Lean: From Theory to Practice — One City’s (and Library’s) Lean Story… Abridged
(FULL) CME Glass (of milk) Half Full copy.pdf
1. PRESENTER: DR FIRHAN BIN HASSAN
SUPERVISOR :A/P DR NG BOON HAU
GLASS (OF MILK) HALF FULL:
ATHERAPEUTIC CHALLENGE
2. OUTLINE:
1. CASE PRESENTATION
2. APPROACH TO PLEURAL
EFFUSION/
CHYLOTHORAX
3. INTRODUCTION
4. AETIOLOGY
5. CLINICAL PRESENTATION
6. RELEVANT
INVESTIGATIONS
7. MEDICAL MANAGEMENT
8. SURGICAL MANAGEMENT
9. TAKE HOME MESSAGE
10. REFERENCES
3. Madam TBC, 33 years old Chinese lady
Presented in early March 2023 for:
1. Progressive dyspnoea over 2 weeks period
2. Orthopnoea and reduced effort tolerance during similar duration
3. Loss of weight and appetite (~4kg in 2 weeks)
1. CASE PRESENTATION
4. EXAMINATION REVEALED:
Cachexia
BP 103/61
PR 90
T 37’C
RR 20
SpO2 98% under RA
Dullness and reduced vocal resonance over bilateral lower hemithorax region.
Diminished breath sounds over bilateral lower hemithorax region.
5. BLOOD INVESTIGATIONS
FBC: TWC 10.4 Hb 14.1 Platelet 414
Renal Profile:
Urea 3.3 Sodium 139 Potassium 4.1
Creatinine 63.6
Liver function test:
Albumin 17 TP 44 ALP 71 AST 29
Bili 2.9
LDH 267
7. She initially attended Anson Medical Centre (6/3/23) with the above complaints
Chest radiograph revealed bilateral pleural effusion of which she underwent bilateral
thoracocentesis. She later underwent CT Thorax 6/3/23 which revealed:
- Bilateral large pleural effusion with collapse lower lobes
- No mass/mediastinal mass/suspicious mediastinal nodes. No suspicious bony lesion.
Transferred out to KPJ Ipoh on 7/3/23 and underwent bilateral pigtail drainage, with right
pleuroscopy + biopsy which HPE revealed non specific inflammation with no granuloma/
malignant cells.
Patient subsequently transferred to HCTM on 13/3/23 for further management.
12. PLEURAL FLUIDANALYSIS
Biochemistry:
Glucose 5.8; Alb 11
TP 24 (ratio: 0.54)
LDH 129 (ratio: 0.48)
TG: 6.62mmol/L (Serum:
0.97mmol/L)
Cytology:
Scattered Neutrophils,
lymphocytes and eosinophils,
no malignant cells seen.
Microbiology:
C&S: No growth
MTB C&S: No MTB detected
Pleural ADA:
TB GeneXpert: negative
Immunophenotyping:
The B and T cells show no
evidence of clonality to suggest
lymphomatous infiltration.
13. BLOOD INVESTIGATIONS
ANA (15/03/23): POSITIVE 1:100 /
Homogenous
Anti DsDNA (15/03/23): Negative
ENA Panel (HUKM, 16/03/23): All Negative
RF: Negative
ESR (15/03/23): 43 mm/Hr
C3 / C4: 93.8 / 28.7 (Normal)
T4: 11.69 pmol/L (Normal) / TSH: 3.51 uIU/mL
(Normal)
Hep B / Hep C / HIV: Negative
Beta 2 microglobulins: 1.6 mg/L (0.8-2.2)
Beta 2 microglobulins: 1600 ng/ml (800-2200)
Lupus anticoagulant : negative
Anti-cardiolipin IgG Antibody: negative
Anti-beta2 glycoprotein1 (IgG/IgM) Antibody: negative
IgA: 437 mg/dL (Normal)
IgM: 198 mg/dL (Normal)
IgG: 2240 mg/dL (Raised, NR 751 – 1560)
OTHER INVESTIGATIONS:
Urine PCI (20/03/23): 0.02 g/mmol
ECHO: EF 65% No RWMA
20. 3. INTRODUCTION
C
hylothorax is a rare condition that results from thoracic duct damage with chyle leakage
from the lymphatic system into the pleural space.
Approximately 2.4 litres of chyle is transported through the lymphatic system every day.
Damage to any part of this structure may lead to an accumulation of chyle in pleural cavity. (1)
I
ts primary role is to carry 60-70% of ingested fat at a concentration of 0.4-0.6g/dl from the
intestines to the circulatory system - thus explained its large amounts of cholesterol,
triglycerides, chylomicrons and fat soluble vitamins. (2)
Untimely identification of the chylothorax may lead to depletion of chyle fluid, ultimately
resulting in malnourishment, compromised immunity, metabolic disorders and even fatality.
21. L
ymph vessels arising in the peritoneal cavity form
the thoracic duct by coalescing at the posterior
aspect of the aorta, inferior to the diaphragm.
F
rom here, the duct follows the course of the aorta
superiorly through the diaphragm before
continuing upwards on the right side of the
thoracic vertebrae.
T
hen it turns left at the level of 3rd or 4th
vertebrae, moves across midline and follows the
course upwards medially behind the
oesophagus - proceeding as far as the cervical region
turning lateral before terminating into subclavian vein.
(3)
3. INTRODUCTION
22. 4.AETIOLOGY
C
hylothorax can be classified as traumatic or non traumatic.
Traumatic cases can be further sub-classified as iatrogenic or
non iatrogenic (20% of traumatic cases).
T
horacic surgery has now replaced physical injury as the leading
cause of trauma with oesophageal surgery having incidence of
4% - with transhiatal approach increases the risk as compared
to thoracic approach. (4)(5)
T
rauma directly damages the duct or leads to tissue damage
close by which eventually results in swelling and blockage with
eventual rupture.
O
ther iatrogenic causes may also include damage from
subclavian catheterisation or blockage due to central venous
catheterisation related venous thrombosis. (6)
23. 4.AETIOLOGY
C
ommonest cause of non traumatic chylothorax is thoracic duct obstruction due to
malignancy - with up to 70% of cases are lymphoma. Rarely metastatic tumour may also
give rise to duct obstruction.
Mediastinal lymphadenopathy may compress the lymphatic vessels preventing drainage of
lymph from the lung periphery resulting in extravasation of chyle into pleural space. (7)
L
ymph vessels disease is extremely uncommon - and list of causes includes
Lymphangioleiomyomatosis (LAM) (2/3 patients experiencing chylothorax); Gorham’s
disease and Yellow Nail Syndrome.
Congenital chylothorax occurs more so due to congenital malformations than trauma during
delivery.
Rarely, chylothorax has also been described as an early and late complication of radiotherapy.
26. 5. CLINICAL PRESENTATION
S
ymptoms - majority of patients present
with dyspnoea due to mechanical
effects of pleural effusion. Onset of
dyspnoea usually relates to aetiology as it is
more gradual for non traumatic causes; and
post surgery causes - onset are more rapid if
volume is high.
Other non specific symptoms may include
heavy feeling in the chest, fatigue and
weight loss.
Some patients may present with
malnourishment - dependant on rate and
degree of loss.
F
ever and chest pain rarely occur as
presence of chyle in pleural space
does not evoke inflammatory response
and rarely become infected due to
bacteriostatic effect of immunoglobulins
that are contained in chyle.
Patients may also be prone to infection in
organ other than pleural space due to loss
of immunoglobulins.
Other clinical presentations may be related
to presentation of aetiology of chylothorax.
28. 6. RELEVANT INVESTIGATIONS
A. PLEURAL FLUID ANALYSIS:
1. Appearance: can be milky, sanguineous, or serous.
2. Cell counts: predominantly lymphocytes.
3. Electrolytes and protein: most have higher protein concentration making them exudative (85% of
time). 8 LDH usually low level - being in the range of transudative except in cases of malignancy.
4. pH ranges around 7.4-7.8
5. Lipid analysis: typically TG level >1.24mmol/L (>110mg/dL) with cholesterol <5.18mmol/L.
Intermediate level of TG (50-110mg/dL) does not exclude chylothorax - consideration for lipoprotein
electrophoresis to detect chylomicrons.
Diagnosis usually established in cases of pleural fluid with typical TG level >1.24mmol/L with predominant
lymphocytes > 70% - along with risk factors.
29. 6. RELEVANT INVESTIGATIONS
B. IMAGING MODALITIES:
1. Enhanced CT of the thorax, abdomen and pelvis: to identify mediastinal, retroperitoneal
lymphadenopathy/masses; course of the thoracic duct, cystic lesion of the thoracic duct/
pulmonary cysts/renal angiomyolipoma of LAM.
2. Lymphatic imaging in select patients when: (9)
i. diagnosis of duct tear/anomalous thoracic duct suspected, OR
ii. site of chyle leak is unknown and intervention is planned to resolve lymph leakage.
3. Magnetic resosance lymphangiography - using heavily T2 weighted imaging provides
detailed visualisation of the entire thoracic duct - usually to assist pre-operative planning.
30. Lymphangioscintigraphy
- performed by injecting technetium99-labelled
HSA-DTPA into subcutaneous region of the
dorm pedis bilaterally.
- Sequential anterior and posterior view of the
thorax then obtained with SPECT/CT - aids
radiolocalization of chyle leaks.
- It is usually limited by its availability and not
ideal for examining anatomic abnormalities of
the lymphatic system.
- but less invasive than Lymphangiography and
easier to perform.
LYMPHATICSIMAGING
31. Lymphangiography
- is a contrast enhanced study of the lymphatics
- performed using bilateral localisation of pedal lymphatics with a subcutaneous injection of
methylene blue (absorbed into lymphatic system) - followed by minor surgical procedure to
cannulate the lymphatic vessel - then injection of Lipiodol contrast with fluoroscopy to follow
the flow of contrast into thoracic duct.
- conventional films and CT images are then obtained.
- aid in identification of areas of chyle leakage.
- May occasionally be therapeutic as iodinated contrast is thought to have a local tissue
sclerosing effect. (10)
LYMPHATICSIMAGING
33. PROGRESS:
• She was started on low fat diet and medium chain
triglycerides TPN on 15/3/23.
• Left pigtail drainage was removed on 14/3/23 and Right pigtail
drainage was later removed on 17/3/23.
• She later underwent F-18 FDG Whole PET CT on 23/3/23 and
Lymphoscintigraphy (on 24/3/23)
• Throughout this period, she required periodic sonographic
assessment with intermittent thoracocentesis from both right
and left pleural space with estimated drainage ranging ~1.5L/
day
• She was later referred to cardiothoracic team for VATS
• However in view of uncertainty of diagnosis (pending serum
ENA, and anti-dsDNA - as EULAR criteria for SLE not met),
decision made by CTC team to postpone VATS after
multidisciplinary discussion on 27/3/23 until review of the
above investigation.
CHEST RADIOGRAPH
FOLLOWING REMOVAL
LEFT PIGTAIL ON 14.3.23
34. IMAGING
CXR 14.3.23
CT THORAX 6.3.23
1. F-18 FDG Whole Body PET CT (23/3/23):
Massive bilateral pleural effusion with low
grade metabolic activity
Short segment of hypermetabolic
thickened pleura at the lower lobe – Ddx
inflammatory / infective changes /
malignancy. Suggest further assessment /
biopsy
No other abnormal hypermetabolic foci or
disease.
2. Left pleuroscopy (11/4/23): thickened
pleura.
3. HPE Left pleural biopsy (11/4/23):
reactive mesothelial cells.
36. • Eventually she required another set of pleural
drainage under seldinger technique for both left
(on 6/4/23) and right pleural reaccumulation (on
8/4/23) as she was symptomatic. (despite
earlier reduction in flow to 50mls/day)
• She underwent left pleuroscopy & biopsy on
11/4/23 which revealed normal findings.
• Left talc pleurodesis subsequently done on
20/4/23 and 25/4/23
• During this period, sonographic assessment
done shows reaccumulation of chylothorax over
left pleural space with daily drainage ranging
~1.5/day
• This led to the decision for Lymphangiogram &
Thoracic duct embolisation (TDE) which took
place on 27/4/23
CHEST RADIOGRAPH FOLLOWING
PRE (IMAGE B) AND POST (IMAGE A)
2ND
LEFT TALC PLEURODESIS ON 25.4.23
IMAGE A IMAGE B
37. • Clinical and serial sonographic assessment on revealed reaccumulation of bilateral space - predominantly
Right > Left, ~1L/day despite measures taken.
• Following multidisciplinary team discussion, she eventually planned for Right Video Assisted Thoracotomy
Surgery (VATS) with decortication, thoracic duct ligation (TDL) and pleurodesis which took place on 8/5/23.
• Findings at Right VATS revealed slow oozing mediastinal lymph with no definite site of leakage. Ligation was
performed above the right hemidaphragm.
• There were also findings of right multilobulated lung empyema and infected chylothorax.
• Following this procedure:
- Both left and apical right seldinger tube was off the following week.
- Eventually basal right seldinger tube off 2 weeks after the procedure.
38. • HPE of thoracic duct shows no signs of atypia or
malignancy. HPE of right lung cortex shows no
granuloma/atypia or malignancy.
• Samples sent for AFB, MTB C&S, C&S and Fungal C&S
were all negative.
• Another multidisciplinary team meeting that took place on
27/5/23 with Cardiothoracic team discussed regarding
patient findings at VATS - thick and plastered right lung
cortex
• Concluded that further op to alter patient outcome will
likely cause more dense adhesion.
• Subsequently patient was discharged home with an
appointment at chest clinic for reassessment.
CHEST RADIOGRAPH 2 WEEKS RIGHT
VATS + TDL + RIGHT TALC
PLEURODESIS + BIOPSY
ON 24.5.23
39. • Following discharge, she was later
readmitted again on 12/6/23 for Left
Loculated pleural effusion.
• She underwent another left talc
pleurodesis on 14.6.23.
• She was later discharge home.
CHEST RADIOGRAPH FOLLOWING
PRE (IMAGE B) AND POST (IMAGE A)
3RD
LEFT TALC PLEURODESIS ON 14.6.23
IMAGE A IMAGE B
40. • She was recently seen in clinic on 30.11.23.
• Clinically she was asymptomatic with
mMRC score 1
• She has gained her weight from 33.5kg to
37.3kg
• Serum Albumin was 40 (prior to discharge
on 22/5/23 was 25)
• Bedside scan shows L1-L6 no effusion.
• R5: thickened pleura, no effusion.
CHEST RADIOGRAPH ON 30.11.23
42. 7. MANAGEMENT - MEDICAL THERAPY
Approach are generally divided based on output
L
ow output chylothorax (if estimated volume is <1L/day) benefit from a staged care plan from least invasive
options to more invasive interventions during several days to weeks of observation.
H
igher output chylothorax (if estimated volume > 1L chyle/day) is most commonly seen in post surgical patients
and those with liver cirrhosis. Early intervention (percutaneous TDE; surgical TD ligation; or lymphangiography
with Lipiodol) needed early since conservative strategies are more likely to fail in this population.
Options include:
• Pleural drainage to control symptoms (via intermittent thoracocentesis or indwelling catheter placement) - generally
limited to less than 14 days to minimise risk of infection/malnutrition/electrolyte losses.
• Dietary modification.
• Adjunctive therapies.
43. DIETARY MODIFICATION
Principles of dietary management is to reduce the flow of chyle through the thoracic duct.
1. Oral or enteral low-fat diet:
- Should be assessed by a dietitian for a high protein, low fat (<10g fat/day) diet.
- Dietary exclusion of long chain-triglycerols (LCTs) is encouraged, as this prevent their conversion into monoglycerides
and free fatty acids (FFAs), which are transported as chylomicrons to intestinal lymph ducts.
- Oral vitamins should be provided, and eventually medium chain triglycerols (MCTs) can be introduced orally as pleural
drainage decreases - as MCTs are absorbed directly and transported to the liver via portal vein (bypassing intestinal
lymph ducts).
- This diet is typically continued for 7-10 days but there is no specific regimen available to time liberalisation of fat intake.
2. Total Parenteral nutrition:
- for patient with high volume chyle leaks - this is generally preferred as dramatic reduction in the flow promotes healing
and prevent acute nutritional deficiencies.
44. ADJUNCTIVE THERAPIES
1. Somatostatin and Octreotide
- Octreotide is a somatostatin analogue that has a longer half life
- Both act by inhibiting gastric, pancreatic and biliary secretions and inhibiting absorption of chyle from the intestine
- hence leading to reduction in volume within the thoracic duct.
- Dosage varies and optimal dose/route of administration are not known.
- Some case series propose IV Somatostatin infusion 6mg/day or SC Somatostatin 50mcg 8hourly for 2 weeks. As
to Octreotide, were given in SC doses of 50-200mcg 8hourly for 2-14 days. (11)
2. Midodrine
- an alpha 1-adrenergic agonist that causes vasoconstriction of the lymph system.
- its success in usage to reduce drainage in post operative/idiopathic chylothorax has been reported in two case
reports. (12)
46. 8. MANAGEMENT - DEFINITIVE THERAPY (SURGICAL)
Selection of intervention modality is usually individualised and depends upon multiple factors:
• Aetiology of chylothorax
• Availability of local surgical and interventional radiologic expertise and preference.
• Rate of chyle loss
• Likelihood of response to chosen intervention
• Location of lymph leakage or thoracic duct obstruction
• Risk of surgery
• Patient preferences
47. 8. MANAGEMENT - DEFINITIVE THERAPY (SURGICAL)
Modality options available are as follows:
•Pleurodesis
•Percutaneous or retrograde lymphatic embolisation or disruption:
A. Thoracic duct embolization (TDE)
B. Thoracic duct disruption (TDD)
•Surgical thoracic duct ligation
•Other less well established options
48. PLEURODESIS
•Is an option for those who fail conservative therapies
in whom early thoracic duct intervention is not
indicated.
•Is a procedure that obliterates the pleural space by
inducing intrapleural inflammation, with consequent
fibrin deposition and scarring - achieved by instilling a
chemical irritant or performing mechanical abrasion.
•Options for sclerosant in chemical pleurodesis
include: talc, tetracycline derivatives (doxycycline),
bleomycin, iodopovidone and silver nitrate.
•Success of pleurodesis generally depends on
adequate drainage of the pleural space prior to the
introduction of chemical or talc, thus concomitant
thoracic duct intervention such as embilization or
ligation is commonly performed.
49. LYMPHATIC EMBOLIZATION/DISRUPTION (1)
•TDE has the advantage of being able to identify the leak and offer a therapy in the same
setting - also less invasive when compared to surgical thoracic duct ligation.
•They are increasingly in favour as an alternative to TDL, chylothoraces that failed with
pleurodesis, chyle ascites (leaks from cisterna chyli), or poor operative candidates.
•Initial lymphangiography with US guided intranodal lymphatic cannulation allows visualisation
of large retroperitoneal lymphatics, which are then most commonly accessed by
tranabdominal percutaneous needle cannulation.
•Some centers access the TD in a retrograde manner via a subclavian vein approach OR by
transnodal lymphangiography via groin nodes.
50. LYMPHATIC EMBOLIZATION/DISRUPTION (2)
•TDE - a catheter is advanced into the
thoracic duct with instillation of contrast
to localise the leak. The affected thoracic
segment is then embolized with coils and
surgical “glue”.
•TDD - the goal is to macerate the
cisterna chill or major lymphatic
channels, thereby decreasing flow to
allow caudal lymphatic leaks to heal -
performed for patients who have
technical issue that doesn’t allow
percutaneous lymphatic access for TDE.
51. THORACIC DUCT
LIGATION
•This option generally reserved for those that have a high-volume chyle leak due to a laceration in
thoracic duct in a post operative setting. Its outcome in low-volume leak cases who failed conservative
therapy varies.
•Is performed via video-assisted thoracoscopy (VATS) or open thoracotomy depending on local
expertise.
•However, locating and repairing the leak during the surgery may be challenging due to tissue
inflammation and adhesions. Thus, localisation of the leak using one of a variety of lymphangiography
imaging techniques is valuable prior to proceeding.
52. THORACIC DUCT LIGATION
•Despite reported success rate, TDL have a morbidity and
mortality of 38% and 2%. And Lymphedema is a known
complication - usually resolves over several months as
collateral lymphatic-venous communications develop. 13
•Many perform concomitant pleurodesis in patients
undergoing TDL since they can be done in same setting.
Data are however not reliable. 14
53. THORACIC DUCT LIGATION
•Identification of thoracic duct leak is
challenging and may usually be done
via preoperative/intraoperative
administration of fat load mixed with
lipophilic green dye; or bilateral inguinal
injection of indocyanine green with
albumin.
54. 9.TAKEHOMEMESSAGE
•Chylothorax can be due to multiple aetiologies, among which malignancy and surgical trauma are the commonest
cause.
•Presentation are usually with typical signs and symptoms induced by mechanical effects of pleural effusion with
onset dependant on its aetiology.
•Requires extensive list of investigation as aetiology varies (traumatic vs non traumatic)
•No algorithm has been universally adopted for the management of patients with chylothorax.
•Management are always MDT approach - varies depending on output - consider for early intervention in high
output chylothorax.
•For patients with low volume (indicating slow accumulation of chyle), waiting for longer periods to note a response
may be appropriate with success rates variable ranging from 25-80%.
•Those who do not respond to or only partially respond to conservative measures (diet modification + pleural
drainage + medical therapy), may require various medical/surgical interventions as previously discussed. A
multidisciplinary approach should facilitate decision making.
55. 10. LIST OF REFERENCES
1. Macfarlane JR, Holman CW. Chylothorax. Am Rev Respir Dis 1972;105(2):287e91.
2. Zilversmit DB. The composition and structure of lymph chylomi- crons in dog, rat, and man.
J Clin Invest 1965;44(10):1610e22.
3. Hillerdal G. Chylothorax and pseudochylothorax. Eur Respir J 1997;10(5):1157e62.
4. McWilliams A, Gabbay E. Chylothorax occurring 23 years post- irradiation: literature review
and management strategies. Respirology 2000;5(3):301e3.
5. Bolger C, et al. Chylothorax after oesophagectomy. Br J Surg 1991;78(5):587e8.
6. Kurekci E, Kaye R, Koehler M. Chylothorax and chylopericar- dium: a complication of a
central venous catheter. J Pediatr 1998;132(6):1064e6.
56. 10. LIST OF REFERENCES
7. Nair SK, Petko M, Hayward MP. Aetiology and management of chylothorax in adults. Eur J Cardiothorac Surg 2007;32(2):
362e9.
8. Maldonado F, Hawkins FJ, Daniels CE, et al. Pleural fluid characteristics of chylothorax. Mayo Clin Proc 2009; 84:129.
9. Mine S, Udagawa H, Kinoshita Y, Makuuchi R. Post-esophagectomy chylous leakage from a duplicated left-sided thoracic
duct ligated successfully with left-sided video-assisted thoracoscopic surgery. Interact Cardiovasc Thorac Surg 2008;
7:1186.
10. Kos S, Haueisen H, Lachmund U, Roeren T. Lymphangiography: forgotten tool or rising star in the diagnosis and therapy of
postoperative lymphatic vessel leakage. Cardiovasc Intervent Radiol 2007; 30:968.
11. Kalomenidis I. Octreotide and chylothorax. Curr Opin Pulm Med 2006; 12:264.
12. Liou DZ, Warren H, Maher DP, et al. Midodrine: a novel therapeutic for refractory chylothorax. Chest 2013; 144:1055.
13. Huggins JT. Chylothorax and cholesterol pleural effusion. Semin Respir Crit Care Med 2010; 31:743.
14. Graham DD, McGahren ED, Tribble CG, et al. Use of video-assisted thoracic surgery in the treatment of chylothorax. Ann
Thorac Surg 1994; 57:1507.
