Recurrent and acquired tracheoesophageal fistulae (TEF)—Minimally invasive management

Journal of Pediatric Surgery xxx (2017) xxx–xxx

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Recurrent and acquired tracheoesophageal fistulae (TEF)—Minimally invasive management Zafar Nazir ⁎, Muhammad Arif Mateen Khan, Javaria Qamar Section of Pediatric Surgery, Department of surgery, The Aga Khan University Hospital, Karachi 74800 (AKUH.K), Pakistan

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Article history: Received 4 January 2017 Received in revised form 9 March 2017 Accepted 18 March 2017 Available online xxxx Key words: Tracheoesophageal fistula Recurrent Acquired Foreign body ingestion Button battery Endoscopic management

a b s t r a c t Objective: Recurrent and acquired fistulae are a serious complication of congenital esophageal atresia and tracheoesophageal fistula (TEF) repair and foreign body ingestion (FBI) (e.g., button battery). We report our experience with a minimally invasive approach to recurrent and acquired TEF. Methods: Medical records of patients referred for management of recurrent and acquired TEF between 2003 and 2015 were reviewed retrospectively. Patients underwent endoscopic procedures (de-epithelization of fistulous tract and fibrin tissue adhesive-TisseelR) under general anesthesia. Results: Nine children (7 male, 2 female) with age range 3 months to 3 years (mean 1.5 year) were managed. TEF closed spontaneously in four patients, whereas in 5 patients the TEF closed after combined endoscopic procedure. Three patients required repeat endoscopic procedures. Follow-up ranged between 7 months to 10 years (mean 4.2 years). Conclusions: Active observation and repeat combined endoscopic procedures are safe alternatives to open surgical repair of acquired and recurrent TEF. Level of evidence: Level IV study. © 2017 Elsevier Inc. All rights reserved.

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are relatively common anomalies managed by pediatric surgeons (incidence 1:4500). Despite innovations in surgical repair and improvement in survival, morbidity remains high [1,2]. Recurrent TEF is reported in 3%–20% of patients following primary repair of EA/TEF [1,3,4]. Acquired TEFs are relatively rare in children and are usually caused by foreign body ingestion (FBI) (e.g., button battery) and iatrogenic trauma [5–8]. Management of children with recurrent and acquired TEF can be difficult. Endoscopic closure of the TEF has evolved as a viable alternative approach as open surgical procedures can be associated with significant morbidity, mortality and recurrence [3–5,9–12]. In this study, we report our experience with a minimally invasive approach to nine children who were referred for management to a university hospital in Southeast Asia. 1. Patients and methods 1. Nine children, age 3 month to 3 years (mean 1.5 years) were referred to the pediatric surgery Service at The Aga Khan University Hospital, Karachi, Pakistan between 2003 and 2015. After approval from Ethical Review Committee (ERC), medical records were reviewed ⁎ Corresponding author at: Section of Pediatric Surgery, Department of Surgery, The Aga Khan University, POB 3500, Stadium Rd., Karachi 74800, Pakistan. Tel.: +92 21 34863911, +92 300 2128878 (Cell); fax: +92 21 34862095. E-mail address: [email protected] (Z. Nazir).

for patient demographics, symptoms and signs, medical and surgical history, location and size of TEF, management and outcome. 2. Diagnosis of recurrent and acquired TEF was based on the presence of symptoms, contrast study of esophagus using water-soluble contrast material, and endoscopy of tracheobronchial and upper gastrointestinal tract. 3. Endoscopic closure of TEF was performed under general anesthesia using video-assisted rigid esophagoscopy and bronchoscopy. Probing with pediatric ureteric catheter (Cook Medical Inc. Bloomington, IN) assisted in identification and measuring the length of the fistula. Fistulous tract and surrounding granulation tissue were fulgurated with Bugbee diathermy electrode (ACMI Corp, South Borough, MA). The current settings varied between 8 and 15 W. After removal of secretions, 0.5–1.0 ml of Fibrin glue (Tisseel, Baxter Corporation, Ontario, Canada) was instilled in the fistulous tract with a 4F ureteric catheter. Excessive adhesive was removed promptly and an esophagoscopy was performed. Feeding was allowed 48-h after the procedure and advanced gradually. Patients were followed in clinic for symptoms of TEF and an esophagogram was performed 2 weeks or later (if needed) to confirm closure of fistula. 4. Case report: A 3-year-old girl, from a remote area of Pakistan, was referred with a 3-week history of feeding difficulty and respiratory distress. A radiograph and CT scan of the chest revealed a ballpoint pen in the esophagus with its tip directed toward the right (Fig. 1a, b). Rigid bronchoscopy revealed the tip of the pen encased by granulation tissue in the right main bronchus. Foreign

http://dx.doi.org/10.1016/j.jpedsurg.2017.03.048 0022-3468/© 2017 Elsevier Inc. All rights reserved.

Please cite this article as: Nazir Z, et al, Recurrent and acquired tracheoesophageal fistulae (TEF)—Minimally invasive management, J Pediatr Surg (2017), http://dx.doi.org/10.1016/j.jpedsurg.2017.03.048

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Z. Nazir et al. / Journal of Pediatric Surgery xxx (2017) xxx–xxx

Fig. 1. A 3-year-old presented with 15-day history of respiratory symptoms and feeding difficulty (a, b, c, d). Chest radiograph and CT scan show the tip (white arrow) and body of the ballpoint pen removed from the esophagus. (d) Contrast study of the esophagus showed fistula between the esophagus and right bronchus (black arrow).

body was disengaged by advancing it into the distal esophagus and removed (Fig. 1c, d). Granulation tissue was fulgurated and a feeding tube inserted. An esophagogram the next day revealed a fistula between the esophagus and the right main bronchus (Fig. 1e). Antibiotics, chest physiotherapy, and nutrition were initiated. Contrast study 8-weeks later showed closure of the fistula. The child is thriving and symptom free on 7-month follow-up. 2. Results The results are summarized in Table 1. Nine children (7 male and 2 female) with acquired (n = 6) and recurrent TEF following EA/TEF repair (n = 3) were managed. Acquired TEF developed 1 to 3 weeks

after removal of esophageal FB—20 mm lithium battery-Maxell 2032 (3), sharp metallic nail (1) ballpoint pen (1) and accidental acid ingestion (1). Most of the patients presented with a history of choking on feedings and recurring respiratory infections. The site of the fistula was mid trachea (5), distal trachea (3) and right main bronchus. Two patients (one each with recurrent and acquired TEF) had undergone unsuccessful open surgical repair before referral. All patients received broad-spectrum antibiotics, chest physiotherapy and nutritional support either through the feeding tube (n = 8) or through gastrostomy (n = 1). Acquired TEF in four patients closed spontaneously within 6 to 11 weeks while on observant management. Five patients (2 acquired and 3 recurrent TEF) had 1–3 endoscopic procedures. The size of the fistula varied from 2 mm to 4 mm. In two patients who had large

Table 1 Summary of patients. Patients

Age at presentation (months)

Etiology

Interventions

Outcome

Follow-up

1 2

15 20

Button battery Acid ingestion

Closed Closed

10 years 10 years

3

36

Button battery

Closed

8 years

4

30

Sharp metallic nail

Closed

8 months

5

20

Button battery

Closed

3 years

6 7

27 3

Ballpoint pen Congenital TEF repair

Closed Closed

7 months 3 years

8

6

Congenital TEF repair

Closed

3 years

9

9

Congenital TEF repair

Diagnostic endoscopy Diagnostic endoscopy Esophageal dilatation × 3 Diagnostic endoscopy Feeding gastrostomy Esophageal dilatation × 3 Diagnostic endoscopy Combined endoscopic procedure × 1 Diagnostic endoscopy Endoscopy + fulguration × 1 Combined endoscopic procedure × 2 Endoscopic removal of FB + fulguration of granulation tissue and fistula Combined endoscopic procedure × 2 Esophageal dilatation × 3 Endoscopy + fulguration of Fistula × 1 Combined endoscopic procedure × 2 Combined endoscopic procedure × 1

Closed

3 years

Please cite this article as: Nazir Z, et al, Recurrent and acquired tracheoesophageal fistulae (TEF)—Minimally invasive management, J Pediatr Surg (2017), http://dx.doi.org/10.1016/j.jpedsurg.2017.03.048

Z. Nazir et al. / Journal of Pediatric Surgery xxx (2017) xxx–xxx

acquired TEF (3–4 mm), fulguration of the fistulous tract and surrounding granulations prior to the definite endoscopic procedure resulted in reduction in the size and spontaneous closure of fistula. A concomitant esophageal stricture in 3 patients required dilatation (Savary-Gilliard Esophageal Dilators-Cook Medical Inc.). Ultimately, the TEF closed in all patients and the patients are symptom-free and thriving on followup ranging from 7 months to 10 years (mean 4.2 years).

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In conclusion, it is difficult to draw absolute conclusions from our limited but variable experience. We suggest customizing the management for each patient and exploiting minutiae of techniques reported in the literature. The endoscopic technique offers a safe alternative for management of recurrent and acquired TEF. Open surgical repair should be reserved for cases which cannot be managed by the minimally invasive approach.

3. Discussion References Recurrent TEF is reported in 3% to 20% of babies following repair of EA and TEF, despite refinements in the surgical technique and improvement in survival [1,3,4]. Acquired TEF is a rare entity in children, and is mostly caused by FBI (e.g., button battery), corrosive ingestion, iatrogenic injuries following tracheostomy as well as endotracheal intubation, and is rarely secondary to malignancy [5–8]. Over the last three decades, ingestions of button batteries have increased because of their easy accessibility (e.g., in toys, shoes and miniature electronic devices) [13,14]. The diagnosis is usually missed or delayed because of limited access to prompt and appropriate medical care, and the lack of high index of suspicion particularly in busy pediatric practices in the developing world. Button batteries in the esophagus cause damage (e.g., burn, stricture, perforation, TEF) by release of caustic substances, electrical current, and necrosis by direct pressure [9,14,15]. In our reported cases, the diagnosis was delayed for 1 to 3 weeks and the removed batteries were large (20 mm) and of poor quality. Acquired TEFs are more difficult to manage than the congenital variety because of variable etiology, delayed presentation, and associated complications [5–7,9,10,12]. A high index of suspicion in the presence of symptoms in a patient who had repair of congenital TEF or difficult removal of FB should prompt an esophagogram using water-soluble contrast. CT scan and MRI of chest can delineate the anatomy and damage to surrounding structures (e.g., trachea, aorta), and can facilitate therapeutic decisions after removal of the neglected FB [13,16]. Several therapeutic approaches are proposed in the literature to manage recurrent and acquired TEF [3–5,9–12,17]. Open procedures are associated with significant mortality (~3%) and morbidity (~6%–60%), and recurrence is reported in 10%–20% of cases [3–5,17]. Tissue friability and fibrosis at the site of the fistula preclude safe dissection, placement of sutures, and interposition of healthy tissues (e.g., pericardium, muscle) between the two suture lines. Similarly, techniques such as esophageal diversion followed by replacement are technically demanding [12]. Two patients in this report had recurrence of the TEF after open repair. Thus, many minimally invasive approaches are reported in the literature with enduring results [3,4,18,19]. Petri et al. [20] have reported successful closure of TEF following lithium disk battery ingestion with hyperbaric oxygen therapy. Optimization of the pulmonary and nutritional status of patients is imperative when planning intervention. We recommend 6–12 weeks of active observation as it may reduce the size of the fistula, improves the general condition of patient prior to endoscopic or surgical repair, and may lead to spontaneous closure of the fistula. Acquired TEF closed spontaneously in four patients while on observant management [21–25]. In mid 1970s, Gdanietz and Krause from Germany reported an endoscopic approach to recurrent TEF. Later, Drablos and Kohler extended the technique to H-type and traumatic TEF [26–29]. Since then, many modifications including de-epithelization of the fistulous tract with chemical sclerosants, mechanical abrasion, electrocautery or Nd-YAG laser followed by instillation of tissue adhesives (e.g., fibrin glue and plastic adhesives) are reported in the literature [26–29]. The reported experience of the endoscopic approach at any institution is limited to case reports and small case series. The overall long-term success rate ranges from 60% to 80% [3,4,18,19]. Repeat procedures can achieve almost 100% long-term success as demonstrated in our present report.

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Please cite this article as: Nazir Z, et al, Recurrent and acquired tracheoesophageal fistulae (TEF)—Minimally invasive management, J Pediatr Surg (2017), http://dx.doi.org/10.1016/j.jpedsurg.2017.03.048