Management of acquired tracheoesophageal fistula with various clinical presentations

Purpose

To determine the best therapeutic approach for acquired tracheoesophageal fistulae in childhood by evaluation of distinct clinical presentations requiring different surgical management based on our clinical experience.

Patients and Methods

Seven patients with acquired tracheoesophageal fistula seen between 1999 and 2010 were retrospectively studied with regard to the presenting findings, diagnostic evaluation, therapeutic approach, and outcomes.

Results

Five girls and two boys with a median age of 36 months (range, 2-156 months) were treated for acquired tracheoesophageal fistula. The presenting symptoms were respiratory difficulty (n = 3), coughing (n = 2), and dysphagia with coughing (n = 2), with a median duration of 30 days (range, 1-730 days). The etiologies were disc battery ingestion (n = 3), placement of endoesophageal prosthesis for caustic esophageal stricture (n = 2), corrosive ingestion with extensive burn (n = 1), and blunt chest trauma with subsequent emergency tracheotomy (n = 1). The site of the fistulae were proximal (n = 3) and middle (n = 1) trachea, left main bronchus (n = 1), and nearly the entire posterior wall of the trachea (n = 2). The patients were variously managed: conservatively with eventual spontaneous closure (n = 1), primary repair (n = 2), and colon interposition after cervical esophagostomy (n = 4) based on the clinical evaluation on admission and the follow-up status. Stenosis of the proximal esophagus (n = 2) and esophagocolonic anastomosis (n = 2) were the only complications encountered after treatment and were successfully managed with dilatation.

Conclusions

The best therapeutic approach for acquired tracheoesophageal fistula can be determined with careful consideration of relevant parameters on admission, including medical history, presenting findings, etiology, and characteristics of the fistula, in addition to the clinical evaluation in the follow-up period. In general, conservative management should precede definitive surgical intervention both to allow for possible spontaneous closure and also to achieve optimal preoperative status. Primary repair or a staged surgical approach can be best selected by giving priority to the patient's airway security.     

Endoscopic management of recurrent tracheoesophageal fistula

Rational

Recurrent tracheoesophageal fistulas (RTEFs) remain a therapeutic challenge because open surgical approaches have been associated with substantial rates of morbidity, mortality, and repeat recurrences. Recently, endoscopic techniques for the repair of RTEF have provided an alternative approach with the potential for improved surgical outcomes. However, previous reports have been limited by small patient numbers and variations in technique. By examining a single institution's experience and performing a systematic review of previously published results, we aimed to identify an optimal approach to managing RTEF endoscopically.

Methods

Retrospective chart review of patients undergoing endoscopic management of RTEF at a single tertiary care institution was performed. Medline search and summated analysis of previously published cases of endoscopically treated RTEF from 1975 to 2007 was conducted.

Results

Four patients with RTEF were identified and selected for endoscopic repair at our institution from 2003 to 2007 (mean age, 11.5 months). Under endoscopic guidance, fistula tracts were de-epithelialized with a Bugbee fulgurating diathermy electrode (5-15 W) and then sealed with fibrin glue (Tisseel with added aprotinin). Closure of RTEF was successful in 3 patients after a single attempt. One revision was required after inadvertent recannulation of the tract with an emergent tracheostomy tube change. No patient has evidence of recurrence (mean follow-up, 16 months). In 15 articles of endoscopically repaired RTEF, 37 cases have been reported from 1975 until present. In general, 3 approaches to endoscopic repair have been explored. Analysis of all reported cases in the literature and results from our patient series suggests that endoscopic techniques designed to both de-epithelialize the fistula tract and seal with fibrin glue have the best chance for cure after a single attempt. Patients with long, thin, and small diameter fistula who have enough distal trachea to accommodate a postoperative cuffed ventilating tube beyond the fistula are ideal candidates for endoscopic repair.

Conclusion

In select patients, endoscopic management of RTEF using Bugbee cautery and tissue adhesives can reduce morbidity and recurrence associated with open approaches and alternative endoscopic techniques.     

Morbid musculoskeletal sequelae of thoracotomy for tracheoesophageal fistula

The standard surgical approach for tracheoesophageal fistula (TEF) is right dorso-lateral thoracotomy. The late musculoskeletal consequences of the operation have been evaluated only rarely. Two hundred and seventy-seven patients with TEF were operated upon during the past 16 years, 117 of whom were available for long term (3 to 16 year) study. Twenty-nine of the patients had significant musculoskeletal deformities: (1) Twenty-one patients (23.8%) had prominent elevation of the right shoulder or "winged" scapula secondary to partial paralysis of the latissimus dorsi muscle; (2) Eighteen (20%) had marked asymmetry of the thoracic wall from atrophy of the serratus anterior muscle; (3) Nine (10%) had fusion of the ribs, in one of whom major respiratory dysfunction was a consequence; (4) Seven (7.8%) had severe thoracic scoliosis. The deformity was not of sufficient severity to warrant surgical correction but all patients required physiotherapy; (5) In two children (2.2%), fixation of the skin cicatrix to the bony thorax limited the mobility of the ipsilateral shoulder; (6) And finally, in three girls (3.3%), the thoracotomy scar disfigured the right breast leading to mammary maldevelopment in one adolescent. The latter child required plastic release of the entrapped breast. The dorso-lateral thoracic incision for tracheoesophageal atresia may lead to significant musculoskeletal complications and, since other alternatives are available, should be reevaluated as the recommended surgical approach.     

Triple fistula: management of a double tracheoesophageal fistula with a third H-type proximal fistula

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a relatively common congenital condition in which there have been several described anatomical variants. The most common type, EA with distal TEF, comprises more than 75% of cases in many reports. Less commonly, a smaller proximal pouch fistula (H-type) will be associated with this most common variant in 1.4% of these cases. Only 2% of all cases of EA/TEF will have 2 large fistulas between the trachea and esophagus in which the end of the upper esophageal pouch connects terminally to the midtrachea and the distal esophagus arises from the trachea near the carina. Here we describe the management of an infant with this type of EA/TEF who was also found to have an H-type TEF of the proximal trachea. The combination of this type of EA/TEF with an associated H-type TEF or “triple fistula” has been previously described in the literature in only 1 other patient.     

Repair of esophageal atresia with tracheoesophageal fistula via thoracotomy: a contemporary series

Background

A recent series detailing thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) reported lower complication rates compared with historic controls. This study provides a contemporary cohort of patients repaired via thoracotomy for comparison with the recent large multi-institutional thoracoscopic series.

Methods

Records of patients with EA/TEF between 1993 and 2008 were reviewed. Attention was focused on demographics and complications including anastomotic leak, recurrent fistulae, stricture formation, and need for fundoplication.

Results

Seventy-two patients underwent repair of EA/TEF via thoracotomy. Complication rates in the current series compared with the thoracoscopic series were anastomotic leak, 2.7% versus 7.6%; recurrent fistulae, 2.7% versus 1.9%; stricture, 5.5% versus 3.8%; and need for fundoplication, 12% versus 24%. Differences in complication rates did not reach statistical significance. Two children in this cohort developed mild scoliosis attributed to congenital vertebral anomalies, neither of whom required intervention.

Conclusions

Thoracoscopic repair of EA/TEF yielded complication rates similar to this contemporary series; however, trends toward increased anastomotic leaks and greater need for fundoplication were noted. No musculoskeletal sequelae were directly attributable to thoracotomy.     

External fixation of airway stents for upper tracheal stenosis and tracheoesophageal fistula

 Open in a separate windowOBJECTIVESStent migration is a common complication of airway stent placement for upper tracheal stenosis and tracheoesophageal fistula. Although several researchers have reported that external fixation is effective in preventing stent migration, the usefulness and safety of external fixation have not been proved because their cohorts were small. We therefore investigated the efficacy and safety of external fixation during upper tracheal stenting.METHODSRecords of patients who underwent airway stent placement from May 2007 to August 2018 in a single centre were retrospectively reviewed. We included only patients whose stent had been placed in the upper trachea with external fixation to the tracheal wall. The primary endpoint of this study was the rate of stent migration.RESULTSAltogether, 51 procedures were performed in 45 patients (32 males, 13 females; median age 60 years, range 14–91 years). The median follow-up period was 9 months (range 0.3–90 months). Among the procedures, 15 were performed for benign disease and 36 for malignancy. Stents were composed of either silicone (n = 42) or metal (n = 9). Stent migration occurred in 3 (6%) patients. The stents with migration were all composed of silicone. Other sequelae were granulation tissue formation in 10 (20%) patients, sputum obstruction in 6 (12%), cellulitis in 3 (6%) and pneumonia in 1 (2%).CONCLUSIONSExternal fixation was an effective method for preventing migration of airway stents placed for upper tracheal stenosis and tracheoesophageal fistula. The complications were acceptable in terms of safety.     

Platysma myocutaneous flap interposition in surgical management of large acquired post-traumatic tracheoesophageal fistula: A case report

INTRODUCTIONAcquired post-traumatic tracheoesophageal fistula (TEF) is an uncommon entity requiring early diagnosis. Among the many strategies in surgical management, we report a case successfully treated with a single-stage tracheal resection and esophageal repair with platysma myocutaneous interposition flap.PRESENTATION OF CASEA 24-year-old man had a motor vehicle accident with head injury and cerebral contusion who required mechanical ventilation support. Three weeks later, he developed hypersecretion, and recurrent episodes of aspiration pneumonia. The chest computed tomography, esophagogastroduodenoscopy, and bronchoscopy revealed a large TEF diameter of 3 cm at 4.5 cm from carina. Single-stage tracheal resection with primary end-to-end anastomosis and esophageal repair with platysma myocutaneous interposition flap was performed. A contrast esophagography was done on post-operative day 7 and revealed no leakage. He was discharged on post-operative day 10. Esophagogastroduodenoscopy at 1 month revealed patient esophageal lumen. At present he is doing well without any evidence of complications such as esophageal stricture or fistula.DISCUSSIONThere are many choices of myocutaneous muscle flaps in trachea and esophageal closure or reinforcement. The platysma myocutaneous flap interposition is simple with the advantage of reduced bulkiness. Concern on the vascular supply is that flap should be elevated with the deep adipofascial tissue under the platysma to ensure that the flap survival is not threatened.CONCLUSIONThe treatment of acquired TEF with platysma myocutaneous flap is an alternative procedure for a large uncomplicated TEF as it is effective, technically ease, minimal donor site defect and yields good surgical results.     

The role of preoperative CT scan in patients with tracheoesophageal fistula: A review

Purpose

The morbidity and mortality associated with esophageal atresia with or without a fistula make it a challenging congenital abnormality for the pediatric surgeon. Anatomic factors like inter-pouch gap and origin of fistula are not taken into consideration in various prognostic classifications. The preoperative evaluation of these cases with computerized tomography (CT) has been used by various investigators to delineate these factors. We reviewed these studies to evaluate the usefulness of this investigation in the intra operative and post operative period.

Materials and Methods

A literature search was done on all peer-reviewed articles published on preoperative computed tomography (CT) in cases of tracheoesophageal fistula using the PUBMED and MEDLINE search engines. Key words included tracheoesophageal fistula, computerized tomography, virtual bronchoscopy, and 3D computerized tomography reconstruction. Further, additional articles were selected from the list of references obtained from the retrieved publications. A total of 8 articles were selected for analysis.

Results

In most of the studies, comprising 96 patients, observations noted in preoperative CT were confirmed during surgery. In a study by Mahalik et al [Mahalik SK, Sodhi KS, Narasimhan KL, Rao KL. Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 2012 Jun 22. [Epub ahead of print]], in 6 patients the fistula could not be identified pre-operatively by CT. One study found a difference of 0.43 cm in the intraoperative gap length compared to that given by CT when compared to +/− 2 mm shown by another [1. Mahalik SK, Sodhi KS, Narasimhan KL, Rao KL. Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int. 2012 Jun 22. [Epub ahead of print], 3. Ratan SK, Varshney A, Mullick S, Saxena NC, Kakkar S, Sodhi PK. Evaluation of neonates with esophageal atresia using chest CT scan. Pediatr Surg Int. 2004 Oct; 20(10):757-61.]. Only three studies showed surgical utility of pre-operative CT, and the surgical plan changed based on the CT findings.

Conclusion

The usefulness of pre-operative computerized tomography in cases of tracheoesophageal fistula is controversial. The safety of this technique is questionable due to limited facilities and associated radiation hazards. Further, limited information obtained which may help in changing the surgical plan can easily be managed intra-operatively by careful and meticulous surgery.Overall, the safety concerns outweigh its efficacy. Thus, the use of pre-operative CT scan cannot be generalized and protocolized as a standard of care in the management of tracheoesophageal atresia.     

Esophageal atresia without distal tracheoesophageal fistula: high incidence of proximal fistula

Background

This retrospective study was performed to test our suspicion that the incidence of esophageal atresia with proximal fistula in our institution is much higher than is generally reported.

Methods

The charts of all patients with esophageal atresia and/or tracheoesophageal fistula admitted in the period 1982 to 2000 were analyzed. The type of atresia and/or tracheoesophageal fistula was noted, and the relative incidence was calculated and compared with the relative incidence in a cumulative series of 3492 patients taken from 9 published studies.

Results

In the period under study, 123 patients with esophageal atresia and/or tracheoesophageal fistula were identified. The relative incidence of esophageal atresia without distal fistula was statistically not different (10.6% in the present series against 8.49% in the reference group). A statistically significant difference in the relative incidence of esophageal atresia with proximal fistula, however, was found: 5.69% in the present series against 1.05% in the reference group (P < .0001). Looking at the subgroup of patients without a distal fistula, more than half of the patients did have a proximal fistula.

Conclusions

The relative incidence of esophageal atresia with proximal fistula in this series of children with esophageal atresia and/or tracheoesophageal fistula is significantly higher than reported in the literature. This is on the account of the subgroup of patients without a distal fistula in which the incidence of a proximal fistula is more than 50%. Especially in this subgroup, the existence of a proximal fistula should be ruled out preoperatively.     

Primary repair of tracheoesophageal fistula secondary to disc battery ingestion: a case report

The authors report on a child with tracheoesophageal fistula secondary to disc battery ingestion. Through a low cervical collar incision with limited sternal split, the fistula was primarily repaired, and the omohyoid muscle and thymus were mobilized to cover the suture lines. There are no signs of recurrent fistula 6 months after the operation. The anterior cervical approach with limited sternal split provided excellent access to the fistula. To the authors’ knowledge, this is the first report of successful primary repair of a tracheoesophageal fistula secondary to disk battery ingestion.     

Oral feeding outcomes in infants with esophageal atresia and tracheoesophageal fistula

Purpose

The purpose of this study was to explore oral feeding outcomes in infants born with type-C esophageal atresia and tracheoesophageal fistula (EA/TEF).

成人先天性食管呼吸道瘘的诊断和治疗

目的 探讨成人先天性食管呼吸道瘘的诊断和治疗经验.方法 回顾性分析1990年1月至2007年11月第四军医大学唐都医院收治的6例成人先天性食管呼吸道瘘的临床资料.本组患者均有饮水和(或)进流质饮食呛咳、咳嗽、咳痰的临床表现.确诊后行食管呼吸道瘘切断缝扎+食管修补+带蒂胸膜加固术.结果 结合病史和影像学检查6例患者均术前确诊,手术探查发现瘘管位于食管气管膜部3例,食管右肺下叶背段2例,食管左肺下叶内基底段1例.直径为0.3～1.0 cm,长度为0.2～1.5 cm.手术治疗效果良好.随访5个月至10年,未见复发.结论 对于反复发作的慢性咳嗽和肺化脓症要考虑到先天性食管呼吸道瘘的可能,综合临床表现及影像学检查可以确诊.手术治疗效果较好,无复发.     

Diagnosis of a proximal tracheoesophageal fistula using three-dimensional CT scan: a case report

Missed proximal tracheoesophageal fistulas (TEF) after repair of an esophageal atresia (EA) with distal TEF are rare. The diagnosis usually is made on the basis of bronchoscopy and esophagoscopy, which can be inaccurate. Recent advances in computerized tomography (CT) have allowed saggital, coronal, and 3-dimensional reconstruction of any structure or organ. The authors report a case in which 3-dimensional CT was used to assist in the diagnosis of a missed proximal TEF in a 16-year-old girl.     

Complications of the tracheal diverticulum after division of congenital tracheoesophageal fistula

In 3 patients, recurrent respiratory symptoms after neonatal repair of congenital tracheoesophageal fistula (Gross type C in 2, D in 1) were associated with a large tracheal remnant of the fistula termed diverticulum. Symptoms resolved after resection of the diverticulum in 1 child with airway obstruction and 1 adult with recurrent tracheoesophageal fistula.     

Esophageal atresia and tracheoesophageal fistula with distal esophageal stenosis: preoperative diagnosis

This is a presentation of a case of preoperative diagnosis of apparent esophageal atresia and an apparent distal and tracheoesophageal fistula from a peripheral hospital. After surgery, histopathologic findings showed the absence of cartilage and bronchial glands, suggesting the etiology as a fibromuscular stenosis.     

Feingold syndome: a rare but important cause of syndromic tracheoesophageal fistula

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are common congenital malformations and are associated with additional anomalies in approximately half of cases. Feingold syndrome is an important genetic cause of syndromic EA-TEF to consider in patients with associated microcephaly and digital anomalies. We present a case report of a male infant with EA-TEF, microcephaly, subtle facial dysmorphism, dysplastic kidney, short fifth fingers, second finger clinodactyly, and increased spacing between the first and second toes bilaterally. His clinical presentation was suggestive of Feingold syndrome, and genetic testing of the MYCN gene confirmed the diagnosis. Feingold syndrome is an autosomal dominant condition, and therefore, the diagnosis has important implications for genetic counseling.     

The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula

During repair of an esophageal atresia with tracheoesophageal fistula (EA/TEF), a right-sided aortic arch (RAA) presents a technical challenge. We reviewed our experience with EA/TEF focusing on the impact of RAA.

Methods

A retrospective review of patients with EA/TEF at our institution from 1990 to 2004 was performed. Of 61 patients, 53 had a left aortic arch (LAA) and 8 had RAA.

Results

The mean gestational age and birth weight were similar between patients with LAA and RAA. There were more vascular rings in patients with RAA than with LAA (37.5% vs 3.8%, P = .009). A preoperative echocardiogram correctly identified the location of the arch in all with LAA but only 62.5% of those with RAA. Operative complications occurred in 25% of patients with RAA and 11% of patients with LAA.

Conclusions

Management of a child with RAA and EA/TEF is technically challenging. Preoperative identification of the great vessel anomalies may impact operative decision making. Therefore, careful diagnostic evaluation of a patient with an aortic arch that cannot be easily defined by ECHO should be considered before definitive repair.     

A gasless abdomen does not exclude distal tracheoesophageal fistula: the value of a repeat x-ray

A “gasless abdomen” in babies with esophageal atresia (EA) has traditionally been thought to imply absence of a distal tracheoesophageal fistula (TEF). We present 3 neonates with a provisional diagnosis of EA, who had a gasless abdomen on an initial x-ray taken within the first 4 hours of life. These children were subsequently shown to have gas in the stomach associated with a distal TEF. Two children were examined on subsequent repeat x-ray of the abdomen, when gas was demonstrated in the stomach. Another child had a laparotomy for a gastrostomy when a distended stomach was found. This baby went on to have a thoracotomy and a routine repair of the distal TEF and an esophageal anastamosis. Based on our data of 65 cases of EA and distal TEF, it is recommended that an x-ray of the abdomen is repeated before surgery is undertaken if the gasless abdomen is documented during the first 4 hours of life.