Clinical Case Conference

Gagan Kumar MD
Fellow
Pulmonary & Critical Care

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

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.

Allergies - NKDA
Family history: Non contributory
Social history:

Non smoker
Occasional EtOH quit 10 years back
Retired steel worker.
Lives at NH

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.

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.

Labs
14.4
22.5

197
40.8

Blood cultures ve

124

87

15

3.9

28

1.0

120

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

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

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

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

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

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 1839% 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

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, 388406

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

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

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).

Acquired Tracheomalacia

Posttraumatic

Emphysema
Chronic infection/bronchitis
Chronic inflammation

Relapsing polychondritis

Chronic external compression of the trachea

Post-intubation
Post-tracheostomy
External chest trauma
Post-lung transplantation

Malignancy
Benign tumors
Cysts
Abscesses
Aortic aneurysm

Vascular rings, previously undiagnosed in childhood

Mounier- Kuhn syndrome

Third or fourth decade of life.
Atrophy of longitudinal elastic fibers and thinning of
the muscularis mucosa
Diagnostic criteria: if
Right mainstem > 2.4cm
Left mainstem > 2.3cm
Trachea exceed > 3.0 cm

Secretions are poorly mobilized, leading to the chronic

accumulation of secretions
Recurrent infections,
Bronchiectasis
Rarely pulmonary fibrosis

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

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?

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

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-tosoft tissue ratio is approximately
4.5 : 1. In patients with TBM,
this ratio is often as low as 2 : 1.

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

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; 63:380387

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

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.

Radiology
Plain films: not good
compression from other structures
may be occasionally seen

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

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;9:350355

Bronchoscopy

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

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

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; 182:8185

Saggital reconstruction

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

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

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 ).

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

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;
21:559567

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

 If the patient is in critical

condition:
noninvasive, positive-pressure
ventilation
short term to keep the airway open
and to facilitate secretion drainage

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.

Stents . . .
Silicone stents
easily inserted, repositioned, and
removed

Problems
rigid bronchoscopy and general
anesthesia
stent migration (new cough)
direct visualization and
repositioning

 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 )

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

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

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.

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; 26:564572

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.

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

Thanks