title: Successful management of plastic bronchitis in a child post Fontan: case report and literature review.
abstract: OBJECTIVE
  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.affiliation: Miller Children's Hospital, 2801 Atlantic Ave. Ground Floor, Long Beach, CA, USA. pcdo78.pedspulm@gmail.com
authors.forename: Paul
authors.initials: P
authors.lastname: Do
authors.forename: Inderpal
authors.initials: I
authors.lastname: Randhawa
authors.forename: Terry
authors.initials: T
authors.lastname: Chin
authors.forename: Kourosh
authors.initials: K
authors.lastname: Parsapour
authors.forename: Eliezer
authors.initials: E
authors.lastname: Nussbaum
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D001991:Bronchitis / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery*; D001999:Bronchoscopy; D002648:Child; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D005100:Expectorants / Q000627:therapeutic use*; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D005100:Expectorants; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D010959:Tissue Plasminogen Activator; D000077300:Bosentan