Mechanisms of gastric distention in esophageal atresia with distal tracheoesophageal fistula

This paper explains how the chest of the crying infant with the common form of esophageal atresia is a pump for ventilating the distal esophagus and inflating the stomach. Esophageal inflation from the trachea occurs during inspiration and esophageal deflation into the stomach during cry.Dr. Arthur A. Siebens died in late January 1996, while this issue was in press.     

气道支架植入术在气道狭窄合并气道食管瘘中的应用

目的观察气道支架植入术在气道狭窄合并气道食管瘘中的应用及治疗效果。方法通过对16例食管癌伴气道狭窄合并气道食管瘘患者的定期随访,观察其在进行膜性支架气道内植入术后的情况和治疗效果。结果术后患者呼吸困难即刻明显缓解,进食呛咳消失。仅13例进行了定期随访,平均生存期为13.6个月,13例患者均行术后放射治疗,其中2例患者治疗中发生大咯血死亡,其余11例无进食后呛咳发生,无再发进行性呼吸困难。结论膜性支架治疗气道狭窄合并气道食管瘘,是一项对患者痛苦少、费用低、并发症少、有效的治疗方法 ,值得临床广泛应用。     

Recovery of esophageal peristalsis after congenital tracheoesophageal fistula repair in an adult

Summary Congenital tracheoesophageal fistulas are usually recognized during early life and are associated with severe esophageal dysmotilities that supposedly persist after fistula repair. We present the case of a patient with abdominal distension diagnosed to result from congenital tracheoesophageal fistula at the age of 17 years. Prior to surgery, manometry showed aperistalsis in the esophageal body that gradually improved during the 14 months of post-operative follow-up. We hypothesized that the dysmotility was due to increased gastroesophageal reflux or to intragastric distension. Therefore, we studied: (1) the correlation between motility and gastroesophageal reflux before and after surgery, and (2) the effect of isotonic gastric distension on esophageal motility. Our studies demonstrated that one year after surgery gastroesophageal acid reflux remained abnormal and that acute experimental gastric distension had no effect on esophageal motility. In summary, esophageal dysmotility in congenital tracheoesophageal fistula may return to normal after surgical repair; neither acute gastric distension nor gastroesophageal reflux are responsible for the reversible esophageal motor abnormalities.     

Late pulmonary function following repair of tracheoesophageal fistula or esophageal atresia

Although tracheoesophageal fistula and esophageal atresia (TEF-EA) are surgically correctable, late respiratory complications have been reported. We administered a respiratory and gastrointestinal symptom questionnaire and performed standard pulmonary function tests (PFT's) and methacholine challenge testing on an unselected group of 25 subjects with TEF-EA who underwent surgery at our institution between 1963 and 1985. Results were compared to predicted normals, as well as 10 sibling controls. While the mean values of lung function test results for the TEF-EA group were within the normal range, they were significantly different from their siblings. Thirteen of the 25 TEF-EA group (52%), but none (0%) of the controls, had abnormal pulmonary function. This was classified as restrictive in 9 (36%). obstructive in 3 (12%), and mixed in 1. In addition, airway hyperreactivity [defined as a positive methacholine challenge (PC₂₀ ? 8 mg/mL)], was found in 6 of 18 TEF-EA subjects and 4 of the 9 controls who were evaluated. Comparison of TEF-EA subjects with normal and abnormal PFTs showed no difference in the incidence of tracheomalacia, esophageal strictures or dilatation, recurrent pneumonias, or gastroesophageal reflux. The respiratory symptom score in the subjects and controls was similar, and did not correlate with abnormal pulmonary function. The cause of the pulmonary function abnormalities remains unexplained. Pediatr Pulmonol. 1995; 20:21–26 . © 1995 Wiley-Liss, Inc.     

Novel magnetic compression technique for establishment of a canine model of tracheoesophageal fistula

BACKGROUND Clinically,tracheoesophageal fistula(TEF)is lack of effective surgical strategies.One reason is due to the lack of appropriate animal models of acquired TEF,which is usually complex and difficult.Recently,the magnetic compression technique has been applied for digestive tract anastomosis or vascular anastomosis in animals.In this study,an animal model of TEF in dogs was developed by using the magnetic compression technique,hoping to provide a new method for mimicking TEF.AIM To establish a TEF model in dogs by using the magnetic compression technique.METHODS Six male beagles were used as models with two Nd-Fe-B permanent magnets for TEF.The parent magnet and the daughter magnet were placed in the cervical esophagus and trachea,respectively.The anterior wall of the esophagus and the posterior wall of the trachea were compressed when the two magnets coupled.After 4-6 d,the necrotic tissue between the two magnets fell off and the parent and daughter magnets disengaged from the target location,leaving a fistula.Gastroscopy/bronchoscopy,upper gastrointestinal contrast study,and histological analysis were performed.RESULTS The establishment of the TEF model in all six beagles was successful.The average time of magnet placement was 4.33±1.11 min(range,3-7 min).Mean time for the magnets to disengage from the target location was 4.67±0.75 d(range,4-6 d).TEFs were observed by gastroscopy/bronchoscopy and esophageal angiography.The gross anatomical structure of the esophagus and the trachea was in good condition.There was no esophageal mucosa or pseudostratified ciliated columnar epithelium at the site of the fistula according to histological analysis.CONCLUSION It is simple,feasible,and minimally invasive to use the magnetic compression technique for the establishment of the TEF model in dogs.     

Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography

Tracheoesophageal fistula (TEF) or bronchoesophageal fistula may be congenital, inflammatory, neoplastic, or secondary to trauma. Congenital TEF or bronchoesophageal fistula is usually associated with eso-phageal atresia and is readily diagnosed in infancy. But if it is not associated with esophageal atresia, it may persist until adulthood. Some theories have been proposed to explain this delay in diagnosis. We present a case of a 70-year-old man with congenital TEF. The TEF was successfully diagnosed by multidetector-row CT esophagography.     

Late manifestation of a large congenital tracheoesophageal fistula in an adult

Congenital tracheoesophageal fistula in an adult is a rare condition. We describe the clinical presentation in a young woman after video-assisted thoracoscopic pleurodesis for spontaneous pneumothorax. She was found to have a very wide and short fistula in her neck. The tracheoesophageal defect was closed by a trap-door flap, using the posterior wall of the trachea. The patient made an uncomplicated recovery.     

Esophageal perforation during pneumatic dilatation for achalasia: A possible association with malnutrition

Pneumatic balloon dilatation of the lower esophageal sphincter is commonly utilized as primary therapy for achalasia. Perforation related to pneumatic dilatation is uncommon (2–6%) but may result in severe morbidity. Factors associated with risk of perforation are not well defined. We noted perforation in three patients undergoing forceful balloon dilatation. All these patients had clinical evidence of significant malnutrition (recent marked weight loss and severe hypoalbuminemia). Malnutrition may be a causal factor for perforation in patients with achalasia undergoing dilatation.     

Swallow-Induced Syncope in a Patient with Achalasia

A 48-year-old man was referred for the evaluation of dysphagia and syncope. He suffered from both dysphagia and transient loss of consciousness when eating solid food, especially bread. An upper gastrointestinal barium examination and esophageal manometry suggested achalasia. Ambulatory ECG revealed marked sinus bradycardia when experiencing a syncopal episode following the ingestion of a solid meal. A permanent pacemaker was implanted and the patient’s syncopal attacks were relieved following this procedure. However, the dysphagia and cardiac arrhythmia completely disappeared only following a successful pneumatic balloon dilatation for achalasia. This observation suggests that swallowing-induced sinus bradycardia and syncope might occur in the course of achalasia and these rhythm abnormalities could be relieved by successful treatment of the achalasia.     

Y 型硅酮气管支架置入治疗恶性气管食管瘘临床体会并文献复习

目的：探讨 Y 型硅酮支架置入方法技巧及气管食管瘘(TEF)封堵的相关临床问题。方法复习我院 Y 型 Dumon 支架置入病例,分析操作准备、气管食管瘘封堵疗效及相关注意问题。结果确定气管支架直径[(横径+纵径)/2]并适形裁剪、打磨,硬质支气管镜下高频通气,支架由推送器经硬镜释放,配合可弯曲支气管镜,可成功置入,术后雾化吸入和随访复查,瘘口封堵良好,未见并发症。结论 Y 型 Dumon 支架对于隆突周围 TEF 疗效肯定、安全。充分病情评估、仔细测量瘘口大小,选择合适直径、医护密切配合是 Y 型硅酮支架置入重要环节,加强术后随访管理可一定程度上预防并发症。     

Congenital bronchoesophageal fistula and tracheoesophageal fistula with esophageal atresia.

A case of initial esophageal atresia and tracheoesophageal fistula in a female newborn, later complicated by pneumonia and a second bronchoesophageal fistula, is reported. She was treated surgically by closure of the tracheoesophageal fistula and by end-to-end esophago-esophageal anastomosis. An esophagram at 1 month of age was normal. Three months later she developed severe, persistent right lower lobe pneumonia that required intensive antibiotic therapy and respiratory support. Esophagography was repeated and revealed a second fistula between the right main-stem bronchus and the lower esophagus. The bronchoesophageal fistula was repaired, and a right lower lobectomy was performed. Postoperative recovery was uncomplicated. Histologic examination indicated that the fistula was congenital in origin. To the best of our knowledge, this is the first reported case of a congenital bronchoesophageal fistula coexisting with a tracheoesophageal fistula and esophageal atresia.     

Motility changes in primary achalasia following pneumatic dilatation

The changes in esophageal motility after pneumatic dilatation were evaluated prospectively in 51 patients with achalasia. The patients were evaluated for a median of 14 months. Pneumatic dilatation led to a clinical improvement in 41 patients. On manometric evaluation, a significant decrease in lower esophageal sphincter pressure was observed (28.4±14.9 mmHg vs. 13.5±7.2 mmHg; p=0.001); the resting pressure of the esophageal body dropped from 4.8±4.2 mmHg above gastric baseline to 0.1±3.9 mmHg below gastric baseline. After therapy, peristaltic activity was present in 10/51 (20%) patients; in 1 case, complete relaxation of the lower esophageal sphincter was recorded. Treatment-induced motility changes could not be predicted by clinical history or the lower esophageal sphincter pressure before or after therapy. However, the resting pressure of the esophageal body before and after therapy was significantly lower in these patients in whom peristalsis recurred after therapy than in patients with an unchanged motility pattern. The reappearance of peristaltic activity after pneumatic dilatation was unrelated to lower esophageal sphincter pressure. In conclusion, motility disturbances of the esophageal body in patients with achalasia do not simply reflect the functional obstruction of the lower esophageal sphincter. These findings support the hypothesis that achalasia is not a distinct motility disturbance but should be regarded as part of a broad spectrum of different interrelated esophageal motility disorders.     

Intermittent gaseous bowel distention: Atypical sign of congenital tracheoesophageal fistula

Three girls, 5‐, 9‐, and 15‐year‐old, were evaluated for recurrent airway infections and pneumonia. Chest X‐rays, which included the upper portion of the abdomen, showed marked gaseous bowels distention, while computed tomography scans of the chest demonstrated the presence of tracheoesophageal fistula (TEF), confirmed by fiberoptic bronchoscopy. Abdominal gaseous distension, a known possible clinical manifestation of TEF in the neonatal period generated by airflow through the fistula into the oesophagus, has not been reported as a clue to the diagnosis in older children. When detected in patients with recurrent respiratory infection, should raise the suspicion of unrecognized TEF. Pediatr Pulmonol. 2009; 44:244–248. © 2009 Wiley‐Liss, Inc.     

Unilateral bronchiectasis and esophageal dysmotility in congenital adult tracheoesophageal fistula

Tracheoesophageal fistulas (TEF) in adults are most commonly neoplastic, and very rarely congenital in nature. We report a 45-year-old Hispanic male with TEF and initial presentation of minimal hemoptysis. The patient had radiographic evidence of unilateral upper lobe (RUL) bronchiectasis, massive esophageal dilatation, and dysmotility. However, there was no evidence of esophageal malignancy, achalasia, or Chagas' disease. Bronchoscopy revealed a large TEF in the posterior wall of trachea, which was not visualized on esophagram or esophagoscopy. Bronchoalveolar lavage (BAL) cultures grew Mycobacterium avium complex (MAC). Our report illustrates that idiopathic, or congenital, TEF can be associated with esophageal dysmotility, adulthood bronchiectasis, and atypical mycobacterial superinfection.     

Y型一体化自膨式覆膜气道金属支架在胸腔胃-气道瘘中的应用

目的 探讨Y型一体化自膨式覆膜气道金属支架(Y型支架)在胸腔胃-气道瘘治疗中的安全性和疗效.方法 回顾性分析2003年4月到2014年10月郑州大学第一附属医院放射介入科收治的108例胸腔胃-气道瘘患者临床及影像学资料,其中男90例,女18例,年龄41 ～75(60±10)岁.根据瘘口的位置、大小及相关气道直径,个体化设计1～3枚Y型支架(其中跨越隆突区域放置的支架简称大Y支架,跨越叶支气管分叉处放置的简称小Y支架).在X线监视下行病变处气道内支架置入,其中单一大Y支架置入65例、单一小Y支架置入16例、大Y+小Y支架组合置入26例,大Y+双小Y支架组合置入1例.结果 所有患者Y型支架均一次置入成功,104例术后即刻气道和食管造影均未见对比剂进入对侧,瘘口封堵完全,4例置入单Y支架后瘘口封堵不严,后改为大Y+小Y支架置入并封堵完全;术后患者卧位刺激性呛咳症状消失,可正常进食水,生活质量明显提高.术后92例患者获得完整随访,其中59例患者于术后3.1 ～9.0个月死亡,死因包括晚期肿瘤消耗(38例)、恶病质进展(14例)、肺部感染(5例)以及大咯血(2例);4例患者单Y支架置入2 ～10个月后瘘口增大,再次给予1枚Y型支架组合置入并证实瘘口封堵完全;余33例患者存活,生活质量满意.结论 Y型一体化自膨式覆膜金属支架气道内置入能有效封堵胸腔胃-气道瘘,技术可行,近期疗效可靠,能显著提高患者生活质量.     

Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia

We report a 19‐year‐old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood. Pediatr Pulmonol. 2010; 45:202–204. © 2010 Wiley‐Liss, Inc.     

Esophageal motility,heartburn, and gastroesophageal reflux: Variations in clinical presentation of esophageal dysphagia

Dysphagia is a potentially important symptom, often leading to the finding of an anatomical or motility disorder of the esophagus. Dysphagia and heartburn represent two of the most common symptoms associated with esophageal motility disorders. To explore the relationship of symptomatic esophageal dysphagia and heartburn and their association with primary esophageal motor disorders, we have performed a retrospective assessment of 1035 patient evaluations performed at our gastrointestinal laboratory. A clear statistical association of symptomatic dysphagia and heartburn was established; however, no pattern diagnostic of a specific motility disorder was discernible. A sizable fraction of our patient population with dysphagia demonstrated normal esophageal motility. A significant portion of dyspeptic patients exhibited both normal motility and acid exposure. The differences observed between the incidence of subjective symptoms and objective dysfunction may be explained in part by an altered or increased esophageal sensitivity of these patients.     

Long‐term respiratory complications of congenital esophageal atresia with or without tracheoesophageal fistula: an update

Despite early surgical repair, congenital esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) has long‐term effects on respiratory and gastrointestinal function. This review updates summarizes research published since 2003 on long‐term respiratory complications in patients with a history of EA ± TEF. Pulmonary hypoplasia appears to not be rare in patients with EA ± TEF. Tracheomalacia is common and is associated with respiratory symptoms in childhood. Aspiration, associated with esophageal dysmotility and/or gastroesophageal reflux, may lead to reduced pulmonary function and bronchiectasis. Pulmonary function is generally normal, although lower than in control patients, and restrictive defects tend to be commoner than obstructive defects. Abnormal airway reactivity is common and, along with respiratory symptoms, is associated with atopy. However, the inflammatory profile in EA ± TEF patients based on bronchial biopsies and exhaled nitric oxide differs from typical allergic asthma. Recent studies suggest that in older patients, respiratory symptoms tend to be associated with atopy, but abnormal lung function tends to be associated with gastroesophageal reflux and with chest wall abnormalities. Early detection and management of aspiration may be important to help prevent decrements in pulmonary function and serious long‐term complications in EA ± TEF patients.     

Medical treatment of esophageal motility disorders

Swallowing is a complex mechanism that is based on the coordinated interplay of tongue, pharynx, and esophagus. Disturbances of this interplay or disorders of one or several of these components lead to dysphagia, non-cardiac chest pain, or regurgitation. The major esophageal motility disorders include achalasia, diffuse esophageal spasm, hypercontractile esophagus (nutcracker esophagus), and hypocontractile esophagus (scleroderma esophagus). Other esophageal diseases such as hypopharyngeal (Zenker's) diverticula or gastroesophageal reflux disease also may be sequelae of primary esophageal motility disorder. Finally, a substantial group of patients referred for evaluation of possible esophageal motor disorders have milder degrees of dysmotility—referred to as nonspecific esophageal motor disorder—that are of unclear clinical significance. Medical treatment of esophageal motility disorders involves the uses of agents that either reduce (anticholinergic agents, nitrates, calcium antagonists) or enhance (prokinetic agents) esophageal contractility. Despite the beneficial effect of the various drugs on esophageal motility parameters, the clinical benefit of medical treatment is often disappointing. From clinical and epidemiological studies there is some evidence for a psychological component in the pathogenesis or perception of esophageal symptoms. Further understanding of esophageal pathophysiology, as well as development of new receptor selective drugs, might increase our chances of successful treatment of esophageal motility disorders.