Plastic bronchitis

LPB is a lymphatic flow disorder characterized by the recurrent formation of branching, rubbery bronchial casts composed primarily of proteinaceous and sometimes chylous material and lymphocytes.

Lymphatic fluids deposited into the airspaces become gelatinous as they cool, forming large string cheese-like casts of the airways, which can obstruct airflow.

The clinical presentation of plastic bronchitis beyond expectoration of casts includes a productive cough, dyspnea, fever and wheezing.

This is followed by short and long term efforts to identify and remediate the underlying condition resulting in the excessive airway leakage or inflammation that is causing the casts to form.

[citation needed] PB can present as a life threatening emergency when the casts obstruct the major airways resulting in acute respiratory distress.

[9] Intranodal lymphangiogram[10] and dynamic contrast-enhanced MR lymphangiography[11][12] have been used to more precisely image the leaks, and in the small number of patients who have been treated to date, embolization of the thoracic duct has been highly successful in controlling cast formation.

[11] Therapeutic interventions with medium-chain triglyceride-enriched low-fat diets, intratracheal heparin, inhaled tissue plasminogen activator, and steroids have also been reported and have met with variable success.

[18][19] Patients with plastic bronchitis that is being caused due to a co-morbid condition generally have a good prognosis once the underlying disease is treated.