Whole Lung Lavage for Pulmonary Alveolar Proteinosis

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Whole-lung lavage is a large-volume BAL that is performed mainly in the treatment of PAP. In brief, it involves the induction of general anesthesia followed by isolation of the two lungs with a double-lumen endotracheal tube and performance of single-lung ventilation while large volume lavages are performed on the nonventilated lung. Warmed normal saline solution in 1-L aliquots (total volumes up to 20 L) is instilled into the lung, chest physiotherapy is performed, then the proteinaceous effluent is drained with the aid of postural positioning. The sequence of events is repeated until such time as the effluent, which is initially milky and opaque, becomes clear. This procedure results in significant clinical and radiographic improvement secondary to the washing out of the proteinaceous material from the alveoli. The whole-lung lavage video details all aspects of the procedure, including case selection, patient preparation and equipment, a step-by-step review of the procedure, and postoperative considerations.

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Whole Lung Lavage for Pulmonary Alveolar Proteinosis

  1. 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. 2. Pulmonary Alveolar Proteinosis (PAP) • Abnormal processing of surfactant by macrophages (primary or acquired) • Deposition of amorphous lipoproteinaceous material (phospholipids and surfactant apoproteins)in the alveoliUchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction inpulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579Greenhill S, Kotton D. Pulmonary alveolar proteinosis: a bench-to-bedside story of granulocyte-macrophage colonystimulating factor dysfunction. Chest 2009; 136:571–577
  3. 3. Primary vs. Acquired• Primary PAP – The cause is unknown yet• Acquired (secondary) PAP – Occupational dust exposure – Atypical infection – Hematologic malignancy – Allogenic bone marrow transplantation
  4. 4. 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 defectSeymour J, Presneill J. Pulmonary alveolar proteinosis: progress in the first 44 years. Am J RespirCrit Care Med. 2002; 166:215–235
  5. 5. Holbert J, Costello P, Li W, et al. CT features of pulmonary alveolar proteinosis. AJR Am JRoentgenol. 2001; 176:1287–1294
  6. 6. Whole-Lung Lavage (WLL)• First line of treatment• Large-volume BAL• Wash out the proteinaceous material from the alveoli – Reestablish effective oxygenation and ventilation
  7. 7. 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
  8. 8. 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%
  9. 9. The Contraindications of Whole-Lung Lavage• Uncorrectable blood dyscrasias• Anesthetic risks• Cardiopulmonary instability
  10. 10. 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
  11. 11. 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
  12. 12. 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
  13. 13. 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
  14. 14. 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)
  15. 15. 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
  16. 16. Procedure (Cont.)• 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
  17. 17. 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
  18. 18. 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
  19. 19. 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
  20. 20. In the case of bilateral disease, the contralateral lung may be lavaged in 24 to 48 h if the patient remains stable
  21. 21. 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 inpulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  22. 22. 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, they have not been found to be 7 necessary in most casesBeccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage inpulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  23. 23. 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
  24. 24. Outcomes and Follow-up • Significant clinical improvement in the majority of patients • Approximately 15% of patients will have a relapsing courseBeccaria M, Luisetti M, Rodi G, et al. Long-term durable benefit after whole lung lavage inpulmonary alveolar proteinosis. Eur Respir J 2004; 23:526–531
  25. 25. 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 alveoliUchida K, Beck D, Yamamoto T, et al. GM-CSF autoantibodies and neutrophil dysfunction inpulmonary alveolar proteinosis. N Engl J Med 2007; 356:567–579Greenhill 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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