Accepted Manuscript Etiology and new treatment options for patients with plastic bronchitis Yoav Dori, MD PhD, Maxim Itkin, MD PII:
S0022-5223(16)30316-6
DOI:
10.1016/j.jtcvs.2016.05.008
Reference:
YMTC 10602
To appear in:
The Journal of Thoracic and Cardiovascular Surgery
Received Date: 2 May 2016 Accepted Date: 3 May 2016
Please cite this article as: Dori Y, Itkin M, Etiology and new treatment options for patients with plastic bronchitis, The Journal of Thoracic and Cardiovascular Surgery (2016), doi: 10.1016/j.jtcvs.2016.05.008. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Etiology and new treatment options for patients with plastic bronchitis
Maxim Itkin MD Hospital of the University of Pennsylvania, Radiology [email protected]
RI PT
Yoav Dori MD PhD Corresponding author The Children's Hospital of Philadelphia, Cardiology [email protected]
M AN U
SC
In this issue of the journal, Ugaki and colleagues describe a case of a 4-year-old child diagnosed with hypoplastic left heart syndrome and plastic bronchitis (PB) that developed shortly after his Fontan operation. The patient failed conventional management including nebulized tPA and Dornase Alpha, and Fontan fenestration. Due to persistent symptoms of PB bilateral groin intranodal lymphangiography was performed. Access to the cisterna chyli was obtained via the transhepatic approach and contrast injection demonstrated an accessory right duct perfusing the right hilar and peribronchial lymphatics. The thoracic duct (TD) could not be selectively cannulated so disruption of the cisterna chyli was performed. The patient had an improvement in his symptoms but ultimately succumbed to hypoxia and other comorbidities.
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Until recently the etiology of PB was poorly understood although lymphatic involvement has been considered to be part of the pathological process.1-3 Poor understanding of the disease process has led to variability in therapies across centers. Therapies have been aimed at either lowering central venous pressure using medications such as sildenafil or catheter techniques by creation of a fenestration or they have focused on prevention of cast formation using inhaled therapies such as inhaled tPA. These therapies have led to symptomatic improvement in some cases but usually do not lead to resolution of symptoms.4 Heart transplantation has been reported in some cases to result in long term resolution of symptoms but has a high mortality risks.5 Recent development of new lymphatic imaging techniques has demonstrated abnormal pulmonary lymphatic perfusion in most patients who have developed the disease after single ventricle palliation.6-8 Furthermore, embolization of the ducts responsible for this flow has been shown to be safe and effective and can result in short term and potentially long-term resolution of symptoms alleviating the need for heart transplant. As a result, percutaneous lymphatic based interventions are now becoming the main therapeutic approach to patient's with this disorder.7,8 The cause of plastic bronchitis in this report is consistent with type I plastic bronchitis due to a single lymphatic channel perfusing the right sided peribronchial lymphatic network.8 Selective catheterization and embolization of these abnormal lymphatic ducts has been demonstrated to resolved PB while maintaining central lymphatic flow.7,8 In this case cannulation of the TD was not successful so disruption of the cisterna chyli was performed. Disruption of the cisterna chyli has been reported as an alternative to TD embolization in treatment of chylothorax, however with lower clinical success.9-11
ACCEPTED MANUSCRIPT
RI PT
Similarly, in this case lymphatic disruption resulted in partial improvement of the child’s symptoms and allowed him to be discharged home. We believe that complete obliteration of the abnormal pulmonary lymphatic flow by catheterization of the TD and embolization of these channels could potentially lead to long term resolution of PB. Further studies should be conducted to determine if lymphatic disruption could achieve the same outcomes as those that have been reported with lymphatic duct embolization. Wiggins J, Sheffield E, Jeffery PK, Geddes DM, Corrin B. Bronchial casts associated with hilar lymphatic and pulmonary lymphoid abnormalities. Thorax. 1989;44(3):226-227.
2.
Hug MI, Ersch J, Moenkhoff M, Burger R, Fanconi S, Bauersfeld U. Chylous Bronchial Casts After Fontan Operation. Circulation. 2001;103(7):1031-1033. doi:10.1161/01.CIR.103.7.1031.
3.
Parikh K, Witte MH, Samson R, et al. Successful treatment of plastic bronchitis with low fat diet and subsequent thoracic duct ligation in child with Fontan physiology. Lymphology. 2012;45(2):47-52.
4.
Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J. A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. The Annals of Thoracic Surgery. 2014;98(2):634-640. doi:10.1016/j.athoracsur.2014.04.015.
5.
Laubisch JE, Green DM, Mogayzel PJ, Reid Thompson W. Treatment of plastic bronchitis by orthotopic heart transplantation. Pediatr Cardiol. 2011;32(8):11931195. doi:10.1007/s00246-011-9989-5.
6.
Dori Y, Zviman MM, Itkin M. Dynamic Contrast-enhanced MR Lymphangiography: Feasibility Study in Swine. Radiology. 2014;273(2):410-416. doi:10.1148/radiol.14132616.
7.
Dori Y, Keller MS, Rychik J, Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics. 2014;134(2):e590-e595. doi:10.1542/peds.2013-3723.
9.
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10.
Dori Y, Keller MS, Rome JJ, et al. Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease. Circulation. 2016;133(12):1160-1170. doi:10.1161/CIRCULATIONAHA.115.019710.
Itkin M, Kucharczuk JC, Kwak A, Trerotola SO, Kaiser LR. Nonoperative thoracic duct embolization for traumatic thoracic duct leak: experience in 109 patients. The Journal of Thoracic and Cardiovascular Surgery. 2010;139(3):584–89– discussion589–90. doi:10.1016/j.jtcvs.2009.11.025.
Binkert CA, Yucel EK, Davison BD, Sugarbaker DJ, Baum RA. Percutaneous Treatment of High-Output Chylothorax with Embolization or Needle Disruption
ACCEPTED MANUSCRIPT
Technique. Journal of Vascular and Interventional Radiology. 2005;16(9):12571262. doi:10.1097/01.RVI.0000167869.36093.43. Pamarthi V, Stecker MS, Schenker MP, et al. Thoracic duct embolization and disruption for treatment of chylous effusions: experience with 105 patients. J Vasc Interv Radiol. 2014;25(9):1398-1404. doi:10.1016/j.jvir.2014.03.027.
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ACCEPTED MANUSCRIPT
S0022-5223(16)30316-6
DOI:
10.1016/j.jtcvs.2016.05.008
Reference:
YMTC 10602
To appear in:
The Journal of Thoracic and Cardiovascular Surgery
Received Date: 2 May 2016 Accepted Date: 3 May 2016
Please cite this article as: Dori Y, Itkin M, Etiology and new treatment options for patients with plastic bronchitis, The Journal of Thoracic and Cardiovascular Surgery (2016), doi: 10.1016/j.jtcvs.2016.05.008. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Etiology and new treatment options for patients with plastic bronchitis
Maxim Itkin MD Hospital of the University of Pennsylvania, Radiology [email protected]
RI PT
Yoav Dori MD PhD Corresponding author The Children's Hospital of Philadelphia, Cardiology [email protected]
M AN U
SC
In this issue of the journal, Ugaki and colleagues describe a case of a 4-year-old child diagnosed with hypoplastic left heart syndrome and plastic bronchitis (PB) that developed shortly after his Fontan operation. The patient failed conventional management including nebulized tPA and Dornase Alpha, and Fontan fenestration. Due to persistent symptoms of PB bilateral groin intranodal lymphangiography was performed. Access to the cisterna chyli was obtained via the transhepatic approach and contrast injection demonstrated an accessory right duct perfusing the right hilar and peribronchial lymphatics. The thoracic duct (TD) could not be selectively cannulated so disruption of the cisterna chyli was performed. The patient had an improvement in his symptoms but ultimately succumbed to hypoxia and other comorbidities.
AC C
EP
TE D
Until recently the etiology of PB was poorly understood although lymphatic involvement has been considered to be part of the pathological process.1-3 Poor understanding of the disease process has led to variability in therapies across centers. Therapies have been aimed at either lowering central venous pressure using medications such as sildenafil or catheter techniques by creation of a fenestration or they have focused on prevention of cast formation using inhaled therapies such as inhaled tPA. These therapies have led to symptomatic improvement in some cases but usually do not lead to resolution of symptoms.4 Heart transplantation has been reported in some cases to result in long term resolution of symptoms but has a high mortality risks.5 Recent development of new lymphatic imaging techniques has demonstrated abnormal pulmonary lymphatic perfusion in most patients who have developed the disease after single ventricle palliation.6-8 Furthermore, embolization of the ducts responsible for this flow has been shown to be safe and effective and can result in short term and potentially long-term resolution of symptoms alleviating the need for heart transplant. As a result, percutaneous lymphatic based interventions are now becoming the main therapeutic approach to patient's with this disorder.7,8 The cause of plastic bronchitis in this report is consistent with type I plastic bronchitis due to a single lymphatic channel perfusing the right sided peribronchial lymphatic network.8 Selective catheterization and embolization of these abnormal lymphatic ducts has been demonstrated to resolved PB while maintaining central lymphatic flow.7,8 In this case cannulation of the TD was not successful so disruption of the cisterna chyli was performed. Disruption of the cisterna chyli has been reported as an alternative to TD embolization in treatment of chylothorax, however with lower clinical success.9-11
ACCEPTED MANUSCRIPT
RI PT
Similarly, in this case lymphatic disruption resulted in partial improvement of the child’s symptoms and allowed him to be discharged home. We believe that complete obliteration of the abnormal pulmonary lymphatic flow by catheterization of the TD and embolization of these channels could potentially lead to long term resolution of PB. Further studies should be conducted to determine if lymphatic disruption could achieve the same outcomes as those that have been reported with lymphatic duct embolization. Wiggins J, Sheffield E, Jeffery PK, Geddes DM, Corrin B. Bronchial casts associated with hilar lymphatic and pulmonary lymphoid abnormalities. Thorax. 1989;44(3):226-227.
2.
Hug MI, Ersch J, Moenkhoff M, Burger R, Fanconi S, Bauersfeld U. Chylous Bronchial Casts After Fontan Operation. Circulation. 2001;103(7):1031-1033. doi:10.1161/01.CIR.103.7.1031.
3.
Parikh K, Witte MH, Samson R, et al. Successful treatment of plastic bronchitis with low fat diet and subsequent thoracic duct ligation in child with Fontan physiology. Lymphology. 2012;45(2):47-52.
4.
Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J. A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. The Annals of Thoracic Surgery. 2014;98(2):634-640. doi:10.1016/j.athoracsur.2014.04.015.
5.
Laubisch JE, Green DM, Mogayzel PJ, Reid Thompson W. Treatment of plastic bronchitis by orthotopic heart transplantation. Pediatr Cardiol. 2011;32(8):11931195. doi:10.1007/s00246-011-9989-5.
6.
Dori Y, Zviman MM, Itkin M. Dynamic Contrast-enhanced MR Lymphangiography: Feasibility Study in Swine. Radiology. 2014;273(2):410-416. doi:10.1148/radiol.14132616.
7.
Dori Y, Keller MS, Rychik J, Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics. 2014;134(2):e590-e595. doi:10.1542/peds.2013-3723.
9.
M AN U
TE D
EP
AC C
8.
SC
1.
10.
Dori Y, Keller MS, Rome JJ, et al. Percutaneous Lymphatic Embolization of Abnormal Pulmonary Lymphatic Flow as Treatment of Plastic Bronchitis in Patients With Congenital Heart Disease. Circulation. 2016;133(12):1160-1170. doi:10.1161/CIRCULATIONAHA.115.019710.
Itkin M, Kucharczuk JC, Kwak A, Trerotola SO, Kaiser LR. Nonoperative thoracic duct embolization for traumatic thoracic duct leak: experience in 109 patients. The Journal of Thoracic and Cardiovascular Surgery. 2010;139(3):584–89– discussion589–90. doi:10.1016/j.jtcvs.2009.11.025.
Binkert CA, Yucel EK, Davison BD, Sugarbaker DJ, Baum RA. Percutaneous Treatment of High-Output Chylothorax with Embolization or Needle Disruption
ACCEPTED MANUSCRIPT
Technique. Journal of Vascular and Interventional Radiology. 2005;16(9):12571262. doi:10.1097/01.RVI.0000167869.36093.43. Pamarthi V, Stecker MS, Schenker MP, et al. Thoracic duct embolization and disruption for treatment of chylous effusions: experience with 105 patients. J Vasc Interv Radiol. 2014;25(9):1398-1404. doi:10.1016/j.jvir.2014.03.027.
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ACCEPTED MANUSCRIPT
