- Email: [email protected]
Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children
Journal of Pediatric Surgery xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children Tutku Soyer a,⁎, Şule Yalcin a, Nagehan Emiralioğlu b, Ebru Arik Yilmaz c, Ozge Soyer c, Diclehan Orhan d, Deniz Doğru b, Bülent Enis Sekerel c, Feridun Cahit Tanyel a a
Hacettepe University, Faculty of Medicine, Department of Pediatric Surgery, Ankara, Turkey Hacettepe University, Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Hacettepe University, Faculty of Medicine, Department of Pediatric Allergy, Ankara, Turkey d Hacettepe University, Faculty of Medicine, Department of Pediatric Pathology, Ankara, Turkey b c
a r t i c l e
i n f o
Article history: Received 13 January 2016 Received in revised form 25 February 2016 Accepted 29 March 2016 Available online xxxx Key words: Plastic bronchitis Bronchial cast Asthma Bronchoscopy Children
a b s t r a c t Aim: Plastic bronchitis (PB) is a rare disorder characterized by formation of bronchial casts (BC) in the tracheobronchial tree with partial or complete airway obstruction. Although lysis of casts with several fibrinolytic agents has been reported, removal of BC with bronchoscope provides better clearance of airways. A retrospective study was performed to evaluate the use of serial rigid bronchoscopy (RB) in the treatment of PB in children. Patients and methods: Between 2011 and 2015, children with partial or complete airway obstruction with PB were evaluated for age, gender, underlying disease, clinical findings, results of bronchoscopic interventions and histopathologic findings. Results: Five patients with 14 RB interventions were evaluated. The mean age of the patients was 7.8 years (min: 3 years — max: 14 years) and male–female ratio was 4:1. All of the patients were diagnosed as asthma and none of them had underlying cardiac disease. Suction of mucus plaques and bronchoalveolar lavage were performed in all patients with flexible bronchoscopy. Also, aerosolized tissue plasminogen activator was used in two patients. During follow-up serial RB was indicated in patients with persistent atelectasis and severe airway obstruction. The most common localization of BC was left main stem bronchus and bilateral cast formation was detected in 7 interventions. Although, removal of BC was challenging in two patients because of cast friability and fragmentation, most of the plugs were successfully removed with optical forceps and rigid suctioning. Two patients underwent repeated RB (min: 3 — max: 8) for recurrent symptoms. Histopathologic evaluation of BC revealed Charcot–Leyden crystals with inflammatory cells in all patients. The time interval between RB interventions was one to five months. Conclusion: BC are tenacious mucus plugs which are firmly wedged to the tracheobronchial tree. The use of optical forceps with rigid suction provides adequate removal of BC during RB. Because of underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial RB when they become unresponsive to standard therapy or develop partial or complete airway obstruction. © 2016 Elsevier Inc. All rights reserved.
Plastic bronchitis (PB) or cast bronchitis is a rare condition characterized by presence of mucofibrinous plugs that can partially or completely obstruct the tracheobronchial tree [1]. Although the pathophysiology of PB formation is not well understood, children with underlying cardiac disease are at risk of developing bronchial casts especially after surgical procedures that direct systemic blood flow into the pulmonary circulation [2]. Casts are more adhesive than mucous plugs, and conform the shape of the tracheobronchial tree [3]. Therefore, it may completely occlude the airway and present with cough, fever, dyspnea and wheezing. Several therapeutic agents including inhaled or systemic corticosteroids, aerosolized acetylcysteine, tissue plasminogen activator (tPA), ⁎ Corresponding author. Tel.: +90 532 6651960. E-mail address: [email protected] (T. Soyer).
and intravenous and aerosolized heparin have been used for PB [4]. Patients who are unresponsive to medical treatment and profound airway obstruction may require flexible and rigid bronchoscopic lavage and direct bronchoscopic removal of casts [5]. However, none of them has been proven to be effective for all patients. In spite of promising results with fibrinolytic agents, BC bronchial cast usually recurs because of underlying disease. Therefore, removal of BC with bronchoscopy provides better clearance of airway. A retrospective study was performed to evaluate the use of serial rigid bronchoscopy in treatment of PB in children.
1. Patients and methods Between 2011 and 2015, children with partial or complete airway obstruction with PB were evaluated for age, sex, underlying disease,
http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017 0022-3468/© 2016 Elsevier Inc. All rights reserved.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
2
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Table 1 Clinical features and flexible bronchoscopic findings of patients. Case 1
Case 2
Case 3
Case 4
Case 5
Sex/age (years) Initial symptom
M/7 Recurrent lung infections Decreased chest sounds on left hemithorax
Fine rales on right lung and bilateral bronchi
M/14 Asthma, recurrent lung infections, left lung atelectasis Bilateral fine rales, lymphedema on left legs
Chest X-ray
Left lower lobe atelectasis
Left lower lobe atelectasis
Left lung total atelectasis
Interstitial infiltration
Chest CT
Left lower lobe subsegmental atelectasis
Left lower lobe subsegmental atelectasis
M/5 Recurrent lung infections Decreased chest sounds on left hemithorax Left lung total atelectasis Left lung total atelectasis
M/3 Recurrent lung infections
Physical examination
F/10 Asthma, left lower lobe atelectasis Decreased chest sounds on left lower lobe
Left lung total atelectasis
Echocardiography Previous asthma history Lung biopsy Flexible bronchoscopy
Normal No
Normal Yes
Normal No
Mediastinal LAP (1.5 cm size maximum) Left upper lobe atelectasis, Left lower lobe bronchial dilatation, Right lower lobe and middle lobe atelectasis PDA No
Bronchial cast in left lower lobe bronchus No No
Bronchial cast in Bronchial cast in left main left main bronchus bronchus and right upper and lower lobe bronchus No Yes Yes (7 days) Yes (12 days)
No
No
Bronchial cast in left upper lobe and lower lobe bronchus tPA with Bronchoscopy No Inhaled tPA No (Only at hospital) Complication No
clinical findings, results of bronchoscopic interventions and histopathologic findings. Flexible and rigid bronchoscopic evaluations were performed under general anesthesia. Optical forceps (Storz R, Germany) and rigid suctions were used during removal of casts. Histopathologic evaluations of bronchial casts were evaluated with hematoxylin–eosin staining and Charcot–Leyden crystals were considered as typical histopathologic finding for PB. Cystic fibrosis was excluded with sweat test. Immune deficiency and tuberculosis were excluded after laboratory examinations. Congenital heart diseases were investigated with echocardiography. Chest CT was performed to all of the patients.
Yes (hemorrhage during the procedure)
Normal Yes Lipid laden macrophages Bronchial cast in left main bronchus
Yes Yes (10 days) No
2. Results Five patients with 14 rigid bronchoscopic (RB) interventions were evaluated. The mean age of the patients was 7.8 years (min: 3 years — max: 14 years) and male–female ratio was 4:1. Initial symptoms, physical findings, and radiologic evaluations of patients were listed in Table 1. None of the patients had underlying cardiac disease and only two of them had a previous history of asthma. All of the patients underwent flexible bronchoscopy (FB). The findings of FB were listed in Table 1. Two of the patients received tissue plasminogen activator (tPA) during FB and three of them received inhaled tPA with a duration
Fig. 1. Chest X-ray before (a) and after (b) rigid bronchoscopy (Case 5).
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
3
3. Discussion
Table 2 Results of rigid bronchoscopy and localizations of casts. Case
Number of RB
Localizations of BC
Complications
tPA with RB
1 2 3 4
1 1 1 3
– – – –
– – – –
5
8
L upper L main L main B main B main L main B main (n = 5) L main (n = 2) R main (n = 1)
–
+
Abbreviations: RB: rigid bronchoscopy, L: left, R: right, B: bilateral, tPA: tissue plasminogen activator.
of 9.3 days (7–12 days). Aerosolized tPA was administered at an initial dose of 12 mg, then a 10-mg dose 1 h later, and finally four 5-mg doses at 2-h intervals for the first day. In the second day and the other days, 5 mg doses at 4 h intervals were given. Suction of mucus plugs and bronchoalveolar lavage were performed in all patients with flexible bronchoscopy. One of the patients had hemorrhage during FB and it was spontaneously controlled with lavage and aspiration. All patients underwent rigid bronchoscopy because of complete or partial airway obstruction and unresponsive to medical treatment (Table 1). During follow-up, serial RB was indicated in patients with persistent atelectasis and severe airway obstruction (Fig. 1). None of the patients needed ventilatory support or supplemental oxygen or intensive care. Since patients were closely followed, none of them required emergent intervention. The mean duration of rigid bronchoscopy was 25 min (min–max: 15–55 min) and longer intervention periods were noted in firmly wedged casts. Number of interventions and cast localization in RB were listed in Table 2. The most common localization of BC was left main stem bronchus and bilateral cast formation was detected in 7 interventions. Although, removal of BC was challenging in two patients because of the cast friability and fragmentation, most of the plugs were successfully removed with optical forceps and rigid suctioning. Desaturation was seen in some of the patients during removing the dense casts that were firmly adherent to bronchial structures. Since patients were ventilated during rigid bronchoscopy, none of them developed bradycardia during the interventions. Repeated RB (min: 2 — max: 8) was performed in two patients for recurrent symptoms. Gross appearances of BC are shown in Fig. 2. Histopathologic evaluation of BC revealed Charcot–Leyden crystals with inflammatory cells in all patients. The time interval between RB interventions was one to five months. The treatment features, followup and the last visits of patients were listed in Table 3.
Plastic bronchitis is a rare condition characterized by thick, mucofibrinous plugs occluding pulmonary bronchial tree resulting in severe respiratory distress. Seerer et al. classified bronchial casts into two histopathologic groups [6]. Type I casts are inflammatory that are mainly composed of fibrous stroma with eosinophilic infiltration. They are called as Charcot–Leyden crystals and are surrounded by inflammation of the respiratory epithelium. Patients with bronchial diseases such as asthma, pneumonia, lymphangiomatosis, cystic fibrosis and acute chest syndrome associated with sickle cell disease have type I BC [7,8]. Type II cases show acellular histopathologic finding that is composed of fibrin and/or mucin without an underlying epithelial inflammation. The second type of BC is commonly seen in patients with congenital cyanotic heart disease [1]. It has been reported that the underlying disease of patients was asthma or allergic disease in 31% of cases and cardiac anomalies in 40% of cases [9]. Brogan et al. suggest that one third of the children with PB do not have an underlying disease [9]. All of our patients were diagnosed as asthma and type I BC were confirmed with Charcot–Leyden crystals in histopathologic evaluations. The pathophysiology of PB is not completely understood, but increased pulmonary venous pressure leading to abnormal response of respiratory epithelium and excessive mucus production may have a role [10]. Leakage of lymph is another proposed mechanism for cast formation. Thus, lymphangiography is suggested as a diagnostic tool in patients with type II casts. Most of the cases present with symptoms of upper respiratory infections, fever, dyspnea, wheezing and expectoration of mucus plugs. Since the mucus plugs take the anatomic configuration of tracheobronchial tree and occlude the airway, life-threatening respiratory problems may be encountered. Physical examination reveals decreased breath sounds, increased respiratory rate, nasal flaring and retractions. The clinical presentation of PB mimics various respiratory conditions. Therefore, high index of suspicion is needed to have the diagnosis. Chest radiographs and computed tomographic scans may show unilateral opacification, bronchiectasis, lung infiltrates and atelectasis [11]. Flexible bronchoscopic evaluation is mandatory to have the accurate diagnosis in most cases. In our series, variable radiologic findings were detected without diagnostic significance which is specific to PB. All of the patients had suction of mucus plugs and bronchoalveolar lavage with flexible bronchoscopy. However, rigid bronchoscopy is performed for better clearance of the airway. There is no information regarding the localization of BC in the literature. We found that left main bronchus was the most frequent localization and bilateral airway involvement was detected in seven bronchoscopic interventions. Several treatment modalities have been reported in children with PB. The fibrinolytic treatment aims the lysis of mucus plugs and removal
Fig. 2. Bronchial casts figuring the involved bronchus.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
4
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Table 3 Medical treatment, follow-up and final outcome.
Treatment
Case 1
Case 2
Case 3
Case 4
Case 5
Inhaled steroid, chest physiotherapy
Inhaled steroid, chest physiotherapy
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy, pulmozyme
2 No symptoms, PE normal, chest X-ray normal, no treatment
2 Missed the follow-up
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy, pulmozyme 2 Cough, PE chest sound decreased in left lung; chest X-ray: left lung atelectasis; low dose systemic steroid, inhaled steroid, pulmozyme, chest physiotherapy
Follow-up (years) 4 Last visit No symptoms, PE normal, chest X-ray normal, continuing inhaled steroid
7 Diagnosed with epilepsy, CD4 deficiency, no symptom, bilateral bronchi Chest X-ray: interstitial infiltration Low dose systemic steroid, inhaled steroid, chest physiotherapy
Abbreviations: PE: physical examination, tPA: tissue plasminogen activator.
with forced expectorations. Even, the results of fibrinolytic treatment are promising, mechanical clearance of the airway is needed in case of tenacious mucus plugs which are firmly wedged to the tracheobronchial tree. Both intratracheal and inhaled treatment of tPA were used in one of our patients with complete airway obstruction. However this patient did not benefit form tPA administration and required several bronchoscopic interventions. The use of bronchoscopic removal of BC is reported by Raghuram et al. in 1997 [12]. Rigid bronchoscopy has dual role in both diagnosis of casts and providing definitive treatment by suctioning and removing with optical forceps [10]. Rigid bronchoscopy provides improved visualization and control of airway with gentle ventilation throughout the procedure. Combination of rigid suctioning and use of optical forceps provides adequate removal in most of the cases. Although tenacious consistency and friability of plugs may challenge the endoscopic removal of the casts, airway clearance was improved with rigid bronchoscopy. In two of our patients, plugs fragmented and firmly wedged to tracheobronchial tree were successfully managed by rigid suctioning. We suggest that bronchoalveolar lavage with rigid suctioning is better than removal with optical forceps in case of friability and fragmentation of casts, whereas firmly adherent casts are easily removed with optical forceps. Since most of the children with PB have an underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial bronchoscopic interventions because of marked airway obstruction. We performed repeated rigid bronchoscopy in two of our patients, and one of them underwent eight different interventions with a shortest time interval of one month between two RB interventions. In conclusion, BC are tenacious mucus plugs which are firmly wedged to tracheobronchial tree. The use of optical forceps with rigid suction provides adequate removal of BC during rigid bronchoscopy. Because of underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial RB when they are
unresponsive to standard therapy or develop partial or complete airway obstruction. Acknowledgement This study is presented at 15th Congress of European Pediatric Surgeon's Association (EUPSA) in Dublin, Ireland in 2014. References [1] Walker PA, Shah SK, Letourneau PA, et al. Treatment of plastic bronchitis using serial flexible bronchoscopy and aerosolized heparin therapy. Eur J Pediatr Surg 2013;23: 157–60. [2] Ishman S, Book DT, Conley SF, et al. Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair. Int J Pediatr Otorhinolaryngol 2003;67:543–8. [3] Hasan RA, Black C, Reddy R. Plastic bronchitis in children. Fetal Pediatr Pathol 2012; 31:87–93. [4] Mateos-Corral D, Cutz E, Solomon M, et al. Plastic bronchitis as an unusual cause of mucus plugging in cystic fibrosis. Pediatr Pulmonol 2009;44:939–40. [5] Preciado D, Verghese S, Choi S. Aggressive bronchoscopic management of plastic bronchitis. Int J Pediatr Otorhinolaryngol 2010;74:820–2. [6] Seerer M, Hui H, Magee F, et al. Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care Med 1997; 155:364–70. [7] Moser C, Nussbaun E, Cooper DM. Plastic bronchitis and the role of bronchoscopy in the acute chest syndrome of sickle cell disease. Chest 2001;120:608–13. [8] Tonan M, Hashimoto S, Kimura A, et al. Successful treatment of severe asthmaassociated plastic bronchitis with extracorporeal membrane oxygenation. J Anesth 2012;26:265–8. [9] Brogan TV, Finn LS, Pyskaty DJ, et al. Plastic bronchitis in children. A case series and review of medical literature. Pediatr Pulmonol 2002;34:482–7. [10] Silva RC, Simons JP, Chi DH, et al. Endoscopic treatment of plastic bronchitis. Arch Otolaryngol Head Neck Surg 2011;137:401–3. [11] Goo HW, Jhang WK, Kim YH, et al. CT findings of plastic bronchitis in children after Fontan operation. Pediatr Radiol 2008;38:989–93. [12] Raghuram N, Pettignano R, Gal AA, et al. Plastic bronchitis: an unusual complication associated with sickle cell disease and acute chest syndrome. Pediatrics 1997;100: 139–42.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
Contents lists available at ScienceDirect
Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children Tutku Soyer a,⁎, Şule Yalcin a, Nagehan Emiralioğlu b, Ebru Arik Yilmaz c, Ozge Soyer c, Diclehan Orhan d, Deniz Doğru b, Bülent Enis Sekerel c, Feridun Cahit Tanyel a a
Hacettepe University, Faculty of Medicine, Department of Pediatric Surgery, Ankara, Turkey Hacettepe University, Faculty of Medicine, Department of Pediatric Pulmonology, Ankara, Turkey Hacettepe University, Faculty of Medicine, Department of Pediatric Allergy, Ankara, Turkey d Hacettepe University, Faculty of Medicine, Department of Pediatric Pathology, Ankara, Turkey b c
a r t i c l e
i n f o
Article history: Received 13 January 2016 Received in revised form 25 February 2016 Accepted 29 March 2016 Available online xxxx Key words: Plastic bronchitis Bronchial cast Asthma Bronchoscopy Children
a b s t r a c t Aim: Plastic bronchitis (PB) is a rare disorder characterized by formation of bronchial casts (BC) in the tracheobronchial tree with partial or complete airway obstruction. Although lysis of casts with several fibrinolytic agents has been reported, removal of BC with bronchoscope provides better clearance of airways. A retrospective study was performed to evaluate the use of serial rigid bronchoscopy (RB) in the treatment of PB in children. Patients and methods: Between 2011 and 2015, children with partial or complete airway obstruction with PB were evaluated for age, gender, underlying disease, clinical findings, results of bronchoscopic interventions and histopathologic findings. Results: Five patients with 14 RB interventions were evaluated. The mean age of the patients was 7.8 years (min: 3 years — max: 14 years) and male–female ratio was 4:1. All of the patients were diagnosed as asthma and none of them had underlying cardiac disease. Suction of mucus plaques and bronchoalveolar lavage were performed in all patients with flexible bronchoscopy. Also, aerosolized tissue plasminogen activator was used in two patients. During follow-up serial RB was indicated in patients with persistent atelectasis and severe airway obstruction. The most common localization of BC was left main stem bronchus and bilateral cast formation was detected in 7 interventions. Although, removal of BC was challenging in two patients because of cast friability and fragmentation, most of the plugs were successfully removed with optical forceps and rigid suctioning. Two patients underwent repeated RB (min: 3 — max: 8) for recurrent symptoms. Histopathologic evaluation of BC revealed Charcot–Leyden crystals with inflammatory cells in all patients. The time interval between RB interventions was one to five months. Conclusion: BC are tenacious mucus plugs which are firmly wedged to the tracheobronchial tree. The use of optical forceps with rigid suction provides adequate removal of BC during RB. Because of underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial RB when they become unresponsive to standard therapy or develop partial or complete airway obstruction. © 2016 Elsevier Inc. All rights reserved.
Plastic bronchitis (PB) or cast bronchitis is a rare condition characterized by presence of mucofibrinous plugs that can partially or completely obstruct the tracheobronchial tree [1]. Although the pathophysiology of PB formation is not well understood, children with underlying cardiac disease are at risk of developing bronchial casts especially after surgical procedures that direct systemic blood flow into the pulmonary circulation [2]. Casts are more adhesive than mucous plugs, and conform the shape of the tracheobronchial tree [3]. Therefore, it may completely occlude the airway and present with cough, fever, dyspnea and wheezing. Several therapeutic agents including inhaled or systemic corticosteroids, aerosolized acetylcysteine, tissue plasminogen activator (tPA), ⁎ Corresponding author. Tel.: +90 532 6651960. E-mail address: [email protected] (T. Soyer).
and intravenous and aerosolized heparin have been used for PB [4]. Patients who are unresponsive to medical treatment and profound airway obstruction may require flexible and rigid bronchoscopic lavage and direct bronchoscopic removal of casts [5]. However, none of them has been proven to be effective for all patients. In spite of promising results with fibrinolytic agents, BC bronchial cast usually recurs because of underlying disease. Therefore, removal of BC with bronchoscopy provides better clearance of airway. A retrospective study was performed to evaluate the use of serial rigid bronchoscopy in treatment of PB in children.
1. Patients and methods Between 2011 and 2015, children with partial or complete airway obstruction with PB were evaluated for age, sex, underlying disease,
http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017 0022-3468/© 2016 Elsevier Inc. All rights reserved.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
2
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Table 1 Clinical features and flexible bronchoscopic findings of patients. Case 1
Case 2
Case 3
Case 4
Case 5
Sex/age (years) Initial symptom
M/7 Recurrent lung infections Decreased chest sounds on left hemithorax
Fine rales on right lung and bilateral bronchi
M/14 Asthma, recurrent lung infections, left lung atelectasis Bilateral fine rales, lymphedema on left legs
Chest X-ray
Left lower lobe atelectasis
Left lower lobe atelectasis
Left lung total atelectasis
Interstitial infiltration
Chest CT
Left lower lobe subsegmental atelectasis
Left lower lobe subsegmental atelectasis
M/5 Recurrent lung infections Decreased chest sounds on left hemithorax Left lung total atelectasis Left lung total atelectasis
M/3 Recurrent lung infections
Physical examination
F/10 Asthma, left lower lobe atelectasis Decreased chest sounds on left lower lobe
Left lung total atelectasis
Echocardiography Previous asthma history Lung biopsy Flexible bronchoscopy
Normal No
Normal Yes
Normal No
Mediastinal LAP (1.5 cm size maximum) Left upper lobe atelectasis, Left lower lobe bronchial dilatation, Right lower lobe and middle lobe atelectasis PDA No
Bronchial cast in left lower lobe bronchus No No
Bronchial cast in Bronchial cast in left main left main bronchus bronchus and right upper and lower lobe bronchus No Yes Yes (7 days) Yes (12 days)
No
No
Bronchial cast in left upper lobe and lower lobe bronchus tPA with Bronchoscopy No Inhaled tPA No (Only at hospital) Complication No
clinical findings, results of bronchoscopic interventions and histopathologic findings. Flexible and rigid bronchoscopic evaluations were performed under general anesthesia. Optical forceps (Storz R, Germany) and rigid suctions were used during removal of casts. Histopathologic evaluations of bronchial casts were evaluated with hematoxylin–eosin staining and Charcot–Leyden crystals were considered as typical histopathologic finding for PB. Cystic fibrosis was excluded with sweat test. Immune deficiency and tuberculosis were excluded after laboratory examinations. Congenital heart diseases were investigated with echocardiography. Chest CT was performed to all of the patients.
Yes (hemorrhage during the procedure)
Normal Yes Lipid laden macrophages Bronchial cast in left main bronchus
Yes Yes (10 days) No
2. Results Five patients with 14 rigid bronchoscopic (RB) interventions were evaluated. The mean age of the patients was 7.8 years (min: 3 years — max: 14 years) and male–female ratio was 4:1. Initial symptoms, physical findings, and radiologic evaluations of patients were listed in Table 1. None of the patients had underlying cardiac disease and only two of them had a previous history of asthma. All of the patients underwent flexible bronchoscopy (FB). The findings of FB were listed in Table 1. Two of the patients received tissue plasminogen activator (tPA) during FB and three of them received inhaled tPA with a duration
Fig. 1. Chest X-ray before (a) and after (b) rigid bronchoscopy (Case 5).
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
3
3. Discussion
Table 2 Results of rigid bronchoscopy and localizations of casts. Case
Number of RB
Localizations of BC
Complications
tPA with RB
1 2 3 4
1 1 1 3
– – – –
– – – –
5
8
L upper L main L main B main B main L main B main (n = 5) L main (n = 2) R main (n = 1)
–
+
Abbreviations: RB: rigid bronchoscopy, L: left, R: right, B: bilateral, tPA: tissue plasminogen activator.
of 9.3 days (7–12 days). Aerosolized tPA was administered at an initial dose of 12 mg, then a 10-mg dose 1 h later, and finally four 5-mg doses at 2-h intervals for the first day. In the second day and the other days, 5 mg doses at 4 h intervals were given. Suction of mucus plugs and bronchoalveolar lavage were performed in all patients with flexible bronchoscopy. One of the patients had hemorrhage during FB and it was spontaneously controlled with lavage and aspiration. All patients underwent rigid bronchoscopy because of complete or partial airway obstruction and unresponsive to medical treatment (Table 1). During follow-up, serial RB was indicated in patients with persistent atelectasis and severe airway obstruction (Fig. 1). None of the patients needed ventilatory support or supplemental oxygen or intensive care. Since patients were closely followed, none of them required emergent intervention. The mean duration of rigid bronchoscopy was 25 min (min–max: 15–55 min) and longer intervention periods were noted in firmly wedged casts. Number of interventions and cast localization in RB were listed in Table 2. The most common localization of BC was left main stem bronchus and bilateral cast formation was detected in 7 interventions. Although, removal of BC was challenging in two patients because of the cast friability and fragmentation, most of the plugs were successfully removed with optical forceps and rigid suctioning. Desaturation was seen in some of the patients during removing the dense casts that were firmly adherent to bronchial structures. Since patients were ventilated during rigid bronchoscopy, none of them developed bradycardia during the interventions. Repeated RB (min: 2 — max: 8) was performed in two patients for recurrent symptoms. Gross appearances of BC are shown in Fig. 2. Histopathologic evaluation of BC revealed Charcot–Leyden crystals with inflammatory cells in all patients. The time interval between RB interventions was one to five months. The treatment features, followup and the last visits of patients were listed in Table 3.
Plastic bronchitis is a rare condition characterized by thick, mucofibrinous plugs occluding pulmonary bronchial tree resulting in severe respiratory distress. Seerer et al. classified bronchial casts into two histopathologic groups [6]. Type I casts are inflammatory that are mainly composed of fibrous stroma with eosinophilic infiltration. They are called as Charcot–Leyden crystals and are surrounded by inflammation of the respiratory epithelium. Patients with bronchial diseases such as asthma, pneumonia, lymphangiomatosis, cystic fibrosis and acute chest syndrome associated with sickle cell disease have type I BC [7,8]. Type II cases show acellular histopathologic finding that is composed of fibrin and/or mucin without an underlying epithelial inflammation. The second type of BC is commonly seen in patients with congenital cyanotic heart disease [1]. It has been reported that the underlying disease of patients was asthma or allergic disease in 31% of cases and cardiac anomalies in 40% of cases [9]. Brogan et al. suggest that one third of the children with PB do not have an underlying disease [9]. All of our patients were diagnosed as asthma and type I BC were confirmed with Charcot–Leyden crystals in histopathologic evaluations. The pathophysiology of PB is not completely understood, but increased pulmonary venous pressure leading to abnormal response of respiratory epithelium and excessive mucus production may have a role [10]. Leakage of lymph is another proposed mechanism for cast formation. Thus, lymphangiography is suggested as a diagnostic tool in patients with type II casts. Most of the cases present with symptoms of upper respiratory infections, fever, dyspnea, wheezing and expectoration of mucus plugs. Since the mucus plugs take the anatomic configuration of tracheobronchial tree and occlude the airway, life-threatening respiratory problems may be encountered. Physical examination reveals decreased breath sounds, increased respiratory rate, nasal flaring and retractions. The clinical presentation of PB mimics various respiratory conditions. Therefore, high index of suspicion is needed to have the diagnosis. Chest radiographs and computed tomographic scans may show unilateral opacification, bronchiectasis, lung infiltrates and atelectasis [11]. Flexible bronchoscopic evaluation is mandatory to have the accurate diagnosis in most cases. In our series, variable radiologic findings were detected without diagnostic significance which is specific to PB. All of the patients had suction of mucus plugs and bronchoalveolar lavage with flexible bronchoscopy. However, rigid bronchoscopy is performed for better clearance of the airway. There is no information regarding the localization of BC in the literature. We found that left main bronchus was the most frequent localization and bilateral airway involvement was detected in seven bronchoscopic interventions. Several treatment modalities have been reported in children with PB. The fibrinolytic treatment aims the lysis of mucus plugs and removal
Fig. 2. Bronchial casts figuring the involved bronchus.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017
4
T. Soyer et al. / Journal of Pediatric Surgery xxx (2016) xxx–xxx
Table 3 Medical treatment, follow-up and final outcome.
Treatment
Case 1
Case 2
Case 3
Case 4
Case 5
Inhaled steroid, chest physiotherapy
Inhaled steroid, chest physiotherapy
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy, pulmozyme
2 No symptoms, PE normal, chest X-ray normal, no treatment
2 Missed the follow-up
Inhaled tPA, systemic steroid, inhaled steroid, chest physiotherapy, pulmozyme 2 Cough, PE chest sound decreased in left lung; chest X-ray: left lung atelectasis; low dose systemic steroid, inhaled steroid, pulmozyme, chest physiotherapy
Follow-up (years) 4 Last visit No symptoms, PE normal, chest X-ray normal, continuing inhaled steroid
7 Diagnosed with epilepsy, CD4 deficiency, no symptom, bilateral bronchi Chest X-ray: interstitial infiltration Low dose systemic steroid, inhaled steroid, chest physiotherapy
Abbreviations: PE: physical examination, tPA: tissue plasminogen activator.
with forced expectorations. Even, the results of fibrinolytic treatment are promising, mechanical clearance of the airway is needed in case of tenacious mucus plugs which are firmly wedged to the tracheobronchial tree. Both intratracheal and inhaled treatment of tPA were used in one of our patients with complete airway obstruction. However this patient did not benefit form tPA administration and required several bronchoscopic interventions. The use of bronchoscopic removal of BC is reported by Raghuram et al. in 1997 [12]. Rigid bronchoscopy has dual role in both diagnosis of casts and providing definitive treatment by suctioning and removing with optical forceps [10]. Rigid bronchoscopy provides improved visualization and control of airway with gentle ventilation throughout the procedure. Combination of rigid suctioning and use of optical forceps provides adequate removal in most of the cases. Although tenacious consistency and friability of plugs may challenge the endoscopic removal of the casts, airway clearance was improved with rigid bronchoscopy. In two of our patients, plugs fragmented and firmly wedged to tracheobronchial tree were successfully managed by rigid suctioning. We suggest that bronchoalveolar lavage with rigid suctioning is better than removal with optical forceps in case of friability and fragmentation of casts, whereas firmly adherent casts are easily removed with optical forceps. Since most of the children with PB have an underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial bronchoscopic interventions because of marked airway obstruction. We performed repeated rigid bronchoscopy in two of our patients, and one of them underwent eight different interventions with a shortest time interval of one month between two RB interventions. In conclusion, BC are tenacious mucus plugs which are firmly wedged to tracheobronchial tree. The use of optical forceps with rigid suction provides adequate removal of BC during rigid bronchoscopy. Because of underlying disease, it is difficult to cure cast formation. Therefore, most of the patients require serial RB when they are
unresponsive to standard therapy or develop partial or complete airway obstruction. Acknowledgement This study is presented at 15th Congress of European Pediatric Surgeon's Association (EUPSA) in Dublin, Ireland in 2014. References [1] Walker PA, Shah SK, Letourneau PA, et al. Treatment of plastic bronchitis using serial flexible bronchoscopy and aerosolized heparin therapy. Eur J Pediatr Surg 2013;23: 157–60. [2] Ishman S, Book DT, Conley SF, et al. Plastic bronchitis: an unusual bronchoscopic challenge associated with congenital heart disease repair. Int J Pediatr Otorhinolaryngol 2003;67:543–8. [3] Hasan RA, Black C, Reddy R. Plastic bronchitis in children. Fetal Pediatr Pathol 2012; 31:87–93. [4] Mateos-Corral D, Cutz E, Solomon M, et al. Plastic bronchitis as an unusual cause of mucus plugging in cystic fibrosis. Pediatr Pulmonol 2009;44:939–40. [5] Preciado D, Verghese S, Choi S. Aggressive bronchoscopic management of plastic bronchitis. Int J Pediatr Otorhinolaryngol 2010;74:820–2. [6] Seerer M, Hui H, Magee F, et al. Bronchial casts in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care Med 1997; 155:364–70. [7] Moser C, Nussbaun E, Cooper DM. Plastic bronchitis and the role of bronchoscopy in the acute chest syndrome of sickle cell disease. Chest 2001;120:608–13. [8] Tonan M, Hashimoto S, Kimura A, et al. Successful treatment of severe asthmaassociated plastic bronchitis with extracorporeal membrane oxygenation. J Anesth 2012;26:265–8. [9] Brogan TV, Finn LS, Pyskaty DJ, et al. Plastic bronchitis in children. A case series and review of medical literature. Pediatr Pulmonol 2002;34:482–7. [10] Silva RC, Simons JP, Chi DH, et al. Endoscopic treatment of plastic bronchitis. Arch Otolaryngol Head Neck Surg 2011;137:401–3. [11] Goo HW, Jhang WK, Kim YH, et al. CT findings of plastic bronchitis in children after Fontan operation. Pediatr Radiol 2008;38:989–93. [12] Raghuram N, Pettignano R, Gal AA, et al. Plastic bronchitis: an unusual complication associated with sickle cell disease and acute chest syndrome. Pediatrics 1997;100: 139–42.
Please cite this article as: Soyer T, et al, Use of serial rigid bronchoscopy in the treatment of plastic bronchitis in children, J Pediatr Surg (2016), http://dx.doi.org/10.1016/j.jpedsurg.2016.03.017