Tracheal growth in congenital tracheal stenosis

Two cases of congenital tracheal stenosis were managed conservatively despite mild to moderate initial respiratory symptomatology in infancy. Serial CT examinations were performed on each child, with tracheal dimensions and cross-sectional areas measured in the region of stenosis at each CT examination. The examinations document increases in tracheal cross-sectional area in the region of stenosis over time, confirming tracheal cartilage growth. We present these data to dispel the hypothesis that tracheal growth does not occur in complete-ring tracheal stenosis. The selection of cases for surgical repair must be considered with the knowledge that this anomaly does not carry an inevitably poor prognosis.     

Slide方法在小儿先天性气管狭窄的应用

目的 总结Slide方法纠治长段气管狭窄的手术疗效.方法 2001年8月至2010年10月,手术纠治先天性气管狭窄21例.手术年龄28 d至8.6岁,平均(21.9±27.1)个月,体重4.2～22.5 kg,平均(9.65±4.24)kg,除2例分别为纵隔肿瘤和气管外伤所致气管短段狭窄外,其余19例均伴有先天性心脏病.根据气管狭窄长度,分为短段气管狭窄9例,长段气管狭窄12例.所有病例均在低温体外循环下行先天性心脏病纠治术,同时行气管狭窄处理.近期8例均采用Slide方法.结果 21例先天性气管狭窄手术死亡2例.1例早期采用人工材料修补,术后3个月出现肉芽,放弃治疗死亡.1例长段气管狭窄采用Slide方法,术后3周出现气管内肉芽,经多次球囊扩张、激光烧灼均无效,死亡.余19例长期随访2个月至8年,气管狭窄症状消失,CT复查效果满意,无一例再次手术.结论 Slide气管成形术采用自体的气管组织重建气道,吻合口牢固稳定,而且保持了正常的气管内膜,保证术后气管内壁的细胞功能和良好生长.是长段气管狭窄的最佳手术方法.由于本组病例较少,还需要定期随访,观察长期疗效.

Abstract：

Objective To evaluate slide tracheoplasty for congenital tracheal stenosis. Methods Between August 2001 and October 2010, twenty-one patients with congenital tracheal stenosis were repaired. The operative age was 28d～8. 6y (mean 21.9 ± 27. 1m) and the weight was 4. 2～22. 5kg (mean 9. 65 ± 4.24 kg) . Except for one case of mediasternal tumour and another one of tracheal trauma, all the restothers had congenital heart disease. There were 9 patients with short tracheal stenosis and 12 patients with long segment tracheal stenosis. The congenital heart disease were repaired underwent low temperature and cardiopulmonary bypass, and the tracheal stenosis were corrected simultaneously. Nine patients with long segment tracheal stenosis were repaired by slide tracheoplasty. Results There was two death in this group. One patient with right and left broncheal stenosis was repaired by synthetic patch. He succumbed 3 months later due todied of granulation tissue on the patch. Another patient with long segment tracheal stenosis was repaired by slide tracheoplasty, who also died from granulation tissue. All other 19 patients were uneventful. These patients were followed up from 2 month to 8 years, there were no complication. CT scan shows that the tracheal anastomosis was patent withand no any stenosis. Conclusions The slide tracheoplasty was used by autologous material,maintaining its contour and function of respiratory epithelium, and keeping its growth. The slide tracheoplasty is best way for correction of long segment tracheal stenosis. Long term follow up is required.     

The current state of congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is an uncommon condition that has challenged pediatric surgeons for decades. Patients with CTS can present with a wide spectrum of symptoms and varying degrees of severity. In addition, a variety of techniques have been devised to repair this malformation. A review of these procedures and our suggestions for clinical standards and practice guidelines will be presented in this paper. A retrospective review of the literature on CTS from 1964 to 31 March, 2006. There is not one standard technique for the repair of CTS, as individualized approach to each patient and airway lesion is necessary to optimize patient management; nevertheless there is a consensus about segmental resection and anastomosis being best for short segment stenosis while slide tracheoplasty is most effective for the long-segment ones. Conservative management is also an option for select group of patients with careful and close follow up. Survival following surgery over the years has improved, but mortality remained high, particularly in a specific subset of patients presenting at the age less than 1 month with associated cardiac malformations. In conclusion, CTS remains a significant challenge for pediatric surgeons. Additional research is required to improve our understanding of the pathogenesis of CTS, and to develop evidence-based treatment protocols for the entire spectrum of presentation including conservative management.     

Perioperative extracorporeal membrane oxygenation for tracheal reconstruction in congenital tracheal stenosis

The management of a critical airway in infants and toddlers with congenital tracheal stenosis (CTS) continues to be an enormous challenge to the surgeon. Until recently, this condition often proved fatal. Improvements in surgical techniques, anesthetic management, and postoperative critical care have resulted in successful outcomes in children not long ago considered untreatable. However, issues such as the best operative approach and the optimal perioperative management are still unresolved. The diagnosis of CTS, often delayed, must be considered in any infant with stridor, wheezing, cyanosis, or recurrent episodes of pneumonia. Associated anomalies are the rule, including frequently vascular rings and rarely pulmonary agenesis. These defects can be repaired with conventional ventilatory support under cardiopulmonary bypass, or using extracorporeal membrane oxygenation (ECMO). We report our experience in which ECMO was used to support two patients with CTS during the perioperative period. ECMO proved to be both safe and practical, allowing unrushed, precise repair of the tracheal stenosis and providing brief postoperative support. Perioperative outcomes were excellent, although one of our patients died months after the repair. A review of the literature and our experience in which ECMO was used to provide cardiopulmonary support during repair of CTS showed uniformly successful perioperative outcomes. Accepted: 3 November 1998     

Borderline congenital hypothyroidism in the neonatal period

One of the most important etiological factors causing prolonged jaundice in the neonatal period is congenital hypothyroidism. Some infants may have abnormal thyroid function test results rather than overt congenital hypothyroidism. Although serum TSH levels are accepted as diagnostic when >20 microIU/l, TSH values higher than 7 microIU/ml cause a hypometabolic condition. In this study, we evaluated infants who had prolonged jaundice for hypothyroidism. A hundred and ten infants suffering from prolonged jaundice were admitted to our clinic during the study period. Among them, 61 infants had normal thyroid function results. Six patients had overt primary hypothyroidism. TRH stimulation test was administered to the 43 patients with mildly elevated TSH levels of between 5 and 20 microIU/ml. Peak TSH values were above 35 microIU/ml in seven patients, and these were considered as having an exaggerated response (borderline hypothyroidism). During the neonatal period, prolonged jaundice is a valuable diagnostic clue for hypothyroidism. In addition, the TRH stimulation test can be a diagnostic tool in evaluating infants with mildly abnormal thyroid function test results.     

Isolated congenital tracheal stenosis in a preterm newborn

Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory.     

Three-dimensional imaging of the pediatric trachea in congenital tracheal stenosis

Technological advances in three-dimensional computer software packages have resulted in fairly widespread acceptance of 3D imaging for musculoskeletal studies. We have recently applied this technology to axial CT images of the pediatric trachea for children with congenital tracheal stenosis, in an effort to replace tracheo-bronchography with a safer, less invasive modality to image the pediatric trachea. This method has provided our surgical colleagues with images which they find easier to interpret because they are more anatomically oriented with respect to regional mediatinal structures, which can be included or removed from the image at will. The images can also be manipulated and rotated through various projections, thus facilitating surgical planning.     

Surgical intervention strategies for congenital tracheal stenosis associated with a tracheal bronchus based on the location of stenosis

Purpose

The aim of this study was to determine the appropriate surgical intervention strategies for congenital tracheal stenosis (CTS) associated with a tracheal bronchus based on the location of stenosis.

Methods

The medical records of 13 pediatric patients with CTS associated with a tracheal bronchus at a single institution between January 2006 and December 2015 were retrospectively reviewed.

Results

Type 1: tracheal stenosis above the right upper lobe bronchus (RULB) (n = 1). One patient underwent slide tracheoplasty and was successfully extubated. Type 2: tracheal stenosis below the RULB (n = 7). Tracheal end-to-end anastomosis was performed before 2014, and one patient failed to extubate. Posterior–anterior slide tracheoplasty was performed since 2014, and all three patients were successfully extubated. Type 3: tracheal stenosis above the RULB to the carina (n = 5). One patient underwent posterior–anterior slide tracheoplasty and was successfully extubated. Two patients with left–right slide tracheoplasty and another two patients with tracheal end-to-end anastomosis for the stenosis below the RULB could not be extubated.

Conclusion

Tracheal end-to-end anastomosis or slide tracheoplasty can be selected for tracheal stenosis above the RULB according to the length of stenosis. Posterior–anterior slide tracheoplasty appears feasible for tracheal stenosis below the RULB or above the RULB to the carina.

     

Tracheobronchography and balloon dilatation in acquired neonatal tracheal stenosis.

Between 1988 and 1992, 18 mechanically ventilated newborn babies (mean weight 1300 g and gestational age 30 weeks) presented with deteriorating respiratory failure at a mean age of 29 days. All developed increased oxygen requirements, hypoxic and hypercapnic episodes, and radiological changes of fixed lobar emphysema or recurrent atelectasis which sometimes changed sides from one day to another. Tracheobronchography with iopydol-iopydone was normal in five (27%) cases, but in 13 showed tracheobronchial stenosis localised to the lower trachea (seven cases), to the right main bronchus (three cases), or including the left main bronchus (four cases). Eleven of these 13 patients underwent endoscopy and balloon dilatation of the stenotic area. Five patients died, one before endoscopy, one immediately after endoscopies, and three subsequently with severe bronchopulmonary dysplasia. The other six babies recovered without any sequelae after balloon dilatation.     

婴幼儿先天性气管狭窄伴复杂先心病的Ⅰ期纠治

目的：探讨婴幼儿先天性气管狭窄伴复杂先心病I期纠治的治疗效果。方法：2例手术年龄分别1.5岁和3岁的先天性气管狭窄伴法洛四联症患儿在体外循环下行气管狭窄纠治和四联症根治术。1例气管狭窄长约1.2cm，切除气管狭窄段，然后端端吻合；另1例气管狭窄长约6cm，切除3cm剪开形成两块补片，分别扩大剪开的气管前壁。总扩大气管狭窄段达6cm。结果：术后恢复良好。随访6个月-1年，呼吸平稳，CT示气管通畅，心脏超声检查无残余分流和梗阻。结论：纠治先天性气管狭窄半先心病，以I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患儿康复。     

新生儿气管狭窄病因及诊疗进展

气管狭窄是由于不同病变所造成的气管腔狭窄,病因尚不清楚,但与先天性心脏病血管环压迫气道、气管软化及气管重塑等有着密切联系.影像学技术尤其是纤维支气管镜和CT对于诊断气管狭窄起到重要作用;手术是常用的治疗手段,气管置入支架是目前临床内科发展的方向.