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Whole-Lung Lavage for Pulmonary
Alveolar Proteinosis
Bassel Ericsoussi, MD
Fellow, Pulmonary and Critical Care Medicine
University of Illinois Medical Center at Chicago
Pulmonary Alveolar Proteinosis (PAP)
• Abnormal processing of surfactant by
macrophages (primary or acquired)
• Deposition of amorphous lipoproteinaceous
material (phospholipids and surfactant
apoproteins)in the alveoli
Uchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction in
pulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579
Greenhill S, Kotton D. Pulmonary alveolar proteinosis: a bench-to-bedside story of granulocyte-
macrophage colonystimulating factor dysfunction. Chest 2009; 136:571–577
Primary vs. Acquired
• Primary PAP
– The cause is unknown yet
• Acquired (secondary) PAP
– Occupational dust exposure
– Atypical infection
– Hematologic malignancy
– Allogenic bone marrow transplantation
Clinical Manifestations of PAP
• Subacute presentation with a gradual onset of
symptoms
• 33% of patients are asymptomatic at
presentation
• Dyspnea, dry cough, fever, malaise, and
respiratory failure
• PFT is consistent with a restrictive defect
Seymour J, Presneill J. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir
Crit Care Med. 2002; 166:215–235
Holbert J, Costello P, Li W, et al. CT features of pulmonary alveolar proteinosis. AJR Am J
Roentgenol. 2001; 176:1287–1294
Whole-Lung Lavage (WLL)
• First line of treatment
• Large-volume BAL
• Wash out the proteinaceous material from the
alveoli
– Reestablish effective oxygenation and ventilation
http://chestjournal.chestpubs.org/site/misc/vid
eos/media1/index.html
The video contains footage and narration to
accompany the from clinical background
through postprocedural care
The Indications of Whole-Lung Lavage
• Pathologic diagnosis of PAP obtained by either
TBBx or SLB
• Severe dyspnea
• Hypoxemia
– PaO2 <65 mmHg,
– A-a gradient > 40 mmHg
– Shunt fraction > 10% to 12%
The Contraindications of Whole-Lung Lavage
• Uncorrectable blood dyscrasias
• Anesthetic risks
• Cardiopulmonary instability
Preprocedural Considerations
• The risks associated with whole-lung lavage
are related to
– General anesthesia
– Double-lumen endotracheal intubation
– Mechanical ventilation
– Lavage itself
• Continued postprocedure ventilatory support
and monitoring in a critical care setting
Equipment
• Warm normal saline solution (37 c) (up to 15 L to 20 L used to
lavage the lungs)
– To maintain the patient’s core temperature, the solution is run through
a blood warmer
• Warming blanket (Bair Hugger) is placed over the patient during the
procedure
• Lavage and drainage limbs (IV tubing with a stopcock)
– Connected to the bronchial lumen of a dual-lumen endotracheal tube
• Multiple drainage receptacles are necessary to collect the effluent
• Procedure table that can be manipulated to allow for
Trendelenburg and reverse-Trendelenburg positioning in order to
facilitate filling and drainage.
• Bronchoscope with an adequate suction channel is used to aspirate
any residual lavage fluid as well as to verify endotracheal tube
position
Procedure
• Performed under general anesthesia
• Double lumen endotracheal tube intubation
• Flexible bronchoscopy is performed to confirm
the appropriate tube placement
• Both the bronchial and the tracheal balloons
are inflated to isolate the lungs
• Mechanical ventilation is initiated
How to Confirm Lung Isolation
• Immerse the end of each lumen of the
endotracheal tubes in water and observing for
air bubbles while the other lung is being
ventilated
Procedure (Cont.)
• Patient placement
– Lateral decubitus position
– The lung being lavaged in the nondependent
position (up)
• Placing supporting pillows in the dependent
axilla, under the head, and between the thighs
(to avoid ischemic complications to the
extremities, the procedure may last a few
hours)
Before initiating the procedure, the tubing limb
to the treatment lung is opened to allow for
the lung to “degas” and ensure appropriate
oxygenation during single-lung ventilation
• Patient’s head end slightly elevated (Reverse-
Trendelenburg position)
• Warm (37°C) normal saline solution is allowed
to flow into the nondependent lung through
the endotracheal tube limb
• After 1 L of normal saline solution has flowed
in, the inflow tubing is clamped
• Chest percussion is performed for
approximately 4 to 5 min
Procedure (Cont.)
Procedure (Cont.)
• The foot of the bed then is elevated
(Trendelenburg position)
• The clamp on the outflow tube is opened to
drain the effluent by gravity into a container
Lavage Fluid Appearance
• The initial effluent is milky in appearance
• The fluid becomes progressively less opaque
• After 10 to 15 lavages the effluent is clear
• Ongoing charting of installed and drained
volumes is important to minimize the risks
related to overdistention of the alveoli from
the residual lavage fluid
Postprocedural Considerations
• With bronchoscopy aspirate any residual
lavage fluid
• Ventilation of the lavaged lung is resumed
• The patient is turned onto his or her back
• A radiograph is performed to ensure that the
patient did not sustain any complications from
the procedure
In the case of bilateral disease, the contralateral
lung may be lavaged in 24 to 48 h if the
patient remains stable
Complications
• Pleural effusion
• Pneumothorax
• Hydropneumothora
– If subsequent lavage is planned, pleural drain may
need to be placed to prevent intraprocedural tension
pneumothorax
– Can be avoided by meticulous charting of the infused
saline solution and the output (not to allow instilled
fluid to exceed the fluid drained by more than a few
hundred milliliters in consecutive lavages)
Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in
pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
Complications (Cont.)
• Low oxygen saturations (percentages in the high
70s to 80s)
– Common in the procedure
– Generally improve throughout the case without any
other intervention
– Hyperbaric oxygen, cardiopulmonary bypass, and
temporary venovenous extracorporeal gas exchange
• All have been used in the past
• More recent studies,7 they have not been found to be
necessary in most cases
Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in
pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
Complications (Cont.)
• Spillage of lavage fluid into the contralateral
(ventilated) lung
– Should be considered if an imbalance is noted
between the instilled and the drained volumes
– Any excess fluid should be aspirated from the
ventilated lung
– The dual-lumen endotracheal tube should be
readjusted
Outcomes and Follow-up
• Significant clinical improvement in the
majority of patients
• Approximately 15% of patients will have a
relapsing course
Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in
pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
GM-CSF therapy
• In refractory cases
• Daily subcutaneous injection
– Increase s alveolar macrophage production
– Reduction in the risk of opportunistic infections
– Reversal of the defect leading to surfactant
accumulation within the alveoli
Uchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction in
pulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579
Greenhill S, Kotton D. Pulmonary alveolar proteinosis: a bench-to-bedside story of granulocyte-
macrophage colonystimulating factor dysfunction. Chest 2009; 136:571–577

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Whole Lung Lavage for Pulmonary Alveolar Proteinosis

  • 1. Whole-Lung Lavage for Pulmonary Alveolar Proteinosis Bassel Ericsoussi, MD Fellow, Pulmonary and Critical Care Medicine University of Illinois Medical Center at Chicago
  • 2.
  • 3. Pulmonary Alveolar Proteinosis (PAP) • Abnormal processing of surfactant by macrophages (primary or acquired) • Deposition of amorphous lipoproteinaceous material (phospholipids and surfactant apoproteins)in the alveoli Uchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579 Greenhill S, Kotton D. Pulmonary alveolar proteinosis: a bench-to-bedside story of granulocyte- macrophage colonystimulating factor dysfunction. Chest 2009; 136:571–577
  • 4.
  • 5. Primary vs. Acquired • Primary PAP – The cause is unknown yet • Acquired (secondary) PAP – Occupational dust exposure – Atypical infection – Hematologic malignancy – Allogenic bone marrow transplantation
  • 6. Clinical Manifestations of PAP • Subacute presentation with a gradual onset of symptoms • 33% of patients are asymptomatic at presentation • Dyspnea, dry cough, fever, malaise, and respiratory failure • PFT is consistent with a restrictive defect Seymour J, Presneill J. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J Respir Crit Care Med. 2002; 166:215–235
  • 7. Holbert J, Costello P, Li W, et al. CT features of pulmonary alveolar proteinosis. AJR Am J Roentgenol. 2001; 176:1287–1294
  • 8. Whole-Lung Lavage (WLL) • First line of treatment • Large-volume BAL • Wash out the proteinaceous material from the alveoli – Reestablish effective oxygenation and ventilation
  • 9. http://chestjournal.chestpubs.org/site/misc/vid eos/media1/index.html The video contains footage and narration to accompany the from clinical background through postprocedural care
  • 10. The Indications of Whole-Lung Lavage • Pathologic diagnosis of PAP obtained by either TBBx or SLB • Severe dyspnea • Hypoxemia – PaO2 <65 mmHg, – A-a gradient > 40 mmHg – Shunt fraction > 10% to 12%
  • 11. The Contraindications of Whole-Lung Lavage • Uncorrectable blood dyscrasias • Anesthetic risks • Cardiopulmonary instability
  • 12. Preprocedural Considerations • The risks associated with whole-lung lavage are related to – General anesthesia – Double-lumen endotracheal intubation – Mechanical ventilation – Lavage itself • Continued postprocedure ventilatory support and monitoring in a critical care setting
  • 13. Equipment • Warm normal saline solution (37 c) (up to 15 L to 20 L used to lavage the lungs) – To maintain the patient’s core temperature, the solution is run through a blood warmer • Warming blanket (Bair Hugger) is placed over the patient during the procedure • Lavage and drainage limbs (IV tubing with a stopcock) – Connected to the bronchial lumen of a dual-lumen endotracheal tube • Multiple drainage receptacles are necessary to collect the effluent • Procedure table that can be manipulated to allow for Trendelenburg and reverse-Trendelenburg positioning in order to facilitate filling and drainage. • Bronchoscope with an adequate suction channel is used to aspirate any residual lavage fluid as well as to verify endotracheal tube position
  • 14.
  • 15. Procedure • Performed under general anesthesia • Double lumen endotracheal tube intubation • Flexible bronchoscopy is performed to confirm the appropriate tube placement • Both the bronchial and the tracheal balloons are inflated to isolate the lungs • Mechanical ventilation is initiated
  • 16. How to Confirm Lung Isolation • Immerse the end of each lumen of the endotracheal tubes in water and observing for air bubbles while the other lung is being ventilated
  • 17. Procedure (Cont.) • Patient placement – Lateral decubitus position – The lung being lavaged in the nondependent position (up) • Placing supporting pillows in the dependent axilla, under the head, and between the thighs (to avoid ischemic complications to the extremities, the procedure may last a few hours)
  • 18. Before initiating the procedure, the tubing limb to the treatment lung is opened to allow for the lung to “degas” and ensure appropriate oxygenation during single-lung ventilation
  • 19. • Patient’s head end slightly elevated (Reverse- Trendelenburg position) • Warm (37°C) normal saline solution is allowed to flow into the nondependent lung through the endotracheal tube limb • After 1 L of normal saline solution has flowed in, the inflow tubing is clamped • Chest percussion is performed for approximately 4 to 5 min Procedure (Cont.)
  • 20. Procedure (Cont.) • The foot of the bed then is elevated (Trendelenburg position) • The clamp on the outflow tube is opened to drain the effluent by gravity into a container
  • 21. Lavage Fluid Appearance • The initial effluent is milky in appearance • The fluid becomes progressively less opaque • After 10 to 15 lavages the effluent is clear • Ongoing charting of installed and drained volumes is important to minimize the risks related to overdistention of the alveoli from the residual lavage fluid
  • 22. Postprocedural Considerations • With bronchoscopy aspirate any residual lavage fluid • Ventilation of the lavaged lung is resumed • The patient is turned onto his or her back • A radiograph is performed to ensure that the patient did not sustain any complications from the procedure
  • 23. In the case of bilateral disease, the contralateral lung may be lavaged in 24 to 48 h if the patient remains stable
  • 24. Complications • Pleural effusion • Pneumothorax • Hydropneumothora – If subsequent lavage is planned, pleural drain may need to be placed to prevent intraprocedural tension pneumothorax – Can be avoided by meticulous charting of the infused saline solution and the output (not to allow instilled fluid to exceed the fluid drained by more than a few hundred milliliters in consecutive lavages) Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  • 25. Complications (Cont.) • Low oxygen saturations (percentages in the high 70s to 80s) – Common in the procedure – Generally improve throughout the case without any other intervention – Hyperbaric oxygen, cardiopulmonary bypass, and temporary venovenous extracorporeal gas exchange • All have been used in the past • More recent studies,7 they have not been found to be necessary in most cases Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  • 26. Complications (Cont.) • Spillage of lavage fluid into the contralateral (ventilated) lung – Should be considered if an imbalance is noted between the instilled and the drained volumes – Any excess fluid should be aspirated from the ventilated lung – The dual-lumen endotracheal tube should be readjusted
  • 27. Outcomes and Follow-up • Significant clinical improvement in the majority of patients • Approximately 15% of patients will have a relapsing course Beccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  • 28. GM-CSF therapy • In refractory cases • Daily subcutaneous injection – Increase s alveolar macrophage production – Reduction in the risk of opportunistic infections – Reversal of the defect leading to surfactant accumulation within the alveoli Uchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction in pulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579 Greenhill S, Kotton D. Pulmonary alveolar proteinosis: a bench-to-bedside story of granulocyte- macrophage colonystimulating factor dysfunction. Chest 2009; 136:571–577