Thoracic injection of contrast material in a patient with plastic bronchitis.
Interventional radiologists at Penn Medicine have developed a program to combine advanced lymphatic imaging and percutaneous embolization for the diagnosis and treatment of plastic bronchitis in adults.
Plastic bronchitis (PB) is an uncommon lung disease defined by the production of occlusive branching casts from the tracheobronchial tree. Casts are composed of lymphocytes or proteinaceous materials and vary in magnitude and branching extent from small bronchial segments (occurring in asthma or pulmonary infection) to the entire lung (>30.5 cm) in individuals with lymphatic disease. PB is thought to arise when elevated central venous pressures and abnormal flow in the thoracic duct cause retrograde flow into the pulmonary lymphatic channels that seeps into the airways and engorges the lung parenchyma. Principally affecting individuals with surgically-repaired congenital heart disease, PB has been identified in patients with sickle cell disease, chronic pulmonary disease and the primary lymphatic disorders, as well.
Plastic bronchitis is a rare syndrome that presents with expectoration of branching bronchial casts
Under the direction of Drs. Maxim Itkin and Gregory Nadolski, interventional radiologists at Penn Medicine have developed an evidence-based initiative to diagnose and treat PB that combines dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) with percutaneous lymphatic embolization. DCMRL was developed to image the central lymphatic system, and involves bilateral injection of gadolinium into the inguinal lymph nodes and image acquisition using time-resolved central k2 space dynamic T1-weighted magnetic resonance imaging. Lymphatic embolization employs a transabdominal catheter to place coils coated with organic endovascular glue into affected lymphatic vessels to block leakage.
In a landmark 2016 study, [1] Dr Itkin and colleagues used DCMRL to demonstrate the long-suspected finding that abnormal pulmonary and mediastinal lymphatic flow from the thoracic duct is a cause of plastic bronchitis in adults. The same study established the efficacy of percutaneous transabdominal lymphatic embolization as a treatment modality for adults with plastic bronchitis.
Case Study
Mr. R, a 67-year-old man, was referred to Penn Interventional Radiology for lymphangiogram and thoracic duct embolization following following a 20-year history of pulmonary disease, including pulmonary alveolar proteinosis, and respiratory complications (e.g., chylothorax, chronic respiratory failure and plastic bronchitis).
Despite obesity (BMI 30.78 kg/m2) and a decades-long daily requirement for oxygen, he was managing relatively well until several months prior to his arrival at Penn, when he began to experience progressive exertional dyspnea, orthopnea and cough productive of casts. At presentation, Mr. R recounted a long series of procedures to clear his airways, including bronchoscopies, vest treatments, hypertonic saline nebulizer treatments, albuterol inhalation and guaifenesin therapy. His most recent procedure, an attempted thoracic duct embolization at a hospital in his home state, was abandoned after 12 hours.
Following this experience, Mr. R was treated for pulmonary nodular amyloidosis (PNA) and his airway clearance regimen increased. To address his persistent oxygen requirement, he had CT pulmonary angiography, which demonstrated acute bilateral pulmonary emboli and a large left-sided pneumothorax, and was discharged on room air at rest and 3L oxygen with ambulation. He continued to cough up small amounts of cast-like material and to experience orthopnea. At this point, arrangements were begun to list him for lung transplant.
Seeking an alternative, Mr. R was referred to Penn Interventional Radiology and the Pulmonary Service at Penn Medicine by his primary care provider for consideration of dynamic contrast MR lymphangiogram with possible thoracic duct embolization. His procedure, which followed a day-long transport by vehicle from his home in the southern United States, was preceded at Penn Medicine by admission to OBS followed by admission to Pulmonary Services.
The Procedure
Under general anesthesia, Mr. R first had intranodal lymphangiography to identify the cisterna chyli and thoracic duct. The cisterna chyli, which sits near the base of the thoracic duct, was then accessed transabdominally via percutaneous means under fluoroscopic guidance with a 22g chiba needle, and a wire placed in the thoracic duct to guide a microcatheter.
Contrast dye was then injected to evaluate the pulmonary lymphatic system (Figure 2), followed by blue dye and bronchoscopy to confirm the leakage and cleanse the lungs of casts. Endovascular glue and coils were then introduced through the catheter to embolize the thoracic duct, and the catheter removed.
Mr. R remained in the hospital for three days and was discharged to home, where he began a full and dramatic recovery. Within weeks of his procedure, his respiration normalized to the point that home oxygen therapy was no longer necessary.
Access
Penn Interventional Radiology
Hospital of the University of Pennsylvania
Ground Dulles
3400 Spruce Street
Philadelphia, PA 19104
Perelman Center for Advanced Medicine
Atrium, Ground Floor
3400 Civic Center Boulevard
Philadelphia, PA 19104
Published on: March 11, 2019
References
1. Itkin MG, McCormack FX, Dori Y. Ann Am Thorac Soc 2016;13:1689–1696.
About Penn Interventional Radiology
A national leader in research, education and patient care, Penn Interventional Radiology is also one of the oldest and largest IR programs in the United States. In addition to performing more than 12,000 procedures annually, Penn Interventional Radiology has an inpatient admitting service, inpatient consult service and daily outpatient consultation and follow-up clinic.
Referring physicians, please use the information below to contact Drs. Itkin and Nadolski at the Penn Center for Lymphatic Disorders:
Administrative Assistant
patricia.hetherington@pennmedicine.upenn.edu
215.662.7892
Lymphatics Nurse Practitioner
Megan.asher@pennmedicine.upenn.edu
Email: PennLymphatics@uphs.upenn.edu
Fax number: 215.615.3545
Penn Faculty Team
Director, Penn Center for Lymphatic Disorders
Professor of Radiology at the Hospital of the University of Pennsylvania
Co-Director, Penn Image-Guided Interventions (PIGI) Laboratory
Chair, Interventional Radiology Resident Selection Committee
Associate Professor of Radiology at the Hospital of the University of Pennsylvania