 2: arrows), which are increasing shortness of breath and tachypnoea with signs of reduced breath sounds, shifting dullness on the affected sides and tracheal deviation to the non-affected side if the effusion is large. Diagnosis is rapid and simple with a biochemical analysis of the pleural fluid for triglyceride levels: In this case, the patient s pleural fluid triglyceride level was mmol/l, which is diagnostic of chylothorax. In the initial aspiration of the pleural fluid, it can be easily mistaken for pus and sent for culture which is usually sterile due to the high contained of lymphocytes and antibody titres in the fluid. Imaging usually confirmed a large effusion (Figure 2: arrows) with possible cause of the effusion as in this case a large anterior mediastinal Hodgkin s lymphoma (Figure 2: arrow heads) which has disrupted the thoracic duct in the left anterior mediastinum (Figure 2b).

55 Treatment include immediate thoracostomy and drainage for symptomatic relief. e Conservative e measures es include putting the patient t on a fat free diet or on middle chain triglycerides diet or complete parenteral nutrition, to reduce the load through the thoracic duct. Chemoradiation may promote resolution of chylothorax and should be used in patients with malignant chylothorax who are not surgical candidates. Somatostatin t ti ( mcg/kg/hr) /h and its analogue octreotide tid has been used successfully in a number of paediatric cases of chylothorax following surgery or other iatrogenic causes, but complications of somatostatin therapy such as diarrhoea, hypoglyceamia and hypotension limits its used. All these conservative therapy will take time to work and patient should be told of the protracted time of treatment which can take up to 2 weeks.

56 Patient who failed medical therapy should be referred for surgical option of right VATS ligation of thoracic duct which is successful in 100% of the time if the thoracic i duct is completedly ltdl ligated and divided. id d To ease the location and identification of the thoracic duct, patient should be given 60-90mls of cream or full cream milk mixed with 1% methylene blue enterally via an NG tube minutes prior to incision. The procedure can be performed using right VATS via 3-4 ports and after opening the pleura inferior to the inferior pulmonary vein, the thoracic duct can be located between the azygos vein and oesophagus. A long segment should be dissected out and ligaclipped. The thoracic duct should then be divided and the ends reflected back and ligaclipped to the rest of the remaining thoracic duct. The effect is almost immediate with reduce production of chyle in the left chest drain which is obvious the next day after a normal diet.

57 Triglyceride levels Diagnosis > 110mg/dL (> 1.25mmol/L) 99% certainty that the fluid is chyle mg/dL ( mmol/L) use lipoprotein analysis to inspect the pleural fluid for chylomicrons or cholesterol crystals. < 50mg/dL (<0.6 mmol/l) 5% certainty that the fluid is chyle A ratio of pleural fluid cholesterol to triglyceride of less than 1 is also diagnostic.