Revealing Secrets: Pulmonary Advanced Diagnostic Center

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Children's Doctor

Understanding pulmonary pathology is often very challenging. While some diagnoses can be made clinically and/or with the aid of imaging, such as a community acquired pneumonia, many pulmonary pathologies are not as straight forward. Standard flexible bronchoscopy is helpful to understand anatomical abnormalities and assess for disease associated pathogens, but it can be limited in its diagnostic capabilities for immune compromised hosts and patients with pulmonary lesions. Open lung biopsies are often the gold standard for pulmonary disease diagnosis, but have many associated comorbidities, and finding the lesion without a wide resection is one limiting factor.

CHOP’s new Pulmonary Advanced Diagnostic Center has set out to address these concerns in our pediatric population.

Side-by-side chest CT images Chest CT images A and B represent examples of pulmonary lesions accessible by EBUS technology. Chest CT image of right-sided mass. Technical advances in flexible bronchoscopy have revolutionized the diagnosis and care of adults with lung cancer. Minimally invasive techniques for transbronchial lung and lymph node biopsies under endobronchial ultrasound (EBUS) or CT-guidance have replaced surgical biopsy as the standard of care. These procedures are typically performed in outpatient settings with substantially lower risks and morbidity than the surgical approaches to diagnosis. Unfortunately, these procedures have not been widely adapted for use in children. Therefore, when diagnostic lung and lymph node tissue sampling is required, surgical biopsy is often the only option.

2 Forms of EBUS Advanced Techniques

While lung cancer is very rare in children, difficult-to-diagnose infections and autoimmune, genetic, and inflammatory disorders are more common problems presenting diagnostic challenges in children. With support from Andrew Haas, MD, PhD, and colleagues in the Interventional Pulmonology Program at the Hospital of the University of Pennsylvania, we began performing EBUS-guided transbronchial lung and lymph node biopsies at CHOP in 2015. EBUS advanced technique has two forms: linear EBUS, which is used for the mediastinal, tracheal and proximal bronchial lymph nodes or lesions and radial EBUS which is helpful in identifying peripheral lesions.

Radial EBUS image Endobronchial Ultrasound (EBUS) Example of radial EBUS image with abnormal findings Immunocompromised children with pneumonia were identified as the first group of children at CHOP likely to benefit from EBUS-guided transbronchial lung biopsy. Compared to the standard approach with bronchoalveolar lavage (BAL) alone, EBUS techniques effectively doubled the diagnostic yield for bacterial pneumonia and substantially increased the yield for other types of infection. The procedures were able to be performed safely, and recovery time was typically less than 24 hours, without major complications or need for escalation of care. The experience was summarized in a recent paper published in the American Journal of Respiratory and Critical Care Medicine1.

Mediastinal lymphadenopathy poses another diagnostic challenge in children. These lymph nodes are difficult to access and often nestled among the great vessels coming off the heart. Use of EBUS-guided transbronchial lymph node aspiration techniques are well-established for adults, but reports of its feasibility and utility in children are sparse. At CHOP, this technique has proven safe and effective for children with lymphoma, solid tumors, and sarcoidosis.

We also use navigational bronchoscopy using CT imaging, which improves our ability to more precisely locate pulmonary lesions.

Additional Advanced Techniques Coming Soon

Even with these advances, we are unwavering in our commitment to advance and develop new ways to diagnose lung disease in children and will add additional techniques as they become available.

The Pulmonary Advanced Diagnostic Center caters to patients from the region, across the nation and around the world. Our team has successfully diagnosed patients with infections, cancers, and interstitial lung disease contributing to a diagnostic-based therapy while decreasing associated morbidities associated with more invasive procedures. This one-of-a-kind program will allow unique and personalized care for children with rare diffuse lung disease and other pulmonary lesions whose diagnoses have been uncertain.

We are happy to discuss any case. For more information or to make a referral, call us at 267-426-6629.

Reference and Suggested Reading

Bouso JM, Yendur O, Hysinger E, Planet PJ, Haas A, Goldfarb S, Piccione J. Endobronchial ultrasound-guided biopsy is feasible, safe, and improves diagnostic yields in immunocompromised children. Am J Respir Crit Care Med. 2020;1;201(3):384-386.

Updated: 9/3/20

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