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.     

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.     

Tracheal diaphragm: a very unusual form of congenital tracheal stenosis

A 3.2-kg newborn was intubated for neonatal respiratory distress owing to a congenital tracheal stenosis. The preoperative assessment showed a sphincter-like stenosis located 1 cm above the carina. The child was cured by resection-anastomosis through sternotomy under cardiopulmonary bypass. The pathological examination showed hypoplastic cartilage islets embedded in a fibroelastic conjunctive tissue. The posterior membranous region was preserved with the existence of smooth muscle cells. This curious malformation resembled a diaphragm at the junction between trachea and carina.     

Using three-dimensional visualization as an optimal tool to plan and validate an aortopexy in a congenital heart disease patient with severe tracheal stenosis

We present a patient with severe tracheal stenosis resulting from a compression by the innominate artery 6 months after an arterial switch operation in a dextro-transposition of the great arteries. Segmentation and three-dimensional (3D) visualization were derived from a contrast-enhanced dual-source computed tomography and post-processing was performed using a dedicated open-source platform (3D Slicer). Post-processing allowed a comprehensible visualization of the relationship of the innominate artery to the trachea when compared to standard computer tomography reformations. Finally, the surgical approach to move the innominate artery anteriorly in order to relieve the tracheal obstruction was emphasized based on the improved 3D visualization of the actual pathology. An effective aortopexy could be performed and the postoperative result was confirmed by a second 3D visualization. About 3 months of follow-up, the patient is completely asymptomatic. Three-dimensional visualization offers excellent opportunities for diagnosis, treatment planning and follow-up in patients with a vascular-related tracheal stenosis in the context of congenital heart disease.     

Tracheoplasty--a new operation for complete congenital tracheal stenosis

This is a report of a case of complete congenital tracheal stenosis confirmed by tracheobronchogram. The stenosis also involved the origin of the right main bronchus. The membranous portion of the trachea was absent. It was repaired through a sternotomy and right thoracotomy aided by partial cardiopulmonary bypass. The posterior trachea was opened from larynx to carina and on into the right main bronchus, and each posterior tracheal edge was sewn to the anterior wall of the esophagus with a running Prolene suture. Three months after repair bronchoscopy showed that the new membranous trachea was epithelialized and the entire airway was of good caliber; the only problem was a diffuse tracheomalacia. He died in his seventh postoperative month after a major airway complication due to tracheotomy, which occurred after an elective bronchoscopy. It is obvious that this operation is technically feasible. It was hoped that his airway would become sufficiently stable to allow the tracheotomy tube to be removed at some time in the future.     

Bronchoplasty for bronchial stenosis in a neonate: a case report

Bronchial stenosis is an uncommon problem in children. Management can be difficult because of the small luminal diameter and proximity of the lung parenchyma to the bronchial lesion. Bronchoplasty procedures have obvious advantages in children because of their long life expectancy. Long-term functional results are superior compared with pneumonectomy because of preserved lung tissue. A premature neonate weighing only 779 g at birth was born with severe respiratory distress syndrome complicated with hyperbillirubinemia and bronchopulmonary dysplasia. The child underwent bronchoplasty using a costal cartilage graft for a right main bronchus stenosis after 2 failed attempts at bronchoscopic dilatations. In spite of all complications, the child improved sufficiently to be discharged without any oxygen dependency. Our case highlights the successful application of bronchoplasty in a very small baby. Preoperative and intraoperative bronchoscopy was helpful in localization of the endobronchial lesion. Understanding the principle, precise attention to technical detail and meticulous postoperative care contribute toward long-term benefit for pulmonary conservation.     

An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia

Background/Purpose

Differences in clinical features between congenital pulmonary airway malformation (CPAM) and bronchial atresia (BA) have not yet been clearly described.

Endoscopic management of congenital esophageal stenosis

Background/Purpose

Congenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES.

Patients and Methods

Patients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary).

Results

Forty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mean age at the diagnosis was 28.3 months (range, 1 day to 146 months). Symptoms were solid food refusal, regurgitation, vomiting, and dysphagia. Congenital esophageal stenosis was located in the distal esophagus. Endoscopic ultrasonography demonstrated TBR and FMH in 6 patients. One hundred forty-eight dilations in 47 patients were performed. The stenosis healed in 45 (95.7%). Complications were 5 (10.6%) esophageal perforations, hydrostatic (3/32, or 9.3%), and Savary (2/116, or 1.7%). At follow-up, 1 patient with FMH CES and 1 patient with TBR CES required operation for persistent dysphagia.

Conclusions

The conservative treatment yielded positive outcomes in CES. Endoscopic ultrasonography allows for a correct diagnosis of TBR/FMH CES. A surgical approach should be reserved for CES not responsive to dilations.     

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.     

Management of congenital tracheal stenosis

Objectives: Congenital tracheal stenosis is a rare disease. Various methods for treatment exist but there is still much debate as to the appropriate surgical procedure. We present our surgical experiences of patch tracheoplasty and slide tracheoplasty as viable methods for the treatment of congenital tracheal stenosis. Methods: From 1994 to 2002, 13 patients were diagnosed with congenital tracheal stenosis. Eight patients (7 symptomatic and 1 asymptomatic) had their stenosis corrected, three by means of pericardial patch tracheoplasty, four by slide tracheoplasty, and one by resection and anastomosis. Concomitant operations were performed on six patients to treat congenital cardiovascular disease. Five patients showing no significant symptoms did not undergo tracheal surgery and received only cardiac procedures. A retrospective review of the hospital course, complications, and long-term results was conducted. Results: Among the patch tracheoplasty group, every patient suffered from granulation tissue formation. One patient died of respiratory acidosis and one was hospitalized due to recurrent granulation tissue, which required frequent bronchoscopy. The third patient from this group is free of all symptoms. Among the slide tracheoplasty group, one patient died of anastomosis disruption. The three remaining patients are alive and well. The one patient who received resection and anastomosis is alive without symptoms. Conclusions: Surgical repair of long-segment congenital tracheal stenosis exhibited high mortality and morbidity rates. Every patient that underwent pericardial patch tracheoplasty suffered from troublesome granulation tissue. As slide tracheoplasty provided relatively good results in the short and mid-term follow-up periods, it seems to be a preferred method for the treatment of long-segment congenital tracheal stenosis.     

Thoracoscopic approach for congenital esophageal stenosis

Congenital esophageal stenosis (CES) is an infrequent entity; however, many cases have been reported during the last years. Its incidence falls between 1 per 25,000 and 1 per 50,000 live births and is associated with other congenital malformations in 17% to 33% of cases (mainly esophageal atresia). Congenital esophageal stenosis is defined as an intrinsic alteration of the esophageal wall given by the presence of ectopic tracheobronchial tissue, membranous diaphragm, muscular hypertrophy, or diffuse fibrosis of the submucosa, among other causes. The therapeutic options include endoscopic dilation and resection plus anastomosis (by either laparotomy or thoracotomy, depending on the level of the stenosis).We present the case of a 1-month-old baby boy with a CES located in the distal esophagus that is associated with anophthalmia and micropenis. We treated the lesion by means of a thoracoscopic resection of the affected segment and an esophageal end-to-end anastomosis. The patient's long-term outcome was uneventful.As far as we know, this is the first report on thoracoscopic resolution of a CES.     

婴幼儿先天性心脏病伴气管狭窄的一期矫治

目的回顾总结一期纠治婴幼儿先天性心脏病（先心病）伴气管狭窄的体会。方法2001年8月至2005年11月，6例先心病伴气管狭窄的婴幼儿行一期手术矫治。手术年龄24d～3岁；体重4．2～10．0kg，平均（7．98±2．03）kg。其中法洛四联症3例，1例伴肺动脉闭锁；室间隔缺损2例，1例伴主动脉缩窄；肺动脉吊带1例。所有病儿均在低温体外循环下行先心病纠治术，同时处理气管狭窄。结果术后早期1例左、右支气管均匀性狭窄，不能脱离呼吸机死亡。1例肺动脉吊带者术后顺利出院，3个月后出现气管内肉芽增生，家属放弃治疗。余4例术后恢复良好，随访6个月至4年，临床上无气促表现。超声复查心功能良好，其中3例经CT复查，显示气管吻合口通畅，无明显狭窄。结论先心病伴先天性气管狭窄，以一期手术治疗为好，不但术中在体外循环下纠治气管狭窄较安全，而且同时纠治先心病有利于手术后病儿康复。     

Stenting for critical airway stenosis under percutaneous cardiopulmonary support

Airway stenting for severe central airway stenosis is inherently a dangerous procedure. There is the risk of critical airway obstruction due to bleeding, tumor debris, and airway perforation during the procedure. Once such situations occur, percutaneous cardiopulmonary support (PCPS) can be one of the most valuable rescue options to prevent critical hypoxic complications. At our institute, four of 49 patients who received stenting or other airway intervention required PCPS support (8%). Two of these cases required PCPS to be performed in an emergency setting during the procedure while the procedure was elective in the other 2. All procedures were performed effectively and safely without any complications caused by PCPS, including massive airway bleeding due to anticoagulant treatment. Patients were able to be weaned off PCPS uneventfully. PCPS is considered to be a valuable procedure in remedying critical hypoxic situations during airway intervention. Read at the Fifty-sixth Annual Meeting of the Japanese Association for Thoracic Surgery, Symposium, Tokyo, November 19–21,2003.     

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 in infant with congenital tracheal stenosis and tracheomalacia after esophageal atresia with tracheoesophageal fistula repair

Slide tracheoplasty can be applied successfully to all types of long segment congenital tracheal stenosis. In case of short segment tracheal stenosis, direct resection and anastomosis is often preferred. We report a case illustrating a new side benefit of the slide tracheoplasty in a patient presenting a relatively short hypoplastic tracheal segment coexisting with tracheomalacia after EA with TEF repair.     

A case of bronchial obstruction by metastatic renal cell carcinoma

A 68-year-old Chinese male with a history of renal cell carcinoma presenting with increasing shortness of breath and hemoptysis was found to have subtotal left main bronchial obstruction secondary to a metastatic endobronchial tumor. The mass was completely resected through a rigid ventilating bronchoscope, and a silicone stent was placed over the tumor bed in the left main bronchus. He remained asymptomatic with no evidence of bronchoscopic recurrence 4 months after the procedure. Obstructive endobronchial metastases from nonpulmonary primaries are rare. Endoluminal resection with stent placement can provide good palliation in this group of patients with limited survival.     

Risk factors for reoperation after relief of congenital subaortic stenosis

Background: Congenital subaortic stenosis entails a lesion spectrum, ranging from an isolated obstructive membrane, to complex tunnel narrowing of the left outflow associated with other cardiac defects. We review our experience with this anomaly, and analyze risk factors leading to restenosis requiring reoperation. Methods: From 1994 to 2006, 58 children (median age 4.3 years, range 7 days–13.7 years) underwent primary relief of subaortic stenosis. Patients were divided into simple lesions (n = 43) or complex stenosis (n = 15) associated with other major cardiac defects. Age, pre- and postoperative gradient over the left outflow, associated aortic or mitral valve insufficiency, chromosomal anomalies, arteria lusoria, and operative technique (membrane resection (22) vs associated myectomy (34) vs Konno (2)) were analyzed as risk factors for reoperation (Kaplan–Meier, Cox regression). Results: There was no operative mortality. Median follow-up spanned 2.7 years (range 0.1–10), with one late death at 4 months. Reoperation was required for recurrent stenosis in 11 patients (19%) at 2.6 years (range 0.3–7.5) after initial surgery. Risk factors for reoperation included complex subaortic stenosis (p = 0.003), younger age (p = 0.012), postoperative residual gradient (p = 0.019), and the presence of an arteria lusoria (p = 0.014). For simple lesions, no variable achieved significance for stenosis recurrence. Conclusions: Surgical relief of congenital subaortic stenosis, even with complex defects, yields excellent results. Reoperation is not infrequent, and should be anticipated with younger age at operation, complex defects, residual postoperative gradient, and an arteria lusoria. Myectomy concomitant to membrane resection, even in simple lesions, does not provide enhanced freedom from reoperation, and should be tailored to anatomic findings.