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Man with chronic granulomatous disease.

Our appreciation is extended to Dr. Larry Stover,
Indiana University Department of Radiology,
for contributing this case.

History:  Man with chronic granulomatous disease.
Click these images to enlarge them.

Click for galleryClick for galleryWhich choices apply? Please respond with TRUE or FALSE.
Cavitary nodules

True or False
Hydropneumothorax

True or False
Pleural-based lesion

True or False
Changes consistent with immunodeficiency state

True or False
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Man with chronic granulomatous disease.

Here are a couple of CT images of the same patient. Click to enlarge.

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Can you select the actual diagnosis?

Rounded atelectasis.Fibrous tumor of the pleura.Tuberculosis.Pulmonary infarct.None of the above.
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Man with chronic granulomatous disease.

On the left are the same 2 images shown on the prior page. On the R are images from a scan 3 months later. Click to enlarge.

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An important clue regarding the diagnosis is actually visible on both of the CT scans. Did you see it? (Revealed on next page of the case).

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Man with chronic granulomatous disease.

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Findings:

Chest PA and Lat- New pleural based opacity projecting over the left costophrenic angle.  Chronic appearing inflammatory changes primarily within right upper lung. 

CT Chest 1 -  There is a subpleural soft tissue mass with diameters of 3 cm x 1.7 cm.  The mass does not extend into the chest wall and the adjacent ribs appear normal.  The soft tissue mass is highly vascularized and fed by a branch off the aorta.

CT Chest 2 - Compared to prior exam, there has been interval enlargement in the left lung mass measuring approximately 6.3 cm x 3.7 cm.  A feeding vessel is again noted to arise off the aorta near the celiac artery.

Image

Differential diagnosis:

  • Pulmonary sequestration
  • Rounded atelectasis
  • Congenital pulmonary airway malformation (CPAM)
  • Arteriovenous malformation
Diagnosis:  Pulmonary sequestration with superimposed infection
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Man with chronic granulomatous disease.


Key points: 

  • Patients are asymptomatic or present with pneumonia like symptoms due to superinfection Most common location is left lower lobe > right lower lobe.
  • Pulmonary sequestration can be subdivided into intralobar type (same pleural covering as lung and venous drainage via a pulmonary vein) or extralobar type (separate pleural covering as lung and venous drainage via a systemic vein). Can be difficult to determine on imaging.
  • Key point- Persistent lung opacity over multiple presentations with pneumonia-like symptoms.
  • Chest radiographs typically show a persistent opacity that is unchanged (unless superinfection has occurred) over multiple radiographs.
  • CT demonstrates opacification with an arterial supply arising from the descending aorta. Air may be present in cases of secondary infection
  • Round atelectasis can appear as a peripheral mass like opacity; however, there should also be pleural disease/effusion present with a convergence of vessels into the atelectatic lung.
  • If infected, sequestration can appear similar to a CPAM which demonstrates a multilocular cystic lesion
  • Pulmonary AVMs typically appear as more nodular densities with feeding arteries and veins present.
  • Treatment is usually surgical resection although some data suggest they can be followed until infection occurs.

References

  1. Fundamentals of Diagnostic Radiology.  3rd Ed.  Brant, William E. and Clyde A. Helms.  Lippincott Williams & Wilkins.  Philadelphia, PA.  2007. Chapter 51 Pediatric Chest. p. 1251.
  2. Primer of Diagnostic Imaging. 4th Ed.  Weissleder, Ralph, et. al.  Mosby Elsevier. Philadelphia, PA.  Chapter 1 Chest Imaging.
  3. STATdx Online. Pulmonary Sequestraion.  www.statdx.comMay 27, 2011.
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Man with chronic granulomatous disease.


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