Document Detail

Successful management of plastic bronchitis in a child post Fontan: case report and literature review.
MedLine Citation:
PMID:  22430124     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Plastic bronchitis is the occlusion of the major bronchial airways by a firm, gelatinous mucoid cast. It is a rare condition, which while classically described in asthma and sickle cell disease has greater mortality in patients with congenital heart disease. The management of this disease is obscure given the lack of clinical data regarding treatment therapies.
METHODS: We describe a case of an 11-year-old female status after Fontan surgery who presented with respiratory distress secondary to atelectasis of the right lung.
RESULTS: A bronchoscopy was performed demonstrating an obstructing bronchial cast with successful extraction. The plastic bronchitis continued to recur and she was placed on multiple inhaled mucolytics as well as inhaled tissue plasminogen activator with temporary resolution. Further evaluation of the etiology of her casts revealed that she had elevated pulmonary arterial pressures. Repeated bronchoscopic removal of the casts was utilized as well as continuation of the aggressive airway clearance. Ultimately fenestration of her Fontan was performed along with treatment of pulmonary vasodilators sildenafil and bosentan. Although there was improvement of the cast formation, her airway clearance could only be weaned to four times a day therapy with which she was discharged home after a 3-month hospitalization. She continues to remain on this therapy and has not required hospitalization since the initial incident over 1 year ago.
CONCLUSIONS: Plastic bronchitis in a patient with Fontan physiology presents a treatment dilemma that may require comprehensive therapy in severe cases such as described.
Paul Do; Inderpal Randhawa; Terry Chin; Kourosh Parsapour; Eliezer Nussbaum
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2012-03-20
Journal Detail:
Title:  Lung     Volume:  190     ISSN:  1432-1750     ISO Abbreviation:  Lung     Publication Date:  2012 Aug 
Date Detail:
Created Date:  2012-07-16     Completed Date:  2013-01-17     Revised Date:  2013-06-18    
Medline Journal Info:
Nlm Unique ID:  7701875     Medline TA:  Lung     Country:  United States    
Other Details:
Languages:  eng     Pagination:  463-8     Citation Subset:  IM    
Miller Children's Hospital, 2801 Atlantic Ave. Ground Floor, Long Beach, CA, USA.
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MeSH Terms
Antihypertensive Agents / therapeutic use
Bronchitis / drug therapy*,  etiology,  surgery*
Combined Modality Therapy
Drug Therapy, Combination
Expectorants / therapeutic use*
Fontan Procedure / adverse effects*
Heart Defects, Congenital / surgery*
Piperazines / therapeutic use
Purines / therapeutic use
Sulfonamides / therapeutic use
Sulfones / therapeutic use
Tissue Plasminogen Activator / therapeutic use*
Treatment Outcome
Vasodilator Agents / therapeutic use
Reg. No./Substance:
0/Antihypertensive Agents; 0/Expectorants; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 0/Vasodilator Agents; 3M7OB98Y7H/sildenafil; EC Plasminogen Activator; Q326023R30/bosentan

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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