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Aggressive bronchoscopic management of plastic bronchitis
International Journal of Pediatric Otorhinolaryngology 74 (2010) 820–822
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Aggressive bronchoscopic management of plastic bronchitis Diego Preciado a,*, Susan Verghese b, Sukgi Choi a a Division of Pediatric Otolaryngology-Head and Neck Surgery, Children’s National Medical Center, George Washington University, 111 Michigan Ave NW, Washington, DC 20010, United States b Division of Anesthesiology, Children’s National Medical Center, George Washington University, Washington, DC, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 December 2009 Received in revised form 2 February 2010 Accepted 3 February 2010
Plastic bronchitis or ‘‘Bronchitis Plastica’’ is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children’s hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week’s period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex. ß 2010 Elsevier Ireland Ltd. All rights reserved.
Keywords: Plastic bronchitis Fontan Bronchial casts
1. Introduction Plastic bronchitis is an extremely rare condition characterized by the presence of large thick, mucofibrinous plugs filling the pulmonary bronchial tree resulting in severe respiratory distress [1,2]. Children with underlying cardiovascular disease are at risk of developing this condition [1–5]. We present three cases of children with plastic bronchitis after having undergone a Fontan procedure for hypoplastic left ventricle syndrome. Their differing clinical evolutions underscore the range of possible outcomes in this particularly difficult to treat clinical entity. IRB approval was obtained to review our recent experience with plastic bronchitis, in a de-identified fashion. 2. Case reports All three children had Fontan procedures during their first year of life. The two children who presented at 3 years of age had a subacute history of expectorating white cheesy material and increasing respiratory difficulties requiring intubation and mechanical ventilation in the cardiac intensive care unit. In all three patients, chest
* Corresponding author. Tel.: +1 202 884 3659; fax: +1 202 884 5038. E-mail address: [email protected] (D. Preciado). 0165-5876/$ – see front matter ß 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2010.02.005
radiographs revealed right-sided diffuse lung opacification with hyperinflation suggestive of bronchial obstruction. Initial rigid bronchoscopy in the patients revealed purely right-sided plastic bronchitis with an occlusive cast. The first child recovered fully after a single bronchoscopic procedure for cast removal (Fig. 1). The second child, however, required 3 separate serial rigid bronchoscopies over a week’s period, for removal of the casts (Fig. 2A–C). Each successive debridement of the casts revealed them to be less solid, but more gelatinous each time. Increasing gross amounts of copious lymph-like fluid were encountered sequentially each time. Extracorporeal membrane oxygenation (ECMO) for 5 days was necessary to maintain effective blood oxygenation. This child was treated with n-acetylcysteine, systemic steroids, and azithromycin. Eventually, pulmonary function returned to normal and bronchoscopic findings normalized. However, he suffered a small hemorrhagic stroke as a consequence of ECMO. Unfortunately, he presented 2 months later with recurrence (Fig. 2D) of casts and after 3 more bronchoscopic interventions for cast removal; he ultimately passed away due to intractable pulmonary failure. Repeated cardiac catheterizations had demonstrated a normally functioning Fontan with normal pulmonary artery pressures. The third child presented at 8 months of age, 6 months after the Fontan procedure, with acute respiratory distress and right-sided bronchial casts requiring bronchoscopic removal and hemodynamic support on ECMO for 7 days. Similar to the second child,
D. Preciado et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 820–822
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Fig. 1. Bronchoscopic view of bronchial cast in Case 1.
Fig. 2. (A) Initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (B) 2 days after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (C) 4 days after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (D) 2 months after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water.
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three separate debridement bronchoscopies were needed during the week the child was on ECMO. Much like the second case, the casts were noted to be increasingly more gelatinous at each endoscopy. This child was also treated with n-acetylcysteine, systemic steroids, and azithromycin. Eventually he was wean off of ECMO, extubated and discharged home after a 3-week period of hospitalization. He has not returned with recurrence and remains symptom free at the 4 months follow-up visit. Cardiac catheterizations demonstrate a normally functioning Fontan with appropriate pulmonary artery pressures. 3. Discussion Plastic bronchitis is characterized by the occurrence of acute and often recurrent bronchial casts. They can occur in patients with inflammatory pulmonary conditions, where the casts are typically composed of fibrinous material with eosinophilic infiltrates, or in patients with underlying cardiovascular disease, where the casts are more often acellular with mucin and no acute inflammatory cells [6]. In the latter instance the bronchiopulmonary endobronchial lymphatic leakage is felt to contribute to their development. This can occur due to lymphatic trauma, adhesions, or elevated central pressures. All of our patients presented with acellular casts, but the second case had gross bronchial lymphatic fluid, which seemed to worsen after each intervention for cast removal. The organized exudate takes the anatomic configuration of the tracheobronchial tree and plugs the airways in a recurrent fashion with respiratory compromise. Adjunctive medical therapeutic options in general for this condition include aerosolized Nacetylcystein, aerosolized urokinase, dornase-alpha, t-PA, steroids combined with a bronchodilator, parenteral heparin and low dose oral azithromycin. The hemodynamic improvements can be achieved with the use pulmonary vasodilators like nitric oxide, sildanefil, and with inotropy, using milrinone infusion combined with diuretics in the CICU. There are many anecdotal surgical treatments for plastic bronchitis that have been reported in plastic bronchitis patients with a history of Fontan procedures, which include, thoracic duct ligation, refenestration of the Fontan circuit, and pacemaker placement [7–10]. The rarity of plastic bronchitis, however, prohibits systematic investigations of its treatment. Our cases highlight the importance of early diagnosis when faced with clinical scenario of respiratory failure in Fontan patients and the reported difficulties in managing this complex condition [11]. One of the main challenges is the need to use high ventilating pressure in Fontan (or single ventricle) patients, who rely on very low pulmonary vascular resistance (PVR) for their cardiac output for management of their respiratory failure and during bronchoscopy. Since the preload to the lungs is from the passive flow of blood from the superior and inferior vena cavae without the
benefit of a pumping ventricle, any rise in PVR from hypoxia, hypercarbia, high airway pressure, positive end-expiratory pressure, and repeated bronchoscopy, will decrease the passive blood flow to the pulmonary system. Keeping this in mind, adequate volume resuscitation, inotropy, limiting the duration of apnea and bronchoscopy, as well as maintaining the patient with slightly head-up position to encourage passive flow of blood from the SVC to the lungs are important measures to optimize the cardiac output in these hemodynamically unstable patients. An ongoing discussion with the anesthesiologists and intensivists who are familiar with this compromising situation is essential for the safe management of these patients. In conclusion, management of this rare and complex clinical situation is both difficult and frustrating. A high degree of suspicion of plastic bronchitis should be kept in mind when faced with a scenario of a child presenting to ER with history of expectorating white cheesy material accompanied by a chest X-ray with complete lung opacification especially after a Fontan repair for single ventricle. Early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary to manage these children who may require ECMO for hemodynamic support. The differing clinical evolutions of cases presented in our series underscore the range of possible outcomes for plastic bronchitis, as some patients appear to fully recover while others are at risk of fulminant recurrence and possible death. References [1] J. Werkhaven, L.D. Holinger, Bronchial casts in children, Ann. Otol. Rhinol. Laryngol. 96 (1987) 86–92. [2] T.V. Brogan, L.S. Finn, D.J. Pyskaty Jr., G.J. Redding, D. Ricker, A. Inglis, et al., Plastic bronchitis in children: a case series and review of the medical literature, Pediatr. Pulmonol. 34 (6) (2002) 482–487. [3] S. Ishman, D.T. Book, S.F. Conley, J.E. Kerschner, Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 543–548. [4] H.J. Zaccagni, L. Kirchner, J. Brownlee, K. Bloom, A case of plastic bronchitis presenting 9 years after Fontan, Pediatr. Cardiol. 29 (2008) 157–159. [5] B. Stiller, F. Riedel, K. Paul, F.K. van Landeghem, Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy? Pediatr. Cardiol. 23 (2002) 90–94. [6] M. Seear, H. Hui, F. Magee, D. Bohn, E. Cutz, Bronchial casts in children: a proposed classification based on nine cases and a review of the literature, Am. J. Respir. Crit. Care Med 155 (1997) 364–370. [7] J. Wilson, J. Russell, W. Williams, L. Benson, Fenestration of the Fontan circuit as treatment for plastic bronchitis, Pediatr. Cardiol. 26 (2005) 717–719. [8] B.J. Barber, G.H. Burch, D. Tripple, S. Balaji, Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology, Pediatr. Cardiol. 25 (2004) 73– 76. [9] S.S. Shah, D.C. Drinkwater, K.G. Christian, Plastic bronchitis: is thoracic duct ligation a real surgical option? Ann. Thorac. Surg. 81 (2006) 2281–2283. [10] M. Chaudhari, O. Stumper, Plastic bronchitis after Fontan operation: treatment with stent fenestration of the Fontan circuit, Heart 90 (2004) 801. [11] M.H. Eberlein, M.B. Drummond, E.F. Haponik, Plastic bronchitis: a management challenge, Am. J. Med. Sci. 335 (2008) 163–169.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Case report
Aggressive bronchoscopic management of plastic bronchitis Diego Preciado a,*, Susan Verghese b, Sukgi Choi a a Division of Pediatric Otolaryngology-Head and Neck Surgery, Children’s National Medical Center, George Washington University, 111 Michigan Ave NW, Washington, DC 20010, United States b Division of Anesthesiology, Children’s National Medical Center, George Washington University, Washington, DC, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 December 2009 Received in revised form 2 February 2010 Accepted 3 February 2010
Plastic bronchitis or ‘‘Bronchitis Plastica’’ is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children’s hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week’s period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex. ß 2010 Elsevier Ireland Ltd. All rights reserved.
Keywords: Plastic bronchitis Fontan Bronchial casts
1. Introduction Plastic bronchitis is an extremely rare condition characterized by the presence of large thick, mucofibrinous plugs filling the pulmonary bronchial tree resulting in severe respiratory distress [1,2]. Children with underlying cardiovascular disease are at risk of developing this condition [1–5]. We present three cases of children with plastic bronchitis after having undergone a Fontan procedure for hypoplastic left ventricle syndrome. Their differing clinical evolutions underscore the range of possible outcomes in this particularly difficult to treat clinical entity. IRB approval was obtained to review our recent experience with plastic bronchitis, in a de-identified fashion. 2. Case reports All three children had Fontan procedures during their first year of life. The two children who presented at 3 years of age had a subacute history of expectorating white cheesy material and increasing respiratory difficulties requiring intubation and mechanical ventilation in the cardiac intensive care unit. In all three patients, chest
* Corresponding author. Tel.: +1 202 884 3659; fax: +1 202 884 5038. E-mail address: [email protected] (D. Preciado). 0165-5876/$ – see front matter ß 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2010.02.005
radiographs revealed right-sided diffuse lung opacification with hyperinflation suggestive of bronchial obstruction. Initial rigid bronchoscopy in the patients revealed purely right-sided plastic bronchitis with an occlusive cast. The first child recovered fully after a single bronchoscopic procedure for cast removal (Fig. 1). The second child, however, required 3 separate serial rigid bronchoscopies over a week’s period, for removal of the casts (Fig. 2A–C). Each successive debridement of the casts revealed them to be less solid, but more gelatinous each time. Increasing gross amounts of copious lymph-like fluid were encountered sequentially each time. Extracorporeal membrane oxygenation (ECMO) for 5 days was necessary to maintain effective blood oxygenation. This child was treated with n-acetylcysteine, systemic steroids, and azithromycin. Eventually, pulmonary function returned to normal and bronchoscopic findings normalized. However, he suffered a small hemorrhagic stroke as a consequence of ECMO. Unfortunately, he presented 2 months later with recurrence (Fig. 2D) of casts and after 3 more bronchoscopic interventions for cast removal; he ultimately passed away due to intractable pulmonary failure. Repeated cardiac catheterizations had demonstrated a normally functioning Fontan with normal pulmonary artery pressures. The third child presented at 8 months of age, 6 months after the Fontan procedure, with acute respiratory distress and right-sided bronchial casts requiring bronchoscopic removal and hemodynamic support on ECMO for 7 days. Similar to the second child,
D. Preciado et al. / International Journal of Pediatric Otorhinolaryngology 74 (2010) 820–822
821
Fig. 1. Bronchoscopic view of bronchial cast in Case 1.
Fig. 2. (A) Initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (B) 2 days after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (C) 4 days after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water. (D) 2 months after initial presentation of Case 2. Bronchoscopic view of bronchial cast in left panel and view of the cast under water.
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three separate debridement bronchoscopies were needed during the week the child was on ECMO. Much like the second case, the casts were noted to be increasingly more gelatinous at each endoscopy. This child was also treated with n-acetylcysteine, systemic steroids, and azithromycin. Eventually he was wean off of ECMO, extubated and discharged home after a 3-week period of hospitalization. He has not returned with recurrence and remains symptom free at the 4 months follow-up visit. Cardiac catheterizations demonstrate a normally functioning Fontan with appropriate pulmonary artery pressures. 3. Discussion Plastic bronchitis is characterized by the occurrence of acute and often recurrent bronchial casts. They can occur in patients with inflammatory pulmonary conditions, where the casts are typically composed of fibrinous material with eosinophilic infiltrates, or in patients with underlying cardiovascular disease, where the casts are more often acellular with mucin and no acute inflammatory cells [6]. In the latter instance the bronchiopulmonary endobronchial lymphatic leakage is felt to contribute to their development. This can occur due to lymphatic trauma, adhesions, or elevated central pressures. All of our patients presented with acellular casts, but the second case had gross bronchial lymphatic fluid, which seemed to worsen after each intervention for cast removal. The organized exudate takes the anatomic configuration of the tracheobronchial tree and plugs the airways in a recurrent fashion with respiratory compromise. Adjunctive medical therapeutic options in general for this condition include aerosolized Nacetylcystein, aerosolized urokinase, dornase-alpha, t-PA, steroids combined with a bronchodilator, parenteral heparin and low dose oral azithromycin. The hemodynamic improvements can be achieved with the use pulmonary vasodilators like nitric oxide, sildanefil, and with inotropy, using milrinone infusion combined with diuretics in the CICU. There are many anecdotal surgical treatments for plastic bronchitis that have been reported in plastic bronchitis patients with a history of Fontan procedures, which include, thoracic duct ligation, refenestration of the Fontan circuit, and pacemaker placement [7–10]. The rarity of plastic bronchitis, however, prohibits systematic investigations of its treatment. Our cases highlight the importance of early diagnosis when faced with clinical scenario of respiratory failure in Fontan patients and the reported difficulties in managing this complex condition [11]. One of the main challenges is the need to use high ventilating pressure in Fontan (or single ventricle) patients, who rely on very low pulmonary vascular resistance (PVR) for their cardiac output for management of their respiratory failure and during bronchoscopy. Since the preload to the lungs is from the passive flow of blood from the superior and inferior vena cavae without the
benefit of a pumping ventricle, any rise in PVR from hypoxia, hypercarbia, high airway pressure, positive end-expiratory pressure, and repeated bronchoscopy, will decrease the passive blood flow to the pulmonary system. Keeping this in mind, adequate volume resuscitation, inotropy, limiting the duration of apnea and bronchoscopy, as well as maintaining the patient with slightly head-up position to encourage passive flow of blood from the SVC to the lungs are important measures to optimize the cardiac output in these hemodynamically unstable patients. An ongoing discussion with the anesthesiologists and intensivists who are familiar with this compromising situation is essential for the safe management of these patients. In conclusion, management of this rare and complex clinical situation is both difficult and frustrating. A high degree of suspicion of plastic bronchitis should be kept in mind when faced with a scenario of a child presenting to ER with history of expectorating white cheesy material accompanied by a chest X-ray with complete lung opacification especially after a Fontan repair for single ventricle. Early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary to manage these children who may require ECMO for hemodynamic support. The differing clinical evolutions of cases presented in our series underscore the range of possible outcomes for plastic bronchitis, as some patients appear to fully recover while others are at risk of fulminant recurrence and possible death. References [1] J. Werkhaven, L.D. Holinger, Bronchial casts in children, Ann. Otol. Rhinol. Laryngol. 96 (1987) 86–92. [2] T.V. Brogan, L.S. Finn, D.J. Pyskaty Jr., G.J. Redding, D. Ricker, A. Inglis, et al., Plastic bronchitis in children: a case series and review of the medical literature, Pediatr. Pulmonol. 34 (6) (2002) 482–487. [3] S. Ishman, D.T. Book, S.F. Conley, J.E. Kerschner, Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair, Int. J. Pediatr. Otorhinolaryngol. 67 (2003) 543–548. [4] H.J. Zaccagni, L. Kirchner, J. Brownlee, K. Bloom, A case of plastic bronchitis presenting 9 years after Fontan, Pediatr. Cardiol. 29 (2008) 157–159. [5] B. Stiller, F. Riedel, K. Paul, F.K. van Landeghem, Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy? Pediatr. Cardiol. 23 (2002) 90–94. [6] M. Seear, H. Hui, F. Magee, D. Bohn, E. Cutz, Bronchial casts in children: a proposed classification based on nine cases and a review of the literature, Am. J. Respir. Crit. Care Med 155 (1997) 364–370. [7] J. Wilson, J. Russell, W. Williams, L. Benson, Fenestration of the Fontan circuit as treatment for plastic bronchitis, Pediatr. Cardiol. 26 (2005) 717–719. [8] B.J. Barber, G.H. Burch, D. Tripple, S. Balaji, Resolution of plastic bronchitis with atrial pacing in a patient with fontan physiology, Pediatr. Cardiol. 25 (2004) 73– 76. [9] S.S. Shah, D.C. Drinkwater, K.G. Christian, Plastic bronchitis: is thoracic duct ligation a real surgical option? Ann. Thorac. Surg. 81 (2006) 2281–2283. [10] M. Chaudhari, O. Stumper, Plastic bronchitis after Fontan operation: treatment with stent fenestration of the Fontan circuit, Heart 90 (2004) 801. [11] M.H. Eberlein, M.B. Drummond, E.F. Haponik, Plastic bronchitis: a management challenge, Am. J. Med. Sci. 335 (2008) 163–169.