Surgical Reconstruction of Tracheal Stenosis inConjunction With Congenital Heart DefectsRichard D. Mainwaring, MD, Michael Shillingford, MD, Ryan Davies, MD,Peter Koltai, MD, Manchula Navaratnam, MBChB, V. Mohan Reddy, MD, andFrank L. Hanley, MD
Divisions of Pediatric Cardiac Surgery, Pediatric Otorhinolaryngology, and Pediatric Anesthesiology, Lucile Packard Children’sHospital, Stanford University School of Medicine, Stanford, California
Background. Surgical reconstruction is the primarymethod of treating airway obstruction in children. Tra-cheal stenosis is frequently associated with congenitalheart defects, which may further complicate the overallmanagement strategy. The purpose of this study was toreview our experience with surgical reconstruction ofairway obstruction in conjunction with congenital heartdefects.
Methods. This was a retrospective review of our surgi-cal experience with tracheal stenosis from February 2003to August 2011. Twenty-seven patients were identified inour database. Six patients had isolated, congenital tra-cheal stenosis, and 21 had tracheal stenosis in associationwith congenital heart defects. There were two identifi-able subgroups. Thirteen patients had airway stenosesidentified concurrently with congenital heart defects andunderwent combined repair. The second group com-
prised 8 patients who had previous correction of their
of Medicine, 300 Pasteur Dr, Falk CVRC, Stanford, CA 94305; e-mail:[email protected]
congenital heart defects and experienced delayed presen-tation of tracheal (n � 6) or bronchial (n � 2) obstruction.
Results. The median age at surgery was 9 months.There were 2 postoperative deaths, both in children withsingle ventricle. The median duration of follow-up forthe entire cohort of 25 surviving patients was 4 years.None of the patients have required reoperations on thetrachea; 5 have had minor reinterventions.
Conclusions. The data demonstrate that tracheal ob-struction is frequently found in conjunction with con-genital heart defects. Nearly one third of our patients haddelayed presentation of airway obstruction that wasidentified subsequent to previous congenital heart defectrepair. Tracheal reconstructive techniques were effectiveregardless of the cause of the airway obstruction.
Tracheal and bronchial stenoses in children manifestwith symptoms of inspiratory stridor and can cause
life-threatening respiratory events [1]. These events fre-quently result in hospitalization and in extreme casesrequire intubation and mechanical ventilation. Becauseairway obstruction is a mechanical issue, medical treat-ment is uniformly ineffective at alleviating symptoms orin preventing further life-threatening events [2]. This isevidenced by the fact that the natural history of patientswith airway obstruction who are managed medically isquite bleak [3]. Thus, surgical reconstruction is the pri-mary option for children with symptomatic airwayobstruction.
During the past 30 years, a variety of techniques hasbeen developed for reconstruction of the trachea. Thework of Grillo and associates [4] and Wright and col-leagues [5] indicated the feasibility of tracheal surgeryand pioneered many of the techniques that are currentlyused. Resection and end-to-end anastomosis is a versa-
Accepted for publication Dec 16, 2011.
Presented at the Fifty-eighth Annual Meeting of the Southern ThoracicSurgical Association, San Antonio, TX, Nov 9–12, 2011.
Address correspondence to Dr Mainwaring, Stanford University School
tile approach that is widely used in adult patients with acircumscribed length of involvement, whether of benignor malignant origin. However, many children with symp-toms of airway obstruction prove to have long-segmenttracheal stenosis, which precludes resection techniquesowoing to the extensive length of abnormal cartilaginousrings [6, 7]. An evolution of procedures has transpired toaddress the challenge of long-segment tracheal stenosisin children, and has been well summarized in the liter-ature [8–10]. These reports demonstrate the excellentresults that can now be achieved with tracheal recon-structive technique [11, 12].
Many children with long-segment tracheal stenosiswill also have associated congenital heart defects [13].These heart defects range from simple to complex and, asa consequence, have a wide-ranging impact on patientpresentation and outcomes. The association betweenpulmonary artery sling and complex, long-segment tra-cheal stenosis is well recognized [14–16], and surgicalmanagement under most circumstances addresses bothproblems concomitantly. Similarly, the strategy for pa-tients with tracheal stenosis in conjunction with relativelysimple congenital heart defects is straightforward, as it isevident that these procedures can be performed concom-
itantly without any additional risk [17]. However, the
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management of patients with complex congenital heartdefects in association with symptomatic airway obstruc-tion can be extremely challenging, as the combinationmay require a change or alteration in conventional strat-egy [18]. Our recent experience has demonstrated anincreasing number of patients with tracheal stenosis andcomplex congenital heart defects. The purpose of thismanuscript is to review this experience with surgicalreconstruction of tracheal stenosis in conjunction withcongenital heart defects.
Material and Methods
The institutional review board at Stanford Universityapproved this study. Medical and clinic records werereviewed, and a written questionnaire was sent to thefamilies of all children surviving tracheal reconstruction.Current follow-up was obtained in all patients throughthese mechanisms.
From February 2003 through August 2011, there were27 patients identified in our database who underwenttracheal or bronchial reconstruction. There were 14 boysand 13 girls. The median age at surgery was 9 months(range, 6 days to 9.1 years), and the median weight was 8kg (range, 2.3 to 18.7 kg). Ten of the 27 patients werehospitalized before surgery as a direct result of airway
Table 1. Summary of Diagnoses for the ConcomitantCongenital Heart Defects in 13 Patients
DiagnosisNo. of
Patients
Pulmonary artery sling n � 5Ventricular septal defect n � 3Tetralogy of Fallot n � 2Complete atrioventricular canal n � 1Pulmonary atresia, intact ventricular septum n � 1Double-outlet right ventricle, single ventricle n � 1
Table 2. Summary of the Diagnoses and PreviousOperations for the 8 Patients Who Experienced AirwayObstruction After Congenital Heart Surgery
Tetralogy of Fallot Tetralogy repair n � 2Truncus arteriosus Truncus repair n � 1Complete A-V canal/
coarctationComplete repair n � 1
Interrupted arch,ventricular septaldefect
Complete repair n � 1
Pulmonary artery sling Sling repair n � 1
A-V � atrioventricular.
symptoms related to airway obstruction. Five of these 10patients were ventilator dependent.
Six of the 27 patients had isolated, congenital trachealstenosis. Two of these 6 patients had short-segmentstenoses, limited to between two and four intrathoraciccartilaginous rings. The remaining 4 patients had long-segment tracheal stenosis affecting between 50% and100% of the intrathoracic trachea. One of these 6 patientswas hospitalized before surgery.
Twenty-one patients had airway obstruction in associ-ation with congenital heart defects. In 13 of these 21children, the diagnosis of airway obstruction was madeconcomitantly with the diagnosis of their heart defect. Asummary of these associated heart defects is shown inTable 1. Nine of these 13 patients were hospitalizedbefore surgery, and 4 were intubated as a result of
Fig 1. Artist’s illustration of long-segment tracheal stenosis. The af-fected length of trachea is composed of complete tracheal rings.These complete rings have limited growth potential in the neonateand infant.
respiratory events.
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There were 8 patients who did not have symptoms ofairway obstruction before heart surgery, and had noother indications of airway obstruction based on thepreoperative studies. These patients presented withsymptomatic airway obstruction from 6 to 18 monthsafter surgical correction of their congenital heart defect,and the diagnosis of tracheal or bronchial obstructionwas confirmed by bronchoscopy in all 8 and confirmedby computed tomographic scan in 5. Four of these 8patients had documentation of complete tracheal rings (3of 6 tracheal and 1 of 2 bronchial) based on their preop-erative evaluation. The diagnoses and previous surgicalrepairs for these 8 patients are listed in Table 2.
Twenty-seven patients underwent tracheal or bron-
Fig 2. Artist’s illustration of the sliding tracheoplasty technique. Thetrachea is transected obliquely from anterior and superior to poste-rior and inferior. Counterincisions are then made on the anteriorsurface of the superior segment of the trachea and the posterior sur-face of the inferior segment. The two segments of the trachea arethen advanced and overlapped.
chial reconstruction. Sliding tracheoplasty was the pre-
dominant surgical approach, accounting for 22 of the 27operations. The specific details of our surgical techniquefor sliding tracheoplasty are illustrated in Figures 1through 3. Figure 1 demonstrates the typical features oflong-segment tracheal stenosis. In Figure 2, the tracheahas been divided obliquely from anterior/inferior toposterior/superior. Counterincisions are then performedalong the posterior surface of the superior segment andthe anterior surface of the inferior segment of the tra-chea. Figure 3 shows the sliding tracheoplasty as the twosegments of trachea are interdigitated. In addition to the22 patients who underwent sliding tracheoplasty, 2 pa-tients had an isolated bronchial stenosis and underwenta “sliding bronchoplasty,” as depicted in Figure 4. The 2patients who had short-segment tracheal stenosis under-went resection and end-to-end anastomosis, and the finalpatient in the series had long-segment tracheal stenosisand had a rib autograft reconstruction performed. Asummary of the surgical techniques used for airwayreconstruction is shown in Table 3.
Cardiopulmonary bypass was used in 25 of the 27procedures. For these patients, the median cardiopulmo-nary bypass time was 115 minutes (range, 65 to 210minutes). Aortic cross-clamp was performed in 6 pa-
Fig 3. Artist’s illustration demonstrating the completion of the slid-ing tracheoplasty technique. The repair has been performed with 6-0polydioxanone suture. Care is taken to avoid both inversion andeversion of the tracheal edges. On completion, the lumen of the tra-cheal has effectively been doubled at the expense of shortening the
trachea by nearly one half.
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tients in whom intracardiac defects were repairedduring the same operation. The median aortic cross-clamp time was 45 minutes (range, 25 to 80 minutes).The two procedures performed without cardiopulmo-nary bypass support were the sliding bronchoplastyoperations, which were performed with single-lungventilation.
All 27 patients underwent preoperative, intraoperative,and immediate postoperative bronchoscopy performedby members of the pediatric otorhinolaryngology service.Six of the 27 patients in this series were noted to havean accessory (“pig”) bronchus to the right upper lobe.There were 2 patients who were discovered duringsurgery to have congenital tracheoesophageal fistulas.Both of these patients were in the group with congen-ital heart defects undergoing concomitant repair, andboth underwent repair of the tracheoesophageal fistulaas well. The patients underwent an additional bron-choscopic evaluation on the fifth postoperative day toassess the integrity of the tracheal reconstruction be-fore extubation.
Results
There were 2 postoperative mortalities in the entirecohort of 27 patients. Both of these deaths occurred in
Fig 4. Artist’s illustration of the “slidingbronchoplasty” technique shown (A) beforeand (B) after repair. The bronchial stenosis isrepaired by oblique transection and advance-ment, as described for the tracheoplastytechnique.
Table 3. Summary of the Surgical Techniques Used forTracheal and Bronchial Reconstruction
Surgical TechniqueNo. of
Patients
Isolated, congenital tracheal stenosisSliding tracheoplasty n � 3Resection and end-to-end anastomosis n � 2Rib graft reconstruction n � 1
Sliding tracheoplasty n � 13Tracheal or bronchial stenosis found after
repair of congenital heart defectsSliding tracheoplasty n � 6
Sliding bronchoplasty n � 2
neonates with tracheal stenosis in conjunction with afunctionally single ventricle. The first patient had pulmo-nary atresia with intact ventricular septum and coronarysinusoids. This patient underwent tracheal reconstruc-tion and a systemic-to-pulmonary artery shunt. The pa-tient had a prolonged intensive care course unrelated toairway obstruction, but eventually exhibited sepsis anddied 58 days after surgery. The second patient was bornwith tracheal stenosis and double-outlet right ventriclewith a functionally single ventricle; this patient wasventilator dependent and had gram-negative sepsis pre-operatively. The patient underwent a combined trachealreconstruction and systemic-to-pulmonary artery shunt,but exhibited recurrent sepsis and died 47 dayspostoperatively.
The median duration of time from surgery to extu-bation was 6 days (range, 6 hours to 13 days). Themajority of patients were intentionally maintained onthe ventilator for a 5-day period as part of a protocol toallow the tracheal reconstruction to begin the healingprocess. Patients received narcotics, sedation, and onoccasion paralytics to facilitate this plan. The causes formaintaining patients on the ventilator longer than thisprescribed time were related to hemodynamic instabil-ity or the development of complications in the interim5 days.
The median duration of hospitalization for the 25survivors was 24 days (range, 5 to 66 days). There were noapparent differences in the duration of hospitalizationbased on patient subgroup. Specifically, the medianduration of hospital stay for patients with isolated con-genital tracheal stenosis was 21 days, for patients whounderwent combined tracheal reconstruction and repairof congenital heart defects was 26 days, and trachealsurgery after repair of congenital heart defects was 19days.
There were four major and six minor complications inthe 27 patients undergoing tracheal surgery. These com-plications are listed in Table 4. None of the patientsrequired reoperation on the intrathoracic trachea. Threepatients experienced sepsis, and 2 of these 3 patientsdied, accounting for both of the mortalities in this series.
The other major complication was a patient who exhib-
5Ann Thorac Surg MAINWARING ET AL2012;xx:xxx TRACHEAL RECONSTRUCTION
ited heart block after repair of tetralogy of Fallot. Therewas 1 patient who required tracheostomy for upperairway obstruction who has subsequently had the tra-cheal cannula removed.
The 25 survivors have been followed for a medianduration of 4.2 years (range, 3 months to 8.5 years). Therehave been no late deaths in this cohort of patients, and noone has required reoperation directly on the trachea orbronchi. Eighteen of the 25 patients have undergone alate follow-up bronchoscopy at a median of 12 months(range, 4 to 28 months) after tracheal surgery. Fivepatients have required minor reinterventions. Four pa-tients had treatment for granulation tissue, and 1 patientunderwent an aortopexy procedure. Two patients havesubsequently undergone conduit replacements sincetheir tracheal surgery.
Comment
This retrospective review was performed to evaluate theshort-term and midterm efficacy of surgical reconstruc-tion for children with airway obstruction. It was ourimpression that an increasing number of patients withairway disease were presenting with associated complexcongenital heart defects. This may be a reflection of thecongenital cardiac practice at our institution, which is areferral center for several complex congenital cardiacdefects. Our review identified 27 patients with tracheal orbronchial obstruction, of whom only 6 had isolatedairway disease. Twenty-one patients had associatedcongenital hearts defects; 13 were identified and re-paired concomitantly, and 8 were identified subse-quent to repair of their heart defects. The surgicalresults demonstrate a mortality rate of 7%, with bothdeaths occurring in patients with tracheal obstructionin conjunction with complex single ventricle. Many ofthe patients in this series have midterm follow-up, andnone of the patients have required major reinterven-tions on the trachea or bronchi. These results wouldindicate that surgical reconstructive techniques pro-vide a durable repair for airway obstruction in the
Table 4. Postsurgical Complications After Tracheal orBronchial Reconstruction
Postsurgical ComplicationsNo. of
Patients
MajorSepsis n � 3Heart block n � 1
MinorSuperficial wound infection n � 2Prolonged pleural effusions n � 2Tracheostomy
(subsequently decannulated)n � 1
Tracheocutaneous fistula(patient had tracheostomypreoperatively)
n � 1
pediatric patient.
Several recent reports would suggest that pediatriccardiac surgery centers are identifying and treating morepatients with the combination of tracheal stenosis andcongenital heart disease. Loukanov and colleagues [13]summarized their experience with 9 patients who un-derwent simultaneous management of tracheal steno-sis and cardiac anomalies in infants. Four of thesepatients had pulmonary artery sling, whereas 5 had adiverse mixture of other heart defects and arch vesselanomalies. Manning and associates [17] reported theirseries of 80 patients who underwent slide tracheo-plasty. In this cohort, 48 patients (60%) had additionalcardiac or great vessel anomalies. Twenty-four of thesepatients had a pulmonary artery sling (17 treatedconcomitantly and 7 treated subsequent to left pulmo-nary artery repair). Other frequently encountered con-genital heart defects included tetralogy of Fallot in 6,ventricular septal defect in 6, atrial septal defect in 5,and double-outlet right ventricle in 2. These diagnosesare comparable to our experience with tracheal steno-sis and congenital heart defects.
We have used the phrase delayed presentation to de-scribe the clinical situation of patients who underwentcongenital heart defect repairs and subsequently pre-sented with symptomatic airway obstruction. Some of thepatients included under this heading had a congenitalcause for their airway problems, as evidenced by thefinding of complete tracheal rings on bronchoscopy andat surgery. This was the case in 4 of the 8 patients in ourseries. Children with congenital complete trachealrings can be asymptomatic from a clinical standpoint,and under these circumstances are usually managedconservatively. However, these 4 patients clearly un-derwent a change in their tracheal physiology subse-quent to their congenital heart repair. We would hy-pothesize that one component of this conversion fromasymptomatic to symptomatic status could be attribut-able to manipulation of a marginal airway. These“internal” factors might be attributable to prolongedintubation, mechanical trauma from suctioning, anddamage from infection (Table 5).
It is conceivable that other factors may contribute tothe development of symptomatic tracheal disease, as 4 ofthe 8 patients in the delayed presentation group did nothave congenital complete tracheal rings. All of thesepatients underwent major rearrangement of the great
Table 5. Potential Causes for Delayed Presentation ofSymptomatic Tracheal Stenosis
Internal factorsProlonged intubationTrauma from suctioningInfection
6 MAINWARING ET AL Ann Thorac SurgTRACHEAL RECONSTRUCTION 2012;xx:xxx
vessels within the mediastinum, and this could result inairway compression as a result of the close juxtapositionof great vessels and the airway. It is possible that scartissue could develop around the airway after mobiliza-tion and rearrangement of the great vessels. Anotherplausible cause would be compromise to the blood sup-ply of the trachea or bronchus, which is possible inpatients with pulmonary atresia and major aortopulmo-nary collaterals in whom collaterals are mobilized off thesurface of the airway. Finally, it is interesting to note that5 of the 8 patients in the acquired group had conotruncalcardiac defects, and thus there is the possibility thatgenetic factors may play a predisposing role in thedevelopment of symptomatic airway disease. These “ex-ternal” factors need not be mutually exclusive, and eachmay have an incremental effect on the airway. Theinterval between the primary cardiac procedure andtracheal repair was 6 to 18 months, indicating that theprocess of developing symptomatic airway disease wasgradual. This observation would support the role forthese external factors.
Manning and coworkers [17] reported that 15 of the 80patients in their series had what they categorized as“acquired tracheal stenosis.” The majority of these cases(13 of 15) were attributed to previous tracheoplasty pro-cedures performed at other institutions. These samepatients had undergone 16 cardiac or great vessel proce-dures. It is unclear from their description whether any ofthe patients could have had a contribution from theirprevious cardiac procedures to the development of ac-quired tracheal stenosis. There were also 2 patients whoexhibited acquired tracheal stenosis after cardiac or greatvessel operations without tracheal surgery. Manning andcolleagues attributed this to “trauma from prolongedintubation or severe tracheal infection with associatedcartilage loss or fibrosis,” because the patients did nothave congenital tracheal rings. These 2 patients appear tobe similar to the 4 patients in our series who exhibitedacquired tracheal stenosis in the absence of completerings.
It is evident that there exists an integral relationshipbetween congenital heart disease and congenital tra-cheal stenosis. This observation should raise our indexof suspicion in searching for this combination of de-fects in a more proactive fashion. It is conceivable thatpatients at high risk (eg, conotruncal abnormalities)could be more actively screened to search for congen-ital tracheal abnormalities. Those patients who areasymptomatic but identified with complete trachealrings could benefit from this knowledge through spe-cial attention to factors such as endotracheal tube size,duration of intubation, and the anticipation for diffi-culties after extubation. These maneuvers potentiallymight reduce the need for surgery in the delayedpresentation category of tracheal stenosis.
In summary, tracheal obstruction in children is fre-quently found in conjunction with complex forms ofcongenital heart disease. The patients with combinedtracheal stenosis and congenital heart defects were di-
vided between those identified concomitantly and those
with delayed presentation. The surgical results of tra-cheal reconstruction were successful regardless ofwhether this was for isolated tracheal stenosis, in con-junction with congenital heart defects identified concom-itantly, or performed in those who underwent congenitalheart repair and were identified late. We would proposethat a more proactive approach to identification of con-genital tracheal abnormalities may be warranted in somegroups of patients at higher risk for the combination ofcongenital heart and tracheal disease.
Illustrations were created by Erin Anne Mainwaring.
References
1. Elliott M, Roebuck D, Noctor C, et al. The management ofcongenital tracheal stenosis. Int J Pediatr Otorhinolaryngol2003;67(Suppl 1):S183–92.
2. Janik JS, Nagari HS, Yacoub U, Groff DB. Congenitalfunnel-shaped tracheal stenosis: an asymptomatic lethalanomaly of early infancy. J Thorac Cardiovasc Surg 1982;83:761– 6.
3. Benjamin B, Pitkin J, Cohen D. Congenital tracheal stenosis.Ann Otol Rhinol Laryngol 1982;90(4 Pt 1):364–71.
4. Grillo HC, Wright CD, Vlahakes GJ, MacGillivary TE. Man-agement of congenital tracheal stenosis by means of slidetracheoplasty or resection and reconstruction, with long-term follow-up of growth after slide tracheoplasty. J ThoracCardiovasc Surg 2002;123:145–52.
5. Wright CD, Graham BB, Grillo HC, Wain JC, Mathisen DJ.Pediatric tracheal surgery. Ann Thorac Surg 2002;74:308 –14.
6. Kim HK, Kim YT, Sung SW, et al. Management of con-genital tracheal stenosis. Eur J Cardiothorac Surg 2004;25:1065–71.
7. Backer CL, Mavroudis C, Gerber ME, Holinger LD. Trachealsurgery in children: an 18-year review of four techniques.Eur J Cardiothorac Surg 2001;19:777–84.
8. Backer CL, Holinger LD. A history of pediatric trachealsurgery. World J Pediatr Congenit Heart Surg 2010;1:344 – 63.
9. Hazekamp MG, Koolbergen DR, Kersten J, Peper J, de MolB, König-Jung A. Pediatric tracheal reconstruction with peri-cardial patch and strips of autologous cartilage. Eur J Car-diothorac Surg 2009;36:344–51.
10. Antón-Pacheco JL, Cano I, Comas J, et al. Management ofcongenital tracheal stenosis in infancy. Eur J CardiothoracicSurg 2006;29:991–6.
11. Manning PB, Rutter MJ, Border WL. Slide tracheoplastyin infants and children: risk factors for prolonged postop-erative ventilatory support. Ann Thorac Surg 2008;85:1187–92.
12. Li X, Cheng L-C, Cheung Y-F, Lun K-S, Chau K-T, ChiuS-W. Management of symptomatic congenital tracheal ste-nosis in neonates and infants by slide tracheoplasty: a 7-yearsingle institution experience. Eur J Cardiothorac Surg 2010;38:609–14.
17. Manning PB, Rutter MJ, Lisec A, Gupta R, Marino BS. One
slide fits all: the versatility of slide tracheoplasty with car-
additional diagnostic evaluation should be pursued? Thank you.
diopulmonary bypass support for airway reconstruction inchildren. J Thorac Cardiovasc Surg 2011;141:155–61.
18. Chiu PPL, Kim PCW. Prognostic factors in the surgical
treatment of congenital tracheal stenosis: a multicenter anal-ysis of the literature. J Pediatr Surg 2006;41:221–5.
DISCUSSION
DR PETER B. MANNING (Cincinnati, OH): Thanks to theprogram committee for the invitation to discuss this, and thankyou to the authors for sending the manuscript in advance.
As the authors have pointed out, tracheal reconstruction is acomplex problem to tackle by itself, but the fact that additionalcongenital cardiac and great vessel anomalies are so frequentlyseen in this group of children only adds to the managementchallenge. I congratulate the authors on their excellent results inthis frequently difficult group of patients.
This paper emphasizes the frequent association of cardiacanomalies with tracheal stenosis which in their series at 78%may have been elevated due to referral patterns, as was men-tioned in their manuscript, but really isn’t too far off from the62% incidence we have seen in Cincinnati, and our referralpattern is probably biased in the other direction, with far morepatients being referred for airway problems primarily.
I agree with the authors that the slide tracheoplasty techniqueis the superior choice for airway reconstruction in children. It isan extremely versatile method which can be applied to virtuallyany length anomaly; because it is a repair using all viable tissue,it has a better record for healing, it also has growth potential,and because it is structurally sound due to the native cartilagesupport of all tissue used in the reconstruction, it allows for earlyextubation in the majority of patients. I noted in the manuscriptthat you followed the protocol of the mandatory 5 days ofmechanical ventilatory support. We felt that there is moreadvantage in early extubation to minimize the trauma to theairway by plastic and positive-pressure ventilation. We strive toextubate the day following operation in most of our cases. Ourexperience now totals 112 cases with an early extubation rate of60%. As your experience with problems will decrease, have youmodified that program in terms of maintaining long intubation?
I noticed that there were 2 patients in your series where therewas bronchial stenosis and both were in the acquired subgroup,and I also noticed that there were 2 patients in this group whohad undergone arch reconstructions as part of their early cardiacrepair. Were these the same 2 patients? It is well described, asyou know, that arch reconstruction, particularly with an openarch, may result in left hilar compression affecting the airway orthe pulmonary artery. If these were the same patients with thebronchial stenosis, I am surprised that bronchoplasty was suc-cessful in managing what I would have expected to be acompression problem. Some have described a posterior aor-topexy to manage this problem. Could you clarify that?
I agree completely with your conclusion that the frequentassociation of cardiac anomalies with airway stenosis in childrenshould raise our index of suspicion for searching for thiscombination proactively. For patients with associated cardiacdefects, which problem influenced presentation more, the car-diac or the airway problem?
While it is easy to screen the relatively small number ofpatients whose airway anomaly is identified first for cardiacdefects, it is a bit more challenging the other way around. Haveyou identified a population of cardiac patients who you wouldclassify at risk for an associated airway problem, and what
DR MAINWARING: Thank you for those comments. I will try torun through them in order to cover all of the questions.
Your first question is an excellent one regarding how long tokeep these patients on the ventilator. We have had a protocol tokeep patients intentionally asleep for 5 days. Obviously, thepotential risk of this strategy is going to be manifested asinfectious complications. When we look at our own data, 12% ofthe patients developed sepsis, but for those who had thiscomplication, the mortality rate was 67%. When you see that, itgives you reason to rethink what you are doing. We have beenwatching your lead on this, and are aware that you have reducedthe median duration of time on the ventilator down to 2 days,while ours is 6. So I think we will be reconsidering our positionon this, and probably follow your lead on this issue in the future.
We had 2 patients who had bronchial obstruction, and forthese patients we adapted the technique of slide tracheoplastyonly we performed a slide bronchoplasty. Both of these had leftmainstem bronchus stenosis. The 1 patient was status postuni-focalization, and the other had undergone an arch reconstruc-tion. This group of patients who had delayed presentation oftracheal stenosis is what caught our attention, as these patientspresented 6 to 18 months after their heart repairs.
Your next question was in regards to the potential causes ofacquired tracheal stenosis. In your papers you have previouslyalluded to what we have called internal factors, which wouldinclude prolonged intubation, trauma from suctioning, andinfection. We have also conceived of external factors playing arole, and these would include great vessel compression and scartissue following arch reconstructions and unifocalization proce-dures, which then traps the trachea and prevents growth.Devascularization is another potential etiology, as the MAPCAs[major aortopulmonary collateral arteries] are peeled right offthe surface of the trachea. Another potential etiology of acquiredtracheal stenosis could be genetic factors, as 5 of our 8 patientshad conotruncal abnormalities.
Your final question was whether we have identified a groupwho we would classify to be at risk for an associated airwayproblem. In our series of 27 patients, there were 23 who hadcomplete tracheal rings, of whom 4 presented late. There werealso 4 patients who had a normal membranous portion to theirtracheobronchial tree. The 4 patients who had complete trachealrings but were asymptomatic presumably had a marginal air-way, and if you knew that up front, you could be a little bit morecareful about the size of the endotracheal tube and the length oftime on the ventilator, and maybe you could prevent thosepatients from needing surgery.
DR MANNING: I agree. It’s that group of kids that really aren’tacquired, but are actually late diagnosed, and you kind of kickyourself when you find out you can’t get the kid extubated afterthe tet[ralogy of Fallot] repair and you realize, we missed thetracheal stenosis. And it’s tough to figure out which kids reallyneed to be screened. Obviously the LPA [left pulmonary artery]slings, even though some of the literature says there’s only a 50%incidence of tracheal stenosis with LPA sling, I think that’s
ridiculous. It’s close to 100%. And anybody with a sling is going
8 MAINWARING ET AL Ann Thorac SurgTRACHEAL RECONSTRUCTION 2012;xx:xxx
to be bronch[oscopi]ed first because they all have to be assumedto have stenosis. But then, you have suggested the conotruncaldefects. Twenty-five percent of our kids have LSVCs [left supe-rior vena cavas]. I don’t know why, and that might be a group inwhom we should have a higher index of suspicion to pick it upearly. I think the acquired is acquired, and as you have pointedout in cases such as unifocalization that this may be related tothe initial operation. But the missed diagnoses are the ones thatyou really want to pick up before you do the cardiac repair. Youwould ideally like to do it simultaneously.
DR MAINWARING: We agree with that. One of our patients inthe delayed diagnosis group had an LPA sling, and initially onlyhad the LPA addressed and not the trachea. So this echoes whatyou just said. Aside from the pulmonary slings, I think it is the
conotruncal abnormalities that are particularly at risk.
DR JORGE D. SALAZAR (Jackson, MS): A comment—in pa-tients referred for surgery, we find that some have symptomsout of proportion to their echo[cardiographic] findings. As aresult, we have lowered the threshold to do CT [computedtomographic] scans preoperatively, and we have found a num-ber of concomitant airway problems. This experience haschanged our management in terms of how we time the opera-tion, and in some of the children we have been able to avoidoperating on the airway by being a little more sensitive toendotracheal tube management.
DR MAINWARING: I have put up the slide demonstrating howwe performed the slide bronchoplasty on the left mainstembronchus. We did these two operations without the use ofcardiopulmonary bypass. We intubated the right mainstem
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