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• DuonebDuoneb 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

124 87 15

3.9 28 1.0120

Blood cultures –ve

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

Excessive dynamic airway collapse?

Are Tracheomalacia & Dynamic airway collapse the same?

1. Tracheobronchial collapse,2. Expiratory tracheobronchial collapse, 3. Expiratory tracheobronchial stenosis,4. Tacheobronchial dyskinesia

Invagination of the posterior membrane

softening of the supporting cartilage and hypotonia of myoelastic elements

Tracheobronchial lumen duringcoughing is 18–39% narrower than the

maximal inspiratory lumen observed during restful respiration

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-sheathtype 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

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

www.chronolab.com/embryo/respiratory.htm

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.

Embryology

1. Stomodeum2. Pharyngeal gut3. Thyroglossal duct4. Tracheobronchial diverticulum

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

• Emphysema• Chronic infection/bronchitis• Chronic inflammation

– Relapsing polychondritis• Chronic external compression of the trachea

– 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-to-soft 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; 63:380–387

http://www.youtube.com/watch?v=j2-61pPx-ZE&feature=related 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;9:350–355

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 standard-

dose 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; 182:81–85

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

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 ).

Shaded-surface display image of central airways in postero-lateralprojection shows diffuse narrowing of trachea and bronchi (arrows ).

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 mid-portion of the curve

*May be seen in moderate-to-severe emphysema

“Break” or notch in the expiratory phase of the flow-volume loop*

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

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. Improvement of respiratory symptoms,2. Clearing of infectious processes,3. Lack of stent complications4. Bronchoscopy5. 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-relatedcomplications.

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.

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

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

Thanks