Jennifer Jean Soares MD Assistant Professor Aerodigestive Program Medical Director Seattle Children s Hospital Pulmonary and Sleep Medicine Division

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1 The Radiologic Evaluation of a Child with Suspected Diffuse Lung Disease: What the Pulmonologist Needs From the Radiologist Jennifer Jean Soares MD Assistant Professor Aerodigestive Program Medical Director Seattle Children s Hospital Pulmonary and Sleep Medicine Division

2 Childhood interstitial lung disease (child) is a subset of pediatric diffuse lung disease. Rare, heterogenous group of lung diseases that affect the airways, alveolar spaces and interstitium. Characterized by: Diffuse abnormalities on imaging Impaired gas exchange Childhood interstitial lung disease (child) Occurs in a variety of clinical contexts including isolated pulmonary disorders, as a consequence of environmental exposures, in the setting of systemic disorders and in the setting of unknown etiology. Though some forms are similar, most childhood ILD is markedly different from adult ILD in etiology, treatment and outcome. Fan LL, Deterding RR, Langston C. Pediatric interstitial lung disease revisited. Pediatr Pulmonol. 2004;38(5):

3 Childhood ILD includes a distinct set of diseases only seen in infants. Category Diffuse Developmental Disorders Lung Growth Abnormalities Specific conditions of uncertain cause Surfactant Dysfunction Specific Diagnosis Acinar dysplasia, Congenital alveolar dysplasia, Alveolar-capillary dysplasia with pulmonary vein misalignment Pulmonary hypoplasia, Chronic neonatal lung disease (BPD), Associated chromosomal disorders (trisomy 21, others), Associated with congenital heart disease Neuroendocrine cell hyperplasia of infancy (NEHI), Pulmonary interstitial glycogenosis (PIG) SFTPB, SFTPC, ABCA3, NKX2.1/TTF1, Histology consistent with surfactant dysfunction but without recognized genetic cause C Kuo, LR Young. Interstitial lung disease in children. Curr Opin Pediatr Jun;26(3):320-7.

4 The diagnosis of child syndrome is made when 3 of the following 4 criteria are met. 1. Respiratory Symptoms Cough, increased work of breathing at baseline, exercise intolerance 2. Respiratory Signs Tachypnea at rest, digital clubbing, crackles, failure to thrive, respiratory failure 3. Hypoxemia 4. Diffuse parenchymal abnormalities on imaging Kurland G, et al. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med Aug 1;188(3):

5 Diagnosis of the specific child diagnosis allows for optimization of therapies and resources for the patient and their family. Systemic steroids Steroid sparing therapies Chemotherapy/Immunotherapy Genetic counseling Supportive Care Oxygen Ventilatory support Nutrition Immunizations Control of comorbidities

6 An individualized stepwise approach is recommended for evaluation of ILD in a child. Exclude more common diseases causing similar signs and symptoms Cystic fibrosis, primary ciliary dyskinesia, chronic aspiration, immunodeficiency, cardiac disease Computed tomography Inspiratory and expiratory views are essential Bronchoscopy with bronchoalveolar lavage Genetic testing Lung biopsy

7 Though lung biopsy remains the gold standard for diagnosis of child, specific forms of ILD can be diagnosed or suggested without lung biopsy. Genetic testing for disorders of surfactant dysfunction SFTPB, SFTPC, ABCA3, NKX2.1/TTF1, GMCSF receptors α and β CT findings can be suggestive of certain diseases Surfactant dysfunction disorders Bronchiolitis Obliterans CT findings can be specific for certain disorders Neuroendocrine cell hyperplasia of infancy (NEHI) Brody AS, Guillerman RP et al. Neuroendocrine Cell Hyperplasia of Infancy: Diagnosis with High Resolution CT. Am J Roentgenol 2010; 194:

8 Computed tomography can define the presence, extent and pattern of disease in a child with ILD and may avoid lung biopsy. ILD can be suggested based on the distribution, size and/or quantity of the following findings on CT interpretation: Hyperinflation; vascular attenuation Ground glass opacities Linear, reticular, or nodular densities Peribronchial or septal thickening Bronchiectasis Cystic lesions Other: calcifications, adenopathy, pleural thickening, and pleural fluid CT chest can also help determine optimal area for lung biopsy when needed. Kurland G, et al. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med Aug 1;188(3):

9 A set of identical twins with NEHI. Ground glass opacities in the right middle lobe, lingula, and perihilar regions, without other abnormalities, demonstrates a pattern consistent with NEHI. Soares JJ. et al. Childhood Interstitial Lung Diseases: An 18 Year Retrospective Analysis. Pediatrics 2013;132:

10 Toddler with Post Infectious Bronchiolitis Obliterans Presented to pulmonary 1-2 months s/p RSV bronchiolitis infection with tachypnea, retractions, wheeze, hypoxemia and FTT. Chest CT confirmed diagnosis of Bronchiolitis Obliterans Syndrome (BOS).

11 1 year old child testing positive for SFTPC mutation. Chest CT of a 1 year old born at term, requiring O2 in the first week of birth with a history of multiple hospitalizations for hypoxemia, tachypnea and baseline respiratory distress in the setting of a family history of pulmonary fibrosis. Genetic sequencing confirmed SFTPC mutation (L181V).

12 Safe and effective imaging in the evaluation of a child for ILD should include multidisiplinary communication. Imaging of infants in the evaluation of ILD may require sedation and controlled ventilation due to their clinical symptoms and the clarity of the images needed. Extreme Tachypnea Need to determine presence of hyperinflation, air trapping, concerns for true abnormalities versus atelectasis/artifact Communication between radiologist, anesthesiologist and pulmonologist prior to imaging allows for best imaging outcomes.

13 References and Suggested Reading Fan LL, Deterding RR, Langston C. Pediatric interstitial lung disease revisited. Pediatr Pulmonol. 2004;38(5): Brody AS, Guillerman RP et al. Neuroendocrine Cell Hyperplasia of Infancy: Diagnosis with High Resolution CT. Am J Roentgenol 2010; 194: Kurland G, et al. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med Aug 1;188(3): C Kuo, LR Young. Interstitial lung disease in children. Curr Opin Pediatr Jun;26(3): Soares JJ. et al. Childhood Interstitial Lung Diseases: An 18 Year Retrospective Analysis. Pediatrics 2013;132: Guillerman RP, Brody AS. Contemporary Perspectives on Pediatric Diffuse Lung Disease. Radiol Clin N Am 49 (2011) Guillerman RP. Imaging of Childhood Interstitial Lung Disease. Pediatric Allergy, Immunology, and Pulmonology; 2010; 23:1: 43-68

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