the road to hrct evaluation of pediatric diffuse lung diseases.part 2

The road to HRCT evaluation of

Dr/Ahmed Bahnassy

Consultant Radiologist

Riyadh Military Hospital

Causes of chILD• Infectious • Aspiration

(GORD)• Environmental

(hypersensitivity pneumonitis)

• Drug-induced • Neoplastic

diseases (&LCH)

• Lymphoproliferative disorders (including HIV)

• Metabolic disorders

• Surfactant disorders

• Neurocutaneous syndromes

• Idiopathic pulm hemosidrosis

• Infectious • Aspiration

(GORD)• Environmental

(hypersensitivity pneumonitis)

• Drug-induced • Neoplastic

diseases (&LCH)

Causes of chILD cont….

• Collagen vascular disease

• Pulmonary vasculitis syndromes

• Radiation-induced

• Amyloidosis• Graft-versus-

host disease

• ARDS (recovering phase)

• Hypereosinophilic syndromes

• Pulmonary veno-occlusive disease

• Sarcoidosis• With chronic

liver, kidney, bowel diseases

• Lymphoproliferative disorders (including HIV)

• Metabolic disorders• Surfactant disorders• Neurocutaneous

syndromes

• Idiopathic pulm hemosidrosis

Causes of ILD

Between Adults and ChILD

NSIP

ILD

DIP

LIP

Neuroendocrine cell hyperplasia of infancy (NEHI)

Ground Glass opacity primarily affecting the middle and lingular lobes

NEHI

• Another typical example of right middle lobe ,and left lingular GGO.

Surfactant Metabolism Dysfunction

• Surfactant is a complex mixture of phospholipids

and proteins (SP-A, -B, -C and -D)& ABCA3.

• ABCA3 an ATP-binding transporter Of lipids.

(chILD) due to ABCA3 gene mutations

Diffuse GG opacity with variableIntelobular septal thickening

Nonspecific interstitial pneumonitis

Bilateral scattered middle zonal GGOBi basilar consolidations.Bronchial dilatation.

HRCT shows a mosaicperfusion pattern and multiple bilateral linear densities

PIG..Pulmonary interstitial Glycogenosis

• GGO

• Interlobular septal thickening.

• Reticular changes.

• Posterior cysts.

BOOP• Diffuse

nodules.

• Mild intralobular septal thickening.

• Patchy GGO.

Hypersensitivity pneumonitis

• Ground Glass and nodular like opacities in lung bases.

Eosinophilic pneumonia

• Reversed Halo sign

• Right peripheral mid-zonal GGO

Pulmonary alveolar proteinosis

• GGO

• +

• Interlobular septal thickening

• =

• Crazy-paving pattern.

Bronchopulmonary Dysplasia septal thickening,

parenchymal bands and multiple hyperlucent areas.

Repeated HRCT at the age of 2 years shows a mosaic pattern andsome residual parenchymal bands

Parenchymal bands in BPD

Bronchial asthma

Normal

Expiratory scan revealed severeAir trapping

Hemosiderosis

ground-glass attenuation due to pulmonary hemorrhage

Langerhans cell histiocytosis

thick- and thin-walled cysts;few micronodules also seen

pulmonary cystic lesions, some located subpleurally, andbilateral pneumothoraces

Bizarre shaped

Lympngiomatosis

Prominent diffuse smooth septal thickening, bronchovascularbundles and ground-glass attenuation

Consider vascular/lymphatic cause

Lesson learned

Most HRCT features are non-specific,

but when related to the clinical findings, they can suggest the proper diagnosis and obviate biopsy.

A new classification system for pediatricinterstitial lung disease evolved out of the recognitionthat clinical setting is an important considerationin the diagnosis of pediatric ILD and thatcombined clinical, imaging, and pathological correlationis a more powerful diagnostic tool, thanany one single component.

This new pediatric interstitial lung disease classification system was validated for infants and very young children in a retrospective review of 186 lung biopsies done

between 1999 and 2004 with accompanying clinical histories and images from children under age 2 contributed by 11 pediatric institutions in North America.

Based on this new classification system, ChILD is classified into three main groups: (1) disorders of infancy; (2) other categories

(not specific to infancy); and (3)unclassifiable.