This clinical case conference document discusses a 95-year-old male patient presenting with progressive shortness of breath. It provides details on the patient's medical history including prior treatment for Mycobacterium avium complex and tracheomalacia. The document summarizes examination findings and test results. It also includes an in-depth discussion of tracheomalacia including classification, causes, symptoms, diagnostic methods such as bronchoscopy, imaging with CT and MRI, and treatment challenges for this condition.

1. Clinical Case Conference
Gagan Kumar MD
Fellow
Pulmonary & Critical Care

2. GL 95yo M
• C/O progressive shortness of breath x 1
month
• Associated with dry cough
• C/O Wheezing on lying down – better with
sitting up.
• No fever/chills/night sweats/weight loss
• No PND/orthopnea
• Treated for CAP with levaquin

3. Past Medical History
•
•
•
•
•
•
•
•
•
•
•
MAI (progressive pulmonary infilterates on CT scan + per
culture reports from BAL in 2007)– started on Azithro + Rifampin
+ Ethambutol but patient took it x 1 month and stopped. No
follow ups.
Tracheomalacia per bronchoscopy in 08/2008, - focal on the
right side.
Tachycardia-bradycardia syndrome, status post pacemaker
placement.
Atrial fibrillation, status post AV ablation.
Diastolic dysfunction.
Hypertension.
Hypothyroidism.
GERD.
History of previous GI bleeding.
Osteoarthritis.
Benign positional vertigo.

4. Allergies - NKDA
• Family history: Non contributory
• Social history:
–
–
–
–
Non smoker
Occasional EtOH – quit 10 years back
Retired steel worker.
Lives at NH

5. Medications
•
•
•
•
•
•
•
•
•
•
•
Combivent 2 puffs 4 times/day
Duoneb TIS
Prednisone 60 mg daily.
Azithromycin 500 mg daily.
Rocephin 1 gram daily.
Cordarone 200 mg daily.
Calcium with Vitamin D 600 mg 1 tab t.i.d.
Lovenox 40 mg subQ at bedtime.
Pepcid 20 mg b.i.d.
Synthroid 150 mcg daily.
Multivitamin with mineral 1 daily.

6. Examination
•
BP 116/62, HR 70. Pulse ox is 99% on 2L/min via NC.
•
GENERAL: AO x 3 pleasant and in no distress.
•
HEENT: No Oral/Nasal lesions or exudates noted.
•
NECK: No JVD, lymphadenopathy or thyromegaly.
•
RESPIRATORY: Chest expansion is equal and bilateral with good effort. Faint bilateral
wheezing throughout.
•
HEART: RRR , normal S1 and S2, No GMR.
•
ABDOMEN: Slightly protuberant, soft, NT/ND , BS + No organomegaly.
•
EXTREMITIES: Warm. No cyanosis, clubbing or edema.
•
NEUROLOGIC: Gait normal. Cranial nerves 2-12 are grossly intact.
•
SKIN: Warm, dry and intact.

7. Labs
14.4
22.5
197
40.8
Blood cultures –ve
124
87
15
3.9
28
1.0
120

8. Radiology
CXR 10/6/10: no focal infiltrates
CT chest 10/12/10:
• Diffuse emphysematous changes
• Patchy opacities in RLL and LLL concerning for
pneumonia
• Multiple linear nodular opacities at apices
• Bilateral pleural effusions with compressive
atelectasis
• Stable RUL nodule, LLL nodule not visualized

9. PFTs
• 10/28/05
– FEV1/FVC – normal
– FEV1 – normal
–
–
–
–
No significant changes with bronchodilator
Flow volume loop – unremarkable
TLC – elevated (shows hyperinflation)
DLco - normal

10. Bronchoscopy
• 8/28/2007 by Dr Hubley
– VC normal
– Trachea revealed significant tracheomalacia seen on
right proximal trachea to cause an approximate
collapse down to 30% of the original diameter with
inspiration.
– No lesions
– Carina – sharp/symmetric
– Mild erythema throughout the airways
– No endobronchial lesions

11. Tracheomalacia
•
•
•
•
Malacia = “softness”
Normal intrathoracic trachea
dilates somewhat with
inspiration and narrows with
expiration
Narrowing is most
prominent when
intrathoracic pressure is
substantially greater than
intraluminal pressure, as it
is during forced expiration,
cough, or the Valsalva
maneuver
Extrathoracic or cervical TM
upper airway collapses
during inspiration

12. Types
• Tracheomalacia - trachea
• Bronchomalacia - one or both of the
main-stem bronchi
• Tracheobronchomalacia - both

13. Are Tracheomalacia & Dynamic
airway collapse the same?
Excessive dynamic
airway collapse?
1.
2.
3.
4.
Tracheobronchial collapse,
Expiratory tracheobronchial collapse,
Expiratory tracheobronchial stenosis,
Tacheobronchial dyskinesia
Tracheobronchial lumen during
coughing is 18–39% narrower than the
maximal inspiratory lumen observed during
restful respiration
Invagination of the posterior membrane
softening of the supporting cartilage and hypotonia of myoelastic
elements

14. Airway lumen during inspiration (A). During expiration there is inward bulging of the posterior membrane.
This process is physiological and is called dynamic airway collapse (DAC) (B). The pathologic
exaggeration of this process results in a reduction in cross sectional area of 50% or more and is called
excessive dynamic airway collapse (EDAC) (C). The pathological collapse of the cartilaginous rings
represents tracheobronchomalacia (TBM). The crescent type TBM occurs when the anterior cartilaginous
wall is softened and results in excessive narrowing of the sagittal airway diameter (D).The saber-sheath
type TBM is due to softening of the lateral walls and excessive narrowing of the transverse airway diameter
(E). Circumferential (combined) type TBM is characterized by anterior and lateral airway walls collapse and
is usually associated with significant airway wall inflammation
Tracheobronchomalacia and excessive dynamic airway collapse Septimiu D. MURGU AND Henri G. COLTRespirology (2006) 11, 388–406

15. History
•
1897 – Czyhlarz : was the first to describe the postmortem finding of
an unusually large trachea and bronchi.
•
1949 - Lemoine : was the first to use bronchoscopy to document
acquired tracheal enlargement in the adult.
•
1950 – Ferraris : described two patients with acquired TM who both
reported “expiratory dyspnea,” the inability to clear secretions, and
recurrent respiratory infections. Both had been labeled and treated as
asthmatic patients
•
1954 - Herzog and Nissen: “Relaxation and expiratory invagination of
the membranous portion of the intrathoracic trachea and the main
bronchi as cause of asphyxial attacks in bronchial asthma and the
chronic asthmoid bronchitis of pulmonary emphysema”. Bone graft in
the membranous trachea to prevent collapse

16. Embryology
1. Stomodeum
2. Pharyngeal gut
3. Thyroglossal duct
4. Tracheobronchial diverticulum
4th week : endodermal lining
of the respiratory diverticulum
gives rise to the epithelial
lining of the larynx, trachea,
bronchi and alveoli.
The cartilaginous and
muscular components of the
trachea and lungs are derived
from the surrounding
splanchnic mesoderm.
www.chronolab.com/embryo/respiratory.htm

17. Classification
•
•
Congenital disease (also called primary): consequence of the
inadequate maturity of tracheobronchial cartilage
– Polychondritis
– Chondromalacia
– Mucopolysaccharidoses: Hunter syndrome and Hurler
syndrome
– Idiopathic “giant trachea” or Mounier- Kuhn
syndrome
– most common associated disease is tracheoesophageal
fistula
• trachea receiving too much tissue during embryologic
separation
Acquired disease (also called secondary).

18. Acquired Tracheomalacia
•
Posttraumatic
•
•
•
Emphysema
Chronic infection/bronchitis
Chronic inflammation
•
Chronic external compression of the trachea
•
Vascular rings, previously undiagnosed in childhood
–
–
–
–
Post-intubation
Post-tracheostomy
External chest trauma
Post-lung transplantation
–
Relapsing polychondritis
–
–
–
–
–
Malignancy
Benign tumors
Cysts
Abscesses
Aortic aneurysm

19. Mounier- Kuhn syndrome
• Third or fourth decade of life.
•
Atrophy of longitudinal elastic fibers and thinning of the
muscularis mucosa
•
Diagnostic criteria: if
•
Secretions are poorly mobilized, leading to the chronic
accumulation of secretions
– Recurrent infections,
– Bronchiectasis
– Rarely pulmonary fibrosis
– Right mainstem > 2.4cm
– Left mainstem > 2.3cm
– Trachea exceed > 3.0 cm

20. Tracheostomy
•
Degeneration of normal cartilaginous support
– Prolonged intubation
– Tracheotomy
– Severe tracheobronchitis
•
Post-intubation ‘malacia’ is most commonly 3 cm in length
and is segmental in nature
•
Predisposing factors
– Recurrent intubation,
– Duration of intubation
– Use of high-dose steroids
– Chronic inflammation
– Irritants, such as cigarette smoke

21. Tracheostomy/Intubation
• SITES
– Stoma
– cuff site
– impingement point
• Mechanism
– Pressure necrosis,
– Impairment of the blood
supply
– Infection
– Mucosal damage caused
by friction
Where does blood supply
to cartilage comes from?

22. Relapsing Polychondritis
•
“recurrent episodes of
inflammation of the cartilage
of various tissues of the
body”
•
Involves tracheal rings in
56% of cases, but the
respiratory symptoms are
found on presentation in
only 14% of cases
•
Worse prognosis and
poorer response to
corticosteroids
World J Radiol. 2010 July 28; 2(7): 237-24
Characteristic thickening of the anterior cartilaginous wall of
the trachea . The posterior membranous wall is uninvolved

23. Histology
•
Pars membranacea is
dilated and flaccid.
•
Anterio-posterior narrowing
of the bronchial lumen
•
Atrophy of the longitudinal
elastic fibers of the pars
membranacea
•
The normal tracheal
cartilage-to-soft tissue ratio
is approximately 4.5 : 1. In
patients with TBM, this ratio
is often as low as 2 : 1.

24. Prevalence
•
1958 - Herzog : reported TBM in 16 of 1,500 patients (1%)
undergoing bronchoscopy for various respiratory symptoms
•
1977 - Jokinen et al: reported bronchoscopic findings for
2,150 patients with a range of symptoms and found that 94
patients (4.5%) had some form of malacia.
– TM -22%, TBM - 62%, isolated BM in15%
•
1992 - Ikeda S, Hanawa T, Konishi T, et al..
– Rate of airway collapse was 50% in 542 of 4,283 patients
(12.7%) with from pulmonary disease who underwent
bronchoscopy

25. Symptoms
• Dyspnea*
• Cough
•
•
Sputum production
Hemoptysis
•
•
•
•
More symptoms during forced exhalation
Inspiratory wheezing or stridor
Barking cough, which has been likened to a barking seal
Syncope associated with forced exhalation or cough
•
Differentials: “emphysema, chronic bronchitis,
cigarette smoking,or asthma”
*Nuutinen J. Acquired tracheobronchomalacia. Eur J RespirDis 1982; 63:380–387

26. http://www.youtube.com/watch?v=j2-61pPx-ZE&feature=related

27. Diagnosis
•In intubated patients,
– positive-pressure ventilatory support
keeps the airway open.
– “experience respiratory distress,
wheezing, and apparent stridor” on
extubation
“Unexplained extubation failure
should prompt evaluation for TM.”

28. Radiology
• Plain films: not good
– compression from other structures may be
occasionally seen

29. Radiology
• 1970s: Tracheograms and
Bronchograms:
– radiopaque material into the trachea, to
outline the bronchial tree and to evaluate
the size of the structures
• Cinetracheograms were used in the
hopes of seeing “tracheal flutter,”
• Fluoroscopy

30. “Gold Standard”
• Direct visualization by bronchoscopy to
document a narrowing of at least 50% in the
sagittal diameter in expiration*
– Mild : obstruction during expiration is to one half
of the lumen
– Moderate : reaches three quarters of the lumen
– Severe : the posterior wall touches the anterior
wall
• Straining/Coughing/Valsalva :
– to elicit airway wall collapse,
– the expiratory effort to achieve collapse has
never been standardized
* Nuutinen J. Acquired tracheobronchomalacia: a clinical study with bronchological correlations. Ann Clin Res 1977;9:350–355

31. Bronchoscopy

32. Dynamic CT scan
• Dynamic CT scan images, although not the
reference standard, are useful in diagnosing TM
• End-expiratory imaging rather than dynamic
expiratory imaging may require a lower threshold
criterion for diagnosing TBM.
Frown face
http://imaging.consult.com/imageSearch?query=lumen&thes=false&resultOffset=11

33. Multi-detector CT
• Permit imaging of the entire central airways in only a
few seconds
• Gilkeson et al (2001): reported agreement between
dynamic expiratory CT scan findings and
collapsibility seen during bronchoscopy
• Zhang et al (2005):
– low-dose CT scan technique is comparable to a standarddose technique for measuring the tracheal lumen
– Air trapping was seen at a higher frequency (TM patients,
100%; control subjects, 60%) and was more severe in the
patients with TM

34. Excessive narrowing of bronchi (black arrows)
Areas of geographically marginated radiolucency (white arrows) within lungs, = air trapping
Zhang J, Hasegawa I, Hatabu H, et al. Frequency and severity of air trapping at dynamic expiratory CT in patients with
tracheobronchomalacia. AJR Am J Roentgenol 2004; 182:81–85

35. Saggital reconstruction

36. Dynamic MRI
• Suto and Tanabe (1998)
– forced expiration and cough to compare
the collapsibility of the trachea in patients
with TM to that of healthy subjects by
using a “collapsibility index”
– CI = (Maxcsa– Mincsa)/Maxcsa
– lack of ionizing radiation

37. Multiplanar CT
• Three-dimensional CT scan reconstructions,
• Virtual bronchoscopy
• WHY multiplanar?
– images are less than ideal for evaluating airways that
course obliquely (eg., the mainstem bronchi)
• In patients who had relative contraindications to
bronchoscopy

38. Shaded-surface
display image of
central airways in
postero-lateral
projection shows
diffuse narrowing
of trachea and
bronchi (arrows ).
Virtual bronchoscopic image obtained at level of bronchus
intermedius during full inspiration shows mildly narrowed
but patent right middle (M) and lower (L) lobe bronchi.
Virtual bronchoscopic image obtained during dynamic
expiration shows marked narrowing of right middle lobe
bronchus (straight arrow ) with complete collapse of lower
lobe orifice (curved arrow ).

39. Pulmonary function studies
• Useful but not diagnostic
• Spirometry is not in proportion to the severity of
malacia
Decreased FEV1 and
a low PFR with a
rapid decrease in
flow
Near complete absence
of the usual sloping
phase of the midportion of the curve
“Break” or notch in
the expiratory phase
of the flow-volume
loop*
*May be seen in moderate-to-severe emphysema

40. Flow oscillations
•
Sequence of alternating decelerations and accelerations of flow,
are often seen on the expiratory curve
•
Also seen in
– redundant pharyngeal tissue, as in obstructive sleep apnea
syndrome,
– structural or functional disorders of the larynx,
– neuromuscular diseases
Vincken W, Cosio MG. Flow oscillations on the flow-volume loop: a nonspecific indicator of
upper airway dysfunction. Bull Eur Physiopathol Respir 1985; 21:559–567

41. Treatment
•
•
•
•
Supportive –
– unless the situation is emergent or progressively
worsening.
TM frequently occurs in patients who also have COPD:
– the obstructive disorder optimally should be treated first.
Bronchospasm must be controlled
– large pressure swings in the thorax
– worsening the degree of collapse of the malacic tracheal
segments
In relapsing polychondritis
– NSAIDs
– Steroids

42. • If the patient is in critical condition:
– noninvasive, positive-pressure ventilation
– ‘short term’ to keep the airway open and
to facilitate secretion drainage

43. Bronchoscopy + Stenting
Metal stents:
• Easily placed by flexible bronchoscopy,
• Are visible on plain radiographs,
• Expand dynamically
• Preserve mucociliary function
Problems:
• Formation of granulation tissue,
• Breakage over time,
• Airway obstruction, airway perforation
• Make future options such as surgical
interventions difficult or impossible
• Can not be removed easily
Not the first choice for patients with TM.

45. Stents . . .
• Silicone stents
– easily inserted, repositioned,
and removed
• Problems
– rigid bronchoscopy and
general anesthesia
– stent migration (new cough)
– direct visualization and
repositioning

46. • Dynamic features of TBM are quite different
from stenosis
• Constant change in size and shape of the airway
predisposes to stent migration and fracture.
• Long-term safety and efficacy data are sparse
• Ernst et al. 2007 : silicone stent placement.
– But they encountered high rate of stent-related
complications in 3 months (n=75):
• 21 partial stent obstructions, 14 infections, and 10 stent
migrations
• Thornton et al. : metallic stents.
– N=40
– Survival at 1, 2, 3, 4, 5 and 6 years as 79, 76, 51, 47,
38 and 23%, respectively. (died of comorbid causes )

47. Surgical Options
• Tracheostomy
– either bypass the malacic segment
– might splint the airway open
• If generalized and extensive TM,
– a longer tube may be necessary,
• Tracheostomy may aggravate the underlying
disorder and is, therefore, not a first-line treatment

48. Surgery
• Bone graft.
• Tracheal implantation of from one to three biocompatible
ceramic rings (Amedee et al) n=16. follow up 6.5 years.
• *TRACHEOPLASTY ( with Prosthetic and autologous
materials):
– Surgical placation of the posterior wall of the trachea with
crystalline polypropylene and high density polyethylene mesh.
• Conventional resection and reconstruction can be
considered for treatment of focal malacia of the trachea

49. Measure of success !!
1.
2.
3.
4.
5.
Improvement of respiratory symptoms,
Clearing of infectious processes,
Lack of stent complications
Bronchoscopy
Imaging technique
If airway stenting does not improve symptoms or the functional baseline
of the patient, the stents should be removed to avoid any stent-related
complications.

50. Gotway MB, Golden JA, LaBerge JM, et al. Benign tracheobronchial stenoses: changes in short-term
and long-term pulmonary function testing after expandable metallic stent placement. J Comput Assist
Tomogr 2002; 26:564–572
Twenty-two patients underwent 34 tracheal and/or bronchial stent placement
procedures for benign airway stenoses and had the results of pulmonary
function tests available. Stent placement indications included bronchomalacia
after lung transplantation (n11), postintubation stenoses (n6), relapsing
polychondritis (n2), and 1 each of tracheomalacia, tracheal compression, and
histoplasmosis.

51. Kelly A. Carden, Philip M. Boiselle, David A. Waltz and Armin Ernst. Chest 2005;127;984-1005

52. Thanks