Current management of type III and IV laryngotracheoesophageal clefts: the case for a revised cleft classification.

PURPOSE OF REVIEW: This review summarizes the paediatric laryngotracheoesophageal cleft (LTEC) literature, with an emphasis on recent trends, evaluation and management, surgical techniques, postoperative care of Type III and IV LTECs, and to propose a revised cleft classification system that more accurately reflects our current understanding of these anomalies. RECENT FINDINGS: There are a number of techniques described to address Type III and IV LTEC, from endoscopic to open approaches with thoracotomy. The surgical approach should be tailored to the length of the cleft and its proximity to important anatomical structures. On the basis of review of the literature, we propose a modified Benjamin-Inglis classification (MBI) with subcategories to address this issue. Postoperative complications are common, namely, tracheoesophageal fistulae and tracheomalacia, which may necessitate subsequent procedures or prolonged tracheostomy dependence. SUMMARY: The medical and surgical management of Type III and IV LTEC is challenging with a high rate of morbidity and mortality. The rarity and difficulties in management of these malformations have made large cohort studies difficult, thus generalizable recommendations have been elusive. Experience and patient selection are critical for successful endoscopic repair. Anterior cervical approach, often with complete laryngofissure, appears to be the most common and preferred method for open repairs, though some use a lateral approach. The proposed MBI classification appears to be a useful adjunct to aid in surgical decision-making for deeper LTEC.

H-type tracheoesophageal fistula in the neonatal period: Difficulties in diagnosis and different treatment approaches. A case series. / Fístula traqueoesofágica en H en el período neonatal: dificultades en el diagnóstico y diferentes abordajes terapéuticos. Serie de casos.

Congenital tracheoesophageal fistula not associated with esophageal atresia, known as H-type fistula, is an uncommon anomaly. It presents with cough, choking, and cyanosis during feeding and/or recurrent pneumonia. Although symptoms are usually present from birth, diagnosis is difficult. The rarity of this disease, non-specific symptoms, and the limitations of radiological and endoscopic confirmation of the fistula often result in a delay between presentation and diagnosis confirmation. Here we describe the clinical manifestations, assessment methods, and management of 3 newborn infants with H-type tracheoesophageal fistula, together with diagnosis recommendations to prevent unnecessary delays in the management of this condition.

La fístula traqueoesofágica congénita sin atresia esofágica asociada, conocida como tipo H, es una anomalía infrecuente. Se manifiesta con episodios de tos, ahogo y cianosis durante la alimentación y/o neumonía recurrente. Si bien los síntomas están usualmente presentes desde el nacimiento, el diagnóstico es difícil. La rareza de esta patología, los síntomas no específicos y las limitaciones en la demostración radiológica y endoscópica de la fístula contribuyen, a menudo, a la demora entre la presentación y la confirmación del diagnóstico. Se describen las manifestaciones clínicas, los métodos de evaluación y el tratamiento de 3 neonatos con esta patología, y se presentan recomendaciones para el diagnóstico a fin de evitar demoras innecesarias en el manejo de las fístulas en H.

Experience of diagnosis and treatment of 31 H-type tracheoesophageal fistula in a single clinical center.

OBJECTIVE: To summarize the experience of the diagnosis and treatment of 31 H-type tracheoesophageal fistula (TEF) at the Children's Hospital of Chongqing Medical University, Chongqing, China. METHODS: A total of 31 patients with H-type TEF were enrolled in this retrospective study from January 2000 to July 2017, and the diagnosis and treatment of the disease were analyzed and summarized. RESULTS: Iodine oil examination of the esophagus, fiberoptic bronchoscopy combined with esophagoscopy and three-dimensional computed tomography (3-D CT) reconstruction of the trachea and esophagus was used separately in 31 patients. 1 patient who had his TEF ligatured directly experienced recurrence 1 month after surgery, but recovered after TEF suturing. Subsequently, the remaining 30 patients were treated by suturing after TEF excision. All patients were followed up for 3 months-2 years and did not suffer from anastomosis orifice stenosis, gastroesophageal reflux, and a recurrent respiratory tract infection. Three patients exhibited vocal cord paralysis and improved spontaneously after a follow-up of 3-6 months. CONCLUSION: H-type TEF is diagnosed by iodine oil examination of the esophagus, fiberoptic bronchoscopy combined with esophagoscopy, and 3-D CT reconstruction of the trachea and esophagus. Excision and suturing of the TEF separately are an effective treatment for the disease and are an excellent outcome.

Acquired Adult Aerodigestive Fistula: Classification and Management.

BACKGROUND: Acquired aerodigestive fistulae (ADF) are rare, but associated with a high mortality rate. We present our experience of the diagnosis, management and outcomes of patients with ADFs treated at a tertiary centre. Utilising our findings, we propose an anatomical classification system, demonstrating how specific features of an ADF may determine management. METHODS: A clinical database was searched and 48 patients with an ADF were identified. A classification system was developed based on anatomical location of the ADF and differences in clinico-pathological features based on this categorisation were performed, with the chi-squared test used for inferential analyses and Kaplan-Meier curves with log-rank test to assess survival. RESULTS: Twenty (41.6%) patients developed an ADF secondary to malignancy, with previous radiotherapy (18.7%), post-operative anastomotic dehiscence and endotherapy (14.6% each) representing other causes. Thirty-one patients were managed with tracheal and/or oesophageal stents and eight underwent surgical repair. The classification system demonstrated benign causes of ADF were either proximally or distally sited, whilst a malignant cause resulted in mid-tracheal fistulae (p = 0.001), with the latter associated with poorer survival. ADFs over 20 mm in size were associated with poor survival (p = 0.011), as was the use of previous radiotherapy. Proximal and distal ADFs were associated with improved survival (p = 0.006), as were those patients managed surgically (p = 0.001). CONCLUSION: By classifying ADFs, we have demonstrated that anatomical location correlates with the size, history of malignancy, previous radiotherapy and aetiology of ADF, which can affect management. The proposed classification system will aid in formulating multi-modality individualised treatment plans.

Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup.

BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup. MATERIALS AND METHODS: We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data. RESULTS: The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001). CONCLUSION: Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.

Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease.

OBJECTIVE: To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. BACKGROUND: H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. METHODS: A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary children's hospital from 2002-2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. RESULTS: We identified 102 patients (median 9.5 per center, range 1-16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. CONCLUSIONS: There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. THE LEVEL OF EVIDENCE RATING: Level IV.

Categorization and repair of recurrent and acquired tracheoesophageal fistulae occurring after esophageal atresia repair.

PURPOSE: Recurrent trachea-esophageal fistula (recTEF) is a frequent (5%-10%) complication of congenital TEF (conTEF) and esophageal atresia (EA) repair. In addition, postoperative acquired TEF (acqTEF) can occur in addition to or even in the absence of prior conTEF in the setting of esophageal anastomotic complications. Reliable repair often proves difficult by endoluminal or standard surgical techniques. We present the results of an approach that reliably identifies the TEF and facilitates airway closure as well as repair of associated tracheal and esophageal problems. METHODS: Retrospective review of 66 consecutive patients 2009-2016 (55 referrals and 11 local) who underwent repair via reoperative thoracotomy or cervicotomy for recTEF and acqTEF (IRB P00004344). Our surgical approach used complete separation of the airway and esophagus, which reliably revealed the TEF (without need for cannulation) and freed the tissues for primary closure of the trachea and frequently resection of the tracheal diverticulum. For associated esophageal strictures, stricturoplasty or resection was performed. Separation of the suture lines by rotational pexy of the both esophagus and the trachea, and/or tissue interposition were used to further inhibit re-recurrence. For associated severe tracheomalacia, posterior tracheopexy to the anterior spinal ligament was utilized. RESULTS: The TEFs were recurrent (77%), acquired from esophageal leaks (26%), in addition to persistent or missed H-type (6%). Seven patients in this series had multiple TEFs of more than one category. Of the acqTEF cases, 6 were esophagobronchial, 10 esophagopulmonic, 2 esophagotracheal (initial pure EA cases), and 2 from a gastric conduit to the trachea. Upon referral, 18 patients had failed endoluminal treatments; and open operations for recTEF had failed in 18 patients. Significant pulmonary symptoms were present in all. During repairs, 58% were found to have a large tracheal diverticulum, and 51% had posterior tracheopexy for significant tracheomalacia. For larger esophageal defects, 32% were treated by stricturoplasty and 37% by segmental resection. Rotational pexy of the trachea and/or esophagus was utilized in 62% of cases to achieve optimal suture line separation. Review with a mean follow-up of 35months identified no recurrences, and resolution of pulmonary symptoms in all. Stricture treatment required postoperative dilations in 30, and esophageal replacement in 6 for long strictures. There was one death. CONCLUSION: This retrospective review of 66 patients with postoperative recurrent and acquired TEF following esophageal atresia repair is the largest such series to date and provides a new categorization for postoperative TEF that helps clarify the diagnostic and therapeutic challenges for management.

Fístula traqueoesofágica recurrente en niños con atresia esofágica reparada y utilidad de la broncoscopia flexible / Recurrent tracheoesophageal fistula in children with repaired esophageal atresia and the usefulness of flexible bronchoscopy

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Treatment of tracheopharyngeal and tracheo-oesophageal fistulas following laryngectomy and fistula classification based on individual silicone casts.

Persistent tracheopharyngeal (TPF) and tracheo-oesophageal (TEF) fistulas after laryngectomy create a therapeutic challenge. The current classification of TPFs and TEFs is based on clinical experience without detailed anatomical information. In this study, casts of TPFs/TEFs were obtained from 16 patients; these were the first steps in manufacturing customised prostheses. Fistulas were classified according to the shape and dimension of the tracheopharyngeal and tracheo-oesophageal silicone casts and prostheses as well as on epithetic requirements. Four different types of fistulas were classified: Type A, a fistula with a straight axis between the neopharynx and oesophagus; Type B, a fistula with a stenosis of the neopharynx but a straight axis; Type C, the axis between the neopharynx and oesophagus is flexed anteriorly; and Type D, neighbouring structures are absent creating a large defect. This classification system might improve the manufacturing processes of customised prostheses in individual cases with challenging tracheopharyngeal and tracheo-oesophageal fistulas.

Clinical outcomes of esophageal atresia: comparison between the Waterston and the Spitz classifications.

INTRODUCTION: Preoperative prognostic predictors are important for surgeons and parents to estimate the survival of patients with esophageal atresia (EA). The aim of this study was to update the clinical outcomes of EA treatment by comparing between the Waterston and the Spitz classification. MATERIALS AND METHODS: Medical records of the patients with EA treated at Queen Sirikit National Institute of Child Heath from 2003 to 2010 were reviewed. All of the patients were categorised into 3 groups of the Waterston and 3 groups of the Spitz risk factor criteria for comparing of the differences in each group and each classification. RESULTS: One hundred and thirty-two patients (81 males and 61 females) were treated for EA during the study period. Applying the Waterston classification, survival rate was 100% in group A, 91.5% in group B and 48.8% in group C. There was no statistical difference between the survival rate in group A and group B (P = 0.119) but significant difference between group B and group C (P = 0.000). Using the Spitz classification, survival rate was 97.4% in group I, 64.4% in group II and 27.3% in group III. There was obviously statistical difference of the survival rate between each group (group I vs group II, P = 0.000; group II vs group III, P = 0.041). CONCLUSION: Comparing with the prognostic predictors, the Spitz classification was more valid than the Waterston criteria. The Spitz classification is suitable to use for preoperative predictor to parental counselling and comparing of treatment outcomes of EA among paediatric tertiary care centres.

Long-gap esophageal atresia: traction-growth and anastomosis - before and beyond.

Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus or esophageal replacement with stomach, colon, or small intestine. Nonetheless, there is a consensus among most pediatric surgeons that the preservation of the native esophagus is associated with better postoperative outcomes. Thus, every effort should be made to conserve the native esophagus. The present study is aimed at critically reporting our experience focused on a standardized protocol based on the preoperative assessment of the gap in all cases and reviewing the present literature because no consensus is available regarding many aspects of LGEA (from definition to treatment). All newborn infants treated since 1995 for esophageal atresia (EA), regardless of type, were included in the present study. Identification of LGEA patients (gap ≥3 vertebral bodies) was performed based on preoperative esophageal gap measurement. The selected patients were grouped based on EA type (A/B vs. C/D) and whether they were referred from an outside institution or not. Postoperative outcome was compared. Statistical analysis was performed with the Fisher's exact test and Mann-Whitney test as appropriate, with P < 0.05 considered statistically significant. Two hundred and nineteen patients have been consecutively treated between 1995 and 2012 with the following EA subtypes: type: A 25 (11.4%); B 6 (2.7%); C 182 (83.1%); D 3 (1.4%); E 3 (1.4%). Fifty-seven patients (26%) were classified as LGEA: type A-B, 31 (54.4%); type C-D, 26 (45.6%). Twenty seven (47%) of these patients were referred after at least one failed attempt at esophageal correction: type A-B, 15 (55%); type C-D, 12 (45%). Only one patient ultimately required esophageal substitution, with an overall survival rate of 94%. A standardized perioperative protocol enhances the possibility of preserving the native esophagus in cases of LGEA. Gap measurement can be accurately defined before surgery in all patients with EA. Esophageal anastomosis (either immediate or delayed repair) is almost always feasible; esophageal substitution should only be considered after a rigorous attempt at achieving end-to-end esophageal anastomosis.

Flexible bronchoscopic cannulation of an isolated H-type tracheoesophageal fistula in a newborn.

Congenital isolated H-type tracheoesophageal fistula (H-TEF) is a rare malformation of the airways. Surgery should not be delayed once the diagnosis is established. Identification of the fistula during surgery is a prerequisite for a successful outcome. Intubation or cannulation of the H-TEF with a catheter can help the surgeon to identify the fistula. A rigid bronchoscope is generally used for cannulation of the fistula. Cannulation of an H-TEF in a newborn with a flexible bronchoscope has the merit of simplicity and safety. We report the insertion of a catheter in an isolated H-TEF in a newborn using a flexible bronchoscope and think that this method can be easily applied.

Tracheoesophageal fistula after primary repair of type C esophageal atresia in the neonatal period: recurrent or missed second congenital fistula.

PURPOSE: The aim of the study is to investigate whether a tracheoesophageal fistula (TEF) found after the primary repair of type C esophageal atresia (EA) is a recannulation of the original fistula, a missed proximal fistula, or other rare foregut malformation. METHODS: Between 2000 and 2009, 143 different types of patients with EA were admitted in our hospital. Seven patients (2 from our series, 5 referred to us by other hospitals with the history of primary repair of type C EA) had late presenting TEF. Esophagogram, 3-dimensional computed tomographic (CT) reconstruction, bronchoscopy, and reoperation were performed to confirm the TEF. Their medical records were reviewed and summarized. RESULTS: Persistent feeding or respiratory problems were the common symptom. The mean age of the first appearance was 17 ± 26 (1-63) months. Preoperative diagnosis was made by esophagograms and bronchoscopy in 6 patients. Reoperations were performed in all patients through thoracotomy. Missed proximal TEF shown as a distinct fistula above the primary anastomosis without much adhesion was confirmed in 5 cases. A recurrent TEF was found in 1 case. A case of communicating bronchopulmonary foregut malformation was confirmed by 3-dimensional CT reconstruction and reoperation. CONCLUSION: A missed proximal TEF after repair of EA may be misdiagnosed as a recurrent TEF. Accurate preoperative diagnosis depends on combined evaluations of radiologic contrast study, 3-dimensional CT, and bronchoscopy.

Esophageal atresia: prognostic classification revisited.

BACKGROUND: Although the Spitz classification is the most widely used prognostic classification for esophageal atresia and/or tracheoesophageal fistula (EA), its discrimination ability remains unclear. We sought to develop a more accurate prognostic classification for EA. METHODS: The records of 121 consecutive infants with EA (1980-2005) were reviewed. The independent variables included 6 clinical characteristics, and the dependent variables were survival and mortality. Stepwise logistic regression analysis was used to construct models predicting mortality and create a revised prognostic classification. The discrimination abilities of the revised classification and the Spitz classification were compared using receiver-operating characteristic (ROC) curves. RESULTS: Birth weight and the presence of major cardiac anomalies were significant prognostic factors for mortality, and major cardiac anomalies affected mortality more than birth weight. The ROC curve for birth weight suggested that 2,000 g was an appropriate cutoff point. The Spitz classification was revised as follows: the revised class I (low-risk group) consisted of patients without major cardiac anomalies and birth weight >2,000 g; class II (moderate-risk group) consisted of patients without major cardiac abnormalities and birth weight <2,000 g; class III (relatively high-risk group) consisted of patients with major cardiac anomalies and birth weight >2,000 g; and class IV (high-risk group) consisted of patients with major cardiac anomalies and birth weight <2,000 g. The ROC comparisons showed that the revised classification provided a significant improvement (P = .049). CONCLUSION: This revised classification can improve the stratification of EA patients and be a useful predictor of survival.

Classification and management of tracheoesophageal and tracheopharyngeal fistulas after laryngectomy.

OBJECTIVES: Tracheoesophageal fistulas (TEF) and tracheopharyngeal fistulas (TPF) are intentionally created for prosthetic or surgical voice restoration after laryngectomy or can develop after radiotherapy or surgical interventions. If the fistula does not shrink or close spontaneously or does not respond to conservative measures, surgical closure of the fistula is indicated. STUDY DESIGN: Retrospective study of 177 patients. METHODS: Data of 168 laryngectomy patients who needed a voice prosthesis (VP) replacement were obtained. Our experiences with nine severe TEF/TPF were analyzed, and a classification of these fistulas depending on the anatomic and clinical appearance was developed. RESULTS: TEF/TPF can be divided into five types: high TEF with leakage through the VP (type Ia), high TEF with leakage around the VP (type Ib), enlarged high TEF (type II), deep TEF (type III), TPF (type IV), and TPF associated with pharynx stenosis (type V). Persisting TEF/TPF after unsuccessful attempts at surgical closure in four patients and the surgical solutions and procedures in these rare cases are discussed in detail. CONCLUSIONS: Leakage of TEF in prosthetic voice restoration usually responds well to conservative measures. If these measures fail, and in all cases of TPF, surgical intervention is necessary for transtracheostomal or transcervical closure with multilayer sutures of the esophagus and trachea. Persisting TEF/TPF after unsuccessful surgical attempts at revision surgery remain challenging. Our experiences show that tracheostoma transposition for dissociation of the cranial end of the trachea and the hypopharynx and esophagus is essential for effective closure. In rare cases of TPF combined with pharyngoesophageal stricture formation, a resection and immediate reconstruction of the stenotic pharyngoesophageal segment with a tube-shaped fasciocutaneous radial forearm flap must be considered.

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.