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| | Patient identification | Presentation of PB | Treatment | Gross description and histopathology | Outcome |
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| 1 | 3 yo M with hypoplastic left heart syndrome. Cardiac surgeries included Norwood, BT shunt, Glenn, and Fontan. | Worsening respiratory distress and expectoration of multiple casts after Fontan. | Multiple bronchoscopic cast removals, budesonide, levalbuterol, direct and inhaled t-PA, spironolactone, inhaled hypertonic saline (3%). Oral azithromycin and spironolactone. | Gross: irregular branching, spongy, soft, tan, and red-brown tissue. Largest specimen 6 × 1.5 × 0.7 cm.
Histology: hypocellular fibrinous casts. | Continued small expectorated casts but without further obstructive casts 6 months after PB diagnosis. |
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| 2 | 3 yo M with tricuspid atresia. Cardiac surgeries included Glenn and Fontan. Course complicated by protein-losing enteropathy and chylothorax. | Presented with chronic cough; expectoration productive of branching mucoid casts. | Bronchoscopic cast removal. Inhaled steroids, albuterol, acetylcysteine, dornase alpha, and alteplase. Oral azithromycin. | Gross: 2.2 × 1.4 × 0.3 cm white fibrous tissue.
Histology: hypocellular, fibrinous cast. | Continued levalbuterol and acetylcysteine with small expectorated casts daily 12 years after PB diagnosis. |
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| 3 | 6 yo M with d-transposition of the great arteries and asthma. Cardiac surgery included arterial switch, closure of ASD, VSD, and PDA ligation. | Respiratory distress and right lung collapse in setting of influenza B infection. Bronchoscopy followed by forceps removal of cast. | Bronchoscopic cast removal, inhaled budesonide, acetylcysteine, dornase alpha, levalbuterol, inhaled t-PA. Oral azithromycin. | Gross: thick, white, extremely viscous material adherent to bronchus wall and obstructing right mainstem bronchus.
Histology: mixture of hypocellular fibrinous casts and inflammatory casts with abundant eosinophils. | Well-controlled asthma with no further casts at 3 years after PB diagnosis. |
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| 4 | 1 yo M with DiGeorge syndrome, tetralogy of Fallot, pulmonary atresia, and MAPCAs with chronic lung disease who was ventilator dependent. Cardiac surgeries included unifocalization to RV-to-PA conduit with VSD closure. | Repeated plugging of tracheostomy with thick mucous. | Inhaled dornase alpha, levalbuterol, albuterol, budesonide. Oral azithromycin. | | Continued on inhaled dornase alpha, budesonide, levalbuterol, and albuterol at discharge, which was 2 months after initial diagnosis of PB. |
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| 5 | 2 yo M with tricuspid atresia. Cardiac surgeries included Glenn and Fontan. Course complicated by chylothorax. | Presented with significant cough which improved after expectoration of cast with delicate strands. | Budesonide, levalbuterol, spironolactone. | | Fontan was fenestrated after PB diagnosis and no further casts at 9 months after PB diagnosis. |
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| 6 | 2 yo F with heterotaxy with atrioventricular septal defect and mitral regurgitation. Cardiac surgery included repair of septal defect and subsequent orthotopic heart transplant. | Acute inability to ventilate while intubated with left lung collapse 1 week after heart transplant. | Bronchoscopic cast removal by side-channel sucker. | Gross: thick, rope-like yellow mucoid secretions. | Resolved after cast evacuation with no further casts at 17 months after PB diagnosis. |
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| 7 | 3 yo M with hypoplastic left heart syndrome. Cardiac surgeries included Norwood with RV-to-PA conduit, aortic arch reconstruction, bidirectional Glenn, and extracardiac Fontan with subsequent Fontan takedown. | Persistent atelectasis and respiratory failure after Fontan takedown and return to Glenn physiology. | Bronchoscopic cast removal and direct instillation of dornase alpha. Inhaled albuterol, levalbuterol, and budesonide. | | Discharged from hospital on albuterol, levalbuterol, and budesonide at 3 months after PB diagnosis. |
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| 8 | 4 yo M with tetralogy of Fallot, pulmonary atresia, and MAPCAs. Cardiac surgeries included right unifocalization to RV-to-PA conduit and left unifocalization to central shunt. | 4 days after unifocalization revision left lung whiteout noted while patient was being mechanically ventilated. | Bronchoscopic cast removal using forceps. Direct and inhaled dornase alpha, inhaled t-PA. | Gross: tenacious mucoid material straddling the carina. | No further cast production after hospital discharge, which occurred 1 month after PB diagnosis. |
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| 9 | 2 yo M with moderate, persistent asthma complicated by pneumonia. | Presented with cough and wheeze with possible foreign body aspiration. | Bronchoscopic cast removal. Inhaled levalbuterol, montelukast, and budesonide. | Gross: several white to tan branching segments, largest 3.9 × 0.2 × 0.2 cm.
Histology: mucinous casts with abundant eosinophils and scattered Charcot-Leyden crystals. | Occasional asthma exacerbation, but overall well controlled with no further episodes of cast formation 4 years after PB diagnosis. |
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| 10 | 15 yo M with exercise-induced asthma. | Presented initially to outside hospital with dyspnea and found to have left mainstem bronchus lesion of unclear etiology. Despite laser resection, obstruction recurred as did respiratory distress. Repeat bronchoscopy with diagnosis 4 months after initial presentation. | Bronchoscopic cast removal. | Gross: 3 × 2 × 0.3 cm irregular, slightly tubular tan, erythematous, soft material.
Histology: mucinous casts with abundant eosinophils and Charcot-Leyden crystals. | Required further bronchoscopic cast removal, most recently documented 3 months after PB diagnosis. |
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| 11 | 9 yo M with history of mild asthma complicated by massive sand aspiration. | Respiratory arrest following massive sand aspiration. Bilateral pneumothoraces, bilateral lung collapse, and tracheal tear requiring ECMO support. | Bronchoscopic cast removal along with lung lavage. | Gross: dark, brown, irregular, hemorrhagic pieces of tissue, largest 3.2 × 1.5 × 0.5 cm.
Histology: hypocellular, fibrinous casts with entrapped red blood cells. | Continued intermittent albuterol use, but no further casts 2 years after initial PB diagnosis. |
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| 12 | 17 mo F with tracheal sling. Tracheoplasty was complicated by prolonged intubation and tracheal stenosis. | After extubation, rigid bronchoscopy performed due to continued stridor which revealed early plastic bronchitis and tracheomalacia. | Inhaled tobramycin, acetylcysteine, levalbuterol, and budesonide. | Gross: thick, yellow, tenacious secretions. | Bronchoscopy 1 week after initial PB diagnosis without casts present. |
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| 13 | 19 yo F with ALL treated with matched sibling bone marrow transplant complicated by graft versus host disease. History of ASD and VSD after repair. | Acute respiratory distress during transplant hospitalization. Large obstructing mucous plug found on bronchoscopy. | Bronchoscopic cast removal using forceps. | Gross: 4 × 2 × 1.7 cm granular, tan-red-green fragment. | No further cast production with most recent followup 2 years after initial PB diagnosis. |
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| 14 | 19 yo F with systemic lupus erythematosus complicated by pulmonary hemorrhage, ARDS, CMV pneumonitis, and aspergillosis. | Initially intubated for pulmonary hemorrhage, but because required increasing pressures, flexible bronchoscopy was performed. This revealed cast in the right mainstem bronchus which was removed using rigid bronchoscopy. | Bronchoscopic cast removal. | Gross: 18 × 6.5 × 1 cm fibrinous cast.
Histology: fibrinous casts with associated acute inflammation and hyphal forms. | Deceased 1 week after cast extraction due to persistent pulmonary hemorrhage resulting in cardiogenic shock. |
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