Pediatric Anesthesia ISSN 1155-5645
REVIEW ARTICLE
Anesthetic techniques to facilitate lung lavage for pulmonary alveolar proteinosis in children—new airway techniques and a review of the literature Caroline A. Wilson, Sally L. Wilmshurst & Ann E. Black Department of Paediatric Anaesthesia, Great Ormond Street Hospital, London, UK
What is already known
• There is no specific equipment available to facilitate lung lavage in small children. • Different techniques have been described with varying success, including using two cuffed tubes alongside one another.
What this article adds
• Increased evidence that two cuffed tubes alongside one another can be used without airway complications and the description of new airway techniques for lung lavage.
• Guidance on appropriate airway techniques for whole lung lavage in children according to their age. Implications for translation
• Increased knowledge of techniques available to successfully facilitate whole lung lavage in small children.
Keywords anesthesia; children; pediatrics; pulmonary alveolar proteinosis; pulmonary ventilation; lung lavage Correspondence Caroline Wilson, Department of Paediatric Anaesthesia, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK Email: [email protected]
Summary Pediatric patients with pulmonary alveolar proteinosis require whole lung lavage to clear the accumulation of lipoproteinaceous material within the alveoli, to maintain respiratory function. Anesthesia for this presents a challenge due to preexisting respiratory failure, and the small diameter and length of the pediatric airway, which is often unable to accommodate existing onelung isolation and ventilation equipment. Novel techniques to facilitate lung lavage on seven occasions are described and placed in the context of the existing literature to date.
Section Editor: Per-Arne Lonnqvist Accepted 1 January 2015 doi:10.1111/pan.12626
Background Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange (1). The disease is classified into congenital, secondary, and acquired forms (2). The clinical course is unpredictable and spontaneous remission has been described. Some children © 2015 John Wiley & Sons Ltd
have stable symptoms and others develop progressive type two respiratory failure (1). Whole lung lavage was first described in 1963 and remains the standard treatment to date for the acquired (or idiopathic) form (3,4). There is no standardized anesthetic approach to facilitating lung lavage in the pediatric population. One lung needs to be isolated to lavage fluid down it, while the other lung is ventilated. In adult patients, use of a cuffed 1
Airway techniques for lung lavage
double lumen tube to protect the airway and allow lavage is the gold standard (4). This technique can also be used in older children with 26F being the smallest cuffed double lumen tube commercially available. In patients too small for these tubes, alternative methods are required to achieve lung isolation, often using two endotracheal tubes (ETTs) alongside one another. Obtaining a tube small enough to enable placement of two alongside each other in the airway, yet long enough for the cuff to extend fully inside the bronchus is challenging, and various methods have been described in the literature to date. One series reports using extracorporeal membrane oxygenation (ECMO) as their method of choice for managing airway lavage in infants and children, which avoids the need for lung isolation techniques (5). ECMO carries a risk of significant morbidity, although it may be necessary in patients with severe refractory hypoxemia. We present case reports, describe the novel techniques utilized within our institution, and place these in the context of the wider literature. Case 1 An 8-year-old child was referred to our tertiary pediatric hospital for whole lung lavage in August 2012. He had pulmonary alveolar proteinosis secondary to hemophagocytic lymphohistiocytosis for which he had received a bone marrow transplant 3 years previously. On examination, he weighed 24 kg, had severe short stature, and had signs of mild respiratory distress. He also appeared cushingoid and had voice changes related to long-term steroid use. He had an oxygen requirement of 1–3 lmin 1 to maintain oxygen saturations at 90–92%, a respiratory rate of 30 breaths per minute and fine inspiratory crepitations throughout his chest. His chest radiograph revealed reticulonodular shadowing, and computerized tomography (CT) scan showed diffuse alveolar ground-glass opacification. The theater team consisted of a consultant physician, perfusionist, radiographer, two anesthetists, an anesthetics nurse, and theater nurse. A cardiopulmonary bypass circuit was used to deliver warmed fluid (0.9% saline) under pressure into the lung being lavaged. This allowed pressures to be monitored, as well as the volumes infused and drained for repeated lung lavages. A rise in pressure indicated that the lung was full of fluid. After preoxygenation, anesthesia was induced with propofol, and atracurium 0.5 mgkg 1 was given to facilitate intubation. A 4.5-mm cuffed tube was placed into the left main bronchus and a 4.0-mm cuffed tube was placed into the trachea. The tubes used contained high-volume, low-pressure cuffs, and although we did not routinely measure cuff pressures, we used the 2
C.A. Wilson et al.
minimal occlusive technique (Where the cuff is slowly inflated until the airflow heard escaping around the cuff during a positive-pressure breath ceases.) to inflate the cuffs. Tube position was confirmed with a flexible bronchoscope. Anesthesia was maintained with isoflurane via the nonlavaged lung. The lavaged lung was oxygenated using a separate circuit between lavages. Saturations were maintained above 90% throughout the procedure. Postoperatively, the patient was intubated with a 5.5-mm uncuffed tracheal tube and was electively admitted to the Pediatric Intensive Care Unit (PICU), and received a course of dexamethasone prior to extubation. The patient required three further lung lavages between September 2012 and February 2013. The same airway management techniques were used as for the first lavage described (see Table 1 for further clinical data). There were two occasions where desaturation occurred, once due to a cuff leak (which improved on changing the tracheal tube), and once at the start of the lavage procedure. The removal of the proteinaceous material (Figure 1) resulted in improved respiratory function. Following his fourth lavage, which was uneventful, the patient was not admitted to PICU and instead was extubated postoperatively. He was awake and talking within half an hour of the procedure with no airway compromise. Since this lavage his disease has remained stable, he has not required further lavages, and he has remained well. Case 2 A 4-year-old patient was referred for a whole lung lavage in 2012. She had been diagnosed with idiopathic pulmonary alveolar proteinosis 1 year previously and was otherwise well. She had an oxygen requirement of 2 lmin 1 to maintain oxygen saturations at 90%, and nocturnal BIPAP (settings 12/6 cm H2O) had been commenced 6 months previously. On examination, she weighed 12 kg, had mild respiratory distress, a respiratory rate of 40 breaths per minute, and fine inspiratory crepitations throughout her chest on auscultation. Her chest radiograph showed extensive airspace opacification and chest CT showed extensive interlobular septal thickening with centrilobular nodularity and diffuse ground-glass changes within the alveolar spaces. She had an inhalational induction of anesthesia with oxygen and 8% sevoflurane. Atracurium (0.5 mgkg 1) was given to facilitate intubation. Anesthesia was maintained with oxygen and isoflurane. A 3.5-mm cuffed ETT was inserted into the patient’s trachea, and a 16 French Foley urinary catheter (5.28-mm external diameter) was placed into the left main bronchus. The end of © 2015 John Wiley & Sons Ltd
C.A. Wilson et al.
Airway techniques for lung lavage
Table 1 Clinical data summary of patients with pulmonary alveolar proteinosis
Date
Age at lavage (years)
Weight (kg)
Left lung
August 2012
8
24
1
Right lung
September 2012
8
22
1
Bilateral
November 2012
8
20
1
Bilateral
February 2013
8
18
2
Bilateral
December 2012
4
12
Case no.
Lavage type
1
2
Bilateral
November 2013
5
13
2
Bilateral
April 2014
5
14
Airway during lavage
Airway postlavage
Complications
4.5 cuffed LMB 4.0 cuffed trachea 4.5 cuffed RMB 4.0 cuffed trachea 4.5 cuffed LMB 4.0 cuffed trachea 4.5 cuffed LMB 4.0 cuffed trachea LLL 3.5 cuffed 16F Foley LMB RLL 3.5 cuffed trachea 4.0 cuffed RMB LLL 4.5 cuffed LMB 3.0 croup RMB RLL 4.5 cuffed RMB 3.0 croup LMB RLL 4.5 cuffed RMB 3.0 croup LMB LLL 4.5 cuffed LMB 3.0 croup RMB
5.5 uncuffed trachea
Uneventful
5.5 uncuffed trachea
Desaturation to 83%, cuff leak
5.5 uncuffed trachea
Desaturation to 71%
5.5 uncuffed trachea
Uneventful
5.0 uncuffed trachea
Desaturation to 40–60% during RLL
5.0 uncuffed trachea 5.0 uncuffed trachea
Desaturation to 38%, ST depression
REVIEW ARTICLE
Anesthetic techniques to facilitate lung lavage for pulmonary alveolar proteinosis in children—new airway techniques and a review of the literature Caroline A. Wilson, Sally L. Wilmshurst & Ann E. Black Department of Paediatric Anaesthesia, Great Ormond Street Hospital, London, UK
What is already known
• There is no specific equipment available to facilitate lung lavage in small children. • Different techniques have been described with varying success, including using two cuffed tubes alongside one another.
What this article adds
• Increased evidence that two cuffed tubes alongside one another can be used without airway complications and the description of new airway techniques for lung lavage.
• Guidance on appropriate airway techniques for whole lung lavage in children according to their age. Implications for translation
• Increased knowledge of techniques available to successfully facilitate whole lung lavage in small children.
Keywords anesthesia; children; pediatrics; pulmonary alveolar proteinosis; pulmonary ventilation; lung lavage Correspondence Caroline Wilson, Department of Paediatric Anaesthesia, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK Email: [email protected]
Summary Pediatric patients with pulmonary alveolar proteinosis require whole lung lavage to clear the accumulation of lipoproteinaceous material within the alveoli, to maintain respiratory function. Anesthesia for this presents a challenge due to preexisting respiratory failure, and the small diameter and length of the pediatric airway, which is often unable to accommodate existing onelung isolation and ventilation equipment. Novel techniques to facilitate lung lavage on seven occasions are described and placed in the context of the existing literature to date.
Section Editor: Per-Arne Lonnqvist Accepted 1 January 2015 doi:10.1111/pan.12626
Background Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange (1). The disease is classified into congenital, secondary, and acquired forms (2). The clinical course is unpredictable and spontaneous remission has been described. Some children © 2015 John Wiley & Sons Ltd
have stable symptoms and others develop progressive type two respiratory failure (1). Whole lung lavage was first described in 1963 and remains the standard treatment to date for the acquired (or idiopathic) form (3,4). There is no standardized anesthetic approach to facilitating lung lavage in the pediatric population. One lung needs to be isolated to lavage fluid down it, while the other lung is ventilated. In adult patients, use of a cuffed 1
Airway techniques for lung lavage
double lumen tube to protect the airway and allow lavage is the gold standard (4). This technique can also be used in older children with 26F being the smallest cuffed double lumen tube commercially available. In patients too small for these tubes, alternative methods are required to achieve lung isolation, often using two endotracheal tubes (ETTs) alongside one another. Obtaining a tube small enough to enable placement of two alongside each other in the airway, yet long enough for the cuff to extend fully inside the bronchus is challenging, and various methods have been described in the literature to date. One series reports using extracorporeal membrane oxygenation (ECMO) as their method of choice for managing airway lavage in infants and children, which avoids the need for lung isolation techniques (5). ECMO carries a risk of significant morbidity, although it may be necessary in patients with severe refractory hypoxemia. We present case reports, describe the novel techniques utilized within our institution, and place these in the context of the wider literature. Case 1 An 8-year-old child was referred to our tertiary pediatric hospital for whole lung lavage in August 2012. He had pulmonary alveolar proteinosis secondary to hemophagocytic lymphohistiocytosis for which he had received a bone marrow transplant 3 years previously. On examination, he weighed 24 kg, had severe short stature, and had signs of mild respiratory distress. He also appeared cushingoid and had voice changes related to long-term steroid use. He had an oxygen requirement of 1–3 lmin 1 to maintain oxygen saturations at 90–92%, a respiratory rate of 30 breaths per minute and fine inspiratory crepitations throughout his chest. His chest radiograph revealed reticulonodular shadowing, and computerized tomography (CT) scan showed diffuse alveolar ground-glass opacification. The theater team consisted of a consultant physician, perfusionist, radiographer, two anesthetists, an anesthetics nurse, and theater nurse. A cardiopulmonary bypass circuit was used to deliver warmed fluid (0.9% saline) under pressure into the lung being lavaged. This allowed pressures to be monitored, as well as the volumes infused and drained for repeated lung lavages. A rise in pressure indicated that the lung was full of fluid. After preoxygenation, anesthesia was induced with propofol, and atracurium 0.5 mgkg 1 was given to facilitate intubation. A 4.5-mm cuffed tube was placed into the left main bronchus and a 4.0-mm cuffed tube was placed into the trachea. The tubes used contained high-volume, low-pressure cuffs, and although we did not routinely measure cuff pressures, we used the 2
C.A. Wilson et al.
minimal occlusive technique (Where the cuff is slowly inflated until the airflow heard escaping around the cuff during a positive-pressure breath ceases.) to inflate the cuffs. Tube position was confirmed with a flexible bronchoscope. Anesthesia was maintained with isoflurane via the nonlavaged lung. The lavaged lung was oxygenated using a separate circuit between lavages. Saturations were maintained above 90% throughout the procedure. Postoperatively, the patient was intubated with a 5.5-mm uncuffed tracheal tube and was electively admitted to the Pediatric Intensive Care Unit (PICU), and received a course of dexamethasone prior to extubation. The patient required three further lung lavages between September 2012 and February 2013. The same airway management techniques were used as for the first lavage described (see Table 1 for further clinical data). There were two occasions where desaturation occurred, once due to a cuff leak (which improved on changing the tracheal tube), and once at the start of the lavage procedure. The removal of the proteinaceous material (Figure 1) resulted in improved respiratory function. Following his fourth lavage, which was uneventful, the patient was not admitted to PICU and instead was extubated postoperatively. He was awake and talking within half an hour of the procedure with no airway compromise. Since this lavage his disease has remained stable, he has not required further lavages, and he has remained well. Case 2 A 4-year-old patient was referred for a whole lung lavage in 2012. She had been diagnosed with idiopathic pulmonary alveolar proteinosis 1 year previously and was otherwise well. She had an oxygen requirement of 2 lmin 1 to maintain oxygen saturations at 90%, and nocturnal BIPAP (settings 12/6 cm H2O) had been commenced 6 months previously. On examination, she weighed 12 kg, had mild respiratory distress, a respiratory rate of 40 breaths per minute, and fine inspiratory crepitations throughout her chest on auscultation. Her chest radiograph showed extensive airspace opacification and chest CT showed extensive interlobular septal thickening with centrilobular nodularity and diffuse ground-glass changes within the alveolar spaces. She had an inhalational induction of anesthesia with oxygen and 8% sevoflurane. Atracurium (0.5 mgkg 1) was given to facilitate intubation. Anesthesia was maintained with oxygen and isoflurane. A 3.5-mm cuffed ETT was inserted into the patient’s trachea, and a 16 French Foley urinary catheter (5.28-mm external diameter) was placed into the left main bronchus. The end of © 2015 John Wiley & Sons Ltd
C.A. Wilson et al.
Airway techniques for lung lavage
Table 1 Clinical data summary of patients with pulmonary alveolar proteinosis
Date
Age at lavage (years)
Weight (kg)
Left lung
August 2012
8
24
1
Right lung
September 2012
8
22
1
Bilateral
November 2012
8
20
1
Bilateral
February 2013
8
18
2
Bilateral
December 2012
4
12
Case no.
Lavage type
1
2
Bilateral
November 2013
5
13
2
Bilateral
April 2014
5
14
Airway during lavage
Airway postlavage
Complications
4.5 cuffed LMB 4.0 cuffed trachea 4.5 cuffed RMB 4.0 cuffed trachea 4.5 cuffed LMB 4.0 cuffed trachea 4.5 cuffed LMB 4.0 cuffed trachea LLL 3.5 cuffed 16F Foley LMB RLL 3.5 cuffed trachea 4.0 cuffed RMB LLL 4.5 cuffed LMB 3.0 croup RMB RLL 4.5 cuffed RMB 3.0 croup LMB RLL 4.5 cuffed RMB 3.0 croup LMB LLL 4.5 cuffed LMB 3.0 croup RMB
5.5 uncuffed trachea
Uneventful
5.5 uncuffed trachea
Desaturation to 83%, cuff leak
5.5 uncuffed trachea
Desaturation to 71%
5.5 uncuffed trachea
Uneventful
5.0 uncuffed trachea
Desaturation to 40–60% during RLL
5.0 uncuffed trachea 5.0 uncuffed trachea
Desaturation to 38%, ST depression
