COD 189 - Idiopathic Pleuroparenchymal Fibroelastosis (iPPFE) - Volumes, Flat Chest and Meandering Trachea

All the findings that go into making a diagnosis of iPPFE

Bhavin Jankharia

Index and Table of Contents

116 Cases with Videos
5 Cases with Posts
30 Snippets
16 Lectures
31 Cases of the Day (CODs)
New Series - ILD Patterns (2 Done)

Index and Table of Contents
Technical and Practice Issues * Case 11: 62-years old misdiagnosed to have interstitial lung disease - mid-inspiratory and expiratory scans * Snippet 03: Radiation risk and CT chest * Case 23: 60-years old - 40-pack years smoker - right upper lobe nodule - resolved using “mean” reconstructions…

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Case

37-years old with gradually progressive dyspnea.

Upper lobe opacities, volume loss

Upper lobe reticular opacities with volume loss, extending below tracheal bifurcation, small left pneumothorax.

CT images show classic findings of pleuroparenchymal fibroelastosis (PPFE), with no other associated etiology, hence idiopathic - iPFFE.

The video describes all the additional findings that often go hand in hand as in

  1. Playthorax - flat chest - progression of platythorax associated with poorer prognosis
    2l Anterior and downward rotation of the ribs - associated with poorer prognosis
  2. Reduced upper lobe lung volumes
  3. Deep suprasternal notch (not present in our patient)
  4. Tracheal deviation and meandering trachea.

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