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 Norovirus evades immune system by hiding out in rare gut cells
12.10.2017 | University of Pennsylvania School of Medicine

nachricht Flexible sensors can detect movement in GI tract
11.10.2017 | Massachusetts Institute of Technology

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: Neutron star merger directly observed for the first time

University of Maryland researchers contribute to historic detection of gravitational waves and light created by event

On August 17, 2017, at 12:41:04 UTC, scientists made the first direct observation of a merger between two neutron stars--the dense, collapsed cores that remain...

Im Focus: Breaking: the first light from two neutron stars merging

Seven new papers describe the first-ever detection of light from a gravitational wave source. The event, caused by two neutron stars colliding and merging together, was dubbed GW170817 because it sent ripples through space-time that reached Earth on 2017 August 17. Around the world, hundreds of excited astronomers mobilized quickly and were able to observe the event using numerous telescopes, providing a wealth of new data.

Previous detections of gravitational waves have all involved the merger of two black holes, a feat that won the 2017 Nobel Prize in Physics earlier this month....

Im Focus: Smart sensors for efficient processes

Material defects in end products can quickly result in failures in many areas of industry, and have a massive impact on the safe use of their products. This is why, in the field of quality assurance, intelligent, nondestructive sensor systems play a key role. They allow testing components and parts in a rapid and cost-efficient manner without destroying the actual product or changing its surface. Experts from the Fraunhofer IZFP in Saarbrücken will be presenting two exhibits at the Blechexpo in Stuttgart from 7–10 November 2017 that allow fast, reliable, and automated characterization of materials and detection of defects (Hall 5, Booth 5306).

When quality testing uses time-consuming destructive test methods, it can result in enormous costs due to damaging or destroying the products. And given that...

Im Focus: Cold molecules on collision course

Using a new cooling technique MPQ scientists succeed at observing collisions in a dense beam of cold and slow dipolar molecules.

How do chemical reactions proceed at extremely low temperatures? The answer requires the investigation of molecular samples that are cold, dense, and slow at...

Im Focus: Shrinking the proton again!

Scientists from the Max Planck Institute of Quantum Optics, using high precision laser spectroscopy of atomic hydrogen, confirm the surprisingly small value of the proton radius determined from muonic hydrogen.

It was one of the breakthroughs of the year 2010: Laser spectroscopy of muonic hydrogen resulted in a value for the proton charge radius that was significantly...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

ASEAN Member States discuss the future role of renewable energy

17.10.2017 | Event News

World Health Summit 2017: International experts set the course for the future of Global Health

10.10.2017 | Event News

Climate Engineering Conference 2017 Opens in Berlin

10.10.2017 | Event News

 
Latest News

Ocean atmosphere rife with microbes

17.10.2017 | Life Sciences

Neutrons observe vitamin B6-dependent enzyme activity useful for drug development

17.10.2017 | Life Sciences

NASA finds newly formed tropical storm lan over open waters

17.10.2017 | Earth Sciences

VideoLinks
B2B-VideoLinks
More VideoLinks >>>