Management of congenital tracheal stenosis in infancy

Objective: Congenital tracheal stenosis (CTS) is a very infrequent malformation. Till recently, the outlook for these patients was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. We review the short- and long-term outcomes of a single institution experience in the management of children with CTS, comparing different treatment modalities. Methods: Between 1991 and 2004, 19 cases of CTS have been managed in our Unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy was performed for diagnostic purposes in all cases; other imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI), bronchography, angiography, doppler-ultrasound) were performed on an individual basis. According to clinical and endoscopical features, patients were classified into three groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomical type, associated anomalies, treatment modality, complications, outcome and time of follow-up. Results: Ten boys and nine girls have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 4 months) and 84% of cases showed associated anomalies. Five patients presented mild or no symptoms and have been managed expectantly. The other 14 cases were operated on because of persistent or severe clinical symptoms. The following procedures were performed: slide tracheoplasty (n = 7), costal cartilage tracheoplasty (n = 5), tracheal resection and reconstruction (n = 3), endoscopical dilatation (n = 3), stent placement (n = 1), and laser resection (n = 1). Three patients required two or more procedures and surgical survival rate is 78%. Overall mortality in the series is 21% and all survivors (15 patients) are asymptomatic or show mild symptoms with respiratory infections only. Follow-up is complete, ranging from 8 months to 12.3 years (mean, 5 years). Conclusions: Bronchoscopy is our preferred diagnostic tool. Selection of the type of treatment depends on the patient's clinical status and the anatomical pattern of the stenosis. In symptomatic cases with short-segment stenosis (<30% of total tracheal length), we prefer tracheal resection with end-to-end anastomosis; for long-segment stenosis (>30%), slide tracheoplasty is our procedure of choice.     

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. __________ This review was submitted at the invitation of the editorial committee.     

Slide tracheoplasty for congenital tracheal stenosis

Background/purpose

The aim of this study was to add to the experience of slide tracheoplasty in infants with congenital tracheal stenosis (CTS), to review the recent literature on this subject, and to evaluate the diagnostic workup in infants with CTS.

Methods

A retrospective review of 3 infants with CTS treated with slide tracheoplasty was conducted at our institution. They all underwent bronchoscopy, tracheobronchography, and echocardiography. Therapy consisted of slide tracheoplasty and simultaneous correction of associated vascular malformations.

Results

A pulmonary artery sling was missed initially on esophagography in 2 patients. Stridor became worse in 2 patients after tracheobronchography. After surgical therapy, 2 patients survived and were discharged 12 days after surgery. Both are without symptoms at follow-up of 12 and 20 months. One patient had inoperable restenosis and died 27 days after the operation.

Conclusions

Esophagography is not a reliable tool to depict associated vascular anomalies for patients with CTS. According to the literature, computer tomography has become more reliable to depict the tracheobronchial tree and is useful to elucidate associated vascular anomalies as well. Compared with other surgical techniques for infants with CTS reported in the literature, slide tracheoplasty has fewer postoperative complications and comparable survival rates.     

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.     

Treatment of postoperative tracheal granulation tissue with inhaled budesonide in congenital tracheal stenosis

Purpose

Tracheal obstruction by granulation tissue can compromise the postoperative course in congenital tracheal stenosis (CTS). Balloon dilatation and stenting may be required. Budesonide is a corticosteroid with topical anti-inflammatory effects. In 2008, we used inhaled budesonide for treatment of postoperative granulation tissue for the first time in CTS, resulting in significant improvement. The aim of this study was to evaluate the efficacy of inhaled budesonide for treatment of postoperative granulation tissue in CTS.

Methods

Retrospective chart review was conducted. From 2004 through 2011, we performed 39 tracheoplasties. Forced stenting ± balloon dilatation (S/B) was required when airway obstruction with tissue granulation was life-threatening. We compared the requirement for S/B between the early group without budesonide (2004–Nov. 2008, Early) and the late group with budesonide (Dec. 2008–2011, Late). Statistical analysis was performed using Fisher’s Exact test.

Results

Eleven of 22 in Early and 8 of 17 in Late were successfully extubated, never having had life-threatening tissue granulation. The remaining patients in each group (11 in Early and 9 in Late) required tracheostomies due to postoperative complication. Ten in Early and 5 in Late with tracheostomies developed granulation tissue. Of these patients, the 10 in Early required S/B, while none of the 5 in Late required S/B (P = .0003). Bronchoscopy demonstrated significant regression of granulation tissue in all cases treated with inhaled budesonide.

Conclusion

Inhaled budesonide is effective for treatment of tracheal granulation tissue in patients with tracheostomies after repair of CTS.     

胆总管囊肿伴肝总管狭窄患儿的腹腔镜手术治疗

目的探讨腹腔镜手术治疗胆总管囊肿伴有肝总管狭窄患儿的治疗方法。方法自2007年1月至2009年12月,共收治胆总管囊肿患儿41例,其中7例伴有肝总管狭窄的患儿均在腹腔镜下完成手术。采用四孔Trocars技术,先在腹腔镜下游离胆囊并将其提出腹壁外,行38％泛影葡胺造影,判断胆总管囊肿及肝总管的情况;电凝钩或超声刀游离切除囊肿;于脐孔处提出空肠行端侧吻合术;腹腔镜探查近端肝总管情况,将狭窄肝总管前壁向近端游离、切开、成形,并探查肝内胆管;行镜下胆肠端侧吻合术。结果7例胆总管囊肿伴有肝总管狭窄的患儿均在腹腔镜下完成手术,术中造影及腔镜探查证实囊肿近端肝总管狭窄,直径1～2mm,长度2mm左右,切开狭窄肝总管前壁至宽敞部,3例伴有肝总管结石,1例伴有蛋白栓,均游离近端肝总管行肝肠吻合。术后黄染、腹疼等症状消失,随访6个月至2年均无胆系感染或胆道梗阻发生。结论胆总管囊肿伴有肝总管狭窄的患儿在腹腔镜下行狭窄肝总管切开成形、胆肠吻合术,比开放性手术更容易操作,预后好,能够预防胆道梗阻及胆管炎的发生。     

Recent challenges in the management of congenital tracheal stenosis: an individualized approach

Background

Congenital tracheal stenosis (CTS) may present as a life-threatening condition often requiring urgent surgical correction. We report our recent experience of 13 consecutive patients with CTS over the past 18 months.

Method

Retrospective analysis of all patients with CTS admitted to our institution from January 2003 to June 2004.

Results

Five of 13 patients (6 boys and 7 girls) were premature at birth. Mean age at repair was 3.8 months (range, 7 days to 9 months). Ten presented with cardiac arrests or “near-death” spells, 4 with copresenting pulmonary infection. Nine patients were local and 4 were national. Surgical repairs included slide tracheoplasty (n = 5), cartilage patch tracheoplasty (n = 4), patch-and-slide tracheoplasty (n = 1), and balloon dilatation (n = 1). Preoperative computed tomographic imaging invariably underestimated the severity of pathology. Two patients with minimal symptoms were treated nonoperatively. Two patients required additional endobronchial stents for bronchomalacia. Cardiopulmonary bypass was used in 8 patients. There were 3 deaths, including 2 planned withdrawal of treatment. Two patients remained in hospital. The remaining patients were discharged from our hospital.

Conclusions

Presentation of CTS can be precipitated by respiratory infection. Preoperative imaging often underestimates the caliber and length of pathology. The treatment options for patients with CTS including observation should be individualized.     

The role of conservative management in congenital tracheal stenosis: an evidence-based long-term follow-up study

Background/Purpose

Surgery has been the management of choice for severe congenital tracheal stenosis (CTS). The role of conservative management of CTS however is not clear. The aim of this study is to characterize the natural history of CTS, review the radiologic evidence of tracheal growth, and evaluate the clinical outcome and selection criteria of conservative management of CTS.

Methods

A retrospective study was carried out on 22 consecutive children with symptomatic CTS admitted into a single institution between 1982 and 2001. The patients were categorized into operation (n = 11) and observation (n = 11) groups. Six patients of the observation group were followed up with serial computed tomography scan. Their tracheal growth was compared with that of healthy children of the same age.

Results

The mortality rates of observation and operation groups were 9% and 27%, respectively, although the latter group consisted of more severely affected patients. The pathologic categorization of the CTS influenced the survival rates (P = .046, χ²), with the long segment type having the worst prognosis (67%). Serial computed tomography scans of 6 conservatively managed patients revealed that all stenotic tracheas continued to grow (P = .039, 2-tailed paired Student's t test). Of the 6 stenotic tracheas, 5 grew at a faster-than-normal rate, and the stenotic tracheal diameters approached those of normal diameters by the age of 9 years.

Conclusions

The management of patients with symptomatic CTS should be individualized. A selected group of patients with CTS can be safely managed nonoperatively.     

Resection for congenital tracheal stenosis

A case of congenital tracheal stenosis is reported. The diagnosis was made on plain X-ray, tomography and computerized axial tomography of the trachea when the child was 8 years old. The operative approach was via a median sternotomy and anaesthesia maintained with dual simultaneous ventilation of the right upper lobe bronchus and distal trachea. Histology of the stenotic area revealed the cartilaginous rings to be completely encircling the trachea.     

Management of bilateral congenital bronchial stenosis in an infant

Congenital bronchial stenosis is a very uncommon condition in children. A full-term neonate showed severe respiratory distress soon after birth. Bronchoscopy and spiral computed tomography with multiplanar reconstruction disclosed a short stenosis of the left main bronchus, next to the carina, and another stenosis in the intermediate bronchus with air trapping in the right middle and lower lobes. The child underwent resection and reconstruction of the left main bronchus, and right middle and lower lobectomies after a failed attempt of bronchoscopic dilatation of the intermediate bronchus. A temporary silicon tracheal stent was necessary to achieve permanent extubation. The patient was discharged in good condition without any oxygen dependency and remains asymptomatic 1 year after surgery. Management of children with airway stenosis is complex, and surgeons involved in it should be familiar with multiple surgical and endoscopic techniques. A team approach in a referral center is the best option to achieve an optimal result.     

Preoperative assessment in patients with postintubation tracheal stenosis

Background Postintubation stenosis remains the most frequent indication for tracheal surgery. Rigid bronchoscopy has traditionally been considered the technique of choice for the preoperative diagnostic assessment. However, this technique is not routinely available, and new techniques such as flexible videobronchoscopy and spiral computed tomography (CT) scan with multiplanar reconstructions have been proposed as alternatives to rigid bronchoscopy. The aim of this study was to compare these techniques in the diagnostic assessment of patients with tracheal stenosis submitted to surgical treatment. Methods Twelve patients who underwent airway resection and reconstruction for postintubation tracheal and laryngotracheal stenosis were preoperatively evaluated with rigid and flexible bronchoscopy and with spiral CT scan with multiplanar reconstructions. The following parameters were examined: involvement of subglottic larynx, length of the stenosis, and associated lesions. The results were compared with the intraoperative findings. Results The accuracy of rigid bronchoscopy, flexible bronchoscopy, and CT scan in the evaluation of the involvement of subglottic larynx was, respectively, 92%, 83%, and 83%. The evaluation of the length of the stenosis was correct in 83%, 92%, and 25% of the patients, respectively, with rigid bronchoscopy, flexible bronchoscopy, and CT scan. A significant correlation was observed between the length of the stenosis measured intraoperatively and preoperatively with rigid (p < 0.001) and flexible bronchoscopy (p < 0.05) but not with CT scan (p = 0.08). The three techniques correctly showed the presence of an associated tracheoesophageal fistula in two patients, but CT scan did not correctly show the exact location of the fistula in relation to the airway. Flexible bronchoscopy was the only effective technique in the assessment of laryngeal function. Conclusions Rigid bronchoscopy remains the procedure of choice in the evaluation of candidates for tracheal resection and reconstruction for postintubation stenosis, and it should be available in centers that perform surgery of the airway. Flexible bronchoscopy and CT scan have to be considered complementary techniques in the evaluation of laryngeal function and during follow-up.     

气管内支架治疗气管良性狭窄11例

目的：探讨气管内支架治疗气管良性狭窄的疗效。方法：１９８２年２月至１９９８年８月间应用气管内支架治疗１１例气管良性狭窄,其中Ｙ形硅胶管支架置入术４例,Ｓｔｒｅｃｋｅｒ金属网状支架置入术１例。结果：随访４￣１８０个月。８例临床效果良好,其中４例拔除内支架。并发症包括肉芽组织形成３例,其中２例更换支架;围术期咳痰困难５例。结论：不同病因良性气和狭窄在选择治疗方法时应严格按照治疗原则,对气管壁全层皆已成     

Surgery for total congenital tracheal stenosis

A 4 1/2-month-old boy with complete tracheal rings throughout the entire trachea and agenesis of the right lung was successfully treated by operative insertion of a cartilagenous graft extending the length of his trachea. The commonly held viewpoint that this lesion is not surgically correctable and these infants will all die, can no longer be supported. Surgical repair should be strongly considered in all these children in the future.     

Patterns of management of congenital tracheal stenosis

Background/purpose

Stenosing airway disease, including congenital and acquired lesions, is rare in the pediatric age group. Untill recently, the outlook for patients with congenital tracheal stenosis (CTS) was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. This report reviews the short and long-term outcomes of a single-institution experience in the management of CTS in children, comparing different treatment modalities.

Methods

From 1991 to 2002, 13 cases of CTS have been managed in the authors unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy established the diagnosis in all cases. According to clinical and endoscopical features, patients have been classified into 3 groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomic type, associated anomalies, treatment modality, complications, outcome, and time of follow-up.

Results

Seven girls and 6 boys have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 8 months), and 77% showed associated anomalies. Four patients presented mild or no symptoms and have been treated expectantly. The other 9 patients have been operated on because of persistent or severe symptomatology. The following procedures have been performed: costal cartilage tracheoplasty (n = 5), tracheal resection (n = 3), slide tracheoplasty (n = 2), endoscopical dilatation (n = 3), and laser resection (n = 1). Three patients required 2 or more procedures, and there were 3 early deaths, all after costal cartilage tracheoplasty. Overall mortality rate in the series is 23%. Follow-up is complete in all survivors (n = 10) ranging from 6 months to 10 years (mean, 4.7 years).

Conclusions

Selection of the type of treatment depends on the patient’s clinical status and the anatomic pattern of the stenosis. In symptomatic cases of short-segment stenoses the authors prefer tracheal resection with end-to-end anastomosis; for long-segment stenoses, slide tracheoplasty is the procedure of choice.     

The anaesthetic management of congenital tracheal stenosis

Congenital tracheal stenosis is a rare and potentially lethal condition. Two case reports are presented which demonstrate the problems encountered in the anaesthetic management of this condition.