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Review article Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management JAMES AUSTIN FRCA * AND TARIQ ALI FRCA *Department of Anaesthesia, Royal Berkshire Hospital, Reading and Paediatric Intensive Care and Anaesthesia, John Radcliffe Hospital, Oxford, UK Summary Tracheomalacia and bronchomalacia are becoming increasingly well recognized. Although pathologically benign conditions, they are responsible for considerable morbidity, occasional mortality and significant difficulties in the operating theatre and intensive care unit. We performed an extensive literature search to identify causal associations, methods of clinical and investigative assessment, treat- ment modalities and anaesthetic experience with these conditions. Keywords: tracheomalacia; bronchomalacia Introduction Tracheobronchomalacia is a rare and serious condi- tion associated with high mortality in children receiving intensive care (1). It is a condition that is becoming increasingly recognized and may often lead to prolonged intubation and ventilation. Anaes- thetists may be called on to provide anaesthesia for a range of procedures in children with this condition, ranging from diagnostic and interventional radiolo- gical procedures, incidental surgery to surgical correction. Anaesthesia can be tricky in these patients and one of the authors has witnessed three critical incidents in recent years; however, informa- tion in the anaesthesia literature relating to this condition is scarce. The intention of this review article is to provide some background to this condi- tion and to provide recommendations with regard to management for Anaesthesia and Intensive Care. What are tracheomalacia and bronchomalacia? Malacia means softness and, in the medical context, is usually used to refer to cartilage or bone. Tracheo- malacia is therefore a softness of the tracheal cartilage; and bronchomalacia, softness of the bron- chial cartilage. The combination of these pathologies is tracheobronchomalacia, and this may coexist with laryngomalacia or generalized chondromalacia. In this review, the term ‘airway malacia’ is used to refer to any malacia of trachea and/or bronchi, but excludes laryngomalacia. What does airway malacia do? Airway malacia allows the affected portion of the airway to collapse, under conditions where the extraluminal pressure exceeds the intraluminal pres- sure. Most cases of airway malacia are intrathoracic; hence, during expiration and particularly forceful Correspondence to: T. Ali, Department of Anaesthetics, John Radcliffe Hospital, Oxford OX3 9 DU, UK. Paediatric Anaesthesia 2003 13: 3–11 Ó 2003 Blackwell Publishing Ltd 3

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Page 1: Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management

Review article

Tracheomalacia and bronchomalacia in children:pathophysiology, assessment, treatment andanaesthesia management

JAMES AUSTIN F R C A* AND TARIQ ALI F R C A†

*Department of Anaesthesia, Royal Berkshire Hospital, Reading and †Paediatric Intensive Careand Anaesthesia, John Radcliffe Hospital, Oxford, UK

SummaryTracheomalacia and bronchomalacia are becoming increasingly well

recognized. Although pathologically benign conditions, they are

responsible for considerable morbidity, occasional mortality and

significant difficulties in the operating theatre and intensive care unit.

We performed an extensive literature search to identify causal

associations, methods of clinical and investigative assessment, treat-

ment modalities and anaesthetic experience with these conditions.

Keywords: tracheomalacia; bronchomalacia

Introduction

Tracheobronchomalacia is a rare and serious condi-

tion associated with high mortality in children

receiving intensive care (1). It is a condition that is

becoming increasingly recognized and may often

lead to prolonged intubation and ventilation. Anaes-

thetists may be called on to provide anaesthesia for a

range of procedures in children with this condition,

ranging from diagnostic and interventional radiolo-

gical procedures, incidental surgery to surgical

correction. Anaesthesia can be tricky in these

patients and one of the authors has witnessed three

critical incidents in recent years; however, informa-

tion in the anaesthesia literature relating to this

condition is scarce. The intention of this review

article is to provide some background to this condi-

tion and to provide recommendations with regard to

management for Anaesthesia and Intensive Care.

What are tracheomalacia andbronchomalacia?

Malacia means softness and, in the medical context,

is usually used to refer to cartilage or bone. Tracheo-

malacia is therefore a softness of the tracheal

cartilage; and bronchomalacia, softness of the bron-

chial cartilage. The combination of these pathologies

is tracheobronchomalacia, and this may coexist with

laryngomalacia or generalized chondromalacia. In

this review, the term ‘airway malacia’ is used to refer

to any malacia of trachea and/or bronchi, but

excludes laryngomalacia.

What does airway malacia do?

Airway malacia allows the affected portion of the

airway to collapse, under conditions where the

extraluminal pressure exceeds the intraluminal pres-

sure. Most cases of airway malacia are intrathoracic;

hence, during expiration and particularly forcefulCorrespondence to: T. Ali, Department of Anaesthetics, JohnRadcliffe Hospital, Oxford OX3 9 DU, UK.

Paediatric Anaesthesia 2003 13: 3–11

� 2003 Blackwell Publishing Ltd 3

Page 2: Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management

expiration or coughing, intrathoracic pressure is

positive and the affected segment of trachea or

bronchus narrows leading to wheeze.

In the less common case of cervical, extrathoracic

tracheomalacia, the collapse takes place during

inspiration. Negative intrathoracic pressure is trans-

mitted to the upper airway which then narrows

under atmospheric pressure, causing inspiratory

stridor.

What is the scale of the problem?

No population incidence studies are available to tell

us how common airway malacia is. Altman identi-

fied 42 cases of paediatric airway malacia in one

hospital over a 5-year period (2) and Burden et al.recorded 62 cases in Paediatric Intensive Care in the

period 1986–95, although they believe that the

condition is underdiagnosed (1). Although tracheo-

malacia usually presents postneonatally, together

with laryngomalacia, it was the most common

diagnosis (23%) in 132 neonates bronchoscoped for

airway difficulties or tracheo-oesophageal malfor-

mations (3).

Airway malacia is particularly common in three

clinical settings, namely bronchopulmonary dyspla-

sia, tracheo-oesophageal malformations (TOM) and

major aortopulmonary malformations. In these

conditions, malacia complicates management, lead-

ing to extended intensive care unit (ICU) stay and

increased morbidity and mortality. Tracheomalacia

is also a significant cause for the need for long-term

tracheostomy in children. In a series of 44 home

tracheostomies, 14 (32%) were for tracheomalacia

(4). However, modern trends in treatment are likely

to decrease this fraction.

Finally, airway malacia may be an unrecognized

cause of Sudden Infant Death Syndrome (SIDS) (5).

An interesting study in 1983 (6) involved extensive

investigation of 58 ‘near-miss SIDS’, with four

diagnoses of tracheomalacia being made.

Classification and associated conditions

Tracheo- or bronchomalacia may be classified as

congenital (also known as primary) or acquired

(secondary). Although no large classification series

is available, a survey of the literature suggests that

the congenital group is slightly more common.

Congenital airway malacia may be further subdi-

vided into isolated (idiopathic) or part of a recog-

nized condition or association (syndromic). Acquired

airway malacia, on the other hand, is typically due to

either compression or to debilitating conditions of

the lungs or cartilage. Table 1 contains a more

detailed list of conditions causing or associated with

airway malacia, some of which are discussed in

detail below.

Premature infants are particularly prone to

airway malacia as part of the spectrum of

Congenital Acquired

Common Tracheo-oesophageal fistula Compression by great vessels:Brachiocephalic

Oesophageal atresia AortaPulmonary artery

Isolated Vascular slingsBronchopulmonary dysplasia

Rare Chromosomal defects: Pulmonary/mediastinal cystsDown’s syndrome

Trisomy 9 Intrathoracic tumours11p13 deletion (Wilms/aniridia) Large/ectopic thymus22q11 deletion Goitres*18–22 translocation Heart-lung transplantChondrodysplasias Liver failure/transplant

Williams–Campbell syndrome Prolonged intubationDi George syndrome TracheostomyCHARGE association Mucocutaneous LeishmaniasisHunter’s syndrome Tetralogy of FallotHurler’s syndrome

* More common cause of airway malacia in adults.

Table 1Clinical associations of airwaymalacia

4 J . AUSTIN & T. ALI

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bronchopulmonary dysplasia (BPD), with incidences

in small bronchoscopic series ranging from 25% to

86% (7–9). As Table 1 suggests, the association

between tracheo-oesophageal malformations and

airway malacia is well documented, although the

mechanism is unclear.

In many cases of TOM, the tracheomalacia is

shown to arise from accompanying compression by

large thoracic vessels or by a dilated oesophageal

pouch (10,11).

Whether malacia contributes to adverse outcome

in TOM is debatable. In one series of 240 patients

(12), tracheomalacia was a major contributor in 17

late deaths. However, in another analysis of 223

patients, it was concluded that tracheomalacia had

no influence on outcome in TOM repair (13).

The mechanism of malacia in aortopulmonary

vascular malformations is straightforward: the

abnormal vessel compresses the airway and restricts

cartilage growth. Thus, even when the vasculature is

corrected, dynamic airway collapse can still occur,

again complicating recovery. In one series (14)

following surgery for congenital cardiovascular

malformations, airway malacia was responsible for

six of 22 prolongations of ICU stay.

Tracheomalacia can also result from prolonged

ventilation, due to internal pressure from a tracheal

tube (15) as well as with tracheostomy (16).

In addition to the conditions listed in Table 1, two

conditions deserve additional attention because of

their complex relationship with airway malacia;

namely, gastro-oesophageal reflux (GOR) and

asthma. GOR aggravates and is aggravated by

airway malacia. One series looking at aortopexy as

treatment for tracheomalacia showed good results,

except in concurrent GOR; however, aggressive

treatment of the GOR restored the good results of

aortopexy (17). Airway malacia can often mimic

asthma (18) and is distinguished by its nonrespon-

siveness to bronchodilators. However, a proportion

of patients will have both conditions.

How does airway malacia manifestclinically?

Tracheo- and bronchomalacia are clinically similar,

with a limited repertoire of clinical manifestations.

These manifestations, however, are nonspecific, and

the diagnosis can be difficult to make unless a high

index of suspicion is held. Table 2 lists the reported

clinical presentations of airway malacia.

Broadly speaking, these presentations fall into

two groups: those that present in hospital, compli-

cating management of some other condition, such as

BPD, TOM or thoracic vessel malformations; and

those that present from home, either as an isolated

finding or as a manifestation of a more subtle

abnormality.

Ventilated children with malacic segments lying

distal to the tracheal or tracheostomy tube will

typically start to become distressed as airway

pressures [and particularly positive end expiratory

pressure/continuousairway pressure (PEEP/CPAP)]

are weaned. Positive airway pressure acts to splint

the airway open and, as this is withdrawn and the

patient starts to breathe spontaneously, airway

collapse occurs. On the other hand, those with

malacia proximal to the end of the tube will clearly

be undistressed until extubation (or tracheostomy

decannulation) itself, when they may require rein-

tubation or noninvasive CPAP (19).

Of those that present from home, many are

initially diagnosed as asthma, with wheeze or

recurrent dry cough. Suspicion of airway malacia

should be raised in those who do not respond to

bronchodilators (18).

Intractable cough, usually brassy in nature, may

be a presenting feature in the older child (20). ‘Dying

spells’, or ‘acute life-threatening events’, are a

dramatic presentation of tracheomalacia; initial

distress caused by a malacic collapse leads to

increased respiratory effort, worsening the collapse

in a vicious cycle. Only when the child loses

consciousness due to hypoxia, does respiration relax

allowing relief of the obstruction (21). Sudden death

may be the extreme result of such episodes (22).

Table 2Clinical presentations of airway malacia

Difficult wean from artificial ventilationFailure of extubation or tracheostomy decannulationEpisodic respiratory distressWheezePersistent dry coughRecurrent chest infectionsPectus excavatumDysphagia‘Dying spells’: acute life-threatening eventsSudden death

TRACHEOMALACIA AND BRONCHOMALACIA 5

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Investigation of airway malacia

Investigations can be divided into three main

groups:

(1) Static imaging: plain X-ray, chest computed

tomography (CT), chest magnetic resonance

imaging (MRI) and 3-D reconstruction CT or

MRI.

(2) Dynamic imaging: flexible bronchoscopy; vid-

eofluoroscopy (± contrast); cine-CT.

(3) Spirometry: flow-volume studies.

As airway malacia is a dynamic process, it follows

that dynamic imaging is the modality of choice.

However, other modalities have specific advantages;

in particular, MRI for delineating compressive cau-

ses, and spirometry for giving a continuous measure

of ventilatory response to therapeutic manoeuvres.

Plain X-ray appears to be a remarkably crude

means of investigating airway malacia, but has the

advantage of being routinely and rapidly available

to most children with respiratory symptoms. As

recently as 1999, Walner et al. claimed a 62%

sensitivity for plain airway X-rays in detecting

tracheomalacia (23). A considerably lower sensitivity

for bronchomalacia might be expected.

‘Static’ chest views with MRI or CT may miss the

dynamic airway closure of malacia, but have the

advantage (particularly MRI) of revealing the anat-

omy of compressive structures (24) and may be

useful in planning surgery.

Until recently, flexible bronchoscopy was consid-

ered the investigation of choice in diagnosing airway

malacia. It is preferably performed on spontaneously

breathing patients to reflect near-normal airway

dynamic, although in the artificially ventilated

patient some airway collapse may still be detectable.

Videotaping, with a slow-motion facility enhances

sensitivity further and enables the degree of collapse

to be graded (25). Early bronchoscopy should be

encouraged in ICU children who have difficulties in

weaning from ventilation. In one PICU study,

bronchoscopy revealed unsuspected stenosis or

malacia in nine of 90 children (26). Bronchoscopy

also has the advantage of being used therapeutically,

to place stents; or as an intraoperative adjunct to

pexy or external splinting procedures.

Fluoroscopy with or without contrast is a useful,

relatively noninvasive alternative to bronchoscopy.

Strauss et al. found contrast fluoroscopy to be as

good as (and in eight of 15 patients superior to)

bronchoscopy in patients with combined stenosis

and malacia (27). Another study combined fluoros-

copy findings with duration of ventilation to form a

powerful predictor of PICU mortality, and found

fluoroscopy to be more useful than bronchoscopy in

this regard (1). Fluoroscopy can also be used with

airway manometry to determine best CPAP (Fig 1)

(28). Whilst bronchoscopy and fluoroscopy may

confirm airway collapse, they provide limited infor-

mation as to the cause of compression. Consequently,

high speed Cine-CT and Cine-MRI are being devel-

oped to improve diagnostic accuracy (29).

Spirometry and flow-volume loops present a

completely different ‘take’ on airway malacia,

examining function rather than structure. It provides

a noninvasive and easily repeatable measure of

airway flow and, by derivation, of resistance. This

enables ready assessment of response to medical

manoeuvres.

In summary, bronchoscopy is considered the ‘gold

standard’ in investigating airway malacia but, in

skilled hands, contrast fluoroscopy may provide as

much information or more, less invasively. With

wider availability, cine radiology may supersede

both, giving the advantage of imaging structures

outside the airway. Spirometry has a completely

different role in continuous assessment and optimi-

zation of treatment.

Treatment of airway malacia

There are essentially five modalities of treatment for

airway malacia. In approximate historical order,

they are: (i) long-term ventilation/CPAP; (ii) resec-

tion of the affected segment; (iii) external splinting;

(iv) pexy procedures (tracheopexy, bronchopexy,

aortopexy, pexy of other vessels) and (v) stenting.

Long-term ventilation was originally the treat-

ment of choice for airway malacia, relying on the fact

that weak cartilage strengthened with time if the

original cause was relieved. CPAP, with or without

ventilatory support, was sufficient to hold the

airway open (30,31). This had the advantage of

being technically easy to apply, noninvasive com-

pared with surgery, and relatively successful for any

size airway. Disadvantages were the high consump-

tion of health care resources and the side-effects of

long-term ventilation, including infections, limited

6 J. AUSTIN & T. ALI

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mobility, delayed speech development and secon-

dary tracheomalacia around the tracheal tube or

tracheostomy site. However, ventilation remains a

slow and complex means of treating airway malacia.

In the search for a more mechanical ‘quick fix’, one

solution (for short-segment malacia) is to resect the

malacic segment and reanastomose the airway (32)

and another is to reinforce the weak cartilage

externally with a splint (Fig 2a). Neither of these

options has become established.

The realization that collapse was not intrinsic, but

depended on positive intrathoracic (and hence

mediastinal) pressure, prompted the introduction

of aortopexy; by lifting the aorta anteriorly in the

mediastinum, it was possible to prevent most of this

pressure being transmitted to the trachea, and hence

prevent (or at least decrease) collapse. This proce-

dure (i.e. the suturing of the aorta anteriorly to the

inner surface of the sternum) seems remarkably

successful even when the aorta is not the obvious

pathological source of the compression (17). It does

not interfere with the later growth of the trachea and

has the advantage of being less invasive than other

intrathoracic procedures, requiring only a small

intercostal window for access (33). In specific cir-

cumstances, the aortopexy principle has been modi-

fied to involve the pulmonary artery (PA-pexy),

ligated patent ductus arteriosus (PDA-pexy) or

innominate (brachiocephalic) artery (34).

The other logical mechanical approach to a malacic

airway is to reinforce it internally, using broncho-

scopically placed stents (Fig 2b). Stents have been

controversial in the past due to their poor results in

early years; however, improvements in material

engineering have overcome most of these problems,

such that stenting is now a competitive mode of

Figure 1(a) Left main bronchomalacia. Fluoroscopy of the respiritory tree showing collapse of the left main bronchus with no PEEP applied.(b) Application of 10 cmH2O PEEP has ’opened’ the left main bronchus. (c) Application of 20 cmH2O PEEP has splinted the left mainbronchus further.

TRACHEOMALACIA AND BRONCHOMALACIA 7

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treatment. Modern expandable reticular stents have

fenestrations allowing ventilation of branching air-

ways, as well as epithelial overgrowth to reestablish

ciliary function. Some stents can be reexpanded at

intervals to allow for growth (35) (Fig 2c).

Complications of stents include displacement

[with one report of a stent being coughed up and

swallowed, with no ill effects (36)], inflammatory

granulomata that may provoke airway obstruction

(37) and fatalities secondary to removal (38). Stents

have the advantage of being easily and rapidly

placed by bronchoscopy, avoiding the resource

outlay and complications of surgery.

Anaesthetic management of patientswith airway malacia

The anaesthetist may be called on to manage airway

malacia in a variety of settings: (i) the patient

undergoing diagnostic (or therapeutic) bronchos-

copy or radiology; (ii) the patient undergoing thor-

acic surgery, with or without correction of the

malacia; and (iii) the patient undergoing surgery

unrelated to the airway malacia.

There is surprisingly little in the literature on

anaesthetic management of airway malacia (39) and

much of it is not available in English. Nevertheless,

Figure 2(a) Splinting of trachea with external plating. (b) Splinting of malacic trachea and left bronchus with expandable internal stents. (c) Ballondilation of internal stents allows for growth.

8 J . AUSTIN & T. ALI

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sensible recommendations can be made in the

understanding of the underlying pathophysiology.

The two main principles are to prevent airway

collapse and air trapping by the use of PEEP/CPAP

and to minimize coughing.

Diagnostic bronchoscopy or radiology for airway

malacia mandates a spontaneously breathing patient

if it is to be of any use. Thus, in broad terms, there

are three anaesthetic options:

Sedation with topical anaesthesia

In skilled hands, this is perfectly practical in any age

group, and arguably provides the best diagnostic

results in terms of realistic airway dynamics. The

emphasis is on adequate sedation and excellent

topical anaesthesia. Strong opioid premedication

provides sedation and suppresses cough; a concom-

itant anticholinergic suppresses secretions and may

decrease vagal responses to airway irritation. Per-

operative supplementation with midazolam will

decrease the discomfort and psychological trauma

to the child. Local anaesthetic is the key to success,

decreasing coughing and minimizing discomfort.

This method is particularly well suited to flexible

bronchoscopy.

General anaesthesia with inhalationalmaintenance

Gas induction is a logical way to induce anaesthesia

while maintaining spontaneous respiration. For

flexible bronchoscopy, this can be continued via

laryngeal mask airway�, with an attachment port

for the fibrescope or, in the case of rigid bronchos-

copy, the anaesthetic circuit can be attatched to the

side port or an insufflation technique employed.

Again, anticholinergic premedication and topical

local anaesthesia to the airway are recommended to

minimize the risk of coughing and laryngeal spasm.

The main drawback to this technique is that the

severity of collapse may be masked by the effects of

general anaesthesia.

General anaesthesia with intravenousmaintenance

This is ideal in the older child. Careful titration of i.v.

anaesthetic, by bolus or infusion, allows the patient

to maintain spontaneous respiration, while freeing

the airway entirely to the bronchoscopist. Oxygen-

ation can be enhanced by insufflation down the side

port.

General anaesthesia for other procedures, whether

using spontaneous or mechanical ventilation, usu-

ally requires the use of CPAP or PEEP to prevent

intraoperative airway collapse. Collapse leads to air

trapping which, in turn, leads to hyperinflation of

the lungs, with each subsequent breath requiring

greater pressure change to achieve the same volume

change. Eventually, intrathoracic pressure may get

so high as to prevent adequate venous return

resulting in dramatic falls in cardiac output and

pulseless electrical activity until adequate expiration

is established, either by halting ventilation or by

manually compressing the chest in combination with

PEEP. The authors’ have witnessed several of these

crises both in theatre and PICU and they have been

associated with morbidity and mortality (40,41).

While there is no good evidence to support any

particular technique, the avoidance of a tracheal tube

where practical by use of a laryngeal mask airway

(42) may decrease postoperative coughing and the

risk of airway collapse on emergence. ‘Deep’ extu-

bation may allow a smooth recovery.

Much of the detail of managing PICU patients

with airway malacia has already been dealt with, but

a high index of suspicion should be maintained in

patients with associated conditions and in those

patients who have difficulty weaning from ventila-

tion, particularly weaning PEEP or CPAP. Failed

extubation complicated by inspiratory stridor or

expiratory wheeze may be the first signs of the

diagnosis. Ready use of bronchoscopy (or contrast

fluoroscopy) should make the diagnosis promptly.

‘Best’ CPAP/PEEP can be established by using a

manometer in the anaesthetic circuit during contrast

fluoroscopy. Bronchodilators are unhelpful in prin-

ciple, unless there is coexisting reactive airways

disease. Stenting or pexy procedures may provide a

rapid alternative to prolonged weaning with the use

of PEEP/CPAP.

Airway malacia is increasingly widely recognized,

and accounts for a significant expenditure of medical

resources, as well as considerable morbidity and

mortality. Unnoticed, it may cause difficulties dur-

ing anaesthesia or in PICU. These problems can

TRACHEOMALACIA AND BRONCHOMALACIA 9

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Page 8: Tracheomalacia and bronchomalacia in children: pathophysiology, assessment, treatment and anaesthesia management

largely be minimized by the judicious use of positive

endexpiratory pressure. Anticipation and prompt

diagnosis can save much distress to the patient and

family. While many of the milder cases will resolve

with time, ongoing improvements in therapy allow

the potential of a good outcome even in the most

severely affected child.

References

1 Burden RJ, Shann F, Butt W et al. Tracheobronchial malaciaand stenosis in children in intensive care: bronchograms helpto predict oucome. Thorax 1999; 54: 511–517.

2 Altman KW, Wetmore RF, Marsh RR. Congenital airwayabnormalities in patients requiring hospitalization. ArchOtolaryngol Head Neck Surg 1999; 125: 525–528.

3 Lindahl H, Rintala R, Malinen L et al. Bronchoscopy during thefirst month of life. J Pediatr Surg 1992; 27: 548–550.

4 Duncan BW, Howell LJ, deLorimier AA et al. Tracheostomy inchildren with emphasis on home care. J Pediatr Surg 1992; 27:432–435.

5 Beal SM, Blundell HK. Recurrence incidence of sudden infantdeath syndrome. Arch Dis Child 1988; 63: 924–930.

6 Finder JD. Primary bronchomalacia in infants and children.J Pediatr 1997; 130: 59–66.

7 Miller RW, Woo P, Kellman RK et al. Tracheobronchialabnormalities in infants with bronchopulmonary dysplasia.J Pediatr 1987; 111: 779–782.

8 Greenholz SK, Hall RJ, Lilly JR et al. Surgical implications ofbronchopulmonary dysplasia. J Pediatr Surg 1987; 22:1132–1136.

9 Wheeler WB, Maguire EL, Kurachek SC et al. Chronic respir-atory failure of infancy and childhood: clinical outcomes basedon underlying etiology. Pediatr Pulmonol 1994; 17: 1–5.

10 Guys JM, Triglia JM, Louis C et al. Esophageal atresia,tracheomalacia and arterial compression: role of aortopexy.Eur J Pediatr Surg 1991; 1: 261–265.

11 Davies MRQ, Cywes S. The flaccid trachea and tracheo-oesophageal anomalies. J Paed Surg 1978; 13: 363–367.

12 Choudhury SR, Ashcraft KW, Sharp RJ et al. Survival ofpatients with esophageal atresia: influence of birth weight,cardiac anomaly, and late respiratory complications. J PediatrSurg 1999; 34: 70–74.

13 Rokitansky AM, Kolankaya VA, Seidl S et al. Recent evaluationof prognostic risk factors in esophageal atresia – a multicenterreview of 223 cases. Eur J Pediatr Surg 1993; 3: 196–201.

14 Bandla HP, Hopkins RL, Beckerman RC et al. Pulmonaryrisk factors compromising postoperative recovery aftersurgical repair for congenital heart disease. Chest 1999; 116:740–747.

15 Windsor HM, Shanahan MX, Cherian K et al. Tracheal injuryfollowing prolonged intubation. Aust NZ J Surg 1976; 46: 18–25.

16 Azizkhan RG, Lacey SR, Wood RE. Anterior cricoid suspensionand tracheal stomal closure for children with cricoid collapseand peristomal tracheomalacia following tracheostomy.J Pediatr Surg 1993; 28: 169–171.

17 Malone PS, Kiely EM. Role of aortopexy in the management ofprimary tracheomalacia and tracheobronchomalacia. Arch DisChild 1990; 65: 438–440.

18 Cohn JR. Localized bronchomalacia presenting as worseningasthma. Ann Allergy 1985; 54: 222–223.

19 Wiseman NE, Duncan PG, Cameron CB. Management oftracheobronchomalacia with continuous positive airway pres-sure. J Pediatr Surg 1985; 20: 489–493.

20 Wood RE. Localized tracheomalacia or bronchomalacia inchildren with intractable cough. J Pediatr 1990; 116: 404–406.

21 Cozzi DA, Bonanni M, Cozzi F et al. Recurrent apparent life-threatening event relieved by glossopexy. J Pediatr Surg 1996;31: 1715–1718.

22 Emery JL, Nanayakkara CF, Wailoo MP. Tracheomalacia –lethal factor in a 17-month-old child. Pediatr Pathol 1984; 2:259–265.

23 Walner DL, Ouanounou S, Donnelly LF et al. Utility ofradiographs in the evaluation of pediatric upper airwayobstruction. Ann Otol Rhinol Laryngol 1999; 108: 378–383.

24 Rimell FL, Shapiro AM, Meza MP et al. Magnetic resonanceimaging of the pediatric airway. Arch Otolaryngol Head NeckSurg 1997; 123: 999–1003.

25 Mair EA, Parsons DS. Pediatric tracheobronchomalacia andmajor airways collapse. Ann Otol Rhinol Laryngol 1992; 101:300–309.

26 Downing GJ, Kilbride HW. Evaluation of airway complicationsin high-risk preterm infants: application of flexible fiberopticairway endoscopy. Pediatrics 1995; 95: 567–572.

27 Strauss HJ, Scheel W, Bartel M. Vergleichende kinematograph-ische, endoskopische und funktionsanalytische Untersuchun-gen zur praoperativen Beurteilung des Schweregrades vonTrachealstenosen. [Comparative cinematographic, endoscopicand functional studies of the preoperative estimation of theseverity of tracheal stenoses]. Z Erkr Atmungsorgane 1989; 172:130–142.

28 Little AF, Phelan EM, Boldt DW et al. Paediatric tracheobron-chomalacia and its assessment by tracheobronchography.Austral Radiol 1996; 40: 398–403.

29 Brody AS, Kuhn JP, Seidel FG et al. Airway evaluation inchildren with use of ultrafast CT: pitfalls and recommenda-tions. Radiology 1991; 178: 181–184.

30 Neijens HJ, Kerrebijn KF, Smalhout B. Successful treatmentwith CPAP of two infants with bronchomalacia. Acta PaediatrScand 1978; 67: 293–296.

31 Kanter RK, Pollack MM, Wright WW et al. Treatment of severetracheobronchomalacia with continuous positive airway pres-sure (CPAP). Anesthesiology 1982; 57: 54–56.

32 Masaoka A, Yamakawa Y, Niwa H et al. Pediatric and adulttracheobronchomalacia. Eur J Carthorac Surg 1996; 10: 87–92.

33 Bullard KM, Scott Adzick N, Harrison MR. A mediastinalwindow approach to aortopexy. J Pediatr Surg 1997; 32:680–681.

34 Kamata S, Usui N, Sawai T et al. Pexis of the great vessels forpatients with tracheobronchomalacia in infancy. J Pediatr Surg2000; 35: 454–457.

35 Nesbitt JC, Carrasco H. Expandable stents. Chest Surg ClinNorth Am 1996; 6: 305–328.

36 Mattison LE, Frye MD, Collop NA. Accidental ingestion of atracheal stent. Chest 1995; 108: 875–876.

37 Furman RH, Backer CL, Dunham ME et al. The use of balloon-expandable metallic stents in the treatment of pediatrictracheomalacia and bronchomalacia. Arch Otolaryngol HeadNeck Surg 1999; 125: 203–207.

38 Filler RM, Forte V, Chait P. Tracheobronchial stenting for thetreatment of airway obstruction. J Pediatr Surg 1998; 33: 304–311.

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� 2003 Blackwell Publishing Ltd, Paediatric Anaesthesia, 13, 3–11

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39 Conroy PT, Bennett NR. Management of tracheomalacia inassociation with congenital tracheo-oesophageal fistula. Br JAnaesth 1987; 59: 1313–1317.

40 Katoh H, Saitoh S, Takiguchi M et al. A case of tracheomalaciaduring isoflurane anesthesia. Anesth Analg 1995; 80: 1051–1053.

41 Menichella G, Pierelli L, Dragani A et al. A preliminary surveyof Italian experience on bone marrow harvesting, processingand manipulation. The Italian Cooperative Study Group on

Cell Manipulation in Hematology. Haematologica 1990; 75:39–42.

42 Yamaguchi S, Takanishi T, Matsumoto T et al. Use of alaryngeal mask airway for anesthesia in a patient withbronchomalacia. Masui 1996; 45: 348–351.

Accepted 30 July 2001

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