H-type Tracheoesophageal Fistula Research Articles

Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

Management of a double H-type tracheoesophageal fistula without esophageal atresia: a case report

BackgroundDouble congenital tracheoesophageal fistula (TEF) without esophageal atresia, known as double H-type fistula, is an extremely uncommon malformation. Due to its nonspecific symptoms and the small size of the fistula in infancy, H-type tracheoesophageal fistula is rarely diagnosed in the neonatal period and is also challenging to repair.Case presentationWe report a rare case of double H-type tracheoesophageal fistula in a 2-day-old newborn, identified with a time-lapse and repaired separately. The diagnosis was established through repeated bronchoscopy, with methylene blue injected through an esophagoscope. A right thoracotomy was performed to undertake the surgical repair after cannulating the fistula with a vascular guide wire. Five months later, the infant, who had a history of recurrent episodes of coughing and shortness of breath, was diagnosed with acute respiratory distress, and a second TEF was detected by bronchoscopy. A cervical incision was used to repair the second H-type fistula.ConclusionsDouble H-type tracheoesophageal fistula should be considered while correcting a tracheoesophageal fistula, especially if the symptoms continue after the first operation. Repeated radiological contrast studies and bronchoscopy with methylene blue injected through an esophagoscope help the early diagnosis.

Retracted: Analysis of the Application Value of Different Esophagography Techniques in the Diagnosis of H-Type Tracheoesophageal Fistula in Neonates.

[This retracts the article DOI: 10.1155/2022/7264343.].

Isolated tracheo-esophageal fistula in children: Analysis of a large multicentric series and proposal of a comprehensive treatment algorithm

BackgroundIsolated tracheo-esophageal fistulas (TEF) are rare and challenging, possibly leading to severe complications. We evaluated the outcomes in a large series of patients treated in 9 referral Centers for airway surgery, and suggested an ideal management algorithm. MethodsA multicentric retrospective study on TEF was performed. Results186 cases were collected. Among 101 patients with recurrent TEF after EA repair, 1 was treated successfully with thoracoscopy, 23 with open surgery with TEF division and tissue interposition, with 15 complete resolution (65 %), 2 TEF recurrence (9 %) and 6 other complications (26 %), and 77 received TCA endoscopic cauterization, with complete resolution in 61 (79 %). Among 54 patients with congenital H-type fistula, 1 received conservative treatment with resolution; 4 underwent thoracoscopy (50 % resolution); 6 had endoscopic cauterization (33 % resolution); 44 received open surgery, with 43 (98 %) successes. In the last group, the majority of TEF was ligated and divided with tissue interposition. Among 26 post-traumatic fistulas (mechanical trauma, battery or caustic ingestion), 6 patients received endoscopic procedure and 5 of them (83 %) reached complete resolution. The other 19 received open surgery (trans-tracheal direct FTE closure with tracheal resection), and 12 had complete resolution (63 %). Two patients eventually died and complication rate was 23 %. Other 5 cases (3 Bronchial-Esophageal Fistulas and 2 TEF after cleft repair) were treated endoscopically or with open approach. ConclusionEndoscopic cauterization can be the preferred treatment for recurrent TEF after EA, while for the congenital H-type and post-traumatic TEF the open approach remains our first choice.

ОПИСАНИЕ СЕРИИ КЛИНИЧЕСКИХ НАБЛЮДЕНИЙ ТОРАКОСКОПИЧЕСКОГО ЛЕЧЕНИЯ ВРОЖДЕННЫХ ИЗОЛИРОВАННЫХ ТРАХЕОПИЩЕВОДНЫХ СВИЩЕЙ Н-ТИПА У МЛАДЕНЦЕВ

Isolated H-type tracheoesophageal fistula is a rare anomaly of the respiratory tract which accounts for 4% of all malformations of the trachea and esophagus. Authors report patients with this developmental anomaly who underwent fistula dissection by the same single surgeon and at different surgical facilities with particular emphasis on preoperative diagnosis and treatment. Thoracoscopic transsection of H-type tracheoesophageal fistula was performed by a single surgeon in 5 patients (3 boys and 2 girls) with R. Gross congenital isolated type E fistulas who were admitted at 2 to 12 weeks of age. The patients were in three surgical facilities located in the cities of Irkutsk and Tomsk (both - Russia), Tashkent (Republic of Uzbekistan). In the final part the methods of preoperative examination, the parameters of the surgical intervention and the results of postoperative observation are given. All 5 procedures were successfully performed thoracoscopically without conversion into open intervention. The duration of the surgical intervention varied from 45 to 135 minutes. The average intervention period was 78.0±35.8 minutes (median - 60.0 [52.5; 112.5] minutes). The mean duration of mechanical ventilation in this group was 33.6±10.0 hours (median - 36.0 [24.0; 42.0] hours) with the longest interval being 48 hours in a patient with pre-existing respiratory distress. The exact time range to complete oral nutrition has been well documented and ranged from 5 to 8 days. Esophageal and tracheal suture failure was not recorded in patients with H-type tracheoesophageal fistula. Two patients required Nissen surgery for hiatal hernia at 6 months and 1 year after initial H-type tracheoesophageal fistula ligation. All patients are currently on full oral nutrition. The follow-up period in this group ranged from 12 to 60 months. These patients showed no signs of recurrence of the fistula. Patients were evaluated for vocal cord paresis if clinical stridor was observed postoperatively. However, no recurrent nerve injury was found in any of the patients in this series of cases. Authors did not record signs of chest asymmetry, pterygoid scapula, laxity of the shoulder girdle or clinically significant scoliosis. Thoracoscopy allows direct visualization and dissection of an H-type tracheoesophageal fistula. Authors believe this technique allows achieving better mobilization of the posterior mediastinal organs than in thoracotomy, which probably expands the scope of thoracoscopic access in the treatment of this disease.

A dual H-type tracheoesophageal fistula; why not being repaired simultaneously? A case report and review of literature

BackgroundH-type Tracheoesophageal Fistula (TEF) is a particular type of congenital esophageal anomalies, in which patients present with non-specific symptoms that can result in delayed diagnosis. Here, we report two pediatric cases with a rarer variant called ‟dual H-type TEFˮ.Case presentationWe present two cases of H-type TEF. The first was a 45-day-old boy with feeding problem and cyanosis while feeding, and the second was a three-month-old girl with cough and choking after feeding from the first day of birth. In both cases, two separate TEFs were detected during diagnostic evaluation by flexible bronchoscopy. Both were repaired simultaneously through a cervical incision. The first patient deteriorated 13 days after the surgery, disturbancing in acid-base balance and expired unfortunately.ConclusionHence, it is necessary to consider the possibility of double TEF in any newly diagnosed H-type TEF.

Endoscopic treatment in pediatric patients with recurrent and H-type tracheoesophageal fistulas - A systematic review and meta-analysis.

Endoscopic treatments for managing recurrent tracheoesophageal fistula (rTEF) and H-type TEF are being utilized lately; however, the preferred technique is yet to be determined. We aimed to systematically review existing publications on endoscopic treatment of rTEF and H-type TEF to analyze their success and complication rates. PRISMA guidelines were followed. MEDLINE, EMBASE, CINAHL and the Cochrane Central Register of Controlled Trials were comprehensively searched in accordance to a priori developed protocol, from 1975 until 2020. English, Spanish and German papers were included. Studies were independently screened and analyzed by two reviewers. 84 full texts were assessed for eligibility out of 581 screened studies, of these, 39 studies with 127 patients were eligible for inclusion (115 rTEF and 12H-type TEF). All included studies were cases reports or case series. Overall success rate was 45% with sealant injection, 87% with de-epithelialization and 80% with sealant injection and de-epithelialization combined. The mean number of required treatments for success was 1.9 (range 1-6). Mixed effect model meta-analysis of case series with n>1 showed that sealant injection had a significantly lower success rate of 50% (95% CI 1-99%, I2 72%) compared to de-epithelialization 90% (95% CI 72-99%, I2 27%), p=0.007 and the combination of both techniques 87% (95% CI 68-99%, I2 11%), p=0.02. Nine patients (7%) had transient respiratory distress. No mortalities reported. Endoscopic treatment for rTEF and H-type fistula is a minimally invasive technique with favorable outcome and considerably less morbidity compared to open surgery, suggesting it as a safe and effective first line treatment option. Repeated endoscopic treatment attempts can be expected to obtain complete closure. De-epithelization techniques with or without combined tissue adhesive injection had significantly better results than sealant injection techniques alone.

11 Cases of H-Type Tracheoesophageal Fistula: How Diagnosis and Treatment May Be Improved

11 Cases of H-Type Tracheoesophageal Fistula: How Diagnosis and Treatment May Be Improved

Management of congenital isolated H-type tracheoesophageal fistula

Management of congenital isolated H-type tracheoesophageal fistula

Analysis of the Application Value of Different Esophagography Techniques in the Diagnosis of H-Type Tracheoesophageal Fistula in Neonates.

Objective The study aimed to analyse the detection rates of the triple-lumen double-balloon catheter technique and conventional esophagography in diagnosing H-type tracheoesophageal fistula (H-TEF) in neonates. Methods The data of 8 neonates diagnosed with H-TEF by surgery in the researchers' hospital between January 2015 and January 2022 were collected. We compared the detection (true positive) rates of H-TEF by the triple-lumen double-balloon catheter technique, conventional esophagography, and multidetector row spiral CT. Results Before surgery, conventional esophagography was applied in all 8 cases, of which the H-TEF diagnosis was confirmed in 5 cases and TEF was suspected in 3 cases. The triple-lumen double-balloon catheter technique was employed in 5 cases, of which 4 were confirmed with H-TEF and 1 was suspected with TEF. Multidetector row spiral CT was performed in 4 cases, and 1 case was confirmed with H-TEF, while no fistula was observed in the other 3 cases. The triple-lumen double-balloon catheter technique yielded a 100% detection rate, while conventional esophagography revealed a 62.5% rate and multidetector row spiral CT showed a 25% rate. By comparative analysis, the true positive rates (TPRs) of the triple-lumen double-balloon catheter technique and conventional esophagography were not significantly different (P=0.118). No significant differences were recorded in TPRs between conventional esophagography and multidetector row spiral CT (P=0.221). However, the triple-lumen double-balloon catheter technique had a significantly higher TPR than multidetector row spiral CT (P=0.118). Conclusion To diagnose congenital H-TEF in neonates, conventional esophagography is a highly valuable yet inconsistently reliable method and the diagnostic value of CT is relatively limited. The triple-lumen double-balloon catheter technique boasts a significantly valuable application for H-TEF diagnosis. Being simple, economical, and effective, it barely requires state-of-the-art facilities, and no complications have occurred during its clinical practice. These advantages justify a possible wider application of the triple-lumen double-balloon catheter technique in clinical practice.

Newborn with annular pancreas and H-Type tracheoesophageal fistula

Esophageal atresia with tracheoesophageal fistula (EA/TEF) and annular pancreas are both rare congenital anomalies, with very few reports of neonates presenting with both. More specifically, there are no reported cases of neonates with both annular pancreas and the rarest form of TEF, the H-type fistula. Here, we present the case of a neonate with annular pancreas diagnosed at birth requiring duodenoduodenostomy with delayed diagnosis of concomitant H-type TEF requiring subsequent fistula ligation with favorable outcomes. When patients with known congenital anomalies have persistent symptoms of unidentified etiology, heightened suspicion for other concomitant congenital anomalies can lead to prompt diagnosis, timely surgical management and good outcomes.

Flexible endoscopic identification and catheterization of congenital H-type tracheoesophageal fistula using a laryngeal mask.

Endoscopy for revealing the orifice of congenital H-type tracheoesophageal fistula (cTEF) is important for diagnostics and therapeutics. To facilitate the identification and catheterization of cTEF, we developed a new modified flexible endoscopy technique using a laryngeal mask with intermittent airflow. A retrospective case series study was conducted from April 2016 to July 2019 at a national regional children's medical center. Twelve infants with cTEF underwent this flexible endoscopy technique. The intermittent positive pressure airflow through laryngeal mask was able to reveal the orifice of cTEF easily in tracheal lumen. Under the visual flexible endoscope, cannulation with a 3-Fr ureteral catheter in fistula was successfully used in all cases. There were no immediate or delayed complications. This case series shows that the flexible endoscopy technique is a safe, easy, and technically efficient approach for diagnosis and cannulation of cTEF.

Repair of congenital H-type tracheoesophageal fistula by electrocautery

Congenital H-type tracheoesophageal fistula is a rare type of congenital tracheoesophageal malformation. In this malformation, the esophagus remains unobstructed, complicating and delaying the diagnosis. We present our experience with three infants with congenital H-type tracheoesophageal fistula who were initially treated by a minimally invasive approach via endoscopy with electrocautery. Two of the three patients ultimately required a surgical procedure after failure to close the fistula via electrocautery despite multiple attempts over several months. One of the three patients was successfully treated via electrocautery after the second procedure. In our center, three infants with congenital H-type tracheoesophageal fistula were initially treated endoscopically with electrocautery. Two out of the three ultimately required a surgical procedure. If considering endoscopy with electrocautery, providers and families should recognize that the procedure may need to be performed several times before the fistula is successfully closed. Additionally, failed attempts may put patients at risk for complications such as respiratory decompensation, pneumonia and poor growth and may prolong hospitalization. • Congenital h-type tracheoesophageal fistula closure with endoscopic electrocautery. • 2 of 3 patients ultimately required surgical repair. • 1 of 3 patients was successfully treated with electrocautery via endoscopy. • Patients requiring surgical repair had complications after endoscopy.

H-Type Tracheoesophageal Fistula Masquerading as Grade V Gastroesophageal Reflux in a Neonate

Tracheoesophageal fistula (TEF) without associated esophageal atresia is a rare congenital anomaly. Diagnosis in neonatal period is usually not made and most of the patients are treated as cases of pneumonia. We report a case of H-type TEF, which was initially diagnosed as grade V gastroesophageal reflux on contrast esophagogram and bronchoscopy done revealed H-type fistula. Through cervical approach, fistula was repaired and baby had uneventful postoperative outcome. High index of clinical suspicion and early diagnosis can provide a better prognosis.

Flexible Bronchoscopy Diagnosis of Uncommon Congenital H-type Tracheoesophageal Fistula, Dual Fistulae, Bronchoesophageal Fistula, and Recurrence of Fistula in Children: A 20-year Experience.

Interventional pediatric flexible bronchoscopy has many advantages over radiologic investigations in diagnosing uncommon congenital H-type tracheoesophageal fistula (TEF), dual TEF, bronchoesophageal fistula (BEF) and fistula recurrence including higher rates of identification and anatomic localization with guide wire cannulation. We compare the diagnostic utility of flexible bronchoscopy to radiologic techniques for congenital aerodigestive fistula. A single center retrospective review was completed of all cases of pediatric TEF and BEF diagnosed with flexible bronchoscopy between January 2000 and November 2020. Fistulae were diagnosed 21 times in 18 patients at a median age of 1.22 years (interquartile range: 0.50 to 2.99). The median time from diagnosis to repair was 17.5 days (interquartile range: 5.5 to 43). Symptoms commonly related to fistula were found in all patients. Uncommon fistulae included single H-type TEF (n=10, 47.6%), dual H-type TEF (n=2, 9.5%), dual proximal and distal TEF with esophageal atresia (n=5, 23.8%), TEF recurrence (n=2, 14.3%), BEF (n=1, 4.8%), and a BEF recurrence (n=1, 4.8%). Flexible bronchoscopy confirmed the diagnosis in all fistulae using a guide wire cannulation or methylene blue dye injection. A combined procedure with simultaneous bronchoscopy and esophagoscopy was used for 6 fistulae. The positive examination rate was 75% for bronchoscopy compared with 2.6% for contrast swallow studies and 28.6% for tube esophagograms. Flexible bronchoscopy should be considered as a first line investigation in uncommon aerodigestive fistulae. In the absence of a skilled bronchoscopist, the best radiologic investigation is a pull-back tube esophagogram but may still require endoscopic confirmation at the time of fistula repair.

Videofluoroscopic Swallow Study in Diagnostics of H-Type Tracheoesophageal Fistula in Children.

Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. It can occur with or without tracheoesophageal fistula (TEF), and to date, there are still no universally recommended diagnostic procedures. The so-called H-type TEF is that without esophageal atresia, and its prevalence is lower than 5% of all TEFs. We present a case report of a newborn with regurgitation, vomiting, feeding problems, dyspnea, and repeated aspiration bronchopneumonia. A wide range of diagnostics procedures had been performed with negative results until we used videofluoroscopy, which revealed the H-type TEF and allowed appropriate treatment of the patient.

The modified Nadeem’s technique for the treatment of H-type tracheoesophageal fistula

An H-type fistula is an unusual category of the tracheoesophageal fistula to occur constituting only 5 % of all cases. The continuity of the esophagus leads to a delayed and vague presentation with a complaint of choking on feed and respiratory issues unlike the inability to feed and frothing soon after birth in other variants of tracheoesophageal fistula. We received a referred case of a 3-months-old male having recurrent respiratory tract infections and episodes of choking. After a thorough evaluation, a suspicion of H-type fistula was encountered that led to refined radiological evaluation. Contrast given per oral also delineated fistula and CT scan with intravenous and oral contrast revealed fistulous communication between two channels. Operated for H-type fistula through the modified Nadeem’s technique via a left cervical approach and discharged after uneventful recovery. The modified Nadeem’s technique via a left-sided cervical approach facilitates access, vision, and intervention easily as compared to the conventional right-sided approach given in the literature.

Congenital and acquired tracheoesophageal fistulas in children

Tracheoesophageal fistulas (TEF) are an anomalous communication between airway and esophagus. There are several types of TEF. Congenital are mainly associated to an esophageal atresia. The type III or C, in which the upper segment of the esophagus ends in a blind pouch and there is distal tracheoesophageal fistula above the carina, accounts for 85% of esophageal atresias. The other are extremely infrequent. H-type or N-type TEF, classified as type 5 or E, is an uncommon variant and accounts for less than 4%.Recurrent TEF is a serious complication after first surgery of esophageal atresia and TEF. The rate of recurrence of TEF is estimated between 3-15%. The treatment is a challenge with a high rate of recurrence after surgery.Classical symptoms of RTEF include coughing especially after drinking, abdominal distension, repeated cyanosis, and respiratory infections. In the case of H-type fistula the symptoms are similar but appear during the first month of life.In this chapter we presented the management and alternative treatments of the congenital and acquired TEF.

Double H-type tracheoesophageal fistula

Abstract Congenital ‘isolated’ tracheoesophageal fistula (TEF) is a rare variant of esophageal atresia, also termed H-type TEF. Even rarer is a double H-type TEF. Here, we report a two-week-old female, with double H-type TEF – one identified pre-operatively, the other during surgery. Pre-operative laryngotracheobronchoscopy (LTB) with guidewire cannulation is considered key to definitive TEF repair, to aid in both pre- and intra-operative identification of the fistula(e). In our experience, only one H-type TEF was identified at the planned pre-operative LTB, with the second H-type fistula identified at the time of cervical dissection. Both fistulae were repaired during the same operation, via cervical approach. This represents the first reported case of a double H-type TEF, repaired primarily via a single cervical incision.

Thoracoscopic repair of congenital isolated H-type tracheoesophageal fistula

Background/purposeThe available literature on congenital isolated H-type tracheoesophageal fistula (TEF) is limited, and preferred approach varies among centers (cervicotomy, thoracotomy, thoracoscopy). We aimed to present one of the biggest case series of thoracoscopic approach for congenital isolated TEF and to assess the method's feasibility and outcomes. MethodsRetrospective review of thoracoscopic TEF repair experiences at 2 European university pediatric surgery centers. Results9 boys and 3 girls were involved in the study (age 5 days–4 years), 8 of them were newborns (mean birth weight 3013 g, mean gestational age 39 weeks). The most common presenting symptoms were desaturations on feeding in neonates and recurrent respiratory tract infections in older children. The diagnoses were established on contrast study and confirmed with rigid bronchoscopy. The fistulas were located at or below the thoracic inlet; the fistulas were 2 cm above the carina to half the height of the trachea. All patients underwent successful thoracoscopic TEF repair. There were no conversions. The postoperative course was uneventful in all but one who had rethoracoscopy for prolonged postoperative chylothorax. All patients had satisfactory vocal cord function. One patient required reoperation for fistula recurrence 8 months after primary surgery. ConclusionThoracoscopic approach for isolated H-type TEF seems to be possible as a procedure of choice with satisfactory results and all benefits of minimally invasive procedure. Level of evidenceIV (case series).