Plastic bronchitis

Plastic bronchitis (PB) is a rare, severe concomitant disease that particularly affects individuals with Fontan circulation. Those affected painfully cough up a gluelike albuminous and fibrinous secretion that develops in the air passages of the lung (bronchia).

Affected individuals experience plastic bronchitis as a great burden which is associated with a high mortality rate. Since little is known even today regarding the causes of this disease, no effective treatment or cure is available. In most cases, patients receive a combination of various drugs (Sildenafil, inhaled sodium chloride, acetylcysteine, dornase alfa, albuterol, steroids, plasminogen activators, MCT diet), which are, however, only able to alleviate the symptoms, if at all.

A team of researchers from Pennsylvania (USA) led by Yoav Dori has recently developed a new procedure that raises hope. While performing an MRI examination on one of their patients, they had found alterations in the lymphatic vessels surrounding the bronchi. They attributed these alterations to an increased production of lymph, which they assumed to be caused by the increased central venous pressure that is typical for Fontan circulation.
Dori and his team believe that the altered lymphatic vessels are somehow connected to the occurrence of plastic bronchitis. They thus closed (embolized) these vessels within the scope of their new procedure.

So far, the new method has turned out to be successful: none of the children who received this treatment has had any symptoms since then.

A different method has been developed by Dr. Hraska at the hospital of St. Augustin (Germany): his procedure aims at reducing the increased pressure in the central lymphatic vessel (thoracic duct) present in Fontan circulation. Within the scope of the treatment, physicians connect the left part of the large vessel which leads the deoxygenated blood from the upper body to the right heart chamber (brachiocephalic vein), directly to the upper apex of the right atrium. In some of the children receiving this treatment, the procedure turned out to be successful (2).

A detailed representation of the lymphatic vessels by means of MRI has also been the focus of scientists from other countries, including Germany. Until now, however, it is still unknown whether the altered lymphatic veins are really the cause of plastic bronchitis in all cases. Furthermore, it remains to be seen if patients who have received the treatment developed by Dori and his team will show signs of newly developing collaterals over time.

If the new procedure of embolization proves to be successful also in the long term, it seems obvious that colleagues from other countries will learn and apply it in their institutions as well.