Innovative treatment of child with plastic bronchitis heralds unique program in lymphatic interventions by CHOP, Penn experts
A case study published recently in the journal Pediatrics describes an innovative, minimally invasive procedure that treated plastic bronchitis, a potentially life-threatening disease, in a six-year-old boy with a heart condition. Using new lymphatic imaging tools and catheterization techniques, physician-researchers eliminated bronchial casts, which are an accumulation of lymphatic material that clogged the child's airway.
Jameson Finley of McKinney, Tex., is the 6-year-old boy who successfully received innovative treatment at CHOP for plastic bronchitis that was clogging his airway.
Credit: The Children's Hospital of Philadelphia
"Our technique represents a new treatment option for plastic bronchitis, which is a rare but often fatal complication of pediatric surgery for single-ventricle disease," said
Yoav Dori, M.D., Ph.D., a pediatric cardiologist in the Cardiac Center at The Children's Hospital of Philadelphia (CHOP), who is the co-author and part of a specialized team which included interventional radiologist Maxim Itkin, M.D., of the Perelman School of Medicine at the University of Pennsylvania, and Marc S. Keller, M.D., an interventional radiologist at CHOP.
Plastic bronchitis is a lymphatic flow disorder, one of a group of diseases characterized by abnormal circulation of lymph, a fluid with a crucial role in immune function and fat and protein transport. Because it has traditionally been difficult to obtain clear images of the lymphatic system, lymphatic flow disorders have often gone undiagnosed. CHOP and Penn are de facto world leaders in lymphatic interventions in children and adults.
The child in the case study, like others with plastic bronchitis, underwent a series of early-childhood heart surgeries, leading to the Fontan procedure, for the birth defect hypoplastic left heart syndrome (HLHS). In HLHS, the left ventricle, one of the heart's pumping chambers, is severely underdeveloped. Some 5 to 10 percent of patients experience plastic bronchitis as a consequence of altered venous and lymphatic pressure resulting from Fontan surgery.
The abnormal circulation causes lymph to ooze into a patient's airways, drying into a fibrous, caulk-like cast formation that takes the shape of the airways. If a child is unable to cough out the cast, the blockage may cause fatal asphyxiation.
In the case study published today, a 6-year-old boy with HLHS had undergone Fontan surgery three years previously. At age five and a half, he suffered respiratory distress and was admitted to a community hospital. Over the next several months, despite a series of evaluations and drug treatments, he continued to have frequent casts.
The boy was referred to CHOP for further evaluation. There the care team used specially developed magnetic resonance imaging (MRI) to visualize the anatomy and flow pattern of lymph in the patient's body, and pinpointed the leakage site: a network of dilated lymphatic vessels surrounding the airway and connected to the thoracic duct through a dilated lymphatic channel. Dori, Itkin and Keller performed the MRI lymphatic techniques they have developed for these children, including dynamic contrast enhanced MRI lymphangiography which provides clearer images of the anatomy and flow of the lymphatic system than conventional MRI.
"Our image analysis allowed us to plan the intervention," said Dori. The team decided on a technique called selective lymphatic embolization. Working in CHOP's interventional cardiac catheterization suite, they injected iodized oil into the leaky small vessel lymphatic network and then glued shut the abnormal dilated feeder lymphatic duct. Stemming the abnormal flow resolved the child's plastic bronchitis. "Eleven months after the procedure, the patient remained free of symptoms, was able to discontinue taking respiratory medicines, and is now playing on a soccer team" said Itkin. Although further investigation must be done in other patients, the authors conclude that selective lymphatic embolization is a potential new treatment for plastic bronchitis. Its targeted, noninvasive application to only the affected site may offer better outcomes for patients.
In fact, "It is conceivable that this technique could even result in a long-term cure for plastic bronchitis," said co-author Jack Rychik, M.D., director of CHOP's Single Ventricle Survivorship Program. "Characterization of the lymphatic system and therapeutic lymphatic intervention may play an important role as we strive to better understand the circulatory consequences of the Fontan operation, in particular those seen in plastic bronchitis" says Rychik. A multi-disciplinary approach involving teams of experts in single ventricle, cardiac catheterization and interventional radiology all focused on the challenges these patients face, as witnessed in this report, is essential for the development of successful outcomes.
CHOP and Penn to Launch Program in Lymphatic Interventions
Plastic bronchitis is one of several lymphatic flow disorders affecting children and adults. Building on their research and clinical expertise in this area, CHOP and Penn Medicine are jointly launching the Lymphatic Imaging and Interventions Program. Among the conditions it will address are chylopericardium and chylothorax, resulting from the leakage of lymphatic fluid into the sac around the heart and into the thoracic cavity, respectively. A similar condition, chylous ascites, occurs when the fluid leaks into the peritoneal cavity.
The center will treat other disorders of lymphatic flow due to diseases such as lymphoangiomatosis, Gorham's disease and lymphangioleiomyomatosis. In addition to plastic bronchitis, some Fontan patients develop another serious lymphatic complication called protein-losing enteropathy that results in leakage of lymphatic fluid and protein into the intestine.
Most of these disorders are individually very rare, so patients requiring this highly specialized care will have access to a dedicated program with clinicians who are world leaders in managing these diseases.
For more information on the Lymphatic Imaging and Intervention Program, visit http://bit.ly/1DxDwsW
Dori et al, "Successful Treatment of Plastic Bronchitis by Selective Lymphatic Sclerosis in a Fontan Patient," August 2014 Pediatrics
About The Children's Hospital of Philadelphia
The Children's Hospital of Philadelphia was founded in 1855 as the nation's first pediatric hospital. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children's Hospital has fostered many discoveries that have benefited children worldwide. Its pediatric research program receives the highest amount of National Institutes of Health funding among all U.S. children's hospitals. In addition, its unique family-centered care and public service programs have brought the 535-bed hospital recognition as a leading advocate for children and adolescents. For more information, visit http://www.chop.edu
Joey McCool Ryan | Eurek Alert!
Scientists develop tiny tooth-mounted sensors that can track what you eat
22.03.2018 | Tufts University
NIH scientists describe potential antibody treatment for multidrug-resistant K. pneumoniae
14.03.2018 | NIH/National Institute of Allergy and Infectious Diseases
An international team of researchers has discovered a new anti-cancer protein. The protein, called LHPP, prevents the uncontrolled proliferation of cancer cells in the liver. The researchers led by Prof. Michael N. Hall from the Biozentrum, University of Basel, report in “Nature” that LHPP can also serve as a biomarker for the diagnosis and prognosis of liver cancer.
The incidence of liver cancer, also known as hepatocellular carcinoma, is steadily increasing. In the last twenty years, the number of cases has almost doubled...
In just a few weeks from now, the Chinese space station Tiangong-1 will re-enter the Earth's atmosphere where it will to a large extent burn up. It is possible that some debris will reach the Earth's surface. Tiangong-1 is orbiting the Earth uncontrolled at a speed of approx. 29,000 km/h.Currently the prognosis relating to the time of impact currently lies within a window of several days. The scientists at Fraunhofer FHR have already been monitoring Tiangong-1 for a number of weeks with their TIRA system, one of the most powerful space observation radars in the world, with a view to supporting the German Space Situational Awareness Center and the ESA with their re-entry forecasts.
Following the loss of radio contact with Tiangong-1 in 2016 and due to the low orbital height, it is now inevitable that the Chinese space station will...
Fraunhofer Institute for Organic Electronics, Electron Beam and Plasma Technology FEP, provider of research and development services for OLED lighting solutions, announces the founding of the “OLED Licht Forum” and presents latest OLED design and lighting solutions during light+building, from March 18th – 23rd, 2018 in Frankfurt a.M./Germany, at booth no. F91 in Hall 4.0.
They are united in their passion for OLED (organic light emitting diodes) lighting with all of its unique facets and application possibilities. Thus experts in...
A new scenario seeking to explain how Mars' putative oceans came and went over the last 4 billion years implies that the oceans formed several hundred million...
For the first time, an interdisciplinary team from the University of Basel has succeeded in integrating artificial organelles into the cells of live zebrafish embryos. This innovative approach using artificial organelles as cellular implants offers new potential in treating a range of diseases, as the authors report in an article published in Nature Communications.
In the cells of higher organisms, organelles such as the nucleus or mitochondria perform a range of complex functions necessary for life. In the networks of...
19.03.2018 | Event News
16.03.2018 | Event News
13.03.2018 | Event News
22.03.2018 | Trade Fair News
22.03.2018 | Earth Sciences
22.03.2018 | Earth Sciences