Long-segment Congenital Tracheal Stenosis Research Articles

Composite Biosynthetic Graft for Repair of Long-Segment Tracheal Stenosis: A Pilot In Vivo and In Vitro Feasibility Study.

Pediatric patients who undergo surgery for long-segment congenital tracheal stenosis (LSCTS) have suboptimal outcomes and postsurgical complications. To address this, we propose a biosynthetic graft comprising (1) a porcine small intestinal submucosa extracellular matrix (SIS-ECM) patch for tracheal repair, and (2) a resorbable polymeric exostent for biomechanical support. The SIS-ECM patch was evaluated in vivo in an ovine trachea model over an 8 month period. Concurrently, the biosynthetic graft was evaluated in a benchtop lamb trachea model for biomechanical stability. In vivo results show that SIS-ECM performs better than bovine pericardium (control) by preventing granulation tissue/restenosis, restoring tracheal architecture, blood vessels, matrix components, pseudostratified columnar and stratified epithelium, ciliary structures, mucin production, and goblet cells. In vitro tests show that the biosynthetic graft can provide the desired axial and flexural stability, and biomechanical function approaching that of native trachea. These results encourage future studies to evaluate safety and efficacy, including biomechanics and collapse risk, biodegradation, and in vivo response enabling a stable long-term tracheal repair option for pediatric patients with LSCTS and other tracheal defects.

Treatment of long-segment congenital tracheal stenosis with congenital cardiovascular defects in infancy.

This study aims to evaluate the outcomes of simultaneous repair for infants with long-segment congenital tracheal stenosis (LSCTS) with congenital cardiovascular defects (CCD). We retrospectively reviewed the clinical data of infants aged less than 1 year with LSCTS and CCD who underwent simultaneous repair at Children's Hospital of Chongqing Medical University from January 2020 to March 2023. A systematic search of PubMed, Embase, and Cochrane Library for the relevant published studies that reported the simultaneous repair of CTS and CCD in infancy was conducted in March 2023. The inverse variance method of DerSimonian-Laird (D+L) was used for estimate synthesis. A total of thirteen infants with a mean age of 5.6±3.1 months and a mean weight of 6.4±0.9Kg underwent slide tracheoplasty with modified procedures and cardiovascular operations. LSCTS was diagnosed in all thirteen patients. Nine infants were ventilator dependent, and four patients were operated on due to persistent wheezing and recurrent respiratory infections. Seven patients underwent pulmonary artery sling repair, and six underwent atrial septal defect repair. All infants were repaired utilizing cardiopulmonary bypass (CPB) support. Significant complications were recorded in three patients. In-hospital deaths were seen in one case. The median tracheal minimum diameter of hospital survivors was significantly larger than the preoperative minimum diameter (p<0.001). The mean follow-up duration was 17.1±7.1 months. There was no late mortality during the follow-up. Twelve studies were included based on our search strategy. The pooled estimate of mortality in the literature was 10.9% (95%CI, 5.3%-17.7%, I2=0). The pooled estimate of airway re-interventions was 28.8% (95%CI, 14.5%-43.2%, I2=74%). Simultaneous repair of LSCTS and CCD in infancy is safe and effective. Slide tracheoplasty with appropriate technical modifications may be valid for LSCTS repair without significant restenosis and reinterventions.

A case of successful slide tracheoplasty for long-segment congenital tracheal stenosis in a neonate with a congenital diaphragmatic hernia and Fallot\u2019s tetralogy

BackgroundCongenital tracheal stenosis (CTS) is a rare and life-threatening airway disorder, which is often associated with cardiac malformations. Among them, neonatal symptomatic CTS with cardiac malformations has an extremely poor prognosis. In contrast to cardiac malformation, congenital diaphragmatic hernia (CDH) has rarely been associated with CTS. We report a neonatal case in which slide tracheoplasty and intracardiac repair were performed simultaneously for CTS and Fallot’s tetralogy (TOF).Case presentationAn infant with left CDH and Fallot's tetralogy (TOF) was born by cesarean section at 38 weeks of gestation. At the time of resuscitation, a 2.5 mm (ID) endotracheal tube could only be inserted just below the vocal cords. After repairing the CDH at 3 days of age, planned extubation was performed at 7 days of age. However, the patient required re-intubation due to life-threatening episodes after 2 days of the extubation. Enhanced CT revealed a long segment CTS from the upper trachea to the right bronchus (length of stenosis: 40 mm, minimum inner diameter: 2 mm). At 24 days of age, veno-arterial extracorporeal membrane oxygenation (ECMO) was introduced due to severe respiratory failure. At 28 days of age, slide tracheoplasty and palliative right ventricular outflow tract reconstruction (RVOTR) was performed with cardiopulmonary bypass (CPB). After tracheoplasty, a 3.5 mm tracheal (ID) tube could be placed in the reconstructed trachea in a patient with CTS. ECMO was completed 7 days after the operation. On the 17th day after the operation, he was extubated successfully. He was discharged 5 months after birth with home oxygenation therapy.ConclusionsWe reported the successful simultaneous correction of slide tracheoplasty and palliative RVOTR for a neonate with CDH. ECMO was used for respiratory management before and after surgery.

Surgical management of long-segment congenital tracheal stenosis with tracheobronchial malacia.

Slide tracheoplasty has become the mainstream treatment for long-segment congenital tracheal stenosis (LSCTS). However, technical improvements are still needed to improve the clinical outcomes of patients exhibiting LSCTS with tracheobronchial malacia. LSCTS patients who underwent tracheoplasty from January 2010 to December 2020 were reviewed. According to the time of surgical technique modifications for reconstructing a supportive carina, the patients were divided into 2 groups: group A (2010-2018) and group B (2019-2020). We identified a well-balanced cohort matched by propensity score to evaluate the differences in surgical outcomes between the 2 groups. There were no significant differences between group A and group B in any of the 8 characteristics before and after propensity score matching. In the propensity score-matched cohort, the number of patients who accepted anterior carina tracheopexy (75/77 vs 4/77, P < 0.001) and in situ pericardium insertion (75/77 vs 14/77, P < 0.001) in group B was significantly greater than that in group A. The mechanical ventilation time [48.3 (interquartile range: 29.6, 116.3) h vs 73.3 (interquartile range: 47.9, 111.6) h, P = 0.009] and cumulative mortality (P = 0.023) were significantly lower in Group B than Group A. Reconstructing a supportive, stable carina of the neotrachea and tracheobronchopexy are helpful to improve the outcomes of slide tracheoplasty.

Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided

BackgroundPulmonary artery sling (PAS) is usually associated with long-segment congenital tracheal stenosis (LSCTS). This combination of abnormalities can also be associated with lung hypoplasia abnormalities (hypoplasia, aplasia, or agenesis). This study analyzed the association of lung hypoplasia abnormalities with combined PAS and LSCTS and its influence on its surgical outcomes. MethodsAll patients (0 to 18 years) who underwent surgical procedures for both PAS and LSCTS from 1995 to 2019 were analyzed retrospectively for mortality, ventilation days, and intensive care unit days by dividing them into those with normal lungs (group 1) and hypoplastic lungs (group 2). ResultsIncluded were 75 patients (30 girls [40%]), who were a median age of 5.7 months (interquartile range [IQR], 2.9-13.3 months), median weight of 5.5 kg (IQR, 4.1-7.9 kg), and had a median follow-up of 99.8 months (IQR, 54.5-152.0 months); of these, 8 patients (10.7%) had hypoplastic right lung, comprising hypoplasia in 7 (87.5%), aplasia in 1 (12.5%), and agenesis in 0 (0%). There was a significant difference in mortality (group 1, 9.0%; group 2, 50%; P = .007) but no significant difference in median ventilation days (group 1, 9.0; group 2, 9.0; P = .89) or in median intensive care unit days (group 1, 14.0; group 2, 11.5; P = .44). ConclusionsLung hypoplasia associated with PAS and LSCTS is usually right-sided. As a result of severe airway obstruction and single-lung physiology, there is a high requirement of preoperative cardiorespiratory support and a significant association with adverse surgical outcomes.

Improving community-based care for children with a rare condition: The example of long-segment congenital tracheal stenosis and perceptions of health professionals, parents and teachers

BackgroundLong segment congenital tracheal stenosis (LSCTS) is a rare, complex condition which is often poorly understood by community-based health professionals (HPs). Anecdotally, such HPs often lack confidence providing care for children, resulting in children being brought to the tertiary centre more frequently than necessary. We wanted to identify the information and support needs of HPs in primary and secondary care looking after a child with LSCTS, the views of those providing education to these children, and elicit parents’ perceptions about community-based services, to improve overall care for children and families. MethodQuestionnaires were sent to 175 community-based HPs and 34 teachers involved in the care of children with LSCTS. Face-to-face or telephone interviews were conducted with 12 parents of patients with LSCTS to establish their perceptions of community-based services. ResultsNinety (51%) completed questionnaires were returned from HPs and 18 (53%) from teachers. Responses indicated low levels of knowledge of LSCTS. Physical, practical and communication information needs were identified by all respondent groups, together with general and condition-specific concerns. Interviews with parents indicated that they thought the level of knowledge about LSCTS in the community was low, which had a negative impact on their willingness to consult local services. Better information provision for HPs, teachers and parents was identified as a means of improving outcomes. ConclusionProfessionals caring for children with LSCTS in the community and in schools have unmet information and support needs, with consequences for children, families, HCPs in both the community and tertiary hospital, and teachers.

Excessive Tracheal Length in Patients With Congenital Tracheal Stenosis

Slide tracheoplasty is the preferred approach for treating long-segment congenital tracheal stenosis (CTS). However, little research has been conducted on the tracheobronchial anatomy before and after slide tracheoplasties in patients with CTS. We reviewed 23 patients with CTS who received slide tracheoplasties. We measured the intrathoracic tracheal length and the carina angle from computed tomography images. To account for each patient's body size, we divided the intrathoracic tracheal length by the length of the thorax to obtain the trachea-thorax ratio (TTR). These measurements were used to compare patients before and after slide tracheoplasties as well as normal control subjects. Two patients had upper tracheal CTS and 21 patients had lower tracheal CTS. For the 21 patients with lower tracheal stenosis, their TTRs before slide tracheoplasty were 0.42 ± 0.04, which were significantly larger than those of the control subjects (0.32 ± 0.04; p < 0.0001). After slide tracheoplasty, the TTR was 0.32 ± 0.04, similar to the control TTRs (p= 0.94). The carina angle was significantly wider in the 21 patients than in the control subjects (120.7 ± 11.7 degrees versus 86.4 ± 13.1 degrees; p< 0.0001). After slide tracheoplasty, the carina angle wassignificantly narrower (from 120.7 ± 11.7 degrees to 92.2 ± 15.2 degrees; p < 0.0001), which was similar to control subjects. The trachea was longer and the carina angle wider in patients with lower tracheal CTS than incontrol subjects. Excessive tracheal length is favorable for slide tracheoplasty. Slide tracheoplasty not only correctsCTS, but also restores tracheobronchial morphology.

Rapid Expansion of Human Epithelial Stem Cells Suitable for Airway Tissue Engineering

Stem cell-based tracheal replacement represents an emerging therapeutic option for patients with otherwise untreatable airway diseases including long-segment congenital tracheal stenosis and upper airway tumors. Clinical experience demonstrates that restoration of mucociliary clearance in the lungs after transplantation of tissue-engineered grafts is critical, with preclinical studies showing that seeding scaffolds with autologous mucosa improves regeneration. High epithelial cell-seeding densities are required in regenerative medicine, and existing techniques are inadequate to achieve coverage of clinically suitable grafts. To define a scalable cell culture system to deliver airway epithelium to clinical grafts. Human respiratory epithelial cells derived from endobronchial biopsies were cultured using a combination of mitotically inactivated fibroblasts and Rho-associated protein kinase (ROCK) inhibition using Y-27632 (3T3+Y). Cells were analyzed by immunofluorescence, quantitative polymerase chain reaction, and flow cytometry to assess airway stem cell marker expression. Karyotyping and multiplex ligation-dependent probe amplification were performed to assess cell safety. Differentiation capacity was tested in three-dimensional tracheospheres, organotypic cultures, air-liquid interface cultures, and an in vivo tracheal xenograft model. Ciliary function was assessed in air-liquid interface cultures. 3T3-J2 feeder cells and ROCK inhibition allowed rapid expansion of airway basal cells. These cells were capable of multipotent differentiation in vitro, generating both ciliated and goblet cell lineages. Cilia were functional with normal beat frequency and pattern. Cultured cells repopulated tracheal scaffolds in a heterotopic transplantation xenograft model. Our method generates large numbers of functional airway basal epithelial cells with the efficiency demanded by clinical transplantation, suggesting its suitability for use in tracheal reconstruction.

Surgical management of congenital tracheal stenosis associated with tracheal bronchus and congenital heart disease.

Congenital tracheal stenosis (CTS) is variable in patients with tracheal bronchus and congenital heart disease (CHD). Tracheoplasty remains a high-risk surgical treatment. From January 2007 to December 2014, 24 CTS patients (10 males and 14 females; age 20.6 ± 13.6 months) with tracheal bronchus and CHD underwent one-stage surgical correction. Clinical features of all patients included dyspnoea, or recurrent pulmonary infections. There was long-segment CTS in 13 cases (54%), and 4 cases were associated with a bridging bronchus. Less than 50% of normal tracheal size was identified in 21 cases. Complete tracheal or bronchial rings were identified in all cases. Operative techniques included: tracheal end-to-end anastomosis in 11 cases and slide tracheoplasty in 13 cases, which included 11 cases of right upper lobe bronchus (RULB) opposite side-slide tracheoplasty. There were 2 operative deaths, due to postoperative tracheomalacia or residual main bronchial stenosis. The duration of postoperative hospital stay was 7-59 days, with an average of 19 days. Follow-up was completed in 19 cases. The follow-up duration was from 1 month to 7 years. Tracheal granulation occurred in 1 case. Clinical symptoms were significantly improved in the remaining patients. Tracheal bronchi have special anatomical features. The techniques of tracheal end-to-end anastomosis or side-slide tracheoplasty can be used to correct tracheal stenosis with satisfactory outcomes.

Clinical outcomes of slide tracheoplasty in congenital tracheal stenosis.

Treatment of long-segment congenital tracheal stenosis (CTS) remains challenging. Recently, slide tracheoplasty has become the standard approach in many centres. The aim of this study was to evaluate the clinical outcomes of slide tracheoplasty. Between 2004 and 2011, 18 patients underwent slide tracheoplasty in our centre. The median patient age was 2.5 months (range, 18 days-4 years) and the median body weight was 4.2 (range, 2.2-17.7) kg at operation. Eleven (61%) patients were on a mechanical ventilator prior to surgery. The median stenotic segment estimated by a computed tomography scan was 52% of the length of total trachea (range, 18-84%). Five (28%) patients had proximal bronchial stenosis, 3 (17%) had tracheal bronchus, 2 (11%) had tracheobronchomalacia and 1 (6%) had agenesis of the right lung. Thirteen (72%) patients had a combined cardiac anomaly, including 8 patients with a pulmonary artery sling. Ten (56%) patients had associated extracardiac anomalies. Slide tracheoplasty was performed on cardiopulmonary bypass in all patients, and cardiac lesions were corrected. There was no early death. The patient with agenesis of the right lung died of left bronchial stenosis 3 months after the surgery. Two (11%) patients were reoperated on for tracheal restenosis. In the other 15 patients, the median duration of ventilator support was 8 (range, 5-34) days and the median duration of hospitalization was 31 (range, 12-79) days. During the follow-up (median duration of 17 months; range, 2-77 months), 13 (72%) patients were symptom-free and 2 (11%) underwent tracheostomy for tracheomalacia. Based on this study, slide tracheoplasty seems to be an effective technique for CTS. However, shortening of the trachea after reconstruction may give rise to recurrent obstruction.

Laryngeal release with slide tracheoplasty for long-segment congenital tracheal stenosis

Slide tracheoplasty is a standard treatment for long-segment congenital tracheal stenosis (LCTS). However, in severe cases of LCTS, aggressive divisions of inferior constrictor muscle from the thyroid cartilage and extensive circumferential dissection of the upper tracheal segment are often necessary to mobilize the upper tracheal segment enough to make an anastomosis, but they increase the risks of anastomotic dehiscence, recurrent nerve injury, and impaired deglutition. Alternatively, laryngeal release provides safe mobilization of the upper tracheal segment, minimizing dissection of the inferior constrictor muscle and preserving the lateral tissue pedicle without circumferential dissection. We successfully performed laryngeal release with slide tracheoplasty on six patients with severe LCTS, and report our findings.

Stem-cell-based, tissue engineered tracheal replacement in a child: a 2-year follow-up study

Stem-cell-based, tissue engineered transplants might offer new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaffold with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report findings after 2 years of follow-up. A 12-year-old boy was born with long-segment congenital tracheal stenosis and pulmonary sling. His airway had been maintained by metal stents, but, after failure, a cadaveric donor tracheal scaffold was decellularised. After a short course of granulocyte colony stimulating factor, bone marrow mesenchymal stem cells were retrieved preoperatively and seeded onto the scaffold, with patches of autologous epithelium. Topical human recombinant erythropoietin was applied to encourage angiogenesis, and transforming growth factor β to support chondrogenesis. Intravenous human recombinant erythropoietin was continued postoperatively. Outcomes were survival, morbidity, endoscopic appearance, cytology and proteomics of brushings, and peripheral blood counts. The graft revascularised within 1 week after surgery. A strong neutrophil response was noted locally for the first 8 weeks after surgery, which generated luminal DNA neutrophil extracellular traps. Cytological evidence of restoration of the epithelium was not evident until 1 year. The graft did not have biomechanical strength focally until 18 months, but the patient has not needed any medical intervention since then. 18 months after surgery, he had a normal chest CT scan and ventilation-perfusion scan and had grown 11 cm in height since the operation. At 2 years follow-up, he had a functional airway and had returned to school. Follow-up of the first paediatric, stem-cell-based, tissue-engineered transplant shows potential for this technology but also highlights the need for further research. Great Ormond Street Hospital NHS Trust, The Royal Free Hampstead NHS Trust, University College Hospital NHS Foundation Trust, and Region of Tuscany.

A New Morphologic Classification of Congenital Tracheobronchial Stenosis

Congenital tracheal stenoses are rare and life-threatening anomalies, associated with considerable variation in both morphology and prognosis. They have been classified previously according to the length of the stenosis or the severity of the symptoms, but not according to bronchial involvement. Data from patients who underwent slide tracheoplasty for long-segment (>50%) congenital tracheal stenosis were collected. We identified four different types of bronchial arborization (normal, n=52; tracheal right upper lobe bronchus, n=10; carina with "trifurcation," n=14; and unilateral bronchial and lung agenesis, n=8). Each type included congenital tracheal (above the carina) or tracheobronchial (extending below the carina) stenosis. Eighty-four children were enrolled in the study. Preoperative ventilation was necessary in 44 patients (52.4%; 75% in patients with a single lung), and preoperative extracorporeal membrane oxygenation was needed in 10 patients (11.9%). Preoperative tracheostomy was present in 3 patients initially treated elsewhere (3.5%), and a left pulmonary artery sling was performed in 44% (37 of 84). The overall mortality was 13% (11 of 84), 7.9% in patients with tracheal stenosis and 28.6% with tracheobronchial stenosis. No deaths occurred in patients with right upper lobe bronchus anatomy. Endoscopic procedures after slide tracheoplasty were required in 34 patients (40.4%). Stents were placed in 18 patients (21.4%), with a higher incidence in those with bronchial trifurcation (42.8%, 6 of 12). This classification appears useful for the morphologic characterization of congenital airway stenosis and could be the benchmark for future prospective studies on the outcome of these patients.

Extracorporeal Membrane Oxygenation, Extubation, and Lung-Recruitment Maneuvers as Rescue Therapy in a Patient With Tracheal Dehiscence Following Slide Tracheoplasty

Long-segment congenital tracheal stenosis is characterized by complete tracheal rings. Surgery is required during infancy to optimize outcomes, and the post-surgery complications include mucus plugging, airway trauma, dehiscence at the surgery site, and death. We report a 5-week-old patient who developed a tracheal-wall dehiscence after a slide tracheoplasty. To safeguard against further dehiscence and to protect her one functional lung, we used extracorporeal membrane oxygenation (ECMO). After she was stabilized on veno-arterial ECMO we extubated and continued ECMO for 5 days. On postoperative day 14 we removed the ECMO and transitioned her to high-frequency oscillatory ventilation, and performed slow lung-recruitment maneuvers every 2 hours. This strategy of ECMO with extubation, then high-frequency oscillatory ventilation is a useful rescue therapy in patients with postoperative tracheal dehiscence.

Repair of Complex Tracheobronchial Stenosis With Left Pulmonary Artery Sling and Bridging Bronchus

Slide tracheoplasty with reimplantation of the left pulmonary artery is one of the preferred techniques for repair of long-segment congenital tracheal stenosis in association with a pulmonary artery sling. Left pulmonary artery sling associated with a right upper bronchus from the trachea (tracheal bronchus) and stenosis of the bridging bronchus is a more complicated situation. We present an infant with left pulmonary artery sling, and stenosis of the bridging bronchus, carina, and both right and left main bronchi. His condition was successfully repaired using the combination of slide tracheoplasty and tracheal autograft techniques.

Management of symptomatic congenital tracheal stenosis in neonates and infants by slide tracheoplasty: a 7-year single institution experience

Congenital tracheal stenosis (CTS) is rare. When it presents early in life, its management can be challenging. Of the surgical techniques that have been devised to correct long-segment CTS, slide tracheoplasty (ST) appears to be superior. We present our 7-year-experience of ST for the treatment of symptomatic CTS in neonates and infants. The hospital records of all 14 neonates and infants who underwent ST between 2001 and 2008 at our hospital were retrieved. Patient characteristics, trachea morphology, co-existing anomalies, operative procedures, techniques, outcomes and clinical courses were reviewed. Patients underwent ST at age 4 days to 22 months (median: 2.4 months). Five (36%) required intermittent ventilator support prior to surgery. All ST was done under cardiopulmonary bypass. The associated cardiovascular anomalies in 10 infants (71%) were also corrected at the same operation. All survived the initial surgical procedures. The in-hospital mortality for the group was 14.3%. The median periods of postoperative intensive care unit and hospital stay of the 12 children, who were successfully extubated within 7 postoperative days, were 9 days (range: 6-28 days) and 28 days (range: 14-375 days), respectively. Follow-up of all 12 midterm survivors was complete, ranging from 5 months to 5.6 years (median: 40 months). A total of four patients had been found to have tracheobronchial malacia postoperatively and were managed with stenting. Of the remaining 10 survivors who had no residual or recurrent airway problems, and no cardiovascular residuum or sequela, two had gross developmental delay. In the management of symptomatic infants with CTS, our limited experience suggests that meticulously performed ST together with vigilant pre- and postoperative care can provide satisfactory short and midterm solution to the airway problem. Some incidental residual clinical problems appear to be unavoidable.

Modified slide tracheoplasty for congenital tracheal stenosis

Slide tracheoplasty has become a standard procedure to treat long-segment congenital tracheal stenosis because it is a reasonable and simple technique. Slide tracheoplasty does not affect the carina during long-segment congenital tracheal stenosis management, and thus lesions of the carina, such as stenosis and tracheobronchomalacia can become important causes of extubation failure after surgery. In this manusript, we describe the effectiveness of our modified slide tracheoplasty, which includes reconstruction of the carina. We have performed this technique on three patients, all of whom were extubated without developing any respiratory symptoms.

When parents opted not to perform surgery for a long-segment congenital tracheal stenosis child: flexible bronchoscopic balloon tracheoplasty as the primary treatment

Congenital tracheal stenosis is a rare airway disorder characterized by a fixed tracheal narrowing. Surgical intervention is usually considered as the treatment of choice for long-segment type. However, due to wide spectrum of this disease, treatment modalities need to be individualized. The long-segment type has the most unfavorable outcome because it is often associated with multiple anomalies and, thus, the most difficult to manage. Here we present a case of long-segment congenital tracheal stenosis (LSCTS) that was managed by several sessions of flexible bronchoscopic balloon tracheoplasty as the primary treatment. In this particular situation, we had to deal with the parents' request of a 'non-surgical' approach for an infant who had respiratory distress that also required a way to increase his tracheal diameter simultaneously. Having done this particular experience, we would like to show that this non-surgical approach can be viable for long-segment tracheal stenosis in selected cases.

Surgical management of congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is a rare but life-threatening disorder, particularly in neonates and infants. The stenotic lesions are commonly composed of complete rings of cartilage varying in length, location, and severity. A definitive diagnosis of CTS may be delayed because of the rarity of this disorder and thus its unfamiliarity among physicians, its variable onset time, its various clinical symptoms, and the diversity of associated clinical conditions due to the cardiovascular disorders that may accompany it. More than half of the patients who manifest clinical symptoms during early infancy show a long-segment stenosis. Long-segment CTS is problematic and challenging to manage. Over the last two decades several surgical techniques for long-segment CTS have been devised and developed, but no definitively advantageous surgical procedure has been established because of insufficient experience and the lack of large-scale studies. Although rib cartilage tracheoplasty and pericardial patch tracheoplasty have provided limited early to midterm success for infants with long-segment CTS, these procedures are associated with early and late complications, including granulation tissue formation, patch collapse, and restenosis necessitating reintervention. By contrast, slide tracheoplasty has given rise to better surgical outcomes. This procedure enables reconstruction of the stenotic trachea using native tracheal walls with preserved blood supply. The trachea is foreshortened by only one-half of the original stenosis, resulting in reduced tension on the anastomosis. Consequently, the technique has several advantages, including less formation of granulation tissue, satisfactory subsequent growth, and infrequent reoperation for restenosis. Slide tracheoplasty is currently recognized as the preferred technique for long-segment CTS.

Long segment congenital tracheal stenosis in twins successfully treated by slide tracheoplasty

Long segment congenital tracheal stenosis (LSCTS), associated with complete tracheal rings, is a rare condition, difficult to manage and historically associated with high mortality rate. We report two pairs of identical twins all affected by LSCTS successfully treated by sliding tracheoplasty. All had severe respiratory distress. Three infants had left pulmonary artery (LPA) sling and one intra-cardiac malformation. Slide tracheoplasty was done under cardiopulmonary bypass, and cardiovascular malformations were corrected at the same time. One child needed plication of paralyzed right hemi-diaphragm and another distal tracheal Palmaz stent insertion due severe tracheobronchomalacia. All children are doing well during 6 months follow-up. Slide tracheoplasty seems to produce the same good early results in twins as for singletons with LSCTS.