61 y.o. male,
never smoker, fro
bilateral severe bronchiectasis i
pulmonary TB in 13 years ago
s/p 6 month course of therapy
(unclear if LTBI vs active infection),
MAI i10 years ago
s/p 6 months of azithro, rifampin, and ethambutol,
MAC 2 years ago (untreated), and now
M. abscessus infection
treatment (IV amikacin, imipenem, azithromycin and linezolid,
LAbs
ANA positive 1:320 diffuse pattern
CF genotype testing negative
Etiology likely secondary to Mounier Kuhn Syndrome compounded by recurrent infections
Imaging
61-year-old male with a history of tracheomegaly (suggestive of Mounier Kuhn syndrome) and varicoid bronchiectasis dominantly involving the middle
lobe and lingula with most recent CT 6 months prior with noted multiple lung nodules, presenting for follow-up. In addition he has been treated
for latent tuberculosis, and pulmonary MAC
CT without contrast reveals the following
Unchanged appearance of multiple pulmonary nodules as above.
Unchanged tracheobronchomegaly and mid-lower lung predominant varicose
bronchiectasis.
61 year old male with a
History of treated mycobacterial infections
and chronic cough
CXR Frontal View Bronchiectasis Shaggy Heart Border
CXR Frontal View Mounier Kuhn Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Frontal view shows shaggy heart borders with bibasilar cystic changes consistent with bronchiectasis in the middle lobe and lingula Ashley Davidoff MD TheCommonVein.net 250Lu 135871
Enlarged Trachea and Bronchiectasis
Lateral CXR – View Mounier Kuhn Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Lateral view shows an enlarged trachea and thick walled cystic changes overlying the heart consistent with known bronchiectasis. There is evidence of hyperinflation Ashley Davidoff MD TheCommonVein.net 250Lu 135872a
Lateral CXR -Mounier Kuhn Syndrome Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Lateral view shows an enlarged trachea and thick walled cystic changes overlying the heart consistent with known bronchiectasis. There is evidence of hyperinflation Lateral view (a magnified in b, and shows an enlarged trachea (white arrowheads) and thick walled cystic changes overlying the heart consistent with known bronchiectasis Ashley Davidoff MD TheCommonVein.net 250Lu 135872ac01L
Tracheomegaly and Mucus
Axial CT – Mounier Kuhn and Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Axial CT at the level of the brachiocephalic vessels shows an enlarged trachea with a strand of mucus straddling the lateral walls. The trachea measures up to 3cms which is abnormally enlarged. There are thin walled cystic changes of the airways along the subsegmental arteries in the upper lobes likely reflecting bronchiectasis Ashley Davidoff MD TheCommonVein.net 250Lu 135873a
Mounier Kuhn Tracheomegaly and Bronchiectasis
Axial CT – Mounier Kuhn Tracheomegaly and Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Axial CT at the level of the brachiocephalic vessels shows an enlarged trachea that measures 3cms which is abnormally enlarged. There are thin-walled cystic changes of the airways along the subsegmental arteries in the upper lobes likely reflecting bronchiectasis Ashley Davidoff MD TheCommonVein.net 250Lu 135874ac
Coronal CT – Mounier Kuhn Syndrome, Tracheomegaly and Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Coronal CT at the level of the trachea shows an enlarged trachea that measures 3cms which is abnormally enlarged. There are both thin-walled and mildly thickened cystic changes of the airways along the subsegmental bronchovascular bundle in the upper lobes and lower lobes reflecting bronchiectasis Ashley Davidoff MD TheCommonVein.net 250Lu 135880
Sagittal CT – Mounier Kuhn Syndrome, Tracheomegaly and Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Sagittal CT at the level of the trachea shows an abnormally enlarged trachea. Mild thin walled bronchiectasis is also noted Flattened hemidiaphragm indicates hyperinflation Ashley Davidoff MD TheCommonVein.net 250Lu 135884
Enlarged Mainstem Bronchi and Bronchiectasis
Axial CT – Mounier Kuhn and Enlarged Mainstem Bronchi and Bronchiectasis 61 year old male with a history of treated mycobacterial infections and chronic cough Axial CT at the level of the carina shows bilaterally enlarged mainstem bronchi that measure 1.9cms. each which are abnormally enlarged. There are both thin-walled cystic changes of the airways along the subsegmental arteries in the upper lobes likely reflecting bronchiectasis . Some of these cystic changes in the right upper lobe (upper panel) have thicker walls Ashley Davidoff MD TheCommonVein.net 250Lu 135875a
Coronal CT – Mounier Kuhn and Enlarged Mainstem Bronchi and Bronchiectasis 61-year-old male with a history of treated mycobacterial infections and chronic cough Coronal CT at the level of the carina shows bilaterally enlarged mainstem bronchi that measure 1.9cms. each which are abnormally enlarged. There are both thin-walled cystic changes of the airways along the subsegmental bronchovascular bundles in the upper lobes reflecting bronchiectasis . Ashley Davidoff MD TheCommonVein.net 250Lu 135881
Lady Windermere Syndrome
Axial CT – Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Axial CT at the level of the mid to lower chest shows mildly ectatic segmental airways to the lower, and middle lobe bronchi but significant bronchiectasis to the middle lobe and lingula involving the subsegmental airways. There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135876
Axial CT – Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Axial CT at the level of the mid to lower chest shows mildly ectatic segmental airways to the lower, and middle lobe bronchi but significant bronchiectasis to the middle lobe and lingula involving the subsegmental airways. There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135876
Axial CT Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Axial CT at the level of the mid to lower chest shows mildly ectatic segmental airways to the lower, and middle lobe bronchi but significant bronchiectasis to the middle lobe and lingula involving the subsegmental airways. There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135877
Axial CT – Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Axial CT at the level of the mid to lower chest shows mildly ectatic segmental airways to the lower, and middle lobe bronchi (teal arrowheads (b and c) but significant bronchiectasis to the middle lobe and lingula involving the subsegmental airways (yellow arrowheads b and c). There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135877cL
Coronal CT – Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Coronal CT at the level of the heart shows significant bronchiectasis to the middle lobe and lingula and as a result abut the right and left heart border accounting for the CXR findings of a “shaggy heart border”. There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135879
Right Sagittal CT – Barrel Chest Lady Windermere Syndrome 61-year-old male with a history of treated mycobacterial infections including MAC and chronic cough. Right sagittal CT shows mildly ectatic segmental airways to the upper, middle and lower lobe airways, but significant bronchiectasis to the middle lobe subsegmental airways. There is a relative paucity of mucus in the ectatic airways. The history of MAC and the distribution of the bronchiectasis in the middle lobe and lingula are reminiscent of the diagnosis of Lady Windermere syndrome. The barrel chest reflects hyperinflation and the obstructive nature of the Mounier Kuhn Syndrome Ashley Davidoff MD TheCommonVein.net 250Lu 135883
Coronal CT – Bronchiectasis 61-year-old male with a history of treated mycobacterial infections and chronic cough Coronal CT at the level of the spinal column shows bilateral mildly ectatic mostly thin-walled cystic changes of the airways along the subsegmental bronchovascular bundles in the upper and lower lobes reflecting bronchiectasis. There is a paucity of mucus accumulation. Ashley Davidoff MD TheCommonVein.net 250Lu 135882
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