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Ann Thorac Surg 2006;81:2281-2283
© 2006 The Society of Thoracic Surgeons

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Case report

Plastic Bronchitis: Is Thoracic Duct Ligation a Real Surgical Option?

Department of Cardiac Surgery, Vanderbilt University, Nashville, Tennessee

Accepted for publication July 5, 2005.

^_* Address correspondence to Dr Shah, Pediatric Cardiac Surgery, 5247 Doctor's Office Tower, 2200 Children's Way, Nashville, TN 37232-9292 (Email: salman1997{at}yahoo.com).

	Abstract

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Plastic bronchitis is an unusual clinical scenario of unknown cause and occurs in multiple clinical settings. The disease is characterized by the development of arborizing, thick, tenacious casts of the tracheobronchial tree that results in airway obstruction. Patients with congenital heart disease who have undergone a Fontan operation are at high risk for having this problem develop. Management of this distressing situation is difficult with only palliative options being available, such as repeated bronchoscopies, inhaled heparin, tissue plasminogen activator, inhaled bronchodilators, or azithromycin. The patients with Fontan circuits have a myriad of unique complications develop, such as atrial arrhythmias, recurrent pleural effusions, chylothoraces, protein-losing enteropathy, and plastic bronchitis. High intrathoracic lymphatic pressures with nondemonstrable lympho-bronchial fistulas were believed to be the cause for the development of these recurrent bronchial casts in plastic bronchitis. Faced with recurrent plastic bronchitis resistant to medical management in 2 Fontan patients with normal Fontan pressures on cardiac catheterization, we decided to explore a surgical solution by performing a thoracic duct ligation. This resulted in complete resolution of the formation of casts in both patients, who were discharged home and remain asymptomatic on continued follow-up. Thoracic duct ligation provides a surgical cure for plastic bronchitis by decreasing intrathoracic lymphatic pressure and flow.

	Introduction

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Plastic bronchitis is an unusual condition that is characterized by the development of thick, tenacious rubbery casts of the tracheobronchial tree. It occurs in different clinical conditions including patients with congenital cardiac conditions palliated with the Fontan operation [1]. Management of this problem is difficult and is mostly symptomatic and palliative. We describe 2 patients who had thoracic duct ligations that were successfully used as a curative surgical option, and we review various treatment options and their efficacy as described in the literature.

	Case Reports

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Patient 1
A 4-year-old boy, who had undergone a fenestrated extracardiac Fontan operation for a double inlet left ventricle with a hypoplastic aortic arch, presented to the hospital with symptoms of respiratory distress. He had reported a recent history of having expectorated multiple bronchial casts for the last few days.

He was admitted and initially started on continuous positive pressure ventilation along with inhaled albuterol breathing treatments and oxygen. Cardiac catheterization revealed normal Fontan pressures with a patent fenestration and good flow to both lungs.

During the course of his hospitalization he started coughing and expectorated bronchial casts again. A chest tube was placed for a pleural effusion, but his clinical situation did not improve. He had been started on inhaled tissue plasminogen activator as well as corticosteroids. Despite adequate medical management he deteriorated and had to be intubated. Bronchoscopy at this time revealed thick multiple casts in the tracheobronchial tree. His overall condition worsened and he required veno-venous extracorporeal membrane oxygenation support. With vigorous medical management his condition improved and he was weaned off of extracorporeal membrane oxygenation support. At that time a decision was made to proceed to the operating room to ligate his thoracic duct, hypothesizing that this would decrease the lymphatic pressure in his chest and hopefully eliminate the production of bronchial casts. Mass ligation of the thoracic duct was done just above the esopahageal hiatus through a right posterolateral thoracotomy. His condition slowly improved and he was extubated. His pleural effusions persisted and he ultimately required a chemical doxycycline pleurodesis. He was discharged home on nasal cannula oxygen (0.5 L). He has been followed-up for 5 months after thoracic duct ligation and has remained free from any episodes of plastic bronchitis.

Patient 2
A patient who had been born with double inlet left ventricle underwent a modified right Blalock-Taussig shunt shortly after birth. Subsequently he underwent a bidirectional Glenn with shunt takedown and then had a completion Fontan done. He remained healthy for the next 3 years and then started to have repeated episodes of coughing up bronchial casts, consistent with plastic bronchitis. He was managed medically with no success, and after multidisciplinary discussions a decision was made to proceed with a thoracic duct ligation. Perioperative cream was given to make the duct prominent. A mass ligation of the thoracic duct was done through a right thoracotomy.

The patient did well after his surgery and had an uneventful postoperative course. He has been followed-up for 2 years since the thoracic duct ligation and has remained free of any further episodes of cast expectoration related to plastic bronchitis.

	Comment

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The Fontan operation is in the final stage of repair for patients with single ventricle congenital heart defects. The operation has undergone several modifications and is now largely done either as an extracardiac conduit or as an intracardiac lateral tunnel, with or without a fenestration. The final result leaves the patient with two circulations in series, so that systemic venous return is returned directly to the pulmonary arteries without the presence of a pumping chamber.

The Fontan physiology renders patients susceptible to the development of chronic pleural effusions, chylothorax, atrial arrythmias, protein losing enteropathy, and plastic bronchitis [1–3]. A decline in cardiac function, elevated pulmonary vascular resistance, and high venous pressures are believed to play a role in the development of some of these conditions.

Plastic bronchitis also known as cast bronchitis is a rare clinical entity that is characterized by the formation of intrabronchial casts [9]. These casts vary from being stringy or rubbery to firm, thick, and hard. In addition to occurring in congenital cardiac disease, this condition can also occur in the setting of asthma, sickle cell disease [10], cystic fibrosis, smoke inhalation, and pulmonary infections [1].

These casts have been classified as type I (inflammatory) or type II (acellular) [11]. The inflammatory casts occur in the setting of underlying pulmonary inflammatory disease, whereas acellular casts are seen more commonly with underlying congneital cardiac disease. Type I casts are believed to occur due to increased mucin production in the setting of poor clearance mechanisms. The cause of type II casts remains unclear.

Declining ventricular function, the presence of a high central venous pressure with high intrathoracic lymphatic pressures and bronchial hypersecretions may be the underlying causes, although these cannot be demonstrated objectively and repeatedly in all patients [12]. Hug and colleagues [8] demonstrated leakage of chyle into the alveoli in a fatal case of plastic bronchitis, substantiating the hypothesis that high intrathoracic lymphatic pressure or obstruction to lymph flow leads to the development of lympho-alveolar fistulae and development of bronchial casts.

Patients usually present with dyspnea increased work of breathing and repeated episodes of coughing and expectoration of casts. Chest roentgenogram usually reveals an infiltrate with atelectasis. The casts can cause severe respiratory embarrassment with asphyxia, cardiac arrest, and death. Mortality has been reported to be as high as 29% in patients with underlying congenital cardiac condition [1].

Different therapies have been explored [4–7]. Inhaled corticosteroids have reported benefit with inflammatory casts, but are not as successful with acellular casts that are more frequent in the setting of congenital heart disease. Inhaled tissue plasminogen activators [13] and inhaled heparin have been used with success in loosening of the casts and facilitating expectoration. Inhaled low dose azithromycin has been reported to result in clinical, spirometric, and radiologic improvement [6]. Similarly inhaled rh-DNAse has been of reported benefit in plastic bronchitis in the setting of acute chest syndrome with sickle cell disease [14]. Repeated toilet bronchoscopies are sometimes required to remove thick tenacious casts [4]. However none of these treatments result in a cure and usually provide short-term symptomatic relief. Improvement in underlying cardiac function and decrease in central venous pressure would theoretically improve the condition. This can be inferred from the success of fenestration of the Fontan circuit [15] and cardiac transplantation in the management of protein-losing enteropathy secondary to failing Fontan circulation [16]. Patients with a failing Fontan circulation who develop plastic bronchitis have also been reported to gain benefit from atrial pacing and restoration of atrioventricular synchrony [17].

The lack of an available, definitive long-term solution and the failure of medical management led us to try thoracic duct ligation as a surgical option. With both patients this was done through a right thoracotomy with mass ligation of all tissues to the right of the esophagus and the aorta and overlying the vertebral column.

Plastic bronchitis is a rare complication of patients having undergone the Fontan operation. Expectoration of casts with the associated airway obstruction is a distressing problem for both the patient and their parents. Rare occurrence combined with an unclear cause has made the management of this problem difficult and frustrating. Palliative management with various inhaled therapies mainly serves to soften the casts and helps with expectoration. This led us to consider a surgical solution in the form of a thoracic duct ligation. The theoretical advantage of this was to decrease intrathoracic lymph flow and pressure, which was believed to be contributing to the development of the bronchial casts. Thoracic duct ligation was attempted in 2 patients who had plastic bronchitis develop after the Fontan operation. In both these patients the surgery was successful and no recurrence of cast formation or expectoration has been reported to date.

	References

Top Abstract Introduction Case Reports Comment References

 

1. Brogan TV, Finn LS, Pyskaty Jr DJ, et al. Plastic bronchitis in childrena case series and review of the medical literature. Pediatr Pulmonol 2002;34:482-487.[Medline]
2. Bowen A, Oudjhane K, Odagiri K, et al. Plastic bronchitislarge, branching, mucoid bronchial casts in children. AJR Am J Roentgenol 1985;144:371-375.[Abstract/Free Full Text]
3. Colloridi V, Roggini M, Formigari R, et al. Plastic bronchitis as a rare complication of Fontan's operation Pediatr Cardiol 1990;11:228.[Medline]
4. Ishman S, Book DT, Conley SF, Kerschner JE. Plastic bronchitisan unusual bronchoscopic challenge associated with congenital heart disease repair. Int J Pediatr Otorhinolaryngol 2003;67:543-548.[Medline]
5. Wakeham MK, Van Bergen AH, Torero LE, Akhter J. Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient Pediatr Crit Care Med 2005;6:76-78.[Medline]
6. Schultz KD, Oermann CM. Treatment of cast bronchitis with low-dose oral azithromycin Pediatr Pulmonol 2003;35:139-143.[Medline]
7. Onoue Y, Adachi Y, Ichida F, Miyawaki T. Effective use of corticosteroid in a child with life-threatening plastic bronchitis after Fontan operation Pediatr Int 2003;45:107-109.[Medline]
8. Hug MI, Ersch J, Moenkhoff M, et al. Chylous bronchial casts after Fontan operation Circulation 2001;103:1031-1033.[Free Full Text]
9. Bowen A. Plastic or cast bronchitis? Am J Dis Child 1990;144:1075-1076.[Abstract/Free Full Text]
10. Raghuram N, Pettignano R, Gal AA, et al. Plastic bronchitisan unusual complication associated with sickle cell disease and the acute chest syndrome. Pediatrics 1997;100:139-142.[Free Full Text]
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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Davis C. Drinkwater Karla G. Christian Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Salman A. Shah, S. Articles by Christian, K. G. Search for Related Content PubMed PubMed Citation Articles by Salman A. Shah, S. Articles by Christian, K. G. Related Collections Mediastinum