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| CASE REPORT |
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| Year : 2021 | Volume
: 8
| Issue : 3 | Page : 142-144 |
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Plastic bronchitis after Fontan surgery presenting as recurrent life-threating airway obstruction
Madhu S Pujar, Vineela Mikkilineni
Department of Pediatrics, JJM Medical College, Davangere, Karnataka, India
| Date of Submission | 04-Nov-2020 |
| Date of Decision | 03-Feb-2021 |
| Date of Acceptance | 10-Feb-2021 |
| Date of Web Publication | 21-May-2021 |
Correspondence Address:
Dr. Madhu S Pujar
Department of Pediatrics, JJM Medical College, Davangere, Karnataka
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jpcc.jpcc_178_20
Plastic bronchitis, a rare but serious clinical condition, is commonly seen after Fontan surgeries in children. It may be a manifestation of suboptimal adaptation to the diversion of systemic blood flow directly into the pulmonary circulation, thereby leading to unfavorable hemodynamics, which causes the formation of bronchial casts. We hereby report a child who had not responded to conventional medical management of the bronchial casts. He was then subjected to cardiac catheterization where balloon dilatation of the Fontan fenestration was done, following which he remained asymptomatic for 3 months of follow-up.
Keywords: Cardiac catheterization, Fontan surgery, plastic bronchitis
How to cite this article:
Pujar MS, Mikkilineni V. Plastic bronchitis after Fontan surgery presenting as recurrent life-threating airway obstruction. J Pediatr Crit Care 2021;8:142-4 |
How to cite this URL:
Pujar MS, Mikkilineni V. Plastic bronchitis after Fontan surgery presenting as recurrent life-threating airway obstruction. J Pediatr Crit Care [serial online] 2021 [cited 2023 Jun 2];8:142-4. Available from: http://www.jpcc.org.in/text.asp?2021/8/3/142/316593 |
| Introduction | |  |
Plastic bronchitis (PB) is a rare and severe respiratory disorder, characterized by the formation of gelatinous plugs in the large airways that take the shape of bronchial “casts.”[1] The hallmark of the disease is expectoration of large branching casts. Casts are more adhesive than mucous plugs and conform the shape of the tracheobronchial tree. Therefore, it may completely occlude the airway and present with cough, cyanosis, dyspnea, and wheezing. Immediate treatment of PB ranges from medical treatment with corticosteroids, different inhaled lytic agents to bronchoscopy, and potentially other surgical procedures.[2] One of the modalities of treatment options is a re-look at the Fontan circuit which helps relieve symptoms remarkably as seen in our case.
| Case Report | | |
A 14 year old boy presented to us with complaints of pertusoid like cough with expectoration, breathlessness, increasing cyanosis and muscle spasms, following episodes of cough. He has been having similar episodes for the past 3 months with progressive increase in the frequency of airway obstruction and expectoration of casts, which were occurring on a daily basis for a week before he came to us. The child had a significant history and was diagnosed first at the age of 1 month with hypoplastic right ventricle. He underwent palliative cardiac procedures including modified Norwood at 1 year of age with the placement of a right ventricle-to-pulmonary artery conduit, modified Blalock–Taussig shunt at 9 years of age, and bidirectional Glenn shunt at 10 years of age. He also underwent extracardiac nonfenestrated Fontan 7 months ago, following which he started having the above symptoms.
On examination, the child had tachypnea, lower chest indrawing, and reduced breath sounds in the left hemithorax. Oxygen saturation in room air was 74%, and chest X-ray showed loss of lung volume on the left side. The child was administered oxygen by a mask and started on nebulization during which the child expectorated a white rubbery chunk of the mucus plug and on distribution was seen to have a bronchial anatomy [Figure 1]. After the expulsion of the cast, the symptoms reduced considerably. The cast was then subjected to histopathology which showed coagulated fibrin and mucin with trapped neutrophils, eosinophilia, and lymphocytes [Figure 2]. Because of recurrent life-threatening airway obstruction and failure to respond to nebulization with both bronchodilators and mucolytics, corticosteroids, and good chest physiotherapy for 2 days, the child was further subjected to a repeat cardiac catheterization and a successful balloon dilatation of the Fontan fenestration was done, following which the child improved and has been symptom free for 4 weeks of follow-up. | Figure 2: Bronchial cast showing coagulated fibrin and mucin with trapped mixed inflammatory infiltrate and few bacterial colonies (H and E, ×100)
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| Discussion | | |
PB is an uncommon condition, but recent evidence suggests that it is underreported as well. It was first reported by Galen (AD 131–200), who described the expectoration of “arteries and veins.” Of note, PB prevalence in Fontan patients has been estimated to be as high as 4%–14%.[3] Early occurrence after Fontan surgery is ominous. If cast bronchitis is provoked by infection or airway reactivity, the prognosis is not too bad.[4] The current understanding suggests a two-step model of insult, with inflammation superimposed on a dysregulated mucus secretion in a vulnerable vascular bed or an endobronchial lymph leakage.[4] This model explains the favorable clinical course in few patients after symptomatic treatment. Recurrent bouts warrant a hemodynamic study to exclude any obstructions in Fontan circuit, as an echocardiogram alone may not be sufficient to exclude such obstructions.
Onset of symptoms is reported within 1–3 years after completion of a Fontan operation. At present, the diagnosis of PB hinges on visualization of bronchial casts that are either expectorated or removed by bronchoscopy.[5] The cast associated with PB may be spontaneously expectorated and patients may cough up large impressions of their tracheobronchial tree. However, life-threatening disease, due to airway obstruction with hypoxia, has been reported in association with PB in children. Both acute cast removal by bronchoscopy and long-term prevention of cast recurrence are the primary modalities of treatment. Inhaled acetyl cysteine, DNase, systemic and inhaled corticosteroids, and chest physiotherapy have all been used. A recent report documented the use of aerosolized urokinase to treat bronchial casts. The efficacy of these therapies remains to be established.[6] Further, such targeted therapy may not always be feasible. Optimization of hemodynamics is done by fenestrating the Fontan circuit, removal of all obstructions (pulmonary artery, vein, aortic narrowing), reconversion to Glenn shunt, and innominate to atrial shunt.[6]
PB is a rare and serious clinical problem after Fontan surgery. As medical line of management may not always give a desirable outcome, the patient can be subjected to cardiac catheterization for a re-look at the Fontan circuit, which can be a diagnostic as well as a therapeutic procedure in these patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Grutter G, Di Carlo D, Gandolfo F, Adorisio R, Alfieri S, Michielon G, et al. Plastic bronchitis after extracardiac Fontan operation. Ann Thorac Surg 2012;94:860-4.  |
| 2. | Swain SK, Bhattacharyya B, Mohanty JN. Plastic bronchitis mimicking with foreign body bronchus in pediatric patient – A review. Indian J Child Health 2019;6:465-9. |
| 3. | Caruthers RL, Kempa M, Loo A, Gulbransen E, Kelly E, Erickson SR , et al. Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis. Pediatr Cardiol 2013;34:256-61. |
| 4. | Singhi AK, Vinoth B, Kuruvilla S, Sivakumar K. Plastic bronchitis. Ann Pediatr Card 2015;8:246-8. [ PUBMED] [Full text] |
| 5. | Quasney MW, Orman K, Thompson J, Ring JC, Salim M, Schoumacher RA, et al. Plastic bronchitis occurring late after the Fontan procedure: Treatment with aerosolized urokinase. Crit Care Med 2000;28:2107-11. |
| 6. | Brogan TV, Finn LS, Pyskaty DJ Jr., Redding GJ, Ricker D, Inglis A , et al. Plastic bronchitis in children: A case series and review of the medical literature. Pediatr Pulmonol 2002;34:482-7. |
[Figure 1], [Figure 2]
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