The management of congenital tracheal stenosis

International Journal of Pediatric Otorhinolaryngology (2003) 67S1, S183—S192

The management of congenital tracheal stenosis�

Martin Elliott*, Derek Roebuck, Clair Noctor, Clare McLaren,Ben Hartley, Quen Mok, Catherine Dunne, Nick Pigott,Chirag Patel, Alpesh Patel, Colin Wallis

The Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK

KEYWORDSTracheal surgery;Stent;Stenosis;Paediatric;Congenital

Summary This paper reviews current concepts and results in the management ofcongenital tracheal stenosis (CTS). Diagnostic options are considered and the require-ments for successful management defined. Chief amongst these is a multi-disciplinaryapproach with individualised patient management. Severe long-segment CTS repre-sents the biggest challenge to clinicians and the worst problems for affected families.Near-death episodes are frequent in affected infants and some cannot be ventilatedand require ECMO. Associated cardiovascular anomalies are frequent. Patients requireimmediate resuscitation and transfer to a specialist unit. After careful assessment,accurate diagnosis and discussion, primary resection and end-to-end repair with aslide technique should always be the first option, with concomitant repair of asso-ciated cardiac anomalies. If this is impossible because of the severity of the lesion,some form of patch tracheoplasty will be indicated. Cardiopulmonary bypass is of-ten required. Patches include pericardium, autograft trachea, carotid artery, carti-lage, and allograft trachea. Mortality ranges from 0 to 30% in the literature, whichlargely comprises single-centre long-term experience. Recurrence is common and canbe managed by stenting and tracheal homograft implantation. Long-term quality oflife of survivors is little reported but seems good. Physiological data are lacking. Toimprove results, we suggest a treatment algorithm to rationalise care.© 2003 British Association for Paediatric Otorhinolaryngology. Published by ElsevierIreland Ltd. All rights reserved.

1. Introduction

Congenital tracheal stenosis (CTS) describes aspectrum of disorders. It is rare, but often lifethreatening and may be difficult and expensiveto treat. It can be very distressing for the pa-tient, their families and for their carers. Symp-

� Research at the Institute of Child health and Great OrmondStreet Hospital for children NHS Trust benefits from Researchand Development funding received from the NHS Executive.

∗Corresponding author. Tel.: +44-2078-298853;fax: +44-2078-138262.

E-mail address: [email protected] (M. Elliott).

toms and signs usually occur in the first monthsof life. Depending on its severity it may presentwith simple stridor or with near-death episodesrequiring resuscitation at home, or worse, inabil-ity to ventilate at all and thus require immediateextra-corporeal membrane oxygenation (ECMO)support [1].

There is no standard definition of LSTS. The re-ports in the literature describe, by means of dia-grams, several variants LSTS. In our own practice(largely but not exclusively congenital) over the lastfew years, we have begun to understand the sig-nificance of this variation. There are at least fourelements that imply severity.

0165-5876/$ — see front matter © 2003 British Association for Paediatric Otorhinolaryngology. Published by Elsevier Ireland Ltd. All rights reserved.doi:10.1016/j.ijporl.2003.08.023

S184 M. Elliott et al.

1. The narrowness of the trachea. We have seenseveral small infants whose trachea is soseverely narrow that ventilation is impossibleand ECMO support has been required. Thereis a big clinical difference between a tracheaof 1mm internal diameter and one of 2.5mminternal diameter.

2. The extent of tracheal involvement. This is prob-ably the classic description of severity, oftenstated in terms of ‘thirds’ of the trachea. LSTSusually implies greater than two-thirds of thetrachea is involved.

3. The involvement of the bronchi. This featureis often ignored, yet may militate against theuse of particular types of repair. In very smallbabies, especially those with associated car-diac lesions, the bronchi are often involved.Associated bronchomalacia has emerged as asignificant risk factor in those with extensivedisease.

4. The presence or absence of complete tra-cheal rings. Complete tracheal rings are com-mon in congenital LSTS. Although they maygrow and there are many children alive withcomplete tracheal rings, some seem to pre-vent adequate growth and precipitate severesymptoms.

We think that all patients with tracheal steno-sis should have their tracheas described accordingto these terms. Morphologic description shouldalso define associated morphologic anomalies ofthe airway (e.g. pig or eparterial bronchus, hy-poplasia or aplasia of the lung) and other as-sociated anomalies particularly of the heart.Each team seeing these patients will have aspecific referral population related to its par-ticular skills. For example, at least 40% of ourpatients have associated cardiac anomalies, re-flecting our role as a quaternary cardiac referralunit.

Interpreting the available literature is difficultbecause of inconsistent definitions and the lack ofagreed standards of severity. Many authors, how-ever, have resorted to pictorial descriptions of thetreated lesions [2]. Until adequate definitions areagreed, these methods are very helpful.

Relatively few authors have contributed rel-atively few patient histories to the literature,mostly in the last 20 years. This reflects the rar-ity of the disorders, as well as their complexityand difficulty in management. These considera-tions argue strongly in favour of specialist unitsand multi-disciplinary dedicated teams. As we willmake clear intense commitment is required to carefor these patients.

2. Presenting symptoms andmanagement

A substantial minority of the patients we arereferred1 have only severe inspiratory and expira-tory stridor as their primary symptoms. Such pa-tients can often be investigated as an outpatient.The majority, however, have presented to their lo-cal hospital in extremis after ‘collapse’ at home.Active resuscitation by parents has frequently beenrequired and most have been intubated with diffi-culty and prove hard to ventilate. Ventilation withhelium as an inert oxygen carrier can be helpful toreduce drag on gas flow and reduce air trapping.Some patients have proved impossible to venti-late, and have needed ECMO. A common statementfrom referring physicians is that the patients ‘keeptrying to die’.

As soon as the patient’s ventilatory state is made‘stable’, the child should be transferred to a spe-cialist unit. Transfer can be extremely hazardousand in our view should be made by a trained paedi-atric retrieval team.

3. Investigation

Once the patient is in the appropriate environ-ment, careful assessment and investigation aremandatory. We perform echocardiography, fibreoptic bronchoscopy and bronchography in all pa-tients. Echocardiography is mandatory because ofthe frequency of associated cardiovascular malfor-mations. Computed tomography (CT) or magneticresonance imaging of the chest is often helpful toassess vascular anatomy. This is important, as morethan half of our patients with tracheal stenosishave associated vascular anomalies, most often apulmonary artery sling [3—6]. Virtual bronchoscopyand three-dimensional reformatting of CT images[7,8] may be useful to show the extent and sever-ity of stenosis, but have significant shortcomings.In particular, they do not allow dynamic evaluationof associated bronchomalacia and they may nothave sufficient spatial resolution to show subtle butimportant anatomical findings such as completetracheal rings. In very small children, transmittedcardiac pulsation causes an inevitable degrada-tion in image quality. Although electron beam CTscanners may avoid this pitfall [9,10], these arenot widely available in children’s hospitals. It isfor these reasons that we continue to performbronchography, a simple and cheap procedure

1 At present, we are referred over 35 patients per year withsymptoms or signs of tracheal stenosis.

The management of congenital tracheal stenosis S185

which is easily combined with bronchoscopy,and which has excellent spatial and temporalresolution.

Microlaryngobronchoscopy may be needed to as-sess the larynx and the segment of the tracheaabove the stenosis if there is any question of ob-struction at more than one level. Angiography isnow rarely indicated for the assessment of vascularanomalies associated with tracheal stenosis.

4. Management at the specialist centre

The primary management must be to stabilize theairway. It is almost never necessary to perform atracheostomy, but ECMO support has been neces-sary in four of our patients over the last 6 years.

After completion of investigations, detailed dis-cussions must take place between the managementteam and the family. All treatment options must bepresented, and stress must be laid upon the uncer-tainty of long-term outcome and the limited dataavailable on which to give advice. The manage-ment of CTS imposes enormous strains on family re-lationships, and these must be taken into accountduring pre-operative counselling. These anticipatedfamily consequences prompt some families to optfor no treatment. Support from a liaison nurse andor social workers is often needed. Long hospitalstays are not uncommon and may be far from thepatient’s family home. This adds significantly to thestress.

The management plan is usually based on theseverity and extent of the stenosis and the natureof associated malformations. For the purposes ofthis paper, it should be assumed that associated le-sions would always be repaired concomitantly withthe trachea.

5. Management options and results

Management is categorised according to the sever-ity and extent of the stenosis. Whenever possible,associated cardiac lesions should be simultaneouslyrepaired.

5.1. For very short segment stenoses

Classically primary resection and end-to-end anas-tomosis is the treatment of choice [11]. In the last6 years, we have done four such repairs, with goodoutcomes except in one boy who had persistent lo-calised malacia requiring tracheostomy support.

Recently, balloon dilatation has become an ac-cepted modality [12—15]and the results are encour-

aging. This can be combined with posterior laseringto release complete tracheal rings [16]. Not enoughpatients have been done to recommend this methodbut it is undoubtedly worth exploring. Various stentshave been employed [17—19], but we can see fewindications for their use in children, where defini-tive repair is of greater value, permitting ratherthan constraining growth as is inherent in the useof stents.

5.2. For medium length (<2/3 trachea)stenoses

For these lesions, we agree with Grillo et al. [2] thatslide tracheoplasty should be the initial treatmentof choice. Slide tracheoplasty was first describedby Goldstraw’s group at the Royal Brompton Hos-pital, London [20]and later popularised by Grillofrom the Massachusetts General Hospital [2,21].In the slide tracheoplasty, the stenotic segment istransected at its midpoint, the upper and lowersegments are incised vertically (anteriorly in onesegment, posteriorly in the other), the corners ofthe segments are trimmed to spatulate them, andthe two ends are slid together and sutured. As Grillohas pointed out, and as has subsequently been con-firmed by Macchiarini et al. in animals [22], thecircumference of the trachea is doubled and thecross-sectional area quadrupled. Only native tissueis used and normal, ciliated tracheal epithelium isimmediately present. Subsequent tracheal growthhas been seen to be satisfactory.

In children, mobilisation of the trachea is usu-ally remarkably easy and it is usually equally easyto perform the slide tracheoplasty. Because of theexcellent mobilisation it is even possible to removethe entire trachea in infants [23] although later re-construction may be required.

Despite these theoretical advantages and theobvious logic which they describe, there are sur-prisingly few data in the literature to support thecontention that slide tracheoplasty should be thedefinitive treatment for LSTS. Grillo’s recent report[2] includes a table listing the relevant publicationsand the number of patients reported. We havemodified that table (Table 1) to include more re-cent publications and our own centres (GOS team)5-year experience. These reports cover a widerange of patients from infants to age 43 years. Thelimited numbers of patients infer limited applica-tion of the technique, even in centres operating ona lot of patients with tracheal stenosis. Some caseselection must be taking place.

It is obvious that slide tracheoplasty is both possi-ble and effective. Other than Grillo’s report, little isknown about long-term results, although Goldstraw


Table 1 Outcome of slide tracheoplasty

Reference Year No. Deaths

[20] 1989 2 1[42] 1997 2 1[43] 1998 1 0[44] 1998 1 0[45] 1999 2 0[46] 2000 1 0[28] 2001 4 0[46] 2001 1 0[2] 2002 8 0[47] 2000 3 1[24] 2002 1 0GOS team 2002 6 1

Total 32 4 (12.5)

This table summarises the results of slide tracheo-plasty from the literature. The GOS team data areunpublished, reflect an entirely infant practice, andrelate to our last 6 years experience. Value given inparenthsis is in percentage.

has reported an excellent long-term outcome in hisindex case [24].

However, there are occasions when it can bedifficult or impossible to perform a slide tracheo-plasty. In particular, when there is a pig or eparte-rial bronchus with a significant length of intermedi-ate airway between the false and true carina, theoperation can be impossible and other proceduresshould be employed.

5.3. For long and very long stenoses

It is for this group that the greatest number ofoptions exist, and for this group that the great-est controversy remains. We believe that resection± slide tracheoplasty should always be the firstoption. However, not all patients can be treatedthis way. For patients with very long, very narrowstenoses (see Fig. 1), and especially for those in-volving the bronchi, some form of patch tracheo-plasty is usually indicated.

The basic principles of patch tracheoplasty aresimple. CPB is usually necessary. The trachea isopened longitudinally anteriorly from above to be-low the stenosis. This incision may extend fromcarina to the bronchial branches. The anteriordefect is then enlarged with a patch. Several ma-terials have been employed over the years, butthe most common and successful have been; au-tologous pericardium [25—27], rib cartilage [28],tracheal autograft [29], tracheal allograft [30—32]and, most recently, carotid artery [33,34]. Hetero-graft tissue and prosthetic material do not work.

Fig. 1 This figure shows the bronchogram of a patientwith total tracheal stenosis and associated bronchial in-volvement. We consider such patients unsuitable for slidetracheoplasty.

Brown’s group [25] recommend the use of suspen-sion sutures on the outside of the pericardial patchto create tent like suspension of the patch. To dif-ferentiate this technique from other pericardialpatch repairs, we have called it ‘suspension’ patchtracheoplasty. The available results of these proce-dures are shown in Tables 1—4. Once again it is im-portant to stress that there are relatively few data,

Table 2 Outcome of pericardial patch tracheoplasty

Author Year Patients Mortality

[48] 1994 13 6 (47)[25] 1996 12 2 (17)[27] 2001 28 7 (25)GOS Team 2002 15∗ 4 (27)∗

Total 68 19 (28)

This table shows representative results of pericardialpatch tracheoplasty. The GOS data (from our past 6years experience) include 12 patients who have hada suspended pericardial patch tracheoplasty (as de-scribed by Bando et al.), of whom two patients (17%)have died late after surgery. Of three patients whohad a simple, unsuspended, patch 2 (67%) died earlyafter operation. We no longer perform this operation.Values given in parentheses are in percentage.


Table 3 Outcome of cartilage tracheoplasty.


[49] 1987 4 1 (25)[50] 1988 5 1 (20)[51] 1997 11 5 (45)[53] 1998 4 0[52] 2001 28 7 (25)GOS Team 2002 0 0

Total 54 14 (26)

This table shows representative results of tracheo-plasty using rib cartilage. We have not used thistechnique at our institution, other than in theupper trachea and sub-glottic region. Values given inparenthesis are in percentage.

mostly from single-centre series over long eras.Interpretation of such data is bound to be difficult.

The tracheal autograft is usually used in conjunc-tion with a pericardial patch as described by Backeret al. [29]. It utilises relatively stiff tracheal au-tograft, obtained by resection of the central partof the narrow tracheal segment, to brace open theinvolved carina. Pericardium is used for the uppertracheal patch.

Tracheal allograft implantation is essentially avariant of patch tracheoplasty, utilising homografttracheal tissue. First utilised as a limited patchin adults by Claus Herberhold, an otolaryngologistfrom Bonn, it was adapted, with his help, by ourteam at GOS for use in longer segment stenoses inchildren when combined with the use of cardiopul-monary bypass [30]. Trachea is harvested at theend of conventional organ donation procedures.After gross cleansing, involving the removal oftrachealis and all other loose tissue, the trachea,bifurcation and bronchi with first divisions on eachside are placed in formaldehyde for 14 days. Thegraft is then transferred to methiolate (thimerosal)for 42 days to remove all antigenic protein and fi-

Table 4 Outcome of tracheal autograft repair.


[29] 2000 6 0GOS Team 2002 3 1∗

Total 9 1 (11)

This table shows the results of tracheal autograftrepair. The one death in our series was a 1.4 kg childoperated on at Guy’s Hospital in London with asso-ciated severe tetralogy of Fallot. The child died ofcardiac reasons on the second post-operative day.Value given in parenthsis is in percentage.

nally the graft is stored in acetone to dissolve allfat. The trachea can then be stored for up to ayear. Standard virology and bacterial tests are doneto prevent transmission of diseases. The graft re-tains rigidity similar to that of fresh trachea. Thus,currently applied tracheal homograft replacement(THR) is essentially chemically stored or ‘pickled’.

The operation is almost always reserved for thosepatients with failed previous tracheal repairs of al-most any type, indeed 95% of homograft replace-ments have been done in patients who have hadprevious repairs. The anterior part of the recipi-ent trachea is removed, extending as far distallyas necessary (including the bronchi if required).The new graft is washed, trimmed to size and su-tured in place over silastic Dumon stents (Novatech,France). A large lumen can be achieved as the graftcan be oversized. Increased blood delivery can beachieved by using strap muscle or rectus abdominisflaps to cover the graft [35].

The Dumon stent can be removed endoscopicallyat about 12 weeks post-operatively. Epithelialisa-tion with functioning respiratory epithelium hasbeen demonstrated, and long-term function is ex-cellent. Quality of life after recovery from theinitial procedure is excellent. There has been noevidence of rejection, no immunosuppressive ther-apy is used and no homograft calcification has beenseen.

Long-term survival is remarkably good [32] with a12-year actuarial survival of 70%. Given that almostall of the patients had undergone major trachealreconstructions in the past, this represents a con-siderable success. Tracheal homograft replacementhas a firmly established place as a fallback proce-dure after failed initial procedures. The operationcan be repeated if necessary.

6. Post-operative complications andtheir management

These are complex and sometimes difficult opera-tions. Post-operative problems are to be expected,and fall into some clear categories.

1. Those related to repair of associated lesions andCPB. Since the majority of associated lesions arecardiac, these relate to the well-described con-sequences of CPB [36] or the cardiac lesion. Dis-cussion of these is beyond the scope of this pa-per.

2. Granulation tissue formation. Granulations oc-cur as a part of the healing process of trachealepithelium. They may result in important andeven life-threatening obstruction to a relatively


narrow airway. Clearly, their prevention andmanagement are important issues in paediatrictracheal surgery. Attention to technical detailduring repair is probably helpful in reducingthe incidence, although there are no firm datato support this contention. We would suggestthat wherever possible sutures should be placeddeep to the tracheal mucosa in an attempt todiminish the stimuli to granulation formation.Since granulation tissue will develop at theinterface between native and patch surfaces,prevention of granulations has been a target ofmany workers. We have been very interestedin the research from Backer’s group in Chicago[37], which has reported animal experiments inwhich vascular endothelial growth factor wasfound to reduce the formation of granulationsand accelerate the rate of healing.• The management of granulations is usually

described as bronchoscopic avulsion withgrasping forceps or lasering. We have ob-served that both these may prolong the prob-lem. In our own practice, we have found thatregular balloon dilation (beginning weeklyand thereafter gradually extending intervals)has dramatically reduced the incidence ofgranulation-related airway obstruction.

3. Malacia. As we have become more aggressiveabout tackling even the most severe trachealstenoses, we have become aware that underly-ing distal malacia can be unmasked. Its manage-ment can be very challenging. Three strategiescan be employed, as we have described else-where [38] in the management of conventionaltracheo-bronchomalacia. Prolonged ventilatorysupport with CPAP may work, but a few patientshave required tracheostomy and ‘home’ BIPAPsupport for up to 2 years. We remain very ner-vous about the use of long-term tracheostomyin tracheal reconstruction because of the riskof precipitating either granulation tissue forma-tion or recurrent stenosis. Thus, recently, wehave chosen to use stents in several such pa-tients. We use largely Palmaz stents regularlydilating them and planning, in the long termto over-dilate them to fracture. We do not anylonger use such stents in the presence of ex-trinsic vascular compression. They rarely workand may erode. Malacia may also develop in thepatched segment of trachea. Autologous peri-cardium is soft and floppy. Backer’s group re-port that the patch will become firm enoughto support spontaneous breathing within a fewdays. This has not been a uniform experience forus, and more than half of our pericardial patch

patients have required intervention by stentingto deal with severe acquired malacia. Backer’steam also claim that the autograft remains rela-tively rigid. Again we have contrary experienceand a number of our autograft patients have re-quired stenting. Because of the risk of malacia,and to encourage trachealisation of patch tissue,Carpentier’s group in Paris has researched [33]the use of autologous aortic tissue as a patch.They have demonstrated metaplasia of aortic totracheal tissue in animals. The Leiden group inHolland has recently reported (in a case report[34]) the use of carotid artery as a patch. Webelieve this approach holds much promise in theinfant.• The management of malacia after repair re-

mains unclear. However, we have used thesame techniques we describe below for recur-rent stenosis, with the exception of balloondilatation.

4. Infection. Whist very rare, infection can becatastrophic. During the initial surgery, the un-sterile tracheal contents are exposed to themediastinum, often during a period of immuno-logic compromise, CPB. We have seen two pa-tients with severe mediastinitis, one of whichlater proved fatal.• Mediastinitis must be taken very seriously in

this group of patients. Aggressive debride-ment and antiseptic irrigation should be triedfirst, but we believe that early muscle flapinsertion is the best way to both deal with theinfection and deliver new blood supply to thetracheal patch [39].

7. Recurrence

Unfortunately, recurrent stenosis does occur. Ab-stracting the true incidence of these events fromthe literature is hard [40]. Most of the series arepublished by the primary operating team, and it isfrequently the case that follow-up is by other (oftenENT) teams in the long term. The more severe andextensive the primary lesion, the more likely somekind of recurrence is to occur. Prevention is betterthan cure, and our management is devoted to boththe early detection and prevention of progress ofstenosis. Regular bronchoscopy and bronchographyis performed. Our strategies for management of re-currence are now clear.

• Recurrent stenosis. At the first sign of significantrecurrence, we perform bronchoscopy and bron-chography, together with balloon dilatation of the


stenotic area. Balloon dilatation can then be per-formed electively at relatively frequent intervalsuntil the stenotic process stabilises.

In practice, we have usually used stents at thefirst diagnosis of recurrent stenosis, based on ourobservation that there is usually an important el-ement of collapse, which would probably not re-spond to ballooning. The most commonly usedstent for recurrent stenosis is the Palmaz. Thisballoon-expandable metal stent is relatively easyto insert, but difficult and sometimes dangerousto remove. The Palmaz stent has the advantage ofdilatability with growth, but the disadvantages ofpotential incorporation into the tracheal wall anderosion into the surrounding blood vessels. This isparticularly likely to occur if a pericardial patchhas been used. Three of our patients have suf-fered stent erosion into the innominate artery oraorta. One was fatal and another required emer-gency tracheal homograft implantation [39]. It isthis experience, which leads us to want to ex-plore the use of carotid artery as a patch.

• Extensive recurrence, or failed stenting. Underthese circumstances we would perform a trachealhomograft repair as indicated above. It repre-sents an excellent fall back position, can be re-peated and there is readily available pool of grafttissue.

For Recurrence1 balloon dilatation

2 stent

3 tracheal allograft

Rules

1 always correct

associated lesions

simultaneously

2 always try

resection +/- slide

first

Diagnosis at Referring

Hospital

Discuss with GOS trachealteam; organise retrieval

Assess airway stability

STABLEProceed to Investigations

Investigations see Text

SSCTS

Slide

Tracheoplasty

if not suitable,use balloon

MSCTS

Slide

Tracheoplasty

almost alwayspossible

LSCTS

Slide

Tracheoplasty

If not possible,patch

tracheoplasty

Extensive CTS

Patch

Tracheoplasty

pericardium,autograft,

carotid artery

UNSTABLE?intubatable

YESventilate ? Helium

NOstart ECMO

Fig. 2 Diagram demonstrating the treatment algorithm for congenital tracheal stenosis currently followed up at TheGreat Ormond Street Hospital for Children NHS Trust, London, UK.

8. Follow-up

Surprisingly few long-term data are available. Mostpapers have been published in the last decade andonly a few of them report long-term outcomes.Grillo [2], and Goldstraw and co-workers [24] em-phasise the satisfactory long-term results of slidetracheoplasty, albeit from a group with relativelyfavourable anatomy. Backer et al. [41] also producegood long-term results from their more complexgroup of patients. It appears that if a child getsover the initial procedure (this can take months),and provided that the trachea grows, late airwayfunction can be very good. There are no long-termdetailed physiological studies. They are badlyneeded.

Since there are so few data, it is vital thatfamilies are aware that they sign a contract withuncertainty when they give consent for treat-ment. Detailed, careful follow-up by a commit-ted and informed team is vital to provide adviceto the child and family and adequately to in-form our decision making in the future. In ourown practice, all relatively local patients are fol-lowed up by a respiratory physician (CW), andall patients living distantly are regularly con-tacted by the Nurse co-ordinator of the team(CN).


9. The future

There are a number of important areas of researchcurrently underway which will impact on this field.Firstly, initial resuscitation will be helped by ad-vances in helium-based ventilation. Secondly, newpatch materials will be developed in the near fu-ture. As well as arterial patches, progress in tissueengineering is now very rapid, and groups in Ger-many and the USA are competing to produce thefirst engineered ‘trachea’. In Japan, most efforthas gone into establishing the role of homografttracheal transplantation, not by chemical storage,but by cryo-preservation. It has already been shownin animals that cryo-preserved tracheas can besuccessfully implanted without immunosuppressionand this work should be watched with interest.

Stent technology is also likely to progress fast.Experience with stents in the vascular bed is grow-ing and there may be a role for drug-eluting stentswithin the trachea. We await a good absorbablestent for use in children.

A great deal of research is needed into the phys-iological and social quality of life of these chil-dren and their families. Current data are simplyinadequate.

10. Conclusions

It is both possible and worthwhile to treatlong-segment tracheal stenoses in children. Resultsare improving fast, and new, sophisticated thera-pies are emerging. Successful strategies for recur-rence exist. The treatment algorithm we currentlyemploy is shown as Fig. 2.

Treatment remains both prolonged and expen-sive. It places a considerable strain on families.However, compared with watching your child try todie and suffer the terrible symptoms of a narrowairway, the vast majority of families are delightedthat some treatment is available.

We must do better. Mortality must fall and mor-bidity must diminish. This is only likely to happenby concentrating skills and referrals and maintain-ing a multi-disciplinary team approach.

Acknowledgements

Tracheal surgery is a team sport. The tracheal teamat GOS deals with internal and external referralsand provides a peripatetic service throughout Eu-rope. We would like to thank all the nursing staffat The Great Ormond Street Hospital for Children

who have cared for our tracheal patients. Theyare remarkable people. We would also like to ac-knowledge the help of the rest of the ENT team;Martin Bailey, David Albert and Susannah Leighton.They carried a huge burden at the beginning ofour program. The ICU teams in both PICU and CICUhave regularly performed miracles in resuscitat-ing these children both before and after surgery.Thank you.

References

[1] A.P. Goldman, R.C. Macrae DJ, K.E. Edberg, G. Mellgren,C. Heberhold, J.P. Jacobs, R.E. Delius, M.J. Elliott, Ex-tracorporeal membrane oxygenation (ECMO) as a bridgeto definitive tracheal surgery in children, J. Pediatr. 128(1996) 386—388.

[2] H.C. Grillo, C.D. Wright, G.J. Vlahakes, T.E. MacGillivray,Management of congenital tracheal stenosis by means ofslide tracheoplasty or resection and reconstruction, withlong-term follow-up of growth after slide tracheoplasty, J.Thorac. Cardiovasc. Surg. 123 (2002) 145—152.

[3] W.E. Berdon, D.H. Baker, J.T. Wung, A. Chrispin, K.Kozlowski, M. de Silva, P. Bales, B. Alford, Completecartilage-ring tracheal stenosis associated with anomalousleft pulmonary artery: the ring-sling complex, Radiology152 (1984) 57—64.

[4] C.L. Backer, C. Mavroudis, M.E. Dunham, L. Holinger,Intermediate-term results of the free tracheal autograftfor long segment congenital tracheal stenosis, J. Pediatr.Surg. 35 (2000) 813—818.

[5] S. Kamata, N. Usui, S. Ishikawa, Y. Kitayama, T. Sawai,H. Okuyama, Y. Fukui, A. Okada, Experience in tracheo-bronchial reconstruction with a costal cartilage graft forcongenital tracheal stenosis, J. Pediatr. Surg. 32 (1997)54—57.

[6] F.J. Lang, M. Hurni, P. Monnier, Long-segment congenitaltracheal stenosis: treatment by slidetracheoplasty, J.Pediatr. Surg. 34 (1999) 1216—1222.

[7] D. Manson, P. Babyn, R. Filler, S. Holowka, Three-dimen-sional imaging of the pediatric trachea in congenital tra-cheal stenosis, Pediatr. Radiol. 24 (1994) 175—179.

[8] M. Remy-Jardin, J. Remy, D. Artaud, M. Fribourg, A.Duhamel, Volume rendering of the tracheobronchial tree:clinical evaluation of bronchographic images, Radiology208 (1998) 761—770.

[9] A.S. Brody, J.P. Kuhn, F.G. Seidel, L.S. Brodsky, Airwayevaluation in children with use of ultrafast CT: pitfalls andrecommendations, Radiology 178 (1991) 181—184.

[10] R.C. Brasch, R.G. Gould, C.A. Gooding, H.G. Ringertz, M.J.Lipton, Upper airway obstruction in infants and children:evaluation with ultrafast CT, Radiology 165 (1987) 459—466.

[11] S. Islam, P.T. Masiakos, D.P. Doody, J.J. Schnitzer, D.P.Ryan, Tracheal resection and reanastomosis in the neonatalperiod, J. Pediatr. Surg. 36 (2001) 1262—1265.

[12] S.B. Brown, G.L. Hedlund, C.M. Glasier, K.D. Williams,L.H. Greenwood, J.D. Gilliland, Tracheobronchial stenosisin infants: successful balloon dilation therapy, Radiology164 (1987) 475—478.

[13] R.B. Jaffe, Balloon dilation of congenital and acquiredstenosis of the trachea and bronchi, Radiology 203 (1997)405—409.


[14] C.E. Bagwell, J.L. Talbert, J.J. Tepas III, Balloon dilatationof long-segment tracheal stenoses, J. Pediatr. Surg. 26(1991) 153—159.

[15] A. Messineo, V. Forte, T. Joseph, M.M. Silver, R.M. Filler,The balloon posterior tracheal split: a technique for man-aging tracheal stenosis in the premature infant, J. Pediatr.Surg. 27 (1992) 1142—1144.

[16] H.B. Othersen Jr., A. Hebra, E.P. Tagge, A new methodof treatment for complete tracheal rings in an infant:endoscopic laser division and balloon dilation, J. Pediatr.Surg. 35 (2000) 262—264.

[17] K. Maeda, M. Yasufuku, T. Yamamoto, A new approachto the treatment of congenital tracheal stenosis: balloontracheoplasty and expandable metallic stenting, J. Pediatr.Surg. 36 (2001) 1646—1649.

[18] R.M. Filler, V. Forte, P. Chait, Tracheobronchial stentingfor the treatment of airway obstruction, J. Pediatr. Surg.33 (1998) 304—311.

[19] J.P. Jacobs, J.A. Quintessenza, L.M. Botero, H.M. vanGelder, J.M. Giroud, M.J. Elliott, C. Herberhold, The roleof airway stents in the management of pediatric tracheal,carinal, and bronchial disease, Eur. J. Cardiothorac. Surg.18 (2000) 505—512.

[20] V. Tsang, A. Murday, C. Gilbe, P. Goldstraw, Slide tracheo-plasty for congenital funnel-shaped tracheal stenosis, Ann.Thorac. Surg. 48 (1989) 632—635.

[21] H.C. Grillo, Slide tracheoplasty for long-segment congeni-tal stenosis, Ann. Thorac. Surg. 58 (1994) 613—621.

[22] P. Macchiarini, E. Dulmet, V. de Montpreville, G.-M. Maz-manian, A. Chapelier, P. Darteville, Tracheal growth afterslide tracheoplasty, J. Thorac. Cardiovasc. Surg. 113 (1997)558—566.

[23] J.P. Jacobs, M.P. Haw, J.A. Motbey, M. Bailey, C. Heberhold,M.J. Elliott, Successful complete tracheal resection in athree-month infant, Ann. Thorac. Surg. 61 (1996) 1824—1827.

[24] C.A. Kutlu, P. Goldstraw, Slide tracheoplasty for congenitalfunnel-shaped tracheal stenosis (a 9-year follow-up of thefirst case), Eur. J. Cardiothorac. Surg. 16 (1999) 98—99(Letter).

[25] K. Bando, M.W. Turrentine, K. Sun, et al., Anterior peri-cardial tracheoplasty for tracheal stenosis: intermediateto long-term outcomes, Ann. Thorac. Surg. 62 (1996) 981—989.

[26] F.S. Idriss, S.Y. DeLeon, M.N. Ilbawi, C.R. Gerson, G.F.Tucker, L. Holinger, Tracheoplasty with pericardial patchfor extensive tracheal stenosis in infants and children, J.Thorac. Cardiovasc. Surg. 88 (1984) 527—536.

[27] C.L. Backer, C. Mavroudis, M.F. Gerber, L.D. Holinger, Tra-cheal surgery in children: an 18-year review of four tech-niques, Eur. J. Cardiothoracic. Surg. 19 (2001) 777—784.

[28] J.A. Matute, R. Romero, M.A. Garcia-Casillas, J.C. deAgustin, C. Marhuenda, F.J. Berchi, J. Vazquez, Surgicalapproach to funnel-shaped congenital tracheal stenosis, J.Pediatr. Surg. 36 (2001) 320—323.

[29] C.L. Backer, C. Mavroudis, M.E. Dunham, L. Holinger,Intermediate-term results of the free tracheal autograftfor long-segment congenital tracheal stenosis, J. Pediatr.Surg. 35 (2000) 813—819.

[30] M.J. Elliott, M. Haw, J.P. Jacobs, M. Bailey, J. Evans, C.Heberhold, Tracheal reconstruction in children using ca-daveric homograft trachea, Eur. J. Cardiothorac. Surg. 10(1996) 707—712.

[31] J.P. Jacobs, M.J. Elliott, M.P. Haw, M. Bailey, C. Heberhold,Paediatric tracheal homograft reconstruction: a novel ap-proach to complex tracheal stenoses in children, J. Tho-rac. Cardiovasc. Surg. 112 (1996) 1549—1560.

[32] J.P. Jacobs, J.A. Quintessenza, T. Andrews, R.P. Burke, Z.Spektor, R.E. Delius, R.J.H. Smith, M.J. Elliott, C. Her-berhold, Tracheal allograft reconstruction: the total NorthAmerican and world-wide pediatric experiences, Ann. Tho-rac. Surg. 68 (1999) 1043—1052.

[33] E. Martinod, G. Zakine, P. Fornes, et al., Metaplasia ofaortic tissue into tracheal tissue: surgical perspectives, C.R. Acad. Sci. III 323 (2000) 455—460.

[34] A. Dodge-Khatami, N.C. Nijdam, E. Broekhuis, I.A. vonRosenstiel, P.G. Dahlem, M.G. Hazekamp, Carotid arterypatch plasty as a last resort repair for long-segment con-genital tracheal stenosis, J. Thorac. Cardiovasc Surg. 123(2002) 826—828.

[35] C. Herberhold, M. Stein, E. Bierhoff, S. Kost, Tracheal re-construction with preserved tracheal homograft–—new as-pects, Laryngorhinootologie 78 (1999) 54—56.

[36] M.J. Elliott, R.A. Jonas (Eds.), Cardiopulmonary Bypassin Neonates, Infants and Young Children, Butterworth-Heinemann, London and Boston, 1994.

[37] A. Dodge-Khatami, C.L. Backer, L.D. Holinger, C.Mavroudis, K.E. Cook, S.E. Crawford, Healing of a free tra-cheal autograft is enhanced by topical vascular endothelialgrowth factor in an experimental rabbit model, J. Thorac.Cardiovasc. Surg. 122 (2001) 554—561.

[38] D.P. Inwald, D. Roebuck, M.J. Elliott, Q. Mok, Current man-agement of tracheo-bronchial malacia and stenosis pre-senting to the paediatric intensive care unit, Int. CareMed. 27 (2001) 722—729.

[39] M. Ricci, G.A. Cohen, D. Roebuck, M.J. Elliott, Manage-ment of complex tracheo-aortic fistula following neona-tal tracheal reconstruction, Ann. Thorac. Surg. 75 (2003)1325—1328.

[40] C.L. Backer, C. Mavroudis, M.E. Dunham, L.D. Holinger, Re-operation after pericardial patch tracheoplasty, J. Pediatr.Surg. 32 (1997) 1108—1111.

[41] C.L. Backer, C. Mavroudis, L.D. Holinger, Repair of con-genital tracheal stenosis, Semin. Thorac. Cardiovasc. Surg.Pediatr. Card. Surg. Annu. 5 (2002) 173—186.

[42] S.H. Dayan, M.E. Dunham, C.L. Backer, C. Mavroudis,L.D. Holinger, Slide tracheoplasty in the management oflong-segment tracheal stenosis, Ann. Otol. Rhinol. Laryn-gol. 106 (1997) 914—919.

[43] R. Houel, A. Serraf, P. Macchiarini, J. Bruniaux, C. Planche,Tracheoplasty in congenital tracheal stenosis, Int. J. Pedi-atr. Otorhinolaryngol. (1998) 4431—4438.

[44] T. Muraji, S. Satoh, C. Tsugawa, et al., Slide tracheoplasty:a case report of successful concomitant reconstructionof extensive congenital tracheal stenosis and pulmonaryartery sling, J. Pediatr. Surg. 33 (1998) 1658—1659.

[45] G.S. Lipschutz, R.W. Jennings, J.B. Lopoo, D. Farmer, M.R.Harrison, C.T. Albanese, Slide tracheoplasty for congenitaltracheal stenosis: a case report, J. Pediatr. Surg. 35 (2000)259—261.

[46] E.A. Garabedian, E. LeBret, A. Corre, G. Roger, E. Pineau,P. Bourel, et al., Tracheal resection associated with slidetracheoplasty for long-segment congenital tracheal steno-sis involving the carina, J. Thorac. Cardiovasc. Surg. 121(2001) 393—395.

[47] A.C. Acosta, C.T. Albanese, D.L. Farmer, R. Sydorak, E.Danzer, M.R. Harrison, Tracheal stenosis: the long and theshort of it, J. Pediatr. Surg. 35 (2000) 1612—1616.

[48] T.M. Andrews, R.T. Cotton, W.W. Bailey, C.M. Myer III, S.R.Vester, Tracheoplasty for complete tracheal rings, Arch.Otolaryngol. Head Neck Surg. 120 (1994) 1363—1369.

[49] T.E. Lobe, C.K. Hayden, D. Nicolas, C.J. Richardson, Suc-cessful management of congenital tracheal stenosis in in-fancy, J. Pediatr. Surg. 22 (1987) 1137—1142.


[50] C. Tsugawa, K. Kimura, T. Muraji, E. Nishijima, Y.Matsumoto, H. Murata, Congenital stenosis involving along segment of the trachea: further experience in re-constructive surgery, J. Pediatr. Surg. 23 (1988) 471—475.

[51] S. Kamata, N. Usui, S. Ishikawa, Y. Kitayama, T. Sawai,H. Okuyama, Y. Fukui, A. Okada, Experience in tracheo-bronchial reconstruction with a costal cartilage graft for

congenital tracheal stenosis, J. Pediatr. Surg. 32 (1997)54—57.

[52] R.D. Jaquiss, R.P. Lusk, T.L. Spray, C.B. Huddleston, Repairof long-segment tracheal stenosis in infancy, J. Thorac.Cardiovasc. Surg. 110 (1995) 1504—1511.

[53] Y. Oshima, M. Yamaguchi, H. Ohashi, et al., Pulmonaryartery sling with tracheal stenosis: primary repair in infan-cy, Jpn. J. Thorac. Cardiovasc. Surg. 46 (1998) 347—353.

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The management of congenital tracheal stenosis