Management of Congenital TrachealStenosisSophie C. Hofferberth, MBBSa, Karen Watters, MB, BCh, BAO, MPHb, Reza Rahbar, DMD, MDb, Francis Fynn-Thompson, MDa

abstract Congenital tracheal stenosis (CTS) is a serious and rare condition. In mostcases, stenotic lesions are composed of complete tracheal rings of cartilage.The severity of symptoms correlates with the length of affected trachea, thepresence of concomitant respiratory conditions, degree of luminal narrowing,and any bronchial involvement. Critically, CTS is a disorder that can lead tolife-threatening respiratory insufficiency in children. Thus, it is a clinical entitythat demands timely diagnosis and treatment. This review will firstly discussthe anatomy and pathophysiology of CTS and outline the various clinicalpresentations associated with the disorder. In addition, methods of diagnosisand treatment strategies will be reviewed, with a focus on contemporarysurgical techniques. Finally, postoperative care of patients with CTS will bereviewed, and a contemporary multidisciplinary management approach willbe presented.

Congenital tracheal stenosis (CTS) isa rare but potentially life-threateningdisorder that often leads to severerespiratory insufficiency, particularlyin neonates and infants. The trueincidence of this complex anomaly isunknown given many infants diebefore the diagnosis is made.1 CTSrepresents a spectrum of stenoticairway lesions that are commonlycomposed of complete tracheal ringsof cartilage that vary in location,length, and severity of luminalnarrowing.1–3 The variability inclinical symptoms, diversity ofassociated cardiovascular anomalies,and scarcity of the disorder presentsignificant challenges to its timely andeffective management. The rarity andcomplexity of CTS demandsa multidisciplinary therapeuticapproach and individualized patientmanagement.4

Although a subset of infants with CTSmay outgrow their tracheal stenosisover time,2,5,6 surgical intervention isoften inevitable for symptomaticpatients. Several operative techniquesfor treating CTS have been described,

including resection with end-to-endanastomosis,7 rib cartilage grafttracheoplasty,8,9 pericardial patchtracheoplasty,5,7,10,11 and slidetracheoplasty.4,5,7,12–17 Additionally,the use of endoscopic stenting as theprimary treatment of CTS was recentlyreported.18 The purpose of this reviewis to (1) provide a comprehensiveoverview of the pathology,presentation, and treatmentindications for CTS, (2) describe theavailable therapeutic options and theiroutcomes, and (3) outlinea contemporary managementapproach for treating patients withCTS. We reviewed all articlespublished from Medline between 1964and 2014 that were identified by usingthe following search terms:“congenital tracheal stenosis,”“complete tracheal rings,”“tracheoplasty,” or “trachealmalformation.” Secondary searcheswere conducted by using the terms“trachea and diagnosis,” “trachea andimaging,” and “endotracheal stent.”The year 1964 marks the firstreported surgical repair of CTS.19

Departments of aCardiac Surgery, and bOtolaryngology,Boston Children’s Hospital, Harvard Medical School, Boston,Massachusetts

Dr Hofferberth conceptualized and designed thestudy, performed data collection, and drafted theinitial manuscript; Drs Watters and Rahbar carriedout the initial analyses, and reviewed and revisedthe manuscript; Dr Fynn-Thompson conceptualizedand designed the study, coordinated and superviseddata collection, and critically reviewed themanuscript; and all authors approved the finalmanuscript as submitted.

www.pediatrics.org/cgi/doi/10.1542/peds.2014-3931

DOI: 10.1542/peds.2014-3931

Accepted for publication Mar 25, 2015

Address correspondence to Francis Fynn-Thompson,MD, Department of Cardiac Surgery, BostonChildren’s Hospital, 300 Longwood Ave, Bader 273,Boston, MA 02115. E-mail: francis.fynn-thompson@cardio.chboston.org

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online,1098-4275).

Copyright © 2015 by the American Academy ofPediatrics

FINANCIAL DISCLOSURE: The authors have indicatedthey have no financial relationships relevant to thisarticle to disclose.

FUNDING: No external funding.

POTENTIAL CONFLICT OF INTEREST: The authors haveindicated they have no potential conflicts of interestto disclose.

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PATHOLOGY

CTS is characterized by a narrowingof the tracheal lumen, mostcommonly secondary to completetracheal cartilage rings and an absentmembranous trachea. Rarely, CTS iscaused by irregular circularcartilaginous plates/ridges, ordisorganized cartilage. Cantrell andGuild19 first described CTS, applyinganatomic criteria to classify theanomaly into 3 morphologicallydistinct types: (1) generalizedhypoplasia; (2) funnel-like stenosis;and (3) segmental stenosis (Fig 1). Intype 1 CTS, the larynx is of normaldiameter, whereas the entire length oftrachea is narrowed from the cricoidto the carina. In type 2 CTS, thesubglottic tracheal diameter is ofnormal caliber; however, the tracheaprogressively narrows more distally,with the maximal point ofconstriction usually located justabove the carina. In type 3 CTS,a short (2–5 cm) segment of tracheais narrowed in an hourglass fashion.An additional classification system onthe basis of functional symptoms wasintroduced in 2003,20 classifying CTSas (1) mild–asymptomatic oroccasional symptoms; (2)moderate–respiratory symptoms,without respiratory embarrassment;and (3) severe–severe symptoms,including respiratory embarrassment.This system also includes anadditional subclassification (A or B)to indicate the presence or absence ofassociated malformations. The global

experience in managing CTS hasdemonstrated a strong correlationexists between anatomic type andfunctional category.

CTS is often associated with abnormalbronchial branching patterns.Commonly seen anomalousarborization patterns include trachealbronchus, bridging bronchus,bronchial trifurcation, and unilateralbronchial and lung agenesis. Trachealbronchus (also referred to as “pigbronchus”) is an anomaly in whichthe origin of the right upper lobebronchus is from the right lateral wallof the trachea.21 Bridging bronchus isa malformation wherein the rightmiddle and lower lobes of the lungare supplied by a horizontal bronchusarising from the left mainbronchus.22–24 Bronchial trifurcationinvolves tracheal division into 3bronchi at the level of the carina. Inchildren with unilateral lung agenesis,the trachea continues into the right orleft main bronchus, with thecontralateral bronchus being eitherabsent or severely hypoplastic.25

Isolated CTS is present in just 10% to30% of patients,13,20,26,27 instead it isfrequently associated with othercardiovascular and extrathoracicanomalies. Cardiovascular anomaliesoccur in up to 70% of patients13 andinclude pulmonary artery sling,patent ductus arteriosus, atrial septaldefect, ventricular septal defect,atrioventricular septal defect, doubleaortic arch, partial anomalouspulmonary venous connection,Tetralogy of Fallot, completetransposition of the great arteries,and tricuspid atresia.28 Thecomplexity of these associatedanomalies often complicates thediagnosis of CTS and compoundsoperative risk.29 Associatedextrathoracic congenital anomaliesinclude gastrointestinal, renal, andskeleton abnormalities, includingvertebral defects-anal atresia-cardiovascular anomalies-tracheoesophageal fistula withesophageal atresia-radial and renaldysplasia-limb defects/vertebral

defects-anal atresia-tracheoesophageal fistula withesophageal atresia-radial and renaldysplasia syndromes and anorectalmalformations.

CLINICAL PRESENTATION

The broad spectrum of stenoticlesions in CTS, ranging from short-segment tracheal narrowing tocomplete tracheobronchialhypoplasia, leads to a wide variabilityin clinical presentations. The severityof airway symptoms generallycorresponds with the degree ofairway obstruction.2 Based on thepattern and severity of symptoms, 3groups of patients with CTS can beidentified:

Minimal Symptoms/IncidentalFinding

In this group, CTS is often diagnosedincidentally, or during workup forbiphasic wheeze, a commonpresentation of mildly symptomaticpatients.30,31 Some patients with mildstenosis may remain undiagnoseduntil late childhood or earlyadolescence, when there is oftena tendency to develop exercise-associated respiratory difficulties. Forthese patients, thorough workup anddiligent monitoring is warranted todetermine an appropriatemanagement strategy.6

Symptomatic-Neonatal Period

This group of patients developsrespiratory distress within the firsthours to days of life. Presentingsymptoms include stridor, cyanoticspells or coarse cough, and assistedventilation is often required forcritical respiratory insufficiency,particularly in the setting of complexassociated cardiovascularmalformations. These patientspresent the greatest challenge forclinicians, with severe associatedcongenital anomalies and concurrentrespiratory infections often leadingthese patients to present ina critically ill state. The outcomes for

FIGURE 1Anatomic classification of CTS from Cantrelland Guild19; type 1 is generalized hypoplasia,type 2 is funnel type stenosis, and type 3 issegmental stenosis.

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this group reflect this; studies havedemonstrated 70% to 100%mortality in newborns with CTS whopresent in a critically ill state, withthe major prognostic factor being thepresence and severity of associatedcardiovascular anomalies.2,20,29 Thecomplexity of these patients demandsa multidisciplinary managementapproach, including appropriatecounseling of parents regarding thepotential for poor outcomes.

Symptomatic-Infancy

This cohort usually presents withrespiratory symptoms near the end ofthe first year of life, when physicalactivity increases. Symptoms ofairflow limitation, including wheeze,exertional shortness of breath, andincreased work of breathing becomeapparent. Some data suggestnonsurgical management is a feasibleoption because some children mayoutgrow their CTS2; however, evenjust a minor respiratory infection canprecipitate acute respiratory distressin these patients. Operative mortalityfor this group has been reported at16% to 20%.29 Patients with Cantrelltype 1 (generalized hypoplasia) CTSmorphology are most likely to beseverely symptomatic in theneonatal/early infancy period, andhave a high rate of associatedcardiovascular anomalies.29,32

DIAGNOSTIC EVALUATION

Several diagnostic approaches havebeen used in patients with CTS, withsome variability in practice patternsamong major referral centers.3,33 Thegold standard for definitive diagnosisof CTS is rigid laryngobronchoscopyunder general anesthesia. Directvisualization of the airway enablesaccurate assessment of the length anddiameter of the stenosed trachealsegment. Care should always be takenin performing rigid bronchoscopy; itrequires general anesthesia, andminor mucosal damage mayprecipitate edema and criticalobstruction in patients with severe

airway stenosis. Some clinicians favortracheobronchography; however, thisis not widely used due to risk ofinducing mucosal edema and causingrespiratory decompensation.34 Othershave advocated the use of virtualendoscopy to evaluate post stenosisbronchi and distal airway anatomy inpatients with extreme airwaynarrowing. This approach uses high-resolution multirow detectorcomputed tomography (CT) scanningto obtain high-resolution 3-dimensional endoluminal images tothe level of the segmental bronchi.35

Contrast chest CT scans with 3-dimensional reconstruction is widelyused to delineate major thoracicvessel and airway anatomy. However,this modality frequentlyunderestimates the degree and lengthof airway narrowing, and henceshould be used as an adjunctiveinvestigative tool only.29 MRI hasbeen demonstrated to be an effectiveand noninvasive imaging modality todelineate associated intracardiac andvascular anomalies in patients withCTS. However, the clinicalcharacteristics of the CTS patientpopulation often limits its utility;general anesthesia is often requiredto obtain an MRI in an infant or child,and patients with severe CTS arefrequently intubated beforeundergoing investigations, therebylimiting accurate imaging of thenative airway. Echocardiography is animportant tool to exclude associatedcardiac malformations. The clinicalpractices of major internationalreferral centers have evolved to nowinclude echocardiography in thediagnostic workup of all patientspresenting with suspected CTS.13,33

Figure 2 outlines a diagnosticalgorithm for the investigation ofpediatric patients presenting withsymptoms concerning for CTS.

THERAPEUTIC APPROACHES ANDTREATMENT INDICATIONS

CTS is a complex disease processthat demands a multidisciplinary

therapeutic approach tosuccessfully meet the diverse needsof a complicated patientpopulation.3,34,36 The contemporarymanagement of CTS at majortertiary centers involves anintegrated, multidisciplinary teamapproach with expertise incardiothoracic surgery,otolaryngology, cardiology,pulmonology, andanesthesiology.13,25,33 An importantconsideration is that inappropriateor untimely intervention haspotential to precipitate a criticalairway event in patients with CTS.This further emphasizes theimportance of treating patients withCTS in a specialized,multidisciplinary center with theexpertise and resources to deal withsuch a complication. Nevertheless,in the event that a child doespresent to an underresourcedcenter with critical airway stenosis,extracorporeal membraneoxygenation support presentsa suitable option as a bridgetherapy. This strategy enables initialstabilization of the patient andfacilitates transfer toa multidisciplinary center where thechild can undergo definitive repairof his or her airway lesion.

Selection of an appropriate treatmentstrategy depends on the following:(1) the patient’s clinical status, (2) theseverity and extent of the trachealstenosis, and (3) the presence ofassociated congenital anomalies.20

Elliot et al3 described 4 keycomponents that indicate the severityof CTS: (1) narrowness of the trachea;(2) extent of tracheal involvement;(3) bronchial involvement; and (4)the presence or absence of completetracheal rings.

Until the 1980s, CTS was managedconservatively, with only a fewgroups reporting success withsurgical intervention.19,37,38 The pooroutcomes associated with earlyattempts at surgical repair led toa general consensus that CTS was not

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amenable to operative repair; instead,many clinicians employed palliativestrategies such as tracheostomy totreat CTS.26 There are some data tosupport the implementation ofa conservative management strategyin a subset of patients with CTS whohave been demonstrated to outgrowtheir tracheal stenosis by the age of7 to 9 years.2 However, it isimperative that careful long-termclinical monitoring is employed,given a significant number of thesepatients will eventually requiresurgical intervention to treatworsening symptoms.2,6,20 Recently,the use of endotracheal stents as theprimary treatment modality wasreported in 5 patients with CTS;however, only modest outcomeswere reported, with excessivegranulation tissue formation andrestenosis being major long-termcomplications.18 Nevertheless, theutility of adjunctive tracheal stenting

to manage residual stenosispostsurgical intervention has beenwidely demonstrated.5,13,33,39,40

Indications for surgery are primarilybased on functional status,20 wherebyany patient presenting withsignificant respiratory symptomsgenerally meets the criteria foroperative intervention. Anycombination of the followingsymptoms may be exhibited:persistent wheeze, dyspnea, repeatedrespiratory infections refractory tomedical therapy, failure to wean fromventilator support, or unsuccessfulventilator support. A pediatric patientcan typically tolerate up to 50%narrowing of the tracheal diameterbefore developing significantrespiratory symptoms20; therefore,overt respiratory symptoms are animportant indicator for surgery.Although some early studies basedtheir management approach solely on

the degree of tracheal stenosis,41–43

the contemporary surgical treatmentparadigm for CTS is fundamentallybased on the clinical status of eachindividual patient.

SURGICAL TECHNIQUES AND OUTCOMES

Cantrell and Guild19 reported the firstsuccessful surgical repair of CTS in1964 when they performed resectionof a bridging bronchus with side-to-side anastomosis. The use of trachealresection and primary anastomosiswas further explored by Carcassonneet al44 in 1973, and others into theearly1980s.31,45,46 However, thisprocedure was found to beunsuccessful in treating patients withlong-segment CTS. In response,Kimura et al8 implemented a trachealgraft technique to widen the airwaydiameter. Since then, numeroussurgical reconstructive techniqueshave been described for CTS(Table 1). Current surgical optionsinclude the following: (1) resectionand primary anastomosis; (2) patchtracheoplasty with nontrachealautologous tissue (costal cartilage,pericardium); (3) slide tracheoplasty;and (4) tracheal transplant withcadaveric tracheal homograft.47–49

Only the most frequently usedtechniques are described here.

Resection and Primary Anastomosis

Primary resection of the stenosedtracheal segment and primaryreconstruction by end-to-endanastomosis is a suitable operativechoice for patients with short-segment CTS (Cantrell type 3;Fig 3).40,44,50–52 Theoretically, thistechnique maintains a normaltracheal diameter; however,anastomotic tension may causesutures to cut through, leading tofibrosis, scarring, and the potentialfor recurrent stenosis. Nevertheless,a number of reported series havedemonstrated excellent outcomesusing tracheal resection in selectedpatients with short segment trachealstenosis.40,53,54 A global mortality

FIGURE 2Proposed diagnostic workup for a child presenting with symptoms suggestive of CTS. LPA, leftpulmonary artery.

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rate of less than 9% has beenreported for patients who haveundergone primary resection andanastomosis, demonstrating thefeasibility of this operative strategyfor patients with short segment CTS.

Patch Tracheoplasty

Rib Cartilage Tracheoplasty

In 1982, Kimura et al8 reported thefirst successful repair of long segmentCTS by using rib cartilagetracheoplasty in a 12-month-oldchild. Their technique consisted ofreconstruction of the anteriortracheal wall by using 2 pieces ofcostal cartilage and was subsequentlyperformed successfully in othercenters.55,56 Jaquiss et al57 reportedfavorable outcomes in their series of6 patients with CTS who underwentanterior tracheal reconstruction byusing rib cartilage as theaugmentation patch, reporting 1 caseof graft dehiscence, and no deaths at4.5 years follow-up. Despite a numberof small series reporting satisfactoryearly outcomes,58–60 several seriouscomplications were observed inpatients treated with this technique.Complications included anastomoticleakage, necrosis, granulation tissueformation, and recurrent stenosis atthe suture line. DeLorimier et al50

observed excessive granulation tissueformation when using rib cartilage

graft $30% of the airwaycircumference. Furthermore, ribcartilage tracheoplasty was shown tobe associated with high rates ofreintervention for residual trachealstenosis, and a high late mortalityrate secondary to airwaycomplications.61

Pericardial Patch Tracheoplasty

Idriss et al62 were the first to reportthe use of pericardial patchtracheoplasty in 5 patients with CTS.To perform this repair,cardiopulmonary bypass (CPB) isinitiated, then a vertical incision ismade into the stenosed trachealsegment, and a patch of harvestedautologous pericardium is sutured inplace. The final step is suspension ofthe patch to surrounding mediastinalstructures to prevent collapse intothe tracheal lumen. Advantages of thistechnique include the following:pliability of the pericardial patch,minimal dissection to expose theanterior tracheal surface, andestablishment of an airtight sutureline. Bando et al63 observed normaltracheal growth and epithelialdevelopment in the follow-up of 12patients with CTS who underwentpericardial patch tracheoplasty.However, a major drawback of thistechnique is the formation ofexcessive granulation tissue arising

from the devascularized surface ofthe pericardial patch. This frequentlyrequires multiple bronchoscopicdebridement procedures to removeexcessive granulation tissue andenable reepithelialization tooccur.62–66

To date, the most robust data on thepericardial patch tracheoplastytechnique is derived from 2 series.Firstly, in 2001, Backer et al40

reviewed their long-term outcomesby using pericardial patchtracheoplasty to treat CTS. In theirseries of 28 patients, the meanpostoperative length of stay was 60days and there were 3 (6%) earlydeaths. Seven (25%) patientsrequired reoperation or endoscopicstenting for residual tracheal stenosisand there were 5 (18%) late deaths.40

More recently, Fanous et al10

reported their long-term outcomesutilizing pericardial patchtracheoplasty in 26 patients with CTS.They reported 3 (11%) in-hospitaldeaths, whereas only 2 (9%) patientsrequired late airway reintervention.All other survivors remainedasymptomatic at a median follow-upof 11 years.

Slide Tracheoplasty

Tsang and Goldstraw17 first proposeda technique termed “slidetracheoplasty” in 1989. This

TABLE 1 Historical Outcomes of Surgical Techniques Used in Repair of CTS

Source No. of Patients Surgical Technique Age, mo Associated CardiovascularDefects (%)

Deaths (%) Survivors/Late AirwayReintervention

Length ofFollow-up, mo

Andrews et al 199477 15 Pericardial patch 7.7 5 (33) 7 (47) 8/5 19Bando et al63 12 Pericardial patch 6.7 8 (67) 1 (8) 10/2 66Kamata et al59 11 Costal cartilage patch 5.1 7 (64) 5 (45) 6/3 7Backer et al40 28 Pericardial patch 6.5 8 (29) 7 (25) 21/6 131

12 Tracheal autograft 4.5 2 (17) 1 (8) 11/4 318 Tracheal resection 4 0 0 8/0 392 Slide tracheoplasty 5 1 (50) 1 (50) 1/0 48

Grillo et al14 8 Slide tracheoplasty 84 5 (63) 0 8/1 70.63 Tracheal resection 6 1 (33) 0 3/1 54

Tsugawa et al61 12 Costal cartilage patch 6.9 7 (58) 4 (25) 8/3 14417 Slide tracheoplasty 10.5 15 (88) 4 (24) 13/6 25

Li et al16 14 Slide tracheoplasty 2.4 10 (71) 2 (14) 12/4 40Manning et al33 80 Slide tracheoplasty 8.7 24 (60) 4 (5) 76/23 12Antón-Pacheco et al 39 14 Slide tracheoplasty 8.7 9 (64) 0 14/3 75Chung et al 201478 18 Slide tracheoplasty 2.5 13 (72) 1 (6) 17/4 17Butler et al13 101 Slide Tracheoplasty 5.8 72 (71) 12 (11) 89/45 55

Inclusion criteria: $10 patients in a reported series.

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operation was initially performed in 2infants with funnel-shaped trachealstenosis; 1 died secondary topulmonary infections, the other hada patent airway at 9 years postrepair. A subsequent series of 4patients was reported by Grillo,30

who augmented the technique toestablish the contemporary slidetracheoplasty operation. In thisprocedure, the trachea is dividedhorizontally at the midpoint of thestenotic segment. The upper stenoticsegment is incised verticallyposteriorly, and the lower segment isincised anteriorly for the full lengthof the stenosis. The right-angledcorners produced by these divisionsare trimmed above and below, andthe 2 ends are then slid over eachother (Fig 4). The circumference ofthe stenosed segment is doubled,resulting in a quadrupled cross-sectional area.30 Further, the stenoticsegment is foreshortened only halfits length, thereby reducinganastomotic tension. A potentialpitfall is the risk of injuring vitalstructures (recurrent laryngealnerve, esophagus, pulmonaryvessels) due to the extensivedissection and reconstruction that isrequired.

Slide tracheoplasty has severaladvantages: (1) less risk ofendoluminal granuloma formation;blood supply is preserved and therepair utilizes native trachealcartilage lined with ciliatedepithelium. This enables shorterpostoperative intubation periods and

fewer bronchoscopicreinterventions13,33; (2) satisfactorytracheal growth post repair14,67; (3)the technique is not limited by lengthof tracheal stenosis68,69; (4) lowincidence of anastomotic problems,including leakage, mediastinitis,fibrosis, or recurrent stenosis13,14,33;and (5) a modified slide tracheoplastycan be successfully performed in alltypes of long-segment CTS, includingpatients with bronchial stenosis andtracheal bronchus anomalies.12,70

Numerous recent reportsdemonstrate slide tracheoplasty haslower mortality and postoperativeairway complication rates comparedwith other techniques.13,33 In a seriesof 80 patients, Manning et al33

reported 4 deaths (5%) and airwayreintervention in 23 (29%) patientsat 12 months follow-up. Butler et al13

performed slide tracheoplasty in 101consecutive patients; overall, 12(12%) deaths occurred, with 45(44%) patients requiring trachealballoon dilatation and 22 (21%)undergoing tracheal stenting postinitial repair.

CPB AND MANAGEMENT OFASSOCIATED CARDIOVASCULARANOMALIES

Contemporary surgical managementof CTS involves a median sternotomy

approach and the use of CPB.13,33 Forpatients undergoing isolated trachealsurgery, single venous cannulationand normothermic CPB is a favoredapproach.33 Associatedcardiovascular anomalies are mostcommonly repaired during the sameprocedure, typically before thetracheal repair. A frequentlyencountered concomitantcardiovascular anomaly is pulmonaryartery sling. Patients with thisanomaly usually undergo trachealrepair on CPB, followed bytransection of the left pulmonaryartery, relocation of the vesselanterior to the trachea, andreimplanted onto the mainpulmonary artery. For associatedintracardiac lesions, bicavalcannulation, moderate hypothermia,and cardioplegic arrest are surgicaltechniques that may be indicateddepending on the specific cardiacanomalies being repaired.13,33,40

POSTOPERATIVE CARE

The extreme heterogeneity andcomplexity of the CTS patientpopulation presents difficultmanagement challenges for clinicians.The standard of care for has evolvedto an integrated, multidisciplinaryteam-based approach.33,39

Multidisciplinary, team-based care

FIGURE 3Examples of short segment (A, distal trachea;B, proximal trachea) CTS treated by using theresection and primary anastomosis techniqueby using running suture.

FIGURE 4Slide tracheoplasty technique. A, Extent of stenosed trachea is identified. Stenotic segment is dividedtransversely in its midpoint. The upper stenotic segment is incised vertically posteriorly and thelower segment is incised anteriorly for the full length of stenosis. B, Right-angled corners producedby these divisions are trimmed above and below. C, The 2 ends are slid together after placement ofrunning sutures around the entire oblique circumference of the tracheoplasty. D and E, The trachealcircumference is doubled, resulting in quadrupled cross-sectional area.

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has significantly improvedperioperative outcomes in patientswith CTS, reducing the duration of

mechanical ventilation, and length ofICU and hospital stay.4 Severalcenters have evolved their

postoperative management to favorearlier extubation of patients withCTS. Many perform bedside fiberoptic

FIGURE 5Postoperative management protocol for CTS. CICU, cardiac intensive care unit; ORL, otolaryngology.

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bronchoscopy at 24 to 48 hourspostsurgery to facilitate earlier weanfrom mechanical ventilation.13,33

Currently, no established protocolexists for postoperative management.Herein is a multidisciplinarymanagement algorithm to help guidepostoperative care (Fig 5) of patientswith CTS. Major postoperative airwaycomplications include anastomoticbreakdown with subsequent air leak,tracheal narrowing secondary toexcessive granulation tissueformation or restenosis at the sutureline.

Post discharge, any child presentingwith symptoms of airway narrowingshould undergo evaluation withdirect laryngoscopy andbronchoscopy. If significant trachealrestenosis is present, rigidbronchoscopic balloon dilatation ofthe stenosed segment is performed.In children who develop excessivegranulation tissue at the anastomoticsite, local steroid injection has beensomewhat effective in reducingfurther granulation tissue andstenosis. The role of topicalapplication of mitomycin-C continuesto be evaluated.71,72 Moreover, recentstudies have demonstrated thatinhaled budesonide can also besuccessful in treating patients whodevelop excessive trachealgranulation tissue post CTSrepair.73,74

CONCLUSIONS

CTS is an important pediatricanomaly that often presents as a life-threatening emergency. Timely andeffective treatment of CTS is oftenchallenging due to the diversity ofclinical presentations, complexassociated anomalies, and rarity ofthe disorder. Optimal outcomes areachieved by managing patients withCTS in specialized centers that havea focused interest andmultidisciplinary expertise. Slidetracheoplasty is now recognized asthe procedure of choice, irrespectiveof airway anatomy and length of

tracheal stenosis, whereas segmentalresection and anastomosis is a viabletreatment option in the subset ofpatients with CTS with discreet,short-segment stenosis. Postoperativeairway stenosis is routinely managedby using endoscopic balloondilatation techniques. Trachealstenting may also be used as anadjuvant therapy, yet this strategycarries its own additional morbidityand is currently only used as salvageprocedure. Nevertheless, trachealstenting shows promise to becomea more feasible adjunctive therapy,with recent reports demonstratingnovel new technologies, such asbioabsorbable materials75 and 3-dimensional printer deriveddevices,76 can be used to designairway stents that are anatomicallyspecific for a given patient.

ABBREVIATIONS

CPB: cardiopulmonary bypassCTS: congenital tracheal stenosis

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