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.     

Esophageal motor function in congenital esophageal stenosis

Background/Purpose: Congenital esophageal stenosis (CES) is a rare condition that is associated with various foregut symptoms. The aim of the current study was to investigate esophageal motor function in pediatric patients with isolated CES.Methods: Four boys with CES (age, 3 weeks to 4 years old) were studied before treatment. The initial symptoms were dysphagia or stridor. The CES was caused by fibromuscular stenosis (FMS) in 2, tracheobronchial remnants (TBR) in 1, and membranous diaphragm (MD) in 1. An esophagram, endoscopy, 24-hour esophageal pH monitoring, and manometry were conducted.Results: The esophagram showed the stasis of contrast medium proximally to the distal esophageal narrowing in FMS/TBR patients. Endoscopic esophagitis was not found in any patients. Three patients were documented with pathologic esophageal acid exposure by 24-hour esophageal pH monitoring. Manometry showed that esophageal contractions predominantly were synchronous in FMS/TBR patients but were peristaltic in an MD patient. Basal lower esophageal sphincter (LES) pressure was at least 20 mm Hg in all. Swallow-induced LES relaxations were incomplete in FMS/TBR patients.Conclusions: The presence of gastroesophageal reflux and impaired esophageal motility are common in patients with CES.     

Circular myectomy for the treatment of congenital esophageal stenosis owing to tracheobronchial remnant

The management of the congenital esophageal stenosis is not well established. The authors present an infant with critical esophageal stenosis owing to tracheobronchial remnant. This lesion was managed successfully by circular myectomy of the esophageal wall without resection. An 1-year-old girl was referred for recurrent vomiting and dysphagia. An esophagogram showed an abrupt stenosis, and endoscopic ultrasonography showed cartilaginous tracheobronchial remnants within the esophageal wall. The extirpation of muscular layer, which contained cartilage, was attempted. Circular muscular layer was resected in 1 cm width leaving mucosal layer intact. Muscular layer was closed horizontally with absorbable sutures. The patient is free from the symptoms and eats normally 1 year after surgery. In case of short segmental stenosis owing to tracheobronchial remnant, this may be the preferred approach.     

High-grade congenital esophageal stenosis owing to a membranous diaphragm with tracheoesophageal fistula

Gross E-type congenital esophageal atresia associated with congenital esophageal stenosis is extremely rare. In a male infant born at 36 weeks of gestation, bubbly vomiting was noted after birth. X-ray films of the chest and abdomen showed coil-up sign of the nasogastric tube and gas in the stomach and small intestines were recognized, so gross C-type esophageal atresia was suspected and surgery was performed on the first day of life. Surgery revealed the presence of a tracheoesophageal fistula in the upper esophagus and membranous stenosis on the distal side.     

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.     

Congenital esophageal stenosis because of tracheobronchial remnant and treated by circular myectomy: a case report

Congenital esophageal stenosis (CES) is a rare anomaly, and appropriate management is not well established. We performed myectomy of the esophageal wall in a child with critical esophageal stenosis caused by tracheobronchial remnant (TBR). An 18-month-old boy was admitted to our hospital having frequent vomiting and failure to thrive. Esophagography and esophagoscopy showed abrupt stenosis at the lower esophageal wall. Balloon dilatation was performed but was ineffective. Surgery was performed under a diagnosis of CES because of TBR. Cartilage was palpable in the stenotic esophageal wall, and extirpation of the muscular layer of the stenotic portion was performed, leaving the mucosal layer intact. The muscular layer was closed loosely using interrupted 5-0 absorbable sutures to match the oral and anal sides together. Postoperatively, the esophageal passage was improved to the point that the patient was able to take solid foods without vomiting. This successful outcome suggests that circular myectomy of the TBR is worth recommending as a surgical procedure for short segment and stenosis of patients with CES because of TBR.     

先天性食管狭窄的诊断和治疗

目的 根据病理结果，探讨小儿先天性食管狭窄的早期诊断和改良的治疗方案。方法 回顾性分析1980～2000年42例先天性食管狭窄病例的呕吐发生年龄、频率，对营养、发育的影响，诊断治疗方法及病理所见，术后正常进食时间，远期营养恢复。结果 经食管镜、消化道造影、手术病理证实，气管软骨异位26例，肌层肥厚11例，膜式狭窄3例，异位胃黏膜2例。42例中11例先行扩张治疗者仅1例膜式狭窄扩张成功；41例手术治疗，10例端端吻合，12例食管胃吻合，19例纵切横缝。9例术后扩张。扩张3个月以上者占55．6％。膜式狭窄、气管软骨异位术后见效快，大多无需扩张。肌层肥厚多数需术后扩张，但随访4～10年效果良好。术后病程1个月即恢复正常进食者达78．6％，6个月9．5％，1年始恢复者为11．9％。结论 注意添加辅食时频繁呕吐影响营养、发育者，可早发现本病；食管镜、消化道造影是诊断依据。膜式狭窄、气管软骨异位，纵切横缝效果好。肌层肥厚行狭窄段切除端端吻合是基本治疗方法；术后1个月扩张疗效满意。     

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.     

经内镜扩张治疗食管狭窄

摘要目的用内镜食管扩张治疗食管狭窄,介绍纤光内镜下扩张器的使用方法。方法用 Savary-Gilliard 探条和Key Med 扩张器,对食管狭窄病人进行内镜扩张。结果 45例食管狭窄病人,作了157次内镜扩张治疗。症状Ⅰ级改善13.3/(6/45),Ⅱ级改善4.4%(2/45),治愈率80%(36/45),总有效率97.8%(44/45),1例食管扩张不成功。全组未发生任何严重并发症和因食管扩张术而死亡者。结论认为这两种扩张器治疗食管狭窄,方法简便、安全、疗效确实,值得推广使用。     

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.     

先天性食道闭锁的转运和早期诊治

目的 探讨先天性食道闭锁的转运和早期诊断及治疗。方法 回顾性分析我院1999年9月至2003年11月收治的16例先天性食道闭锁患儿的外科资料。结果 16例中13例行手术治疗,3例放弃治疗,13例患儿中11例治愈,治愈率84．6％。术后随访6个月至3年,2例术后有食道吻合口狭窄,行球囊扩张治愈,其余9例进食良好。结论 合理的转运处理和早期诊断、及时的手术治疗对提高先天性食道闭锁手术治愈率有重要作用。     

新生儿先天性食管闭锁及气管食管瘘围术期的呼吸管理

目的探讨新生儿先天性食管闭锁及气管食管瘘围术期的呼吸管理,术中单肺通气(OLV)的临床应用及其安全性。方法 18例年龄6h～10d、体重1380～3100g、行食管闭锁及气管食管瘘食管端端吻合术患儿,麻醉诱导静脉注射阿托品0.01mg/kg、芬太尼2μg/kg、维库溴铵0.1mg/kg,面罩吸2%～4%七氟醚1min,气管插管尽可能插过气管食管瘘口。使用压力控制通气呼吸模式。麻醉维持吸入1%～3%七氟醚。术中压迫右侧肺,使其尽量萎陷,造成左肺OLV,调节呼吸参数,维持SpO2>90%。术中监测ECG、SpO2、PETCO2、BP、T。记录麻醉诱导前后、OLV10min、30min及术毕时的SpO2、PETCO2、HR、T。结果 OLV后所有患儿SpO2均有不同程度下降,适当调节呼吸参数,3例使用呼气末正压通气(PEEP),其中1例肺部感染重的患儿间隔恢复双肺通气,使SpO2维持90%以上。OLV30min,17例患儿SpO2较OLV10min时升高(P<0.05)。OLV10、30min时HR均较诱导后减慢。术中T维持在35.5～37.0℃。全部患儿术后安全返回病房。结论新生儿先天性食管闭锁及气管食管瘘围术期,采用单腔气管插管,术中人工肺萎陷法OLV麻醉时,恰当的呼吸管理,应用压力控制通气,七氟醚吸入维持麻醉是安全有效的。     

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.     

Tracheoplasty for congenital stenosis of the entire trachea

Congenital stenosis involving the entire length of the trachea has generally been regarded as a fatal disease. Tracheoplasty using costal cartilage grafts to enlarge the lumen was successfully employed in such a case, and the technique is described. A 12-mo-old female was referred with recurrent severe respiratory distress since birth. By tracheoscopy and bronchography, the entire trachea was seen to be stenotic. The left bronchus was of normal caliber by bronchogram and the left lung was over inflated, while the right lung was aplastic. Through a midsternal thoracotomy, the left bronchus was incised and cannulated for ventilation. Longitudinal incision of the entire length of the anterior wall of the trachea permitted the advance of a nasotracheal tube along the inner surface of the divided trachea to the carina. Two pieces of costal cartilage were used to fill the defect in the anterior wall of the trachea. The grafts were attached to the tracheal edges by interrupted 5-0 Dexon sutures. The endotracheal tube was successfully removed two months later. The subsequent course of the patient has been satisfactory.     

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.