Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

New treatment resolves a hazardous airway complication in child with heart disease

22.10.2014

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!

Further reports about: CHOP HLHS MRI Philadelphia airway diseases disorders hazardous heart disease lymph lymphatic lymphatic system plastic

More articles from Health and Medicine:

nachricht The genes are not to blame
20.07.2018 | Technische Universität München

nachricht Targeting headaches and tumors with nano-submarines
20.07.2018 | Universitätsmedizin der Johannes Gutenberg-Universität Mainz

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Future electronic components to be printed like newspapers

A new manufacturing technique uses a process similar to newspaper printing to form smoother and more flexible metals for making ultrafast electronic devices.

The low-cost process, developed by Purdue University researchers, combines tools already used in industry for manufacturing metals on a large scale, but uses...

Im Focus: First evidence on the source of extragalactic particles

For the first time ever, scientists have determined the cosmic origin of highest-energy neutrinos. A research group led by IceCube scientist Elisa Resconi, spokesperson of the Collaborative Research Center SFB1258 at the Technical University of Munich (TUM), provides an important piece of evidence that the particles detected by the IceCube neutrino telescope at the South Pole originate from a galaxy four billion light-years away from Earth.

To rule out other origins with certainty, the team led by neutrino physicist Elisa Resconi from the Technical University of Munich and multi-wavelength...

Im Focus: Magnetic vortices: Two independent magnetic skyrmion phases discovered in a single material

For the first time a team of researchers have discovered two different phases of magnetic skyrmions in a single material. Physicists of the Technical Universities of Munich and Dresden and the University of Cologne can now better study and understand the properties of these magnetic structures, which are important for both basic research and applications.

Whirlpools are an everyday experience in a bath tub: When the water is drained a circular vortex is formed. Typically, such whirls are rather stable. Similar...

Im Focus: Breaking the bond: To take part or not?

Physicists working with Roland Wester at the University of Innsbruck have investigated if and how chemical reactions can be influenced by targeted vibrational excitation of the reactants. They were able to demonstrate that excitation with a laser beam does not affect the efficiency of a chemical exchange reaction and that the excited molecular group acts only as a spectator in the reaction.

A frequently used reaction in organic chemistry is nucleophilic substitution. It plays, for example, an important role in in the synthesis of new chemical...

Im Focus: New 2D Spectroscopy Methods

Optical spectroscopy allows investigating the energy structure and dynamic properties of complex quantum systems. Researchers from the University of Würzburg present two new approaches of coherent two-dimensional spectroscopy.

"Put an excitation into the system and observe how it evolves." According to physicist Professor Tobias Brixner, this is the credo of optical spectroscopy....

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

Leading experts in Diabetes, Metabolism and Biomedical Engineering discuss Precision Medicine

13.07.2018 | Event News

Conference on Laser Polishing – LaP: Fine Tuning for Surfaces

12.07.2018 | Event News

11th European Wood-based Panel Symposium 2018: Meeting point for the wood-based materials industry

03.07.2018 | Event News

 
Latest News

A smart safe rechargeable zinc ion battery based on sol-gel transition electrolytes

20.07.2018 | Power and Electrical Engineering

Reversing cause and effect is no trouble for quantum computers

20.07.2018 | Information Technology

Princeton-UPenn research team finds physics treasure hidden in a wallpaper pattern

20.07.2018 | Materials Sciences

VideoLinks
Science & Research
Overview of more VideoLinks >>>