Elsevier

Clinics in Chest Medicine

Volume 37, Issue 3, September 2016, Pages 405-408
Clinics in Chest Medicine

Plastic Bronchitis

https://doi.org/10.1016/j.ccm.2016.04.003Get rights and content

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Key points

  • Plastic bronchitis associated with congenital heart disease or with lymphatic anomalies is caused by aberrant pulmonary lymphatic vessels and drainage. This true form of plastic bronchitis can usually be treated by selective lymphatic vessel ablation.

  • Plastic bronchitis is probably more common than reported. This speculation is based on the observation that many clinicians are unfamiliar with the disease and may fail to recognize milder forms of the syndrome.

  • Plastic bronchitis with cohesive

Patient evaluation overview

The diagnosis of PB is confirmed by a history of expectoration of branching airways casts, or by removing branching casts at the time bronchoscopy. Life-threatening respiratory distress can occur because of obstruction of airways with casts in children with congenital heart disease or as a consequence of lymphatic engorgement following surgical correction of congenital heart disease.1 The casts of PB often contain an abundance of mucin but unlike mucin polymers in normal mucus, which are

Disease associations

PB in children is usually associated with congenital heart disease and especially those with single-ventricle, Fontan physiology (Box 1).5 The occurrence, severity, and the frequency of exacerbations of PB vary markedly among patients with congenital heart disease, sometimes first appearing years after surgery. Some patients have subclinical disease with the expectoration of small casts or resolution of casts between exacerbations. PB has been associated with lymphatic abnormalities both in

Pharmacologic treatment options

Because PB is an uncommon condition, most reports of effective therapy are based on subjective criteria detailed in case reports or small case series (Box 2). Further, most patients in these small studies have received many different medications, making it difficult to ascertain which of these therapies, if any, are effective. Evaluation of data from the Plastic Bronchitis Registry (http://rubinlab.pediatrics.vcu.edu/research/plastic-bronchitis/) suggests that there is no benefit to the use of

Nonpharmacologic and surgical treatment options

In patients with lymphatic abnormalities, the most effective therapy for PB is MRI-guided selective lymphatic embolization.7 Improving cardiac output has been reported to reduce the severity of PB, but surgical attempts to optimize Fontan physiology by fenestration of the atrial septum or taking down the shunt are generally unsuccessful. Cardiac transplant in patients with heart failure has also been reported to improve PB.17

Airway clearance using standard pulmonary toilet techniques seems to

Evaluation of outcomes and long-term recommendations

The primary considerations when treating PB are facilitating the clearance of casts that have formed in the airways, and preventing future casting and airway obstruction. Cast clearance by bronchoscopy allows inhaled medications to gain access to the distal conducting airways and promote airway clearance and reduce inflammation and bronchospasm. In addition, extensive cast removal may make surgical and radiologic interventions easier for patients to tolerate.

The future

Through the US National Institutes of Health, Office of Rare Diseases, an International Plastic Bronchitis Registry has been established to collect data on patients with PB throughout the world (ClinicalTrials.gov identifier NCT01663948). This database will help clinicians to generate hypotheses that can be tested clinically, including the use of genomic or inflammasome screening for potential causes for this debilitating disease. Through the Office of Rare Diseases, a tissue repository of

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Disclosure: Dr Rubin is a Board member of the Plastic Bronchitis Foundation, he directs the International Plastic Bronchitis Registry and Specimen Repository, and his lab is partially funded by the American Respiratory Care Foundation to study this condition.

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