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Introduction Despite various improvements in technology and patient care, tracheal intubation and tracheostomy still result in significant tracheal injuries like stenosis and airway fistula. Pressure necrosis by the inflated balloon is the commonest culprit. Post-intubation tracheal stenosis is a major indication for tracheal resection and anastomosis. This study was done to find out the indications and results of surgery for post-intubation tracheal lesions. Methods It was a descriptive observational study of patients with postintubation tracheal lesions managed in Manmohan Cardiothoracic Vascular and Transplant Center over a period of 20 years (2001 to 2021). Results Twenty five patients were treated for post-intubation tracheal lesions. Four had isolated tracheo-esophageal fistula (TEF); eighteen had an isolated tracheal stenosis and three had both. Most had a tracheal resection and end-end anastomosis (REEA) via a cervical approach, resecting an average of 2.7 cm of tracheal length. The average size of stenotic lumen was 4.32 mm. There were no operative deaths. Two patients expired within a month of surgery: one due to fatal restenosis and the other due to sepsis. There were a few immediate and a few long-term complications. The average follow-up period was 5 years. Conclusion Among the various post-intubation lesions, tracheal stenosis is the commonest indications for surgery. The management is challenging but safe and feasible, with a multidisciplinary team approach. [ABSTRACT FROM AUTHOR]

Acquired Tracheo-esophageal fistula (TEF) is a challenging and complicated condition. The laryngeal protection is lost in acquired TEF cases due to the established connection between the esophagus and the airways leading to aspiration, pneumonia, and acute respiratory distress syndrome. Malignancy contributes to about 80% of acquired TEF. Nonmalignant causes for TEF include prolonged ventilation, trauma (iatrogenic, penetrating, or blunt injury), foreign bodies, corrosive burns, and granulomatous infections. With the advancements in critical care, the incidence of TEF post-ventilation is on the rise in recent decades. We would like to share our experience managing ten cases of nonmalignant acquired cervical TEF by the lateral cervical approach at our institute. Apart from the isolated TEF cases, one patient with concomitant tracheal stenosis was repaired simultaneously with good postoperative results. TEF was identified in two cases following removal of T-tube and solid stent respectively and was repaired successfully with lateral cervical approach with strap muscle flap interposition. [ABSTRACT FROM AUTHOR]

We report a 49‐year‐old patient with COVID‐19 who developed lung abscess, pleural empyema, and tracheo‐esophageal fistula. He underwent cervicotomy, segmental tracheal resection, esophageal‐tracheal fistula excision, and suturing the esophagus, and a classic thoracotomy was performed. Despite the severe infection, the patient was discharged home after successful surgical treatment. [ABSTRACT FROM AUTHOR]

Summary: Persistent tracheoesophageal fistulas requiring flap repair of the trachea in the mediastinum are uncommon. A 44-year-old man developed a 5 cm x 2 cm persistent posterior tracheal defect after failed tracheo-esophageal fistula repair. The defect was patch repaired using the de-epithelialized skin paddle of an extended pectoralis major flap tunneled into the mediastinum. The use of cross-field ventilation, second rib removal and an anterior tracheostomy slit were crucial for posterior tracheal repair. No major complication occurred. The patch repair with the de-epithelialized skin paddle of pectoralis major flap mucosalized in 2 weeks and the patient was discharged one month postoperatively. The extended skin paddle of the pectoralis major flap was a useful method for tracheal wrap-around reconstruction.

Abstract We report a 49‐year‐old patient with COVID‐19 who developed lung abscess, pleural empyema, and tracheo‐esophageal fistula. He underwent cervicotomy, segmental tracheal resection, esophageal‐tracheal fistula excision, and suturing the esophagus, and a classic thoracotomy was performed. Despite the severe infection, the patient was discharged home after successful surgical treatment.

Aim: We have conducted a study of the clinical profile of esophageal atresia (OA) at our institution over a period of 9 years with an aim toward improving our results. We have attempted to find the significant indicators of mortality to be able to focus on improving the survival of these patients in future. To achieve this end, we applied the Waterston's prognostic classification and the Spitz' prognostic criteria to our patients and studied the effects of each variable independently as a cause of mortality. We have also compared the two prognostic criteria to see, which has more significance for us at our institution. Materials and Methods: Over a period of 9 years, from 2006 to 2014, 73 neonates were treated for OA at our institute. There were 27 mortalities. We analyzed our results using the Waterston and the Spitz criteria and studied each independent variable within the two prognostic classifications for the more highly significant cause of mortality. The variables considered as significant indicators of mortality were low birth weight (LBW), pneumonia, associated congenital heart disease (CHD), other associated anomalies, and very LBW. Results: The Waterston prognostic classification was very highly significant (P < 0.0005) as an indicator of prognosis in this study. The two most significant variables affecting survival were LBW, as described by Waterston (P = 0.005) and the association of major CHD (P = 0.006). We had 63% survival and none below the birth weight of 1.8 kg. Conclusion: The study clearly points out that LBW is a highly significant indicator of prognosis followed by the association of major CHD. Waterston's risk classification continues to be a highly significant indicator of prognosis. Further studies are required to assess the combined effects of more than one variable on babies with OA.

Radiation therapy for broncho-pulmonary malignancies can lead to fistula formation between the digestive and respiratory tracts. Treatment options have been largely palliative in nature. Here, we report a combined pneumonectomy and esophagectomy, followed by staged retrosternal gastric pull-up esophageal reconstruction, for treatment of a broncho-esophageal fistula with good functional outcomes following reconstruction. [ABSTRACT FROM AUTHOR]

Acquired tracheo-esophageal fistulas (TEFs) are challenging. The most common causes are prolonged intubation, malignancy, and trauma whereas granulomatous infections like tuberculosis are rare. Endoscopic intervention with esophageal or tracheal stenting or clipping is of unproven benefit in the management of such lesions, where surgical repair is almost invariably required. We report a case of a 32-year-old man, with a case of multidrug-resistant pulmonary tuberculosis. He had no history of malignancy or trauma. The patient developed spontaneous TEF probably due to mediastinal lymph node necrosis. Multiple attempts were made using staplers, clips, and atrial septal defect (ASD) device closure but were unsuccessful. The nuanced complication leads to very individualized course of treatment which was optimal for this patient. [ABSTRACT FROM AUTHOR]

Gastro-esophageal reflux disease (GERD) is a pathological entity in which the mixture of gastric contents (with low pH because of the high content of HCl acid) and biliary juice (rich in proteolytic enzymes like pepsin and trypsin) causes lesions on the mucosa lining the esophagus. A higher prevalence of GERD was discovered in patients who had a total laryngectomy. Concerning laryngectomies with tracheo-esophageal speaking valves, it has been demonstrated that GERD is an independent risk factor for failure of this method of speech rehabilitation. The authors performed an analysis of English language articles found following a search of the major medical scientific databases (NCBI®, EMBASE®, Cochrane®) containing the following keyword string: vocal prosthesis, total laryngectomy, GERD, acid reflux, tracheo-esophageal fistula, failure. Studies showed that exposure to the protelolytic enzymes from the reflux juice increases the diameter of the tracheo-esophageal fistula and permits local granulation tissue to form. Progressively, the increasing fistula diameter leads to complications ranging from microaspiration of liquids and saliva to speaking valve expulsion or ingestion and temporary incapacity of oral feeding. Medical therapy in the postoperative period as well as long-term (6 months or more) correction of GERD (PPI therapy, H2 blockers, prokinetics) is also demonstrated to reduce the complication rate in this category of patients. GERD has a high prevalence in the total laryngectomy population of patients and it has multiple etiologic factors. In patients with tracheoesophageal vocal prostheses, GERD causes an increase in the fistula diameter – with important consequences that affect the patient's quality of life - his ability to phonate and his ability to swallow. These complications come with added costs because of the need to more frequently change the vocal prostheses with ones increasing in diameter, as well as multiple hospitalizations (even surgery to recalibrate or close the tracheo-esophageal fistula). Recognizing and treating this condition therefore decreases the risk of complications following vocal prosthesis speech rehabilitation.

This is the first description of a trachea-tracheal mucosal tunnel, which occurred after multiple procedures to close a recurrent trachea-oesophageal fistula. Moreover, we are the first to describe completely endoscopic treatment of this unusual condition.

Background: Esophageal Atresia (EA) with or without associated tracheo-esophageal fistula (TEF) is one of the common congenital anomaly that can be life threatening if left unattended. In low and middle income countries like Pakistan, the management and outcome of such type of cases depends upon many factors related to resource limitation. Objective: To prospectively evaluate the management and outcome of esophageal atresia at Children Hospital, Pakistan Institute of Medical Sciences (PIMS), Islamabad, Pakistan. Study design, settings and duration: An Observational- descriptive study was conducted at the Department of Pediatric Surgery, The Children Hospital, PIMS, Islamabad from October 2017 to August 2018. Methodology: Consecutive patients diagnosed with esophageal atresia were included in the study. Demographic data, investigations, procedure performed and outcome were collected on a pre designed proforma and results were analysed. Results: Total 140 consecutive patients of esophageal atresia were enrolled in study. Out of 140 patients, 79 (56.4%) were male and 61 (43.6%) were female. Mean age at presentation of esophageal atresia was 5.5 days (ranged from 1-30 days). Mean weight was 2.43 kg. Regarding type of esophageal atresia, 10 (7.1%) patients had type A, 97 (69.3%) had type C, 1 (0.7%) had type E and 1 (0.7%) had type F esophageal atresia. Nine patients with type A underwent cervical esophagostomy along with feeding gastrostomy. Right thoracotomy was performed in 98 cases. End to end esophageal anastomosis was possible in 76 patients. Twenty one patients had long gap EA for which cervical esophagostomy and feeding gastrostomy was done. Sepsis was the main complication post operatively (29.3%) followed by pneumonia (14.3%), Anastomotic leak (7.9%) and surgical site-infection (2.1%). Overall mortality was 57.9% (81/140) with pre operative mortality of 21.4% (30/140) and post operative mortality of 36.5% (51/140). Low birth weight and post operative sepsis, anastomotic leak and pneumonia had statistically significant relationship with mortality using SPSS version 21. Conclusion: With the improvement of medical facilities, better survival rates of patients with esophageal atresia can be achieved.

Management of complex esophageal problems in children is challenging. We report our experience with the use of esophageal stents in three children with esophageal strictures, leaks, or airway-esophageal fistulae refractory to conventional treatment. The stent played a key role in allowing extubation of a child with a large tracheo-esophageal-pleural fistula and in the resolution of pulmonary infection in a child with esophago-bronchial fistula, both followed by surgery. In the third child, with stricture, stents were complicated with migration, esophageal erosion, and esophago-bronchial fistula. In our experience, esophageal stents were useful mainly as a bridge to definitive surgical repair.

Background: Esophageal atresia (EA) and treacheo-esophageal fistula occur in 1 out of every 3500 live births. Children born with EA have a higher incidence of prematurity than the general population EA. The treatment of EA and tracheo-esophageal fistula, although still a challenge, represents one of the true successes of newborn surgery. Objective: The aim of this study cases with EA and/or tracheo-esophageal fistula in Rapareen pediatric surgery center, Erbil, Iraq, regarding management, and early outcome. Materials and Methods: Fifty-three neonates were enrolled in this study from October 2011 to September 2015. Preoperative investigations included chest X-ray, ultrasound of the abdomen, and echocardiography. All patients were resuscitated before surgical intervention. Statistical Package for the Social Sciences version 20 was used for data analysis. Results: Out of 53 cases, 21 survived and 32 died. Thirty patients were male and 23 were female (male-to-female ratio 1.3:1). Twelve were premature and 41 term babies. The most common type was EA and distal fistula in 47 cases, pure atresia in 5 cases, and EA with both distal and proximal fistula in one case. Presenting features were excessive salivation in all cases, failure to pass nasogastric tube in 98.1%, cyanosis in 69.8%, and chocking in 37.7%. Prenatal history of polyhydramnios was present in 67.9%. Eighteen cases had associated anomalies, most of them were cardiac. Conclusion: EA with distal tracheo-esophageal fistula is the most common type of anomaly. Early diagnosis, weight, maturity, and associated anomalies are the most important factors that affect the outcome. Postoperative respiratory care is necessary, especially for those who have a preoperative chest infection.

Background: Improved surgical techniques, as well as pre and postoperative care, have dramatically changed survival of children with esophageal atresia (EA) with trachea-esophageal fistula (TEF) over last decades. The increase in survival caused a number of young children with post-operative esophageal strictures referred for endoscopic management. Objective: To assess efficacy and safety of endoscopic dilatation of anastomotic strictures in patients with post-surgical repair of EA and TEF. Materials and methods: We retrospectively analyzed clinical endoscopic data of 52 patients with post-surgical anastomotic strictures from March 1994 to December 2019. All procedures were performed under local anesthesia with use of fluoroscopic guidance. Outcome parameters measured included the location of stricture, number of dilations, procedural success rates, and complications such as esophageal perforation. Results: Of 52 patients analyzed, 16 (31%) underwent dilatation with SG dilators used in initial part of the study i.e., from 1994 to 2004. With the availability of CRE balloons, further patients were dilated with CRE balloon 36/52 (69%). Strictures were located centrally in 46 patients (88.6%) and eccentrically in 6 patients (11.5%). Associated pseudodiverticula above the stricture noted in 14(26.9%). The number of dilatations varied from 2 to 16 sessions, mean being 6.3 sessions. Complications secondary to procedure were observed in 7 patients with fever in 4 (7.6%), minor bleed in 2(3.8%) and perforation in one (1.9%) and were treated conservatively. We have not encountered any mortality. No significant difference between SG dilators and CRE balloon group. Conclusions: Endoscopic dilatation of post-surgical esophageal strictures in patients with EA and TEF with SG dilators CRE balloon dilatation is safe and effective with less number of complications. Endoscopic dilatation under local lignocaine anesthesia is feasible, effective and safe. [ABSTRACT FROM AUTHOR]

Filippo Parolini,1 Anna Lavinia Bulotta,1 Sonia Battaglia,1 Daniele Alberti1,2 1Department of Pediatric Surgery, “Spedali Civili” Children’s Hospital, 2Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy Abstract: Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. In recent years, because of the advances in prenatal diagnosis, neonatal critical care, and surgical procedures, overall outcomes have improved substantially, including for premature children. Nowadays, most of the research is focused on medium- and long-term morbidity, with particular reference to respiratory and gastroesophageal problems; the high frequency of late sequelae in esophageal atresia warrants regular and multidisciplinary checkups throughout adulthood. Surprisingly, there are few studies on the impact of prenatal diagnosis and there is continuing debate over the prenatal and preoperative management of these complex patients. In this review, we analyze the literature surrounding current knowledge on the management of newborns affected by esophageal atresia, focusing on prenatal management and preoperative assessment. Keywords: prenatal diagnosis, esophageal atresia, tracheoesophageal fistula, ultrasound scan, tracheobronchoscopy

Background: Esophageal Atresia (EA) with or without associated tracheo-esophageal fistula (TEF) is one of the common congenital anomaly that can be life threatening if left unattended. In low and middle income countries like Pakistan, the management and outcome of such type of cases depends upon many factors related to resource limitation. Objective: To prospectively evaluate the management and outcome of esophageal atresia at Children Hospital, Pakistan Institute of Medical Sciences (PIMS), Islamabad, Pakistan. Study design, settings and duration: An Observational-descriptive study was conducted at the Department of Pediatric Surgery, The Children Hospital, PIMS, Islamabad from October 2017 to August 2018. Methodology: Consecutive patients diagnosed with esophageal atresia were included in the study. Demographic data, investigations, procedure performed and outcome were collected on a pre designed proforma and results were analysed. Results: Total 140 consecutive patients of esophageal atresia were enrolled in study. Out of 140 patients, 79 (56.4%) were male and 61 (43.6%) were female. Mean age at presentation of esophageal atresia was 5.5 days (ranged from 1-30 days). Mean weight was 2.43 kg. Regarding type of esophageal atresia, 10 (7.1%) patients had type A, 97 (69.3%) had type C, 1 (0.7%) had type E and 1 (0.7%) had type F esophageal atresia. Nine patients with type A underwent cervical esophagostomy along with feeding gastrostomy. Right thoracotomy was performed in 98 cases. End to end esophageal anastomosis was possible in 76 patients. Twenty one patients had long gap EA for which cervical esophagostomy and feeding gastrostomy was done. Sepsis was the main complication post operatively (29.3%) followed by pneumonia (14.3%), Anastomotic leak (7.9%) and surgical site-infection (2.1%). Overall mortality was 57.9% (81/140) with pre operative mortality of 21.4% (30/140) and post operative mortality of 36.5% (51/140). Low birth weight and post operative sepsis, anastomotic leak and pneumonia had statistically significant relationship with mortality using SPSS version 21. Conclusion: With the improvement of medical facilities, better survival rates of patients with esophageal atresia can be achieved. [ABSTRACT FROM AUTHOR]

Background: Repair of large, upper thoracic, cuff-induced, tracheo-esophageal fistula (TEF) is technically demanding and is conventionally performed by open surgery. Minimal access approach is, hitherto, unreported. Technique & Case: Minimally invasive repair of TEF involving fistula isolation - by thoracoscopic oesophageal exclusion, and simultaneous establishment of alimentary continuity - by laparoscopy-assisted sub-sternal colonic transposition, is described. The technique was successfully employed in repairing a large (4.5 centimetres), cuff-induced, upper thoracic TEF, in a 25-year-old woman. The rationale behind the technique, its pros and cons are analysed and contrasted against conventional techniques of TEF repair. Conclusion: Large upper thoracic, cuff-induced TEF can be successfully repaired employing minimal access. [ABSTRACT FROM AUTHOR]

Gastro-esophageal reflux disease (GERD) is a pathological entity in which the mixture of gastric contents (with low pH because of the high content of HCl acid) and biliary juice (rich in proteolytic enzymes like pepsin and trypsin) causes lesions on the mucosa lining the esophagus. A higher prevalence of GERD was discovered in patients who had a total laryngectomy. Concerning laryngectomies with tracheo-esophageal speaking valves, it has been demonstrated that GERD is an independent risk factor for failure of this method of speech rehabilitation. The authors performed an analysis of English language articles found following a search of the major medical scientific databases (NCBI®, EMBASE®, Cochrane®) containing the following keyword string: vocal prosthesis, total laryngectomy, GERD, acid reflux, tracheo-esophageal fistula, failure. Studies showed that exposure to the protelolytic enzymes from the reflux juice increases the diameter of the tracheo-esophageal fistula and permits local granulation tissue to form. Progressively, the increasing fistula diameter leads to complications ranging from microaspiration of liquids and saliva to speaking valve expulsion or ingestion and temporary incapacity of oral feeding. Medical therapy in the postoperative period as well as long-term (6 months or more) correction of GERD (PPI therapy, H2 blockers, prokinetics) is also demonstrated to reduce the complication rate in this category of patients. GERD has a high prevalence in the total laryngectomy population of patients and it has multiple etiologic factors. In patients with tracheoesophageal vocal prostheses, GERD causes an increase in the fistula diameter - with important consequences that affect the patient's quality of life - his ability to phonate and his ability to swallow. These complications come with added costs because of the need to more frequently change the vocal prostheses with ones increasing in diameter, as well as multiple hospitalizations (even surgery to recalibrate or close the tracheo-esophageal fistula). Recognizing and treating this condition therefore decreases the risk of complications following vocal prosthesis speech rehabilitation. [ABSTRACT FROM AUTHOR]

Tracheo-esophageal fistula (TEF) without associated esophageal atresia or H-type fistula is a rare congenital anomaly. H-type fistula is usually missed in the neonatal period as the presenting symptoms are either of recurrent pneumonia or gastro-esophageal reflux which always lead to delay in diagnosis and infant undergoes unnecessary treatment. We report a case of H-type of TEF, diagnosed within 12 days of birth based upon choking and cyanosis on the first trial of spoon feeds. Diagnosis was confirmed with contrast esophagogram. The infant was operated for it and was successfully discharged.

Objective: Tracheal stenosis is usually iatrogenic, a result of an accident or due to tracheal tumors. Anesthesia for tracheal resection and reconstruction is a challenging job and requires expertise. The crux of anesthetic management is securing the airway early and maintenance of ventilation and oxygenation during resection and reconstruction. This study is aimed to share the anesthetic management and outcome of 21 cases of tracheal resection and reconstruction surgery for tracheal stenosis. Methodology: This prospective, descriptive study was carried out at PNS Shifa Hospital Karachi and Combined Military Hospital Rawalpindi between October 2014 and July 2018. All 21 patients undergoing tracheal resection and reconstruction surgery for tracheal stenosis at these centers were enrolled. Informed consent was obtained from all patients and institutional ethics committee approval was secured. The site of tracheal stenosis, type of anesthesia used, ventilation and oxygenation before and during resection and anastomosis of trachea and the type of surgery and the outcome was noted. The data were collected in MS Excel sheet and simple statistical analysis done to present the results. Results: Out of 21 patients, 9 (43%) were males and 12 (57%) were females, between 6 to 66 years of age of ASA II-IV. Post intubation stenosis was the leading cause of stenosis followed by tumor, trauma and corrosive ingestion. Fourteen patients had high cervical / subglottic stenosis and were operated by high anterior cervical collar incision, while five had lower tracheal lesions, and 2 had carinal lesion and were operated by right thoracotomy. Seven patients were anesthetized through tracheostomy tube, one by fiberoptic intubation, and the rest with 5-7 mm ETT with or without muscle relaxant. One patient developed cardiac arrest during surgery, but was revived successfully. Four (19%) out of 21 had to be put on ventilator postoperatively while remaining 17 (89%) were extubated on operating table. Two patients on ventilator were weaned of successfully. Outcome was excellent in 19 (90%) cases while in 2 (10%) patients, operation was unsuccessful and they landed up with permanent tracheostomy. Conclusion: The study highlights the importance of prevention of post-intubation tracheal stenosis with strict vigilance and high quality professional nursing care. Thorough preoperative assessment and preparation, intra operative management, a backup plan and close communication between the surgeon and anesthesiologist are necessary for successful outcome. Most of these patients require general anesthesia and profound relaxation. [ABSTRACT FROM AUTHOR]

Heterotopic gastric mucosa (HGM) is occasionally found at endoscopy in the proximal esophagus of adults and children, when it manifests as an asymptomatic small island of reddish pink mucosa just below the upper esophageal sphincter. There are few reports of esophageal HGM detected by endoscopy after repair of esophageal atresia (EA) with tracheo-esophageal fistula (TEF). We report a child with multiple patches of HGM in the proximal and distal esophagus seen at endoscopy after EA/TEF repair. No obvious symptoms were related to the HGM and she remains under endoscopic surveillance. The incidence of esophageal HGM may be increased in patients with EA and its distribution can be more extensive than a simple “inlet patch”. There is evidence to suggest that esophageal HGM increases the risk of developing Barrett's esophagus and has a malignant potential. Heterotopic gastric mucosa extends the spectrum of potential pathologies affecting the esophagus in patients with EA/TEF and supports current international guidelines for endoscopic surveillance of these patients. [ABSTRACT FROM AUTHOR]

Esophageal atresia (OA) represents one of the commonest and most severe developmental disorders of the foregut, the most proximal segment of the gastrointestinal (GI) tract (esophagus and stomach) in embryological terms. Of intrigue is the common origin from this foregut of two very diverse functional entities, the digestive and respiratory systems. OA appears to result from incomplete separation of the ventral and dorsal parts of the foregut during development, resulting in disruption of esophageal anatomy and frequent association with tracheo-oesophageal fistula. Not surprisingly, and likely inherent to OA, are associated abnormalities in components of the enteric neuromusculature and ultimately loss of esophageal functional integrity. An appreciation of such developmental processes and associated defects has not only enhanced our understanding of the etiopathogenesis underlying such devastating defects but also highlighted the potential of novel corrective therapies. There has been considerable progress in the identification and propagation of neural crest stem cells from the GI tract itself or derived from pluripotent cells. Such cells have been successfully transplanted into models of enteric neuropathy confirming their ability to functionally integrate and replenish missing or defective enteric nerves. Combinatorial approaches in tissue engineering hold significant promise for the generation of organ-specific scaffolds such as the esophagus with current initiatives directed toward their cellularization to facilitate optimal function. This chapter outlines the most current understanding of the molecular embryology underlying foregut development and OA, and also explores the promise of regenerative medicine.

This report describes a successful outcome in a preterm baby with an esophageal atresia and tracheo-esophageal fistula, who initially underwent a primary esophageal repair; but a persistent nonexpanding lung on the side of surgery led to further investigations. A further diagnosis of an esophageal lung resulted in pneumonectomy and prophylactic placement of an intra-thoracic prosthesis to prevent post-pneumonectomy syndrome. To the best of our knowledge, this is the first report of a prophylactic placement of an intra-thoracic prosthesis in a neonate with the condition of esophageal atresia and tracheo-esophageal fistula and associated esophageal lung.

Tracheoesophageal fistula (TEF) is one of the most common congenital anomaly requiring surgical correction in neonatal period. The important goal of airway management is to avoid excessive gastric distension and ensure adequate ventilation prior to surgical ligation of the fistula. If a large fistula is present close to carina, excessive loss of delivered tidal volume may lead to ineffective ventilation. In addition, gastric distension elevates diaphragm and diminishes the lung compliance. If lung compliance is already impaired due to pre-existing lung pathology, situation becomes much more demanding. We report the successful airway management of a patient with large precarinal fistula and bilateral pneumonitis using the novel Microcuff tube. The unique design of microcuff makes it suitable to be used for this purpose. To the best of our knowledge, the use of microcuff ETT for perioperative airway management in case of a large precarinal fistula in a neonate with respiratory pathology has not been reported in the past.

Prune belly syndrome is a rare abnormality; its association with VACTERL is even rarer. This association has been reported in literature a few times since first reported in 1993 and so far the majority have either been stillbirths or died shortly after birth. We present a case of Prune belly syndrome associated with VACTERL who is now one year old.

The incidence of tracheo-esophageal (TO) fistula is on the rise, especially after palliative management for esophageal malignancies. We report a case of cancer of esophagus who after chemotherapy and radiotherapy developed TO fistula. Placement of an esophageal stent helped him in taking food orally, but his cough and dyspnoea continued to worsen. Fibreoptic bronchoscopy demonstrated a severely compressed trachea secondary to protrusion of esophageal stent which responded very well to an Ultraflex-covered tracheal stent and the patient achieved relief from cough and dyspnoea.

Children with congenital tracheo-esophageal fistula and esophageal atresia need surgical intervention for their survival. Apart from the several gastro-intestinal and respiratory problems that often follow surgery, there is an increased risk of impaction of foreign body above the reconstructed esophagus that becomes stenosed and/or dysmotile. Here we illustrate a case of a six-year-old boy who underwent repair of a sporadic form of congenital tracheo-esophageal fistula with esophageal atresia at the age of three months, presenting with impaction of a glass marble above the repaired esophageal segment. This report adds to the long-term care of reconstructed aerodigestive tract in children in the form of strict vigilance from the parents and care-givers so as to keep them safe from small objects that can potentially result in impacted foreign bodies. [ABSTRACT FROM AUTHOR]

Two cases of hypertrophic pyloric stenosis (HPS) developed after a few weeks of repair of an esophageal atresia and tracheo-esophageal fistula (EA&TEF). Both cases were dealt successfully with laparoscopic pyloromyotomy.

Tracheo-esophageal fistula (TEF) without associated esophageal atresia or H-type fistula is a rare congenital anomaly. H-type fistula is usually missed in the neonatal period as the presenting symptoms are either of recurrent pneumonia or gastro-esophageal reflux which always lead to delay in diagnosis and infant undergoes unnecessary treatment. We report a case of H-type of TEF, diagnosed within 12 days of birth based upon choking and cyanosis on the first trial of spoon feeds. Diagnosis was confirmed with contrast esophagogram. The infant was operated for it and was successfully discharged. [ABSTRACT FROM AUTHOR]

INTRODUCTION Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge. PRESENTATION OF CASE Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms. DISCUSSION While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances. CONCLUSION A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes. [ABSTRACT FROM AUTHOR]

Esophageal Atresia (EA) is a severe developmental defect of the foregut that presents with or without a Tracheo-Esophageal Fistula (TEF). The prevalence of EA/TEF over time and around the world has been relatively stable. EA/TEF is manifested in a broad spectrum of anomalies: in some patients it manifests as an isolated atresia or fistula, but in over half it affects several organ systems. While the associated malformations are often those of the VACTERL spectrum (Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal and Limb), many patients are affected by other malformations, such as microcephaly, micrognathia, pyloric stenosis, duodenal atresia, a single umbilical artery, and anomalies of the genitourinary, respiratory and gastrointestinal systems. Though EA/TEF is a genetically heterogeneous condition, recurrent genes and loci are sometimes affected. Tracheo-Esophageal (TE) defects are in fact a variable feature in several known single gene disorders and in patients with specific recurrent Copy Number Variations and structural chromosomal aberrations. At present, a causal genetic aberration can be identified in 11-12% of patients. In most, EA/TEF is a sporadic finding; the familial recurrence rate is low (1%). As this suggests that epigenetic and environmental factors also contribute to the disease, non-syndromic EA/TEF is generally believed to be a multifactorial condition. Several population-based studies and case reports describe a wide range of associated risks, including age, diabetes, drug use, herbicides, smoking and fetal alcohol exposure. The phenotypical and genetic heterogeneity seen in EA/TEF patients indicates not one underlying cause, but several. Unraveling the complex multifactorial and heterogeneous etiology of EA/TEF and associated features will require large cohorts of patients. Combined statistical analysis of component findings, genome sequencing, and genome wide association studies will elucidate new causal genetic defects and predisposing loci in the etiology within specific sub-populations. Improved knowledge of environmental risk factors, genetic predisposition and causal genetic syndromes may improve prediction and parental counseling, and prevent co-morbidity. [ABSTRACT FROM AUTHOR]