Liang Teresa,  Lee Edward

Final Pr. ID: Poster #: EDU-090

Childhood interstitial (diffuse) lung disease (chILD) in infants consists of a rare and heterogeneous group of disorders previously classified with clinical, radiologic, and pathologic features. The purpose of this article is to discuss imaging techniques and provide a pattern-based approach for chILD in infants. Read More

Authors:  Liang Teresa , Lee Edward

Keywords:  childhood interstitial lung disease, Lung, Infants

Deboer Emily,  Deterding Robin,  Lynch David,  Humphries Stephen,  Jacob Joseph,  Devaraj Anand,  Ley-zaporozhan Julia,  Griese Matthias,  Schiwek Marilisa,  Stowasser Susanne,  Clerisme-beaty Emmanuelle

Final Pr. ID: Paper #: 034

Children’s interstitial lung diseases (chILD) comprise a large spectrum of rare diffuse lung disorders, with a subset of patients developing chronic fibrosing lung disease. While imaging criteria for lung fibrosis are established for adults and correlate with prognosis and outcome, there are limited data on imaging for diagnosis, prognosis, and outcomes in children with fibrotic lung disease. To fill this gap, a group of ILD specialists aimed to define the imaging features for diagnosis of lung fibrosis for a forthcoming randomized trial of nintedanib versus placebo in pediatric patients with fibrosing ILD (1199-0337; NCT04093024). Read More

Authors:  Deboer Emily , Deterding Robin , Lynch David , Humphries Stephen , Jacob Joseph , Devaraj Anand , Ley-zaporozhan Julia , Griese Matthias , Schiwek Marilisa , Stowasser Susanne , Clerisme-beaty Emmanuelle

Keywords:  childhood interstitial lung disease, computed tomography, fibrosis criteria

Singh Jaspreet,  Tuburan Smyrna

Final Pr. ID: Poster #: CR-077

We report the case of a neonatal male with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). ACD/MPV is a rare, fatal disorder of the development of the alveolar vasculature. In this disorder, alveolar capillaries are reduced in number and are improperly located within the walls of the alveoli impeding oxygen exchange. In addition to alveolar capillary abnormalities, there are other associated abnormalities with the cardiovascular, gastrointestinal tract, and genitourinary systems. In this report, we describe the case of a term male neonate with a prenatal diagnosis of hypoplastic left heart who developed respiratory distress shortly after birth. The patient required prolonged ECMO and underwent evaluation with CT angiography of the chest. CT findings included diffuse bilateral interstitial thickening with enhancing airspace disease scattered in the left lung. The respiratory distress continued to be unresponsive to treatment, with difficulty weaning ECMO and respiratory support. Life support was withdrawn on day 30. Pathologic examination at autopsy demonstrated ACD/MPV. Our case adds to the limited literature available in describing imaging findings of ACD/MPV and support for early diagnosis with lung biopsy as it can prevent aggressive futile interventions. Read More

Authors:  Singh Jaspreet , Tuburan Smyrna

Keywords:  Interstitial Lung Disease, diffuse lung developmental disease

Bezzant Braydan,  Guo Grace,  Roberie Dustin

Final Pr. ID: Poster #: CR-048

Down syndrome, typically caused by Trisomy 21, is the most common chromosomal abnormality resulting in intellectual disability affecting 1 in 600-800 pregnancies worldwide. Respiratory problems are the leading cause of hospitalizations and excess mortality in DS, accounting for 42% of admissions through age 2 years. Respiratory issues include pulmonary vascular disease, recurrent respiratory infection, sleep-disordered breathing, airway abnormalities, or subpleural cysts. Abnormalities like craniofacial anomalies, developmental delay, chronic aspiration, and hypotonia contribute to the severity of respiratory issues. Independent of congenital heart defect status, the lungs of DS children have widened alveolar ducts, and fewer and enlarged alveoli. These alveolar changes along with connective tissue abnormalities are believed to contribute to the development of subpleural cysts found in up to 20-36% of DS children. The clinical significance of these cysts is variable. It is important for radiologists to be familiar with subpleural cysts associated with DS as it may prevent misdiagnosis with other serious conditions. Additionally, subpleural cysts may increase the risk for pneumothorax or parenchymal damage during mechanical ventilation or surgery. Generally, subpleural cysts are poorly seen on radiographs and have been primarily identified using CT. We present a 5 year old male with DS, born at 36 weeks gestation, who presented to the emergency room with cough, rhinorrhea, and fever. He had no history of respiratory issues and received routine vaccinations. Chest radiograph revealed bilateral apical predominant subpleural cystic lucencies, perihilar fullness, and peribronchial cuffing. He was diagnosed with acute upper respiratory infection and discharged home with symptomatic management and outpatient follow-up. Sedated CT of the chest two weeks later had findings of diffuse bilateral subpleural and perilymphatic cysts, small subpleural calcifications, and mild scattered interlobular septal thickening. Since he did not have respiratory issues at baseline or after his illness, there was less concern for progressive disease requiring intervention and he was managed with conservative monitoring. Our case highlights the importance of recognizing pulmonary manifestations frequently associated with DS to improve disease recognition and prevention of complications. Read More

Authors:  Bezzant Braydan , Guo Grace , Roberie Dustin

Keywords:  Cystic Lung Disease

Sharma Priya,  Schaiberger Gregory,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-076

In this educational exhibit, we will present a series of childhood interstitial lung diseases (chILD) cases from our institution and present the x-ray and computed tomography (CT) images. We will describe a wide variety of pathologies that are encountered but are rare. We will discuss key imaging features and include teaching points of these chILDs. Read More

Authors:  Sharma Priya , Schaiberger Gregory , Rajderkar Dhanashree

Keywords:  chILD, Interstitial Lung Disease, Infectious

Gual Fabiana,  Sameshima Yoshino,  Otto Debora,  Braga Fernanda,  Cardoso Mariana,  Fernandes-ferreira Rafael,  Souza Antonio

Final Pr. ID: Poster #: EDU-084

Ultrasonography is a very powerful non-invasive diagnostic tool widely employed for examining multiple organs and systems located both superficially and deeply. This is possible thanks to a wide range of transducers that work on different frequencies and sound lengths to explore all layers of anatomical structures. Nowadays, the reduction of exposure to ionizing radiation in children is advocated by the “as low as reasonably achievable” (ALARA) principle, and therefore, ultrasound (US) has become the main investigation tool in many assessments of lung pediatric disorders. The purpose of this educational exhibit is to describe the normal artefacts produced by LUS in a healthy lung to distinguish them from pathologic patterns and the application of LUS in pediatric diseases and procedures (ultrasound-Guided Lung Recruitment). Read More

Authors:  Gual Fabiana , Sameshima Yoshino , Otto Debora , Braga Fernanda , Cardoso Mariana , Fernandes-ferreira Rafael , Souza Antonio

Keywords:  Ultrasonography, Lung Diseases, ALARA

Hwang James,  Chen Danling,  Kuehne Alexander,  Benyakoub Amine,  Tu Long,  Lisse Sean,  Ehrlich Lauren

Final Pr. ID: Poster #: CR-061

Filamin A (FLNA) is a widely expressed X-linked dominant gene that encodes Filamin A, a protein that crosslinks actin filaments and plays a crucial role in the structure of the cell cytoskeleton. With such a broad role, FLNA mutations are especially consequential and can result in cardiovascular malformations, intellectual disability, skeletal dysplasia, and neuronal migration abnormalities. Additionally, recent literature has linked FLNA mutation to pediatric-onset interstitial lung disease.
A three-month-old female with a history of atrial septal defect, patent ductus arteriosus, pulmonary valve dysplasia, micrognathia, and recent hospitalization for parainfluenza infection causing respiratory failure presented to the emergency department with worsening hypoxemia and respiratory distress. Initial chest radiograph demonstrated an interval increase in bilateral patchy opacifications suggestive of worsening multifocal infection. The patient was subsequently admitted. The patient’s clinical status failed to improve after multiple days of admission. A non-contrast CT study of the chest revealed multiple regions of air trapping, atelectasis, coarsened interstitial markings, and diffuse septal thickening, differential diagnosis included interstitial lung disease versus congenital alveolar abnormality. Of note, MR imaging of the brain obtained showed diffuse nodularity along the subependymal lining of the lateral ventricles and temporal horns, compatible with gray matter heterotopia. This prompted genetic testing which revealed Filamin A deficiency.
Filamin A mutation typically presents radiographically with hyperinflation of the lungs with scattered atelectasis, which can be complicated by concomitant pneumonia. This appearance can mimic pulmonary emphysema and bronchopulmonary dysplasia. Cardiac anomalies are a common manifestation of the mutation; notably, our patient had a history of a dysplastic pulmonary valve, ASD and PDA. CT imaging patterns vary and can present as a combination of cystic and diffuse ground-glass changes, hyperinflation, and emphysema. After extensive consultation, our patient transitioned to palliative care and ultimately passed away at 5 months of age. Maintaining a high index of suspicion for this rare but important entity is crucial to accurately diagnosing this inherited mutation, treating affected patients appropriately, and providing patients and their families with essential information regarding prognosis and inheritance patterns. Read More

Authors:  Hwang James , Chen Danling , Kuehne Alexander , Benyakoub Amine , Tu Long , Lisse Sean , Ehrlich Lauren

Keywords:  Interstitial Lung Disease, Genetics, Chest Computed Tomography (CT)