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    
Abstract/OtherAbstract:
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.
Authors:
Paul Do; Inderpal Randhawa; Terry Chin; Kourosh Parsapour; Eliezer Nussbaum
Related Documents :
24087784 - Addisonian pigmentation and vitamin b12 deficiency: a case series and review of the lit...
2288384 - Improvement of action myoclonus by an administration of 5-hydroxytryptophan and carbido...
2300064 - Structural basis of carcinogenicity in rodents of genotoxicants and non-genotoxicants.
22168524 - Giant dedifferentiated liposarcoma of the right hemifacial area involving the oral cavity.
8249594 - Multiple melanocytic tumours in a case of ocular melanocytosis.
14738174 - Suboptimal care and perinatal mortality in ten european regions: methodology and evalua...
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    
Affiliation:
Miller Children's Hospital, 2801 Atlantic Ave. Ground Floor, Long Beach, CA, USA. pcdo78.pedspulm@gmail.com
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Antihypertensive Agents / therapeutic use
Bronchitis / drug therapy*,  etiology,  surgery*
Bronchoscopy*
Child
Combined Modality Therapy
Drug Therapy, Combination
Expectorants / therapeutic use*
Female
Fontan Procedure / adverse effects*
Heart Defects, Congenital / surgery*
Humans
Piperazines / therapeutic use
Purines / therapeutic use
Sulfonamides / therapeutic use
Sulfones / therapeutic use
Tissue Plasminogen Activator / therapeutic use*
Treatment Outcome
Vasodilator Agents / therapeutic use
Chemical
Reg. No./Substance:
0/Antihypertensive Agents; 0/Expectorants; 0/Piperazines; 0/Purines; 0/Sulfonamides; 0/Sulfones; 0/Vasodilator Agents; 3M7OB98Y7H/sildenafil; EC 3.4.21.68/Tissue Plasminogen Activator; Q326023R30/bosentan

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


Previous Document:  L: -citrulline Prevents Alveolar and Vascular Derangement in a Rat Model of Moderate Hyperoxia-induc...
Next Document:  Dickkopf-1 (DKK-1) interrupts FAK/PI3K/mTOR pathway by interaction of carbonic anhydrase IX (CA9) in...