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1.  Simple strategy of anesthesia for the neonate with tracheoesophageal fistula: a case report 
A 3-day-old neonate, given a diagnosis of esophageal atresia (EA) with tracheoesophageal fistula (TEF), which is large and just above the carina, was scheduled for TEF repair. Routine anesthetic management focuses on adequate ventilation and avoidance of gastric distension during positive pressure ventilation. Using a balloon-tipped embolectomy catheter or a Fogarty catheter to block the fistula under the guidance of fiberoptic scope has been described. In most of the medical centers, however, the pediatric fiberoptic scope may not be available. We present a case of a newborn undergoing type C EA/TEF repair and describe a simple intra-operative technique that could temporarily occlude the gastroesophageal junction, allowing stable vital signs of patient and definitive repair of the tracheoesophageal fistula.
PMCID: PMC3902279  PMID: 24482727
Esophageal atresia (EA); tracheoesophageal fistula (TEF); repair; occlude; ligation
2.  Tracheoesophageal fistula following disc battery ingestion and foreign body impaction  
Background: Ingestion of foreign bodies may result in the formation of a tracheoesophageal fistula (TEF), which causes severe morbidity in children. We describe four cases of TEF, who underwent emergent surgery for repair.
Case presentation: In this report, we present about four patients aged between 9 months to 2.5 years, who referred due to disc battery ingestion. There were two boys and two girls. The common symptoms were cough, cyanosis, and dysphagia, choking and vomiting. The diagnosis was performed through an x-ray, barium swallow and CT Scan. All batteries were impacted in the esophagus, two in upper, one in the middle, and one in lower esophagus position. All disc batteries were removed endoscopically, but had tracheoesophageal fistula (TEF). All the patients underwent TEF repaired surgically. There was no morbidity in four patients, but one patient developed moderate esophageal stenosis, which was repaired by staged dilatation. There was no mortality in our cases.
Conclusion: Long-term impaction of foreign bodies may result in tracheoesophageal fistula. This complication may be seen earlier with alkaline disc batteries. Removal of these foreign bodies should be followed carefully for the diagnosis and treatment of these fistulas.
PMCID: PMC3895833  PMID: 24551442
Esophageal foreign body; Disc battery; tracheoesophageal fistula; Children.
3.  Esophageal atresia in newborns: a wide spectrum from the isolated forms to a full VACTERL phenotype? 
Background
VATER association was first described in 1972 by Quan and Smith as an acronym which identifies a non-random co-occurrence of Vertebral anomalies, Anal atresia, Tracheoesophageal fistula and/or Esophageal atresia, Radial dysplasia. It is even possible to find out Cardiovascular, Renal and Limb anomalies and the acronym VACTERL was adopted, also, embodying Vascular, as single umbilical artery, and external genitalia anomalies.
Methods
Data on patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) admitted in the Neonatal Intensive Care Unit (NICU) between January 2003 and January 2013 were evaluated for the contingent occurrence of typical VACTERL anomalies (VACTERL-type) and non tipical VACTERL anomalies (non-VACTERL-type). The inclusion criterion was the presence of EA with or without TEF plus two or more of the following additional malformations: vertebral defects, anal atresia, cardiovascular defects, renal anomalies and lower limb deformities, like radial dysplasia.
Results
Among 52 patients with EA/TEF, 20 (38,4%) had isolated EA and 7 (21,8%) had a recognized etiology such a syndrome and therefore were excluded. Among 32 infants with EA and associated malformations, 15 (46,8%) had VACTERL association. The most common anomalies were congenital heart defects (73,3%), followed by vertebral anomalies (66,6%). Many patients also had additional non-VACTERL-type defects. Single umbilical artery was the most common one followed by nervous system abnormalities and anomalies of toes. Between the groups of infants with VACTERL type and non-VACTERL-type anomalies, there are several overlapping data regarding both the tipically described spectrum and the most frequently reported non-VACTERL-type malformations. Thus, it is possible to differentiate infants with a full phenotype (VACTERL full phenotype) and patients that do not meet all the criteria mentioned above, but with some homologies with the first group (VACTERL partial phenotype).
Conclusion
The high frequency of non-VACTERL-type anomalies encountered in full and partial phenotype patients would suggest the need for an extension of the clinical criteria for the diagnosis of VACTERL association and also for pre- and post-operative management and follow-up in the short and long term.
doi:10.1186/1824-7288-39-45
PMCID: PMC3726359  PMID: 23842449
Esophageal atresia; Tracheo-esophageal fistula; VATER; VACTERL; Association; Congenital malformations; Anomalies
4.  Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre 
Objective:
To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome.
Materials and Methods:
A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading.
Results:
EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C.
Conclusion
Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.
doi:10.4103/0971-9261.42564
PMCID: PMC2810819  PMID: 20177477
Associated anomalies; esophageal atresia; prognosis; tracheoesophageal fistula
5.  Current knowledge on esophageal atresia 
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.
doi:10.3748/wjg.v18.i28.3662
PMCID: PMC3406418  PMID: 22851858
Esophageal atresia; Tracheoesophageal fistula; Esophageal stenosis; Long-gap; Gastro-esophageal reflux
6.  Isolated congenital tracheal stenosis in a preterm newborn 
European Journal of Pediatrics  2011;170(9):1217-1221.
Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory.
doi:10.1007/s00431-011-1490-x
PMCID: PMC3158335  PMID: 21590265
Congenital tracheal stenosis; Neonate; Respiratory failure; VACTERL association
7.  Application of MPVR and TL-VR with 64-row MDCT in neonates with congenital EA and distal TEF 
AIM: To assess the application of multiple planar volume reconstruction (MPVR) and three-dimensional (3D) transparency lung volume rendering (TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula (TEF).
METHODS: Twenty neonates (17 boys, 3 girls) with EA and distal TEF at a mean age of 4.6 d (range 1-16 d) were enrolled in this study. A helical scan of 64-row MDCT was performed at the 64 mm × 0.625 mm collimation. EA and TEF were reconstructed with MPVR and TL-VR, respectively. Initial diagnosis of EA was made by chest radiography showing the inserted catheter in the proximal blind-ended esophageal pouch. Manifestations of MDCT images were compared with the findings at surgery.
RESULTS: MDCT showed the proximal and distal esophageal pouches in 20 cases. No significant difference was observed in gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR. The lengths of gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR correlated well with the findings at surgery (R = 0.87, P < 0.001). The images of MPVR revealed the orifice of TEF in 13 cases, while TL-VR images showed the orifice of TEF in 4 cases.
CONCLUSION: EA and distal TEF can be reconstructed using MPVR and TL-VR of 64-row MDCT, which is a noninvasive technique to demonstrate the distal esophageal pouches and inter-pouch distance in neonates with EA and distal TEF.
doi:10.3748/wjg.v17.i12.1649
PMCID: PMC3070138  PMID: 21472133
Children; Computed tomography; Congenital malformation; Esophagus; Tracheoesophageal fistula
8.  Congenital tracheoesophageal fistula and coordination of care: Expectations and realities 
Paediatrics & Child Health  2006;11(7):395-399.
BACKGROUND
Tracheoesophageal fistula (TEF) is a rare congenital anomaly with chronic morbidities. Aside from health care costs, the authors suspected that additional burden rests on the family due to hospitalizations, radiological and surgical procedures, and frequent outpatient visits. It was speculated that this complex care is poorly coordinated. The objective of the present study was to document utilization and coordination of health care services for children with TEF at the Alberta Children’s Hospital (Calgary, Alberta).
METHODS
Medical records of children with TEF (primary surgical repair at Alberta Children’s Hospital between April 1994 and September 1999) were reviewed for demographics, TEF type, associated anomalies, age at diagnosis and repair, health services utilization data, and radiological and surgical procedures.
RESULTS
Twenty-two children were identified, of whom 18 survived beyond one year. Ten of these 18 children were male. Average gestational age and birth weight were 37.4 weeks (range 32 to 42 weeks) and 2512 g (range 780 g to 3950 g), respectively. Seventy-eight per cent of children had at least one associated anomaly. Median initial hospital and intensive care unit stays were 27.5 days and 12 days, respectively. During year 1 of life, there was a median of 31 radiological examinations, five surgical procedures, 2.5 hospital admissions and five outpatient clinic visits. Coordination of procedures and outpatient visits was poor. In year 2 of life, children had fewer procedures, admissions and outpatient visits; between years 2 and 5, procedures or admissions were rare.
CONCLUSIONS
Children with TEF endure multiple procedures and lengthy hospital admissions in early life. There was easy access to, but minimal coordination of, subspecialist care, procedures and admissions. A dedicated multidisciplinary clinic for children with TEF should improve care.
PMCID: PMC2528634  PMID: 19030308
Child; Chronic disease; Delivery of health care; Tracheoesophageal fistula
9.  Delayed primary reconstruction of esophageal atresia and distal tracheoesophageal fistula in a 471-g infant 
INTRODUCTION
Waterston et al. have classified the risk of morbidity in infants with esophageal atresia and tracheoesophageal fistula. However, few cases of esophageal atresia with distal tracheoesophageal fistula in extremely low birth weights infants have been reported. In such infants, the selection of primary reconstruction or staged repair remains controversial. In the present report, we describe the difficulties of perioperative management of such small infants and discuss how to rescue them.
PRESENTATION OF CASE
A 471-g female infant was delivered at 28 weeks’ gestation via cesarean section. Esophageal atresia with distal tracheoesophageal fistula was diagnosed. Esophageal banding and gastrostomy were performed via laparotomy on day 1. On day 74, when the infant weighed almost 1000 g, airway management was discontinued. On day 136, endotracheal intubation again became necessary because of respiratory problems, and the esophagus was reconstructed on day 141. Despite this operation, the patient died on day 276 because of continuing respiratory problems.
DISCUSSION
Esophageal banding is considered an appropriate treatment for respiratory problems in such extremely low weight infants. However, the timing of dissection of the tracheoesophageal fistula after esophageal banding is extremely important.
CONCLUSION
In the present case, ligation of the tracheoesophageal fistula and esophageal reconstruction should have been performed as soon as possible.
doi:10.1016/j.ijscr.2012.11.010
PMCID: PMC3540228  PMID: 23276758
Esophageal atresia; Extremely low weight infants; Esophageal banding
10.  An 18-year experience of tracheoesophageal fistula and esophageal atresia 
Korean Journal of Pediatrics  2010;53(6):705-710.
Purpose
To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit.
Methods
A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007.
Results
The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37+2 weeks and 2.5±0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age.
Conclusion
Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.
doi:10.3345/kjp.2010.53.6.705
PMCID: PMC2994136  PMID: 21189942
Tracheoesophageal fistula; Esophageal atresia; VACTERL association; Mortality; Morbidity; Growth
11.  Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario? 
Aims:
To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages.
Materials and Methods:
A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed.
Results:
Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis.
Conclusions:
In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.
doi:10.4103/0971-9261.98123
PMCID: PMC3409897  PMID: 22869975
Anorectal malformation; esophageal atresia; neonatal intensive care; tracheoesophageal fistula
12.  EMERGENCY OPERATIONS IN THE NEWBORN 
California Medicine  1958;88(3):221-227.
With the present-day development and understanding of anesthetic methods, fluid and electrolyte therapy, antibiotic medications and pediatric care, many congenital anomalies once uniformly fatal are now being successfully treated by emergency operations in the neonatal period. The eight most common of these which demand emergency operation in the immediate postnatal period are esophageal atresia and tracheoesophageal fistula, diaphragmatic hernia with dislocation of the abdominal viscera into the chest, malrotation of the intestine with obstruction, intestinal atresia, meconium ileus, imperforate anus, omphalocele and myelomeningocele.
Although infants born with any of these serious problems often are born prematurely and often have more than one congenital anomaly, survival rates in the surgical treatment of these conditions are steadily improving. Early diagnosis and prompt treatment are the most important factors in the continued improvement of these survival rates.
PMCID: PMC1512397  PMID: 13511214
13.  Associated congenital anomalies between neonates with short-gap and long-gap esophageal atresia: a comparative study 
Background:
Predicting the presence of long-gap esophageal atresia (EA) prior to the surgery is of clinical importance. No comparison between short-gap and long-gap EA for the prevalence of VACTERL and non-VACTERL-type anomalies has yet been performed.
Objective:
The aim of this study was to compare VACTERL and non-VACTERL-type anomalies between patients with short-gap and long-gap EA.
Methods:
Retrospectively, medical records of all newborns managed for EA/tracheoesophageal fistula (TEF) in Tabriz Children’s Hospital and Tehran Mofid Hospital between 2007 and 2010 were evaluated. Demographic data and associated anomalies including both the VACTERL and non-VACTERL-type defects were listed. The VACTERL spectrum defects covered vertebral/costal, anorectal, cardiovascular, TEF, and renal- or radial-type limb anomalies. The non-VACTERL-type anomalies included hydrocephalus, orofacial defects, respiratory system anomalies, gastrointestinal anomalies, genital anomalies, and non-VACTERL limb defects. Demographic data, and the VACTERL and non-VACTERL-type anomalies were compared among children with long-gap EA and those with short-gap EA.
Results:
Two hundred and seventy-six children were included in the study: 230 (83.3%) in the short-gap EA group and 46 (16.7%) in the long-gap EA group. Although prevalence of the VACTERL spectrum anomalies did not differ between the two groups, the non-VACTERL anomaly was more common in the long-gap EA group (P = 0.02). Among the VACTERL-type defects, TEF was detected in 30 (65.2%) and 218 (94.7%) patients in long-gap and short-gap EA groups, respectively (P = 0.0001).
Conclusion:
The non-VACTERL-type anomalies, but not the VACTERL spectrum defects, are more frequent in patients with long-gap EA than those with short-gap EA.
doi:10.2147/IJGM.S19301
PMCID: PMC3133517  PMID: 21760750
esophageal atresia; short-gap; long-gap; tracheoesophageal fistula; anomaly; VACTERL
14.  Anorectal Malformations Associated with Esophageal Atresia in Neonates 
Purpose
Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia.
Methods
Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted.
Results
The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems.
Conclusion
This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.
doi:10.5223/pghn.2013.16.1.28
PMCID: PMC3746047  PMID: 24010103
Anorectal malformation; Esophageal atresia; Neonates
15.  Delayed diagnosis of high proximal tracheoesophageal fistula in esophageal atresia and a novel approach to the treatment of tracheomalacia by submanubrial tracheopexy 
SpringerPlus  2014;3:113.
An infant with esophageal atresia (EA) had delayed diagnosis of proximal tracheoesophageal fistula (TEF) and severe tracheomalacia. We recommend bronchoscopy via laryngeal mask or rigid bronchoscopy to rule out associated TEF in infants diagnosed with esophageal atresia, as flexible bronchoscopy via endotracheal tube may not provide complete visualization of the trachea. We also describe a novel cervical approach to tracheopexy via neck incision for treatment of associated severe tracheomalacia in this infant.
doi:10.1186/2193-1801-3-113
PMCID: PMC3951653  PMID: 24634808
Esophageal atresia; Tracheoesophageal fistula; Tracheopexy
16.  A case of VACTERL and non-VACTERL association without the “V and L” 
VACTERL is a cluster of congenital malformations based on the non-random association of various congenital malformations in a single patient. Here “V” denotes vertebral defects or vascular anomalies (single umbilical artery), “A” anal atresia, “C” cardiac abnormalities, “TE” tracheoesophageal fistula, “R"renal (kidney) abnormalities and “L” for limb anomalies) It is called an association, rather than a syndrome because the complications are not pathogenetically related, tend to occur more frequently than expected and are thought to be linked to embryonic mesodermal defects. Studies have reported the coexistence of various other congenital malformations such as respiratory, cerebral anomalies, which are frequently referred as non-VACTERL-type of associations. Diagnosis of VACTERL association is done only when at least three of the above mentioned congenital malformations are identified in a patient. Although 80% of these cases have vertebral defects, our case is unique as patient does not have one of the commonest occuring association i.e., vertebral anomalies, but has all other associations and an additional non VACTERL brain anomaly, hitherto unreported in the literature. The other highlight of this case is although reports say that VACTERL babies with ipsilateral renal disorder have the same side limb defects, our case has a renal anomaly with no limb anomaly. Finally VACTERL and non VACTERL association was considered in our patient in view of ventricular septal defect, tracheo esophageal fistula, anal atresia, renal anomaly, seizure disorder and global developmental delay due to pontocerebellar hypoplasia.
doi:10.4103/0972-3919.125776
PMCID: PMC3928753  PMID: 24591785
Crossed fused ectopic kidney; gastro esophageal reflux scintigraphy; methylene diphosphonate bone scan; tracheo esophageal fistula; vertebral defects; vascular anomalies; anal atresia; VSD; renal and limb anomalies
17.  Early presentation of postintubation tracheoesophageal fistula: Perioperative anesthetic management 
Tracheoesophageal fistula (TEF) in adults occurs as a result of trauma, malignancy, cuff-induced tracheal necrosis from prolonged mechanical ventilation, traumatic endotracheal intubation, foreign body ingestion, prolonged presence of rigid nasogastric tube, and surgical complication. Anesthetic management for repair of TEF is a challenge. Challenges include difficulties in oxygenation or ventilation resulting from placement of endotracheal tube in or above the fistula; large fistula defect causing loss of tidal volume with subsequent gastric dilatation, atelactasis, and maintenance of one lung ventilation. The most common cause of acquired nonmalignant TEF is postintubation fistula, which develops after prolonged intubation for ventilatory support. Acquired TEF, which occurs after prolonged intubation, usually develops after 12–200 days of mechanical ventilation, with a mean of 42 days. We present a rare case of TEF that developed after 7 days of intubation. It was a difficult case to be diagnosed as patient had a history of polytrauma, followed by emergency intubation and both these conditions can contribute to tracheobronchial injury.
doi:10.4103/0970-9185.92460
PMCID: PMC3275943  PMID: 22345958
Mechanical ventilation; postoperative; tracheosophageal fistula
18.  Congenital tracheoesophageal fistula: A rare and late presentation in adult patient 
Annals of Thoracic Medicine  2012;7(1):48-50.
Congenital H-type tracheoesophageal fistula (TEF) in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.
doi:10.4103/1817-1737.91553
PMCID: PMC3277043  PMID: 22347352
Adult; congenital tracheoesophageal fistula; late presentation
19.  Tracheoesophageal Fistula with Esophageal Atresia with Absent External Ear: A Unusual Association 
The Indian Journal of Surgery  2012;74(4):337-339.
Esophageal atresia with tracheoesophageal fistula (TEF with EA) is a common disease which is associated with many other congenital abnormalities. The most common association is with VACTERL syndrome.. Various types of ear malformations have been reported in CHARGE syndrome associated with EA and TEF. However, absence of external ear has not been described which is being reported in this article. We could not found this association even on extensive search of literature.
doi:10.1007/s12262-012-0410-2
PMCID: PMC3444599  PMID: 23904729
Esophageal atresia; Tracheoesophageal fistula; Ear deformity; Absent pinna
20.  Tracheoesophageal fistula: New option in management 
Tracheoesophageal fistula is a life threatening condition. Patients not managed surgically ultimately die of their disease. Surgical management is the treatment of choice. We present a case of a patient that developed a tracheoesophageal fistula after tracheostomy. Surgical repair was done which failed due to infection. The patient was managed with the help of an esophageal stent and Trichloroacetic Acid cautery. This approach can be used in selected patients, depending upon the size and site of TEE Larger fistulae and those situated lower down e.g. supra carinal cannot be managed by this technique.
doi:10.1007/BF03050850
PMCID: PMC3450399  PMID: 23120323
Tracheoesophageal fistula(TEF); Tracheostomy; Nasogastric tube; esophageal stent; trichloroacetic Acid (TCA)
21.  The Application of a New Cyanoacrylate Glue in Pediatric Surgery for Fistula Closure 
Surgical glues have been used in pediatric surgery because of the fragility of tissue, and to prevent major surgeries. The present report describes our experience with using a new cyanoacrylate Glubran 2 (Viareggio, Italy) in the treatment of five cases of tracheoesophageal atresia with fistula (one fistula protection, three recurrent fistula, and one unstable patients), two cases of hypospadias, one case of vesicutanouse fistula after bladder extrophy, and one case of cloacal extrophy from January–December 2008. Three cases of recurrent tracheoesophageal atresia with fistula were treated by bronchoscpic glue injection. The other two cases benefited from glue through its ability to plug the fistula and to act as a protecting layer on anastomosis. In two cases with hypospadias excessive use of the glue caused skin necrosis, which was repaired. The wounds of cloacal extrophy were protected from nearby colostomy contamination and infection, and the vesicocutanouse fistula was closed by deepithelialization and sealing with glue. Based on the outcomes of the cases, it may be possible to suggest that Glubran 2 may be used safely in Pediatric Surgery as a sealant for the prevention and treatment of fistulas.
PMCID: PMC3559118  PMID: 23365480
Glubran 2; surgical glues; tracheoesophageal atresia with fistula; hypospadias; fistula
22.  Posterior Vertebral Column Resection for VATER/VACTERL Associated Spinal Deformity: A Case Report 
HSS Journal  2006;3(1):71-76.
The VATER/VACTERL association is a syndrome notable for congenital vertebral malformations, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, and renal or limb malformations. Vertebral malformations may include the entire spectrum of congenital spinal deformities, including kyphosis, as was seen in this case. A 14-year-old girl presented to our institution with severe rigid sagittal deformity in the thoracolumbar spine that had recurred following three prior spinal fusion surgeries: the first posterior only, the second anterior and posterior, and the third a posterior only proximal extension. These surgeries were performed to control progressive kyphosis from a complex failure of segmentation that resulted in a 66° kyphosis from T11 to L3 by the time she was 9 years old. Our evaluation revealed solid arthrodesis from the most recent procedures with resultant sagittal imbalance, and surgical options to restore balance included anterior and posterior revision spinal fusion with osteotomies, multiple posterior extension osteotomies with circumferential spine fusion, and posterior vertebral column resection with circumferential spine fusion. She was advised that multiple posterior extension osteotomies would likely be insufficient to restore sagittal balance in the setting of solid arthrodesis from anterior and posterior surgery, and that the posterior-only vertebral column resection would provide results equivalent to revision anterior and posterior surgery, without the morbidity of the anterior approach. She successfully underwent posterior vertebrectomy and circumferential spinal fusion with instrumentation and is doing well 2 years postoperatively. Severe rigid sagittal deformity can be effectively managed with a posterior-only surgical approach, vertebrectomy, and circumferential spinal fusion with instrumentation.
doi:10.1007/s11420-006-9021-4
PMCID: PMC2504096  PMID: 18751773
VATER; VACTERL; kyphosis; kyphectomy; spinal deformity; recurrent
23.  Etiology of Esophageal Atresia and Tracheoesophageal Fistula: “Mind the Gap” 
Current Gastroenterology Reports  2010;12(3):215-222.
Esophageal atresia and tracheoesophageal fistula (EA/TEF) are major congenital malformations affecting 1:3500 live births. Current research efforts are focused on understanding the etiology of these defects. We describe well-known animal models, human syndromes, and associations involving EA/TEF, indicating its etiologically heterogeneous nature. Recent advances in genotyping technology and in knowledge of human genetic variation will improve clinical counseling on etiologic factors. This review provides a clinical summary of environmental and genetic factors involved in EA/TEF.
doi:10.1007/s11894-010-0108-1
PMCID: PMC2874026  PMID: 20425471
Congenital anomaly; Foregut; VACTERL; Feingold syndrome; CHARGE syndrome; AEG syndrome; Genes
24.  Compromised ventilation caused by tracheoesophageal fistula and gastrointestinal endoscope undergoing removal of disk battery on esophagus in pediatric patient -A case report- 
Korean Journal of Anesthesiology  2011;61(3):257-261.
Ingestion of disk batteries may have serious complications such as esophageal burn, perforation, and tracheoesophageal fistula, particularly when the battery is caught in the esophagus. Proper placement of the tracheal tube is critical when tracheoesophageal fistula was occurred from esophageal impaction the battery. Endoscopy of upper gastrointestinal tract in infants and children is an important and effective tool for the diagnosis and treatment of foreign body ingestion. But upper gastrointestinal endoscopy in infant and children has very high risk of tracheal compression and airway compromise. We present a case of ventilatory compromise during insertion of the upper gastrointestinal endoscopy in 16-month-old child with tracheoesophageal fistula secondary to disk battery ingestion.
doi:10.4097/kjae.2011.61.3.257
PMCID: PMC3198189  PMID: 22025950
Disk battery ingestion; Gastrointestinal endoscopy; Tracheoesophageal fistula; Ventilatory compromise
25.  CONGENITAL ATRESIA OF THE ESOPHAGUS AND TRACHEOESOPHAGEAL FISTULA—Review of 48 Cases 
California Medicine  1963;99(5):308-309.
Forty-eight cases of esophageal atresia and tracheoesophageal fistula observed at the University of California Medical Center, San Francisco, over a 25-year period (1937-1961) were reviewed. Four of the patients were born at the University of California Hospitals, a frequency of 1 in 6,823 births. In 43 of the 44 referred cases, the referring physician established the diagnosis before admission by passing a tube into the blind esophagus or by radiography.
Survival rates are comparable to those of other medical centers and emphasize the importance of prompt diagnosis, skillful repair and meticulous preoperative and postoperative care.
PMCID: PMC1515280  PMID: 14081773

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