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Objectives: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA)., Method: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery., Results: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types., Conclusions: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients., (© 2024 European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Autologous tissue-assisted regenerative procedures have been considered effective to close different types of fistula, including the leakage around tracheoesophageal puncture. The aim of this study was to retrospectively review 10 years of lipotransfer for persistent periprosthetic leakage in laryngectomized patients with voice prosthesis. Clinical records of patients who experienced periprosthetic leakage from December 2009 to December 2019 were reviewed. Patients receiving fat grafting were included. The leakage around the prosthesis was assessed with a methylene blue test. Twenty patients experiencing tracheoesophageal fistula enlargement were treated with fat grafting. At the one-month follow-up, all patients were considered improved with no leakage observed. At six months, a single injection was sufficient to solve 75% of cases (n 15), whereas 25% (n 5) required a second procedure. The overall success rate was 80% (n 16). Results remained stable for a follow-up of 5.54 ± 3.97 years. Fat grafting performed around the voice prosthesis, thanks to its volumetric and regenerative properties, is a valid and lasting option to solve persistent periprosthetic leakage.

Rationale: Videofluoroscopic swallowing study (VFSS) is a noninvasive radiographic procedure that examines the oral, pharyngeal, and cervical esophageal stages of swallowing. Tracheoesophageal fistula (TEF) is difficult to diagnose depending on its size and location. However, how VFSS can be of benefit in the diagnosis of TEF has not been reported yet., Patient Concerns: A 64-year-old man who had been tracheostomized post spinal tumor resection surgery at the cervical level 1 to 2, had his tracheostomy tube removed approximately 25 years ago. After decannulation, he reported coughing while swallowing food, foreign sensation in the neck and repeated bouts of pneumonia ever since., Diagnosis: VFSS revealed, for the first time, acquired TEF after tracheostomy decannulation as the cause of repetitive aspiration pneumonia., Intervention: VFSS was performed in this case., Outcomes: In the background of suspected TEF based on VFSS results, the patient underwent a computed tomography scan of the chest and airway in the prone position, followed by bronchoscopy, which confirmed the existence of a TEF. He then underwent primary closure of the fistula. The patient had an uneventful recovery and is currently symptom-free 10 months after the surgery., Lessons: This case alerts clinicians to the possibility of TEF as a diagnosis when the aspirate leaks from the upper esophagus and through the posterior wall of trachea in the esophageal phase of VFSS., Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article. The authors have no funding and conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on "strong", "moderate" or "weak" agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients' age were also developed., (© 2020 Wiley Periodicals LLC.)

Introduction:  Esophageal atresia (EA) is often accompanied by tracheobronchial malformations leading to stridor, recurrent bronchitis, and occasionally to life-threatening obstructive apnea after surgical repair. The aim of this study was to identify the presence of tracheomalacia in patients with EA and tracheoesophageal fistula (TEF) pre- and postoperatively and to find endoscopic correlates leading to clinical airway symptoms., Methods:  In a cohort of 362 patients with EA-TEF who underwent 595 tracheoscopies at the Children's Hospital of Cologne between January 1983 and December 2002, impaired tracheal lumen, localization of TEF, tracheal pulsations, and corresponding clinical symptoms were retrospectively analyzed., Results:  The incidence of tracheomalacia was higher in patients with EA and TEF (Gross B-D) compared with patients with EA alone (Gross A) and average tracheal collapse does not significantly change before and after surgical repair of the esophagus in all types. Patients with cyanosis while eating and obstructive apnea presented with an average tracheal collapse of 89%. The presence of respiratory symptoms such as cough, stridor, or bronchitis was not associated with a higher grade of tracheal collapse compared with patients without any airway symptoms (average tracheal collapse of 37% in symptomatic patients vs. 33% in nonsymptomatic patients)., Conclusion:  Tracheomalacia tends to be present independently of surgical procedure. Tracheomalacia should be measured by tracheoscopy (in % of tracheal collapse). Patients with a tracheal collapse of >80%, a ventral pulsation, and obstructive apnea or cyanosis in combination, are at risk for life-threatening situations and further surgical treatment should be considered., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

The study aimed to assess concerns of caregivers of children with EA-TEF related to feeding-swallowing difficulties, compare the concerns according to type of atresia and repair time, and investigate its relationship with time to start oral feeding. Caregivers accompanying 64 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding were noted. Parents completed the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) to assess the concerns of caregivers related to feeding-swallowing difficulties. The T-FS-IS has three subscales including daily activities, worry, and feeding difficulties. The median age of patients was 3 (min = 1, max = 12) years, of which 57.8% were male. 43.8% of cases were isolated-EA, and 56.3% were EA-distal TEF. 57.8% of cases received early repair, and 42.2% had delayed repair. The median time to start oral feeding was 4 weeks (min = 1, max = 128). The mean scores of daily activities, worry, feeding difficulties, and total score from the T-FS-IS were 2.43 ± 1.18, 2.73 ± 1.28, 2.10 ± 0.97, and 2.44 ± 1.09, respectively. Caregivers of children with isolated-EA reported more problems in total score and all subscales of the T-FS-IS than EA-distal TEF (p < 0.01). Caregivers of children who received delayed repair reported more problems in total score and all subscales of the T-FS-IS than children with early repair (p < 0.05). Moderate to strong correlations were found between the T-FS-IS and time to start oral feeding (p < 0.01, r = 0.55-0.65). This study suggests that caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding-swallowing difficulties.

Introduction: Tracheoesophageal Fistula (TF) is a rare complication of Bevacizumab. Thoracic radiotherapy may be a contributing factor to TF formation. To the best of our knowledge, we report the first case of Chinese patient with non-small cell lung cancer (NSCLC) who developed TF after completion of chemotherapy with bevacizumab and thoracic radiotherapy., Patient Concerns: A 54-year-old male patient was diagnosed with NSCLC. He received definitive thoracic radiotherapy with concurrent pemetrexed and cisplatin chemotherapy. Two months after the treatment, the disease progressed with enlargement of right inguinal lymph node and chemotherapy of docetaxel, carboplatin and bevacizumab was administrated. Eighteen days after 4 cycles, the patient presented a sudden onset of acute cough after drinking., Diagnosis: Esophageal barium swallow revealed a TF. Gastroscopy confirmed a fistula in the esophagus., Interventions: A jejunal feeding tube was placed for nutrition for a month. After that a covered esophageal stent was placed in the esophagus., Outcomes: At the 6-month follow-up visit, the patient recovered well and had not developed any complication related to the stent placement., Conclusion: TF is a rare but life-threatening complication of bevacizumab. Careful observation is imperative for those patients who are administered bevacizumab, particularly in patients treated previously with thoracic radiotherapy.

Background: Tracheobronchial stents are widely used devices in interventional pulmonology; however, the current literature on the effectiveness and complication rates of the different types of stents is limited., Objective: We report the largest case series of airway Bonastent placement and describe the efficacy and early (<30 days) and late (≥30 days) complication rates., Methods: We performed a retrospective review of our prospectively collected database of patients who underwent therapeutic bronchoscopy with stent placement. All adult patients who had a tracheal/bronchial Bona-stent placed between July 1, 2017, and July 30, 2019, for any indication at our institution were included. The efficacy as well as intraoperative and short- and long-term complications of Bonastent placement were evaluated., Results: Sixty Bonastents were placed in 50 patients. The etiology was malignant in 90% of the cases, while 2 patients had a tracheoesophageal fistula. All procedures were performed via rigid bronchoscopy. The most common location for stent placement was the bronchus intermedius, followed by the trachea, in 32 and 30% of the cases, respectively. Seventy percent of the patients (35/50) had improvement of respiratory symptoms within 30 days. Twenty-eight stents (48%) were removed at a mean of 74 days. Seventeen patients (34%) died within 30 days of stent placement. The overall complication rate was 54% (27/50 patients) at a mean follow-up of 111 days. The stent-related complication rate was 23.3% (14/60 cases) within <30 days and 53% (18/34 cases) at ≥30 days., Conclusions: The tracheobronchial Bonastent is effective for the treatment of patients with central airway obstruction and tracheoesophageal fistulae with an acceptable safety profile., (© 2020 S. Karger AG, Basel.)

Objectives:  Endoscopic follow-up after esophageal atresia (EA) tracheoesophageal fistula (TEF) repair is recommended to detect esophageal histopathological complications. We investigated the prevalence of histopathologically proven esophageal complications (peptic esophagitis, gastric metaplasia, and eosinophilic esophagitis) and assessed the predictors of these complications in children with EA-TEF., Materials and Methods:  This is a prospective longitudinal cohort study performed between September 2005 and December 2014 comprising 77 children with EA-TEF followed-up until February 2017. Univariate analysis was performed using the Wilcoxon's rank-sum test for continuous variables and the Pearson's chi-square test for categorical variables. Multivariable analysis was performed using a Cox regression hazard model. The association between clinical factors and histopathologically proven complications was estimated using a Cox regression hazard model with time until the appearance of complications as the time scale., Results:  All 77 children received proton pump inhibitors (PPIs) ( n  = 73) or H2 receptor antagonists (H2RA). A total of 252 endoscopies were performed in 73 children (median 2.6/child, range: 1-29). Median age at study completion was 4.9 years (range: 2.3-11.5 years). Histopathologically proven complications occurred in 38 children (52%): peptic esophagitis ( n  = 32, 44%), eosinophilic esophagitis ( n  = 15, 21%), and gastric metaplasia ( n  = 9, 12%). A total of 82% patients were on PPI or H2RA at the time of diagnosis of histological complication. Multivariable Cox regression analysis showed that patients with recurrent anastomotic strictures (>3 dilations) had a higher risk of occurrence of histopathologically proven complications over time (hazard ratio: 3.11, 95% confidence interval [CI]: 1.53-6.34). On univariate analysis, the result of the first endoscopy was not associated with the occurrence of histopathologically proven complications (odds ratio: 0.8, 95% CI: 0.16-3.95)., Conclusion:  Histopathologically proven complications with potential long-term consequences occurred in approximately 50% of children after EA-TEF repair. A history of recurrent anastomotic strictures is associated with the occurrence of these complications. The result of the first endoscopy does not predict the histopathological outcome. Children with EA-TEF warrant close and systematic long-term follow-up at specialized multidisciplinary clinics with endoscopic evaluation., Competing Interests: None declared., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Scoliosis has been reported after repair of esophageal atresia with or without tracheoesophageal fistula (EA-TEF). This study aims to investigate the prevalence and natural history of scoliosis and associated congenital vertebral anomalies in patients operated for EATEF., Methods: A retrospective review of patients operated for EA-TEF with radiological examination for the presence of scoliosis or associated spine congenital anomalies was done on 106 patients (ages 5-19 years)., Results: Scoliosis was found in 53 patients (49%) for which 46 of these were in the thoracic region and 33 were right-thoracic curves. After a follow-up ranging from 5 to 14 years, four patients (3.7%) out of 106 were operated for scoliosis. Right-sided thoracotomy (RST) was the identifiable risk factor for scoliosis development; all patients with scoliosis had their EA repaired through RST. Congenital vertebral anomalies were found in 8 of those patients (7.5%). After a median follow-up of 6.5 years, no patients progressed enough to require operation., Conclusion: Scoliosis affects one of every two patients operated for EA; it may progress to the indication of surgery. RST was the identifiable risk factor for scoliosis development., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Oesophageal atresia with or without tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease are surgical malformations of the gastrointestinal tract typically diagnosed early in the neonatal period and varying in severity and prognosis. This report describes a full-term male newborn presenting simultaneous oesophageal atresia with distal tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease. In addition to the complex types of gastrointestinal malformations involved, the combination of ileal atresia and Hirschsprung's disease, as well as ganglion cells distal to intestinal atresia, resulted in a challenging diagnosis. Despite a successful outcome, the patient presented increased morbidity and prolonged hospitalisation. We highlight some important findings that may aid the early diagnosis of Hirschsprung's disease in this clinical setting. To our knowledge, the association of oesophageal atresia/tracheo-oesophageal fistula, ileal atresia and Hirschsprung's disease has not been previously reported., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Development of hypertrophic pyloric stenosis (HPS) after a few weeks of repair of an oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) is a rare condition in early infancy. Although vomiting or feeding intolerance in operated cases of OA+TOF are attributed to oesophageal stricture, gastro-oesophageal reflux and oesophageal dysmotility, it may also be caused by HPS. Herein, we report a newborn infant who had OA and TOF operation on day 2 of life and diagnosed to have HPS at 15th day of age. Even though it is a rare anomaly, HPS should be kept on mind in the presence of persistent vomiting following repair of OA., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) is a complex disorder, and most outcome data are confined to mortality and feeding-related morbidities. Our objective was to examine mortality, growth and neurodevelopmental outcomes in a large recent cohort of infants with EA/TEF., Methods: Single center study of EA/TEF infants referred from January 2000 to December 2015. Data collected included associated defects, neonatal morbidity and mortality and growth and neurodevelopmental outcomes at age 12-36months. Multiple regression analysis was used to determine variables associated with adverse outcome., Results: Of the 253 infants identified, 102 infants (40%) were preterm. Overall mortality was 8.3%, the majority from major cardiac malformations (p<0.001) Neurodevelopmental assessments (n=182) showed that 76% were within normal, while some delay was seen in 24%, most often in expressive and receptive language. Nine infants had hearing impairment and 5 had visual impairment. Gastrostomy tubes were required in 47 patients and 15% continued to have weight growth velocities less than the 10th centile. A number of specialist interventions were required, Speech/Language being frequent., Conclusion: Mortality in EA/TEF is primarily related to concomitant anomalies, especially cardiac. Multidisciplinary follow up is important for early identification and intervention for growth failure and developmental delay., Type of Study: Retrospective study LEVEL OF EVIDENCE: Level II., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: The estimated incidence of esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) is 1:3500 live births. During childhood these patients have various co-morbidities, but the overall quality of life among adults is similar to that of the general population., Objectives: To evaluate short- and long-term co-morbidities and quality of life among infants born with EA ± TEF at a large single medical center., Methods: Medical records of 65 children born over a 21 year period were reviewed for short- and long-term medical data. Telephone interviews were conducted with 46 of their parents regarding medical problems and quality of life after home discharge., Results: The main long-term co-morbidities during the first 2 years of life, 4-6 years of age, and during adolescence (12-16 years) included gastro-esophageal reflux disease (GERD) in 56.5%, 35.8%, and 18.7%, respectively; stridor in 84.8%, 45.2%, and 12.5%, respectively; hyper-reactive airway disease (HRAD) in 43.5%, 35.5%, and 36.5%, respectively; recurrent pneumonia in 43.5%, 32.3%, and 18.8%, respectively; and overall recurrent hospitalizations in 87%, 41.9%, and 25%, respectively. The quality of life was reportedly affected among 100%, 75%, and 33.3% respectively., Conclusions: Long-term follow-up of patients with EA ± TEF indicates a high burden of co-morbidities during the first 6 years of life, with a gradual decrease in symptoms thereafter. Nevertheless, HRAD continued to impact the daily life of about one-third of the older adolescents, and GERD one-fifth. A long-term multidisciplinary follow-up should be conducted to prevent late onset complications that may affect the quality of life.

Background: Tracheoesophageal fistula (TEF) and esophageal atresia (EA) are rare anomalies in neonates. Up to 50% of neonates with TEF/EA will have Vertebral anomalies (V), Anal atresia (A), Cardiac anomalies (C), Tracheoesophageal fistula (T), Esophageal atresia (E), Renal anomalies (R), and Limb anomalies (L) (VACTERL) association, which has the potential to cause serious morbidity., Purpose: Timely management of the neonate can greatly impact the infant's overall outcome. Spreading latest evidence-based knowledge and sharing practical experience with clinicians across various levels of the neonatal intensive care unit and well-baby units have the potential to decrease the rate of morbidity and mortality., Methods/search Strategy: PubMed, CINAHL, Cochrane Review, and Google Scholar were used to search key words- tracheoesophageal fistula, esophageal atresia, TEF/EA, VACTERL, long gap, post-operative management, NICU, pediatric surgery-for articles that were relevant and current., Findings/results: Advancements in both technology and medicine have helped identify and decrease postsurgical complications. More understanding and clarity are needed to manage acid suppression and its effects in a timely way., Implications for Practice: Knowing the clinical signs of potential TEF/EA, clinicians can initiate preoperative management and expedite transfer to a hospital with pediatric surgeons who are experts in TEF/EA management to prevent long-term morbidity., Implications for Research: Various methods of perioperative management exist, and future studies should look into standardizing perioperative care. Other areas of research should include acid suppression recommendation, reducing long-term morbidity seen in patients with TEF/EA, postoperative complications, and how we can safely and effectively decrease the length of time to surgery for long-gap atresia in neonates.

Competing Interests: Competing interests: None

Aim  The aim of this study was to evaluate the results of Swallowing Rehabilitation Protocol (SRP) on swallowing function (SF) of esophageal atresia and tracheoesophageal fistula (EA-TEF) patients with pharyngeal swallowing disorder. Materials and Methods  In this study, 24 children with EA-TEF who had deglutitive and respiratory problems were grouped into either study ( n  = 12) or control group ( n  = 12) by basic randomization. Study group received the SRP including neuromuscular electrical stimulation, thermal tactile stimulation, and hyolaryngeal mobilization. The control group received nonnutritive stimulations. SF was evaluated with 3 mL liquid and pudding barium by videofluoroscopic swallowing study (VFSS) before and after 20 sessions of interventions. Results  No statistical differences were found between groups in terms of descriptive characteristics including age, sex, weight, height, type of atresia, repair type, repair time, and start time of oral intake ( p  > 0.05). There were no statistical differences between groups in term of swallowing parameters ( p  > 0.05), except reflux ( p  = 0.004) according to VFSS findings. After 20 sessions of interventions, the study group showed improvement in penetration-aspiration scale scores, oral phase dysfunction, delay in swallowing reflex, and residue in valleculae and pyriform sinuses after pudding swallow. The control group showed improvement only in oral phase dysfunction. Conclusion  SRP can be recommended to improve SF in patients with EA/TEF who have pharyngeal swallowing disorders., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Esophageal atresia (EA) and tracheoesophageal fistula (TEF) represent major therapeutic challenges, frequently associated with serious morbidities following surgical repair. The aim of this longitudinal study was to assess temporal changes in morbidity and mortality of patients with EA/TEF treated in a tertiary-level center, focusing on postoperative complications and their impact on long-term gastroesophageal function., Methods: One hundred nine consecutive patients with EA/TEF born between 1975 and 2011 were followed for a median of 9.6 years (range, 3-27 years). Comparative statistics were used to evaluate temporal changes between an early (1975-1989) and late (1990-2011) study period., Results: Gross types of EA were A (n = 6), B (n = 5), C (n = 89), D (n = 7), and E (n = 2). Seventy (64.2%) patients had coexisting anomalies, 13 (11.9%) of whom died before EA correction was completed. In the remaining 96 infants, surgical repair was primary (n = 66) or delayed (n = 25) anastomosis, closure of TEF in EA type E (n = 2), and esophageal replacement with colon interposition (n=2) or gastric transposition (n=1). Long-gap EA was diagnosed in 23 (24.0%) cases. Postoperative mortality was 4/96 (4.2%). Overall survival increased significantly between the two study periods (42/55 vs. 50/54; P = 0.03). Sixty-nine (71.9%) patients presented postoperatively with anastomotic strictures requiring a median of 3 (range, 1-15) dilatations. Revisional surgery was required for anastomotic leakage (n = 5), recurrent TEF with (n = 1) or without (n=9) anastomotic stricture, undetected proximal TEF (n = 4), and refractory anastomotic strictures with (n = 1) or without (n = 2) fistula. Normal dietary intake was achieved in 89 (96.7%) patients, while 3 (3.3%) remained dependent on gastrostomy feedings. Manometry showed esophageal dysmotility in 78 (84.8%) infants at 1 year of age, increasing to 100% at 10-year follow-up. Fifty-six (60.9%) patients suffered from dysphagia with need for endoscopic foreign body removal in 12 (13.0%) cases. Anti-reflux medication was required in 43 (46.7%) children and 30 (32.6%) underwent fundoplication. The rate of gastroesophageal reflux increased significantly between the two study periods (29/42 vs. 44/50; P = 0.04). Twenty-two (23.9%) cases of endoscopic esophagitis and one Barrett's esophagus were identified., Conclusions: Postoperative complications after EA/TEF repair are common and should be expertly managed to reduce the risk of long-term morbidity. Regular multidisciplinary surveillance with transitional care into adulthood is recommended in all patients with EA/TEF.

In the past decades improved surgical techniques and better neonatal supportive care have resulted in reduced mortality of patients with esophageal atresia (EA), with or without tracheoesophageal fistula, and in increased prevalence of long-term complications, especially respiratory manifestations. This integrative review describes the techniques currently used in the pediatric clinical practice for assessing EA-related respiratory disease. We also present a novel algorithm for the evaluation and surveillance of lung disease in EA. A total of 2813 articles were identified, of which 1451 duplicates were removed, and 1330 were excluded based on review of titles and abstracts. A total of 32 articles were assessed for eligibility. Six reviews were excluded, and 26 original studies were assessed. Lower respiratory tract infection seems frequent, especially in the first years of life. Chronic asthma, productive cough, and recurrent bronchitis are the most common respiratory complaints. Restrictive lung disease is generally reported to prevail over the obstructive or mixed patterns, and, overall, bronchial hyperresponsiveness can affect up to 78% of patients. At lung imaging, few studies detected bronchiectasis and irregular cross-sectional shape of the trachea, whereas diffuse bronchial thickening, consolidations, and pleural abnormalities were the main chest X-ray findings. Airway endoscopy is seldom included in the available studies, with tracheomalacia and tracheobronchial inflammation being described in a variable proportion of cases. A complete diagnostic approach to long-term respiratory complications after EA is mandatory. In the presence of moderate-to-severe airway disease, patients should undergo regular tertiary care follow-up with functional assessment and advanced chest imaging., (Copyright © 2016. Published by Elsevier B.V.)

Terminal 7q deletion is rarely reported in the literature. Holoprosencephaly and sacral dysgenesis are found in association with this deletion, due to haploinsufficiency of SHH and HLBX9 genes respectively. We report on a 2-year-old boy with 7q35-36.3 deletion encompassing SHH identified by oligonucleotide array comparative genomic hybridization. In addition to other frequent features, the patient presented with esophageal atresia and tracheoeosophageal fistula diagnosed at birth. This case, together with two others previously described, one presenting with esophageal atresia, the other with congenital esophageal stenosis, confirms the possible association between congenital esophageal malformations and 7q terminal deletion including SHH., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Oesophageal atresia and tracheo-oesophageal fistula are congenital anomalies of the oesophagus requiring surgical repair in infancy, either by open or thoracoscopic approach. Although mortality rates associated with this procedure are low, children may go on to have complications throughout childhood and into adulthood, most commonly related to ongoing gastrointestinal and respiratory symptoms. This review outlines the early, mid and long-term outcomes for these children in terms of quality of life and incidence of symptoms., (© 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a rare congenital malformation. Digestive and nutritional problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting with gastroesophageal reflux and its complications, such as anastomotic strictures. Esophageal dysmotility is constant, and can have important consequences on feeding and nutritional status. Patients with EA need a systematic follow-up with a multidisciplinary team., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Objective: To investigate long-term prognosis of infants with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF)., Methods: The data of patients with EA were investigated from their medical files. For the neurodevelopmental evaluation, they were requested to come for a return visit to authors' polyclinic. Intellectual development was assessed by Ankara Developmental Screening Inventory (ADSI) (for 0-6 age) and Wechsler Intelligence Scale for Children-Revised (WISC-R) (for 6-16 age)., Results: Of the 57 patients, 50 had EA+ distal TEF (87.7 %), six had isolated EA (10.5 %) and one had isolated TEF (1.8 %). Of the total patients, 18 cases (31 %) died and remaining 39 cases (69 %) survived. In the surviving cases, the most common long-term complication was dysphagia (n = 37, 94.8 %). Intellectual levels of the 24 patients assessed by ADSI were normal and of the remaining 15 cases evaluated by WISC-R ranged between 95 and 110 points., Conclusions: The long-term complications and hospital visits are common in surviving cases of EA; however, they have normal cognitive functions and physical developmental characteristics.

Background: Newborn babies with esophageal atresia/tracheoesophageal fistula (EA/TEF) are prone to respiratory tract disorders. Functional residual capacity (FRC) and lung clearance index (LCI) are commonly considered useful and sensitive tools to investigate lung function and early detecting airways diseases. The aim of the present study is to report the first series of EA/TEF infants prospectively evaluated for FRC and LCI., Methods: Prospective observational cohort study of all patients treated for EA/TEF. Lung volume and ventilation inhomogeneity were measured by helium gas dilution technique using an ultrasonic flow meter. Babies were studied both in assisted controlled ventilation (sedated) and in spontaneous breathing (quiet sleep). Three consecutive FRC and LCI measurements were collected for each test at three different time points: before surgery (T0), 24hours after surgery (T1) and after extubation (T2)., Results: 16 EA newborns were eligible for the study between December 2011 and July 2013. Three were excluded because of technical problems. At T0 FRC values were in the normal range regardless the presence of TEF but worsened afterwards at T1, with a subsequent recovering after extubation; a significant improvement after surgery was observed concerning LCI while no differences were found in tidal volume., Conclusion: Helium gas dilution technique is a suitable method to measure the effect of surgery on lung physiology, even in ventilated infants with EA. The changes observed could be related to the ventilatory management and lung compression during surgical procedure., (Copyright © 2016 Elsevier Inc. All rights reserved.)

A case of successful therapy of the patient with viral and bacterial pneumonia, prolonged ventilation (137 days) and tracheoesophagealfistula presented. The possibility of optimizing the gas exchange in the lungs by selecting appropriate modes of mechanical ventilation with the use ofproportional assist ventilation of the lungs (PVVL or PAV +), rather than suppressing patient's own attempts is shown.

Aim: A retrospective study was performed to evaluate the functional disorders of deglutition with videofluoroscopy (VFS), in children operated for esophageal atresia (EA) and/or tracheoesophageal fistula (TEF)., Methods: Patients with the repair of EA-TEF were evaluated in respect to the type of malformation, operative procedure, postoperative complications, deglutitive and respiratory symptoms, and gastroesophageal reflux disease (GERD). The dysphagia score, VFS findings of oral, pharyngeal and esophageal phases, and penetration-aspiration scale (PAS) score were recorded in the evaluation of the deglutitive functions., Results: Thirty-two cases with a median age of 48months (2-120months), and male to female ratio of 14:18 were included in the study. Most of the cases had Gross type C anomaly (n=26, 81.3%), and the others were type A (n=3), D (n=2) and E (n=1). The incidence of associated anomalies was 71.8%. The patients underwent primary (n=26, 81.3%) or delayed (n=6, 18.7%) anastomosis. Postoperative complications including anastomotic stricture (n=12), leak (n=2) and recurrent fistula (n=2) were managed by dilatation, conservative approach and repair of the fistula, respectively. Recurrent pneumonia (n=13), cough with liquid intake (n=10) and food impaction (n=7) were recorded in the history. Management of GERD included medical (n=11) and surgical (n=7) treatment. The median dysphagia score was 3.5 (min: 0-max: 27). The oral phase of VFS was normal in most of the cases (n=29, 90.6%). Only three had mild or moderate impairment, and none had severe. The pharyngeal phase showed no impairment in 23 of the cases (71.8%), and severe impairment was observed only in 3 of all, for the parameters of hyolaryngeal elevation and airway closure. Opposite to the first two phases of the deglutition, the esophageal phase was normal in only 2 of the cases (6.3%). Among the other 30 cases with impairment, only two had mild, and the rest had moderate to severe problems. Esophageal backflow, motility and residue were the most severely impaired parameters of this phase. The PAS evaluation revealed no penetration and aspiration in 26 of the cases (81.3%), while 1 had penetration and five had aspiration., Conclusion: The patients with repaired EA-TEF may reveal deglutitive and respiratory symptoms in follow-up, necessitating certain investigations. The deglutition is functionally evaluated with VFS. While the disorders of oral and pharyngeal phases are less frequent and prominent, the esophageal phase reveals disorders with higher incidence and severity., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Feingold syndrome (FS) is an autosomal dominant disorder characterized by microcephaly, short stature, digital anomalies, esophageal/duodenal atresia, facial dysmorphism, and various learning disabilities. Heterozygous deletion of the miR-17-92 cluster is responsible for a subset of FS (Feingold syndrome type 2, FS2), and the developmental abnormalities that characterize this disorder are partially recapitulated in mice that harbor a heterozygous deletion of this cluster (miR-17-92∆/+ mice). Although Feingold patients develop a wide array of learning disabilities, no scientific description of learning/cognitive disabilities, intellectual deficiency, and brain alterations have been described in humans and animal models of FS2. The aim of this study was to draw a behavioral profile, during development and in adulthood, of miR-17-92∆/+ mice, a genetic mouse model of FS2. Moreover, dopamine, norepinephrine and serotonin tissue levels in the medial prefrontal cortex (mpFC), and Hippocampus (Hip) of miR-17-92∆/+ mice were analyzed.Our data showed decreased body growth and reduced vocalization during development. Moreover, selective deficits in spatial ability, social novelty recognition and memory span were evident in adult miR-17-92∆/+ mice compared with healthy controls (WT). Finally, we found altered dopamine as well as serotonin tissue levels, in the mpFC and Hip, respectively, of miR-17-92∆/+ in comparison with WT mice, thus suggesting a possible link between cognitive deficits and altered brain neurotransmission.

Background: In the past decade, targeted therapy with antiangiogenic drugs has become standard of care for most types of metastatic, progressive thyroid cancer. While these drugs were thought initially to be less toxic than traditional chemotherapy, they can have rare but serious and fatal toxicities. Once such toxicity that has been reported in other tumor types is upper airway fistula formation, which can be life-threatening., Summary: Here, we describe three patients treated with antiangiogenic tyrosine kinase inhibitors at two academic institutions who developed aerodigestive fistula. All three patients had risk factors for fistula formation, which included external beam radiation and/or large tumor with invasion of the tracheal wall., Conclusions: Fistula formation is a known but rare side effect of antiangiogenic tyrosine kinase inhibitors. Knowledge of the risk factors that may predispose thyroid cancer patients to this serious adverse event is vital prior to prescribing antiangiogenics. Particular caution should be observed when using these drugs in patients undergoing radiation therapy or surgery, or in patients whose tumor is invading vital structures of the neck, as they may be at higher risk of developing this rare complication. In these patients, antiangiogenic tyrosine kinase inhibitors should be used cautiously, patients should be aware of the risk, and physicians should monitor patients for symptoms of fistula.

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., (© 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.)

Esophageal atresia (EA) is one of the congenital neonatal anomalies whose immediate consequence for the newborn is the inability to feed. Most centers strive to minimize the effects of surgeries and subsequent postoperative complications such as esophageal strictures, respiratory problems, and gastrointestinal reflux on the child's ability or motivation to feed. Feeding difficulties in early infancy may not only interrupt maternal expectations of becoming providers of nutrition to their infants but may also influence the infant's development of sensory motor skills and parent-child relationships. Early involvement by a multidisciplinary team consisting of occupational therapist, nutritionist, and psychologist is an important addition to the surgical and medical team. The team assists in preparing mothers for feeding-related difficulties, providing anticipatory guidance to improve feeding abilities and relationships, especially for children with multiple surgical involvements and prolonged periods of non-oral feeding., (© 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.)

Background: With advances in minimally invasive surgery, thoracoscopic repair of oesophageal atresia has become popular in many centres worldwide and indeed has been described as the pinnacle of neonatal surgery. Here, we report our experience in two tertiary referral centres., Methods: Thoracoscopic technique was introduced in 2007. Thus, a retrospective review of all patients diagnosed with oesophageal atresia was carried out. Patients who had thoracoscopic repair were included, and those who had open repair due to co-morbidities were excluded. Patient demographics, operative data, complications, and associated anomalies were noted., Results: A total of thirty-three patients underwent thoracoscopic repair during the time period. Thirty-one were successfully repaired thoracoscopically. Two patients had conversions due to intra-operative instability. The mean body weight of the neonates was 2.58 kg. The mean operative time was 146 min. Three patients suffered from minor anastomotic leaks, which healed on conservative management. Seven patients had anastomotic strictures, which responded successfully to endoscopic dilatation. Two patients died in the post-operative period due to pneumonia. One patient had a recurrent fistula 3 months after the primary repair, and he subsequently underwent a successful second repair., Conclusions: In experienced hands, thoracoscopic repair of oesophageal atresia is at least as good as open surgery but with less surgical trauma. Standard of post-operative care contributes significantly to post-operative outcome. Thoracoscopic technique is now our preferred approach., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Persistence of postintubation esophago-tracheal fistulas is a difficult therapeutic problem. Authors present and discuss two successfully operated cases. Surgical reconstruction was done via left lateral cervical approach, including dissection of the fistulous tract, closure of the defect on both sides by suture, exclusion of the esophagus with a linear stapler beyond a loop esophagostomy, separation the suture lines with interposing of omohioid muscle flap, and making a Pezzer-catheter splinted esophagostomy. In the first case the spontaneous recanalization of the occluded esophagus was prolonged and the fistula recurred. Reoperation was done by the same procedure without exclusion of the esophagus. The second patient needed intervention because of the prolonged closure of esophagostomy. Recovery of both patients was successful. In conclusion, it can be stated that adaptation of surgical techniques for the individual pathologic situation helped the authors to find the way to the successful outcome.

Background: Long-term impairment of pulmonary function in trachea-esophageal fistula (TEF) patients is, at least in part, commonly ascribed to gastro-esophageal reflux disease (GERD). The objective of this study was to examine the independent effects of the underlying condition and GERD on cardiopulmonary function., Methods: Cardiopulmonary function of TEF patients, who had (severe) GERD (s-GERD) requiring antireflux surgery (TEF + GERD, n = 11) and TEF patients who did not have s-GERD (group TEF-GERD, n = 20) were compared with control patients who had isolated s-GERD requiring antireflux surgery (group GERD, n = 13). All patients performed spirometry, lung volume measurements, measurement of diffusion capacity and maximal cardiopulmonary exercise testing (CPET)., Results: Mean age of the participants was 13.8 ± 2.7 (group TEF + GERD). 13.2 ± 2.9 (group TEF-GERD), and 14.7 ± 1.5 years (group GERD). FVC and TLC were significantly lower in patients with TEF (with and without s-GERD) when compared to patients with isolated s-GERD. Most pulmonary function parameters were similarly affected in both TEF groups, but FEV(1) was lower in the TEF + GERD group than in the TEF-GERD group. Cardiopulmonary exercise parameters were similar in all groups., Conclusions: TEF patients had restrictive lung function impairment when compared to patients with isolated s-GERD. This difference may be due to several causes, including thoracotomy. FEV(1) was lower in TEF + GERD when compared to TEF-GERD indicating that GERD may affect large airway function in TEF patients. Other differences between TEF patients with and without s-GERD were not significant, suggesting only a minor role for GERD., (Copyright © 2010 Wiley-Liss, Inc.)

Background: Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA) are congenital anomalies commonly associated with pulmonary complications during early childhood. This study investigated the role of the forced oscillation technique (FOT) in assessing lung function in young children with repaired TOF/OA., Methods: Forty children with repaired TOF/OA of median (range) age 8.0 (3.3-10.6) years, and 20 healthy children without TOF aged 6.1 (3.1-10.8) years were studied. FOT measurements were attempted in all subjects and spirometry only in those 6 years and above. Resistance and reactance (both hPasL(-1)) at 6 Hz (Rrs6 and Xrs6, respectively) and 8 Hz (Rrs8 and Xrs8) measured using FOT, and forced expired volume in 1 sec (FEV(1)), forced vital capacity, functional residual capacity, total lung capacity, and residual volume (all L) obtained from spirometry or plethysmography were compared with reference values and expressed as z-scores., Results: Technically acceptable measurements of Rrs6, Rrs8, Xrs6, Xrs8, Fdep, and Fres were obtained in 37 children with TOF and 20 healthy children without TOF, respectively. Those with TOF had significantly higher mean (SD) z-scores for Rrs6 [0.99 (0.75)] versus healthy children without TOF [0.31 (0.69)] and lower mean (SD) z-scores for Xrs6 [-1.04 (1.07)] versus healthy children without TOF [-0.34 (0.83)]. Spirometry was successful in 24 of the 29 with TOF in whom it was attempted and all healthy children without TOF. Mean (SD) z-score for FEV(1) was significantly lower in those with TOF [-0.86 (1.13)] versus healthy children without TOF [0.67 (0.54)]. z-Scores for Rrs6 and FEV(1) were significantly correlated (r = -0.49; P = 0.003)., Conclusions: Children with repaired TOF have diminished lung function compared with healthy children. FOT is sensitive and correlates well with standard spirometry. It can be used to measure lung function in younger children when spirometry is difficult to perform and should be considered as an objective method for monitoring clinical progress in young children with TOF.

Erb's palsy occurs in neonates following traumatic delivery, where excessive traction on the neck stretches these nerve roots. Nonobstetric causes of Erb's palsy are rare in neonates. The authors report the presentation of a female neonate with Erb's palsy following a postero-lateral thoracotomy. The infant underwent surgery on day 3 of life for esophageal atresia and presented with right upper limb weakness on day 21 of life. She demonstrated features of Erb's palsy with normal higher mental functions. An electromyography and nerve conduction study confirmed Erb's palsy. The surgical procedure in the index case did not involve the brachial plexus. However, her right upper limb was positioned hyperabducted during the intraoperative period, which possibly had led to the palsy. The key message of this report is that prolonged stretching of the brachial plexus roots during surgery of the neck and thorax can be an important nonobstetric cause of Erb's palsy in neonates.

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.

Acquired tracheal diverticulum is a rare clinical entity with only limited reports in the literature. We report a case of a tracheal diverticulum that was identified as part of a work-up for a worsening cough. In this case the diverticulum was observed 2 years after surgical management of a retropharyngeal abscess. It was felt that pooling of secretions in the acquired pouch was a contributing factor in the exacerbation of the patient's chronic cough. Because of the patient's extensive comorbidities, observation and serial computed tomography scans were recommended.

Background: Much of pediatric medicine is focused on prevention of disease and injury. Although accidental ingestions of various household chemicals and medicines are well described and the treatment is supported by local poison control hotlines, the ingestion of button batteries by children is less publicized, and the dangers are less understood by both parents and health care providers., Methods: We describe a case report of a 17-month-old girl with no significant medical history who presented with respiratory distress, cough, and fever and subsequently was discovered to have ingested a button battery., Results: The formation of a traumatic tracheoesophageal fistula required intensive management that escalated to cardiopulmonary bypass and eventual pericardial patch closure of the tracheal defect after the failure of conventional mechanical ventilation., Conclusions: Esophageal button battery impaction places the patient at high risk for full-thickness damage to the esophagus and tracheal structures with fistula formation in as little as a few hours. The key to successful therapy is prompt diagnosis and removal, but in nonverbal pediatric patients, this often is not achievable. Because of the complications associated with this disease (tracheoesophageal fistula) and subsequent difficulties associated with oxygenation and ventilation, these patients should be managed at an institution with the skilled capability of providing cardiopulmonary bypass quickly as a potentially lifesaving therapy.

Oesophageal atresia (OA) encompasses a group of congenital anomalies comprising of an interruption of the continuity of the oesophagus with or without a persistent communication with the trachea. In 86% of cases there is a distal tracheooesophageal fistula, in 7% there is no fistulous connection, while in 4% there is a tracheooesophageal fistula without atresia. OA occurs in 1 in 2500 live births. Infants with OA are unable to swallow saliva and are noted to have excessive salivation requiring repeated suctioning. Associated anomalies occur in 50% of cases, the majority involving one or more of the VACTERL association (vertebral, anorectal, cardiac, tracheooesophageal, renal and limb defects). The aetiology is largely unknown and is likely to be multifactorial, however, various clues have been uncovered in animal experiments particularly defects in the expression of the gene Sonic hedgehog (Shh). The vast majority of cases are sporadic and the recurrence risk for siblings is 1%. The diagnosis may be suspected prenatally by a small or absent stomach bubble on antenatal ultrasound scan at around 18 weeks gestation. The likelihood of an atresia is increased by the presence of polyhydramnios. A nasogastric tube should be passed at birth in all infants born to a mother with polyhydramnios as well as to infants who are excessively mucusy soon after delivery to establish or refute the diagnosis. In OA the tube will not progress beyond 10 cm from the mouth (confirmation is by plain X-ray of the chest and abdomen). Definitive management comprises disconnection of the tracheooesophageal fistula, closure of the tracheal defect and primary anastomosis of the oesophagus. Where there is a "long gap" between the ends of the oesophagus, delayed primary repair should be attempted. Only very rarely will an oesophageal replacement be required. Survival is directly related to birth weight and to the presence of a major cardiac defect. Infants weighing over 1500 g and having no major cardiac problem should have a near 100% survival, while the presence of one of the risk factors reduces survival to 80% and further to 30-50% in the presence of both risk factors.

Objective: To describe our experience with the use of trachea-oesophageal puncture (TEP) after total laryngectomy., Material and Method: We have studied retrospectively 96 patients who received a voice prosthesis through tracheoesophageal puncture between October 2000 and December 2005., Results: The punctures effected were 86 primary (89.6 %) and 10 secondary (10.4 %); of the prostheses, 81 (84.4 %) were Provox(R) type and 74 % of patients used it as their normal means of communication. Reasons for withdrawal included insufficiency and aspiration in 66.7 % (14/21), lack of use in 23.8 % (2/21), granulation tissue, and inflammation in 4.7 % (1/21)., Conclusions: Voice rehabilitation by TEP and prosthesis was successful in 74 % of our patients with acceptable complications and better results than those obtained by tracheaoesophageal speech. We recommend the use of TEP in those patients who have the motivation and a good oncological and physical assessment.

Background: The organogenesis of esophageal atresia with tracheoesophageal fistula remains unclear. We have previously demonstrated that the fistula tract develops from a trifurcation of the embryonic lung bud and displays pulmonary lineage traits. Unlike the lung, the fistula grows without branching. Bone morphogenetic proteins (BMPs) are known to be important in lung branching. We studied possible BMP signaling defects as a potential cause for the absence of branching in the fistula tract., Methods: Adriamycin was administered to pregnant rats on days 6-9 of gestation to induce tracheoesophageal fistula. Microdissection was performed at E13 and E17 isolating the foregut. Tissues were analyzed using immunohistochemistry for BMP ligand (BMP2, BMP4, BMP7) and receptor (BMPRIA, BMPRIB, BMPRII) expression., Results: Immunohistochemistry revealed the presence of all 3 BMP ligands at E13, localized specifically to the esophageal mucosa but absent in the fistula and lung. At E17, the ligands were again present in the esophageal mucosa, and additionally in the fistula tract mucosa, but remained absent in the lung. At E17, all of the BMP receptors were also localized to the luminal surface of esophagus and fistula. However, in the lung epithelium, only BMPRII was found, whereas BMPRIA and BMPRIB remained absent., Conclusions: The normal expression pattern of BMP4 was increased at the branch tips and low between branches. Among other results, we show here a constant expression level of BMP ligands throughout the entire epithelium of the fistula tract. This diffuse expression suggests defective BMP signaling in the fistula tract and explains its nonbranching phenotype.

Malignancy is the most common cause of tracheoesophageal fistulas. The malignancies commonly implicated in the development of tracheoesophageal fistulas are primary bronchial or esophageal carcinomas. Hodgkins disease rarely leads to such a fistula. We report a case of Hodgkin's disease with a tracheoesophageal fistula as well as a left recurrent nerve palsy at presentation. This presentation has no precedent in literature. The patient was treated with systemic chemotherapy and involved field radiotherapy. On follow up 1 year after the completion of treatment, he was clinically and radiologically disease free.

A 9-year-old male child presented with features of recurrent episodes of pneumonia. Following administration of different types of antibiotics, resolution of pneumonia was not complete. Antituberculous drugs were given on two occasions for sufficient period of time without any improvement. Following admission, history of a choking sensation during taking food pointed to perform barium swallow examination of oesophagus which revealed the diagnosis of tracheo-oesophageal fistula.

Background: Two severe birth defects, congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF), have traditionally been analyzed separately in epidemiological studies. Lung hypoplasia (LH), part of the CDH spectrum, is not usually associated with EA/TEF, yet both are foregut malformations., Methods: We conducted an epidemiological study of two combinations of the defects in the population of 3,318,966 live births and stillbirths monitored from 1983 to 1996 by the California Birth Defects Monitoring Program (CBDMP)., Results: A total of 433 cases had a Bochdalek type CDH/LH (0.13 per 1000 births), 893 had EA/TEF (0.27 per 1000 births), and 646 had LH (0.19 per 1000 births). Among them, 18 cases had CDH/LH with EA/TEF (0.005 per 1000 births), and 53 had EA/TEF and LH (0.02 per 1000 births); both prevalences are significantly higher than expected. Sixteen of 17 cases of CDH/LH with EA/TEF, and 34 of 40 cases of EA/TEF with LH were stillborn or died; 72% and 74%, respectively, had an autopsy. The male to female sex ratios were 1.43 and 1.13, respectively. In both groups, infants had similar proportions of additional severe defects, except for genitourinary and anal defects and syndromes/associations, which were more prevalent in the EA/TEF with LH group. We reviewed human studies and experimental animal models for factors reported to cause any combination of the defects., Conclusions: Several genetic and environmental factors could affect the significant co-occurrence of the defects. Future studies should include storage of patients' biological materials for DNA analysis, karyotyping, and environmental exposure evaluation.

Objective: Post-operative cerebrospinal fluid (CSF) fistula following neurosurgery is associated with increased morbidity and mortality. This prospective study evaluates the efficacy of a new bioadhesive--BioGlue, as a dural sealant in preventing CSF fistula. The complications associated with its use are investigated and the literature regarding dural closure reviewed., Methods: BioGlue was applied to the dura mater as a sealant in 210 patients undergoing 216 neurosurgical procedures over a period of 22 months at the Royal Melbourne Hospital. It was used where watertight closure of the dura mater could not be ensured by primary suture alone and for reconstruction of the sellar floor following transsphenoidal adenohypophysectomy. It was used in 114 supratentorial (52.7%), 53 infratentorial (24.5%) craniotomies, 41 (18.9%) transsphenoidal adenohypophysectomies and 8 spinal (3.7%) procedures. The incidence of CSF fistula as a complication of surgery with intradural exposure was analysed., Results: The incidence of CSF fistula post-operatively was significantly low. Two patients (0.93%), both having undergone posterior fossa craniotomy--for evacuation of a cerebellar haematoma and redo excision of a metastasis respectively and both complicated by hydrocephalus, developed CSF fistula. There were no complications associated with the use of BioGlue., Conclusion: BioGlue reduced the incidence of complications associated with neurosurgery. It is an effective adjunct in dural closure to prevent CSF fistula with enhanced bonding properties and is simple to use. In this study there were no complications associated with its use.

Acquired TEF is a rare complication that can occur from a variety of causes. The most common etiology of nonmalignant TEF is as a complication of intubation with cuff-related tracheal injury. Most patients present with increased secretions, pneumonia, and evidence of aspiration of gastric contents while the patient is on mechanical ventilation. When diagnosed after extubation, the most frequent sign of TEF is coughing after swallowing. A high index of suspicion is required in patients at risk for developing a TEF. The diagnostic evaluation is by bronchoscopy and esophagoscopy. When the diagnosis has been made, the immediate goal should be to minimize tracheobronchial soilage by placing the cuff of a tracheostomy tube distal to the fistula. Reflux of gastric contents is diminished by placement of a gastrostomy tube, and adequate nutrition is facilitated by inserting a jejunostomy tube. Surgical correction is required because spontaneous closure is rare, but surgery should be postponed until the patient is weaned from mechanical ventilation because positive pressure ventilation after tracheal repair carries an increased risk of anastomotic dehiscence and restenosis. An anterior cervical collar incision can be used for most cases of post-intubation TEFs. The esophagus should be closed in two layers over a nasogastric tube and buttressed with a pedicled strap muscle flap. If the tracheal defect is small, primary repair can be employed. In most cases, however, the best results can be achieved with tracheal resection and reconstruction. The patient should be extubated at the completion of the case, if possible. With this strategy, as first described by Grillo and colleagues [27], single-stage repair can be performed safely and with a high success rate. Malignant TEFs cannot be cured because of the underlying incurable disease process. As with nonmalignant TEFs, the principal complications are tracheo-bronchial contamination and poor nutrition. Without prompt palliation, death occurs rapidly, with a mean survival time of between 1 and 6 weeks in patients who are treated with supportive care alone. The most common primary tumor causing malignant TEF is esophageal carcinoma. The other frequent cause is lung cancer. Patients present with signs and symptoms typical of TEF, including coughing after swallowing. Diagnosis is made by barium esophagography, and the location and size of the fistula is determined by bronchoscopy and esophagoscopy. Treatment must correct the two problems of airway contamination and poor nutrition. The most effective treatments are esophageal bypass and esophageal stenting. Bypass is demonstrated to resolve respiratory soilage and allow fairly normal swallowing, but it should be reserved for patients who can tolerate a major operation. Stenting can be offered to nearly all patients regardless of their physiologic condition. Stenting also limits aspiration and allows swallowing. Esophageal exclusion is rarely indicated in the current era of familiarity with stenting techniques. Direct fistula closure and fistula resection do not yield satisfactory results. Radiation therapy and chemotherapy combined might offer a survival benefit compared with supportive care alone. The complication of TEF secondary to malignancy is a devastating problem that carries a bleak prognosis, but when it is performed promptly after the diagnosis of a malignant TEF, esophageal bypass or stenting improves survival and quality of life for these unfortunate patients.

Dysphagia, gastroesophageal reflux (GER) and esophageal metaplasia are reported with various incidence in the long term follow-up of patients treated at birth for esophageal atresia (EA). To evaluate the long term outcomes 26 patients treated at birth for EA with Tracheo Esophageal Fistula (TEF) were examined 8-28 (mean 15.8) years later by clinical evaluation, including barium meal, fiberoptic upper GI endoscopy, 24 hour ambulatory two-channel pH-monitoring and stationary esophageal manometry. 50% of patients complained of dysphagia. Mild esophagitis was found in 20% of patients but GER was detected in only 16.7% of the cases. By morphological X-ray, esophageal anomalies were detected in 31% of cases without significant functional relevance. Hundred percent of patients had a disorganized peristaltic esophageal activity and a low amplitude of the esophageal contractions was observed in 58% of them. In our series, esophageal dismotilty seems to be the main consequence of EA without any relevant disturbance of normal nutritional habit.