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1.  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.
2.  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
3.  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
4.  Passage of nasogastric tube through tracheo-esophageal fistula into stomach: A rare event 
Esophageal atresia with tracheo-oesophageal fistula (TEF) occurs in 1 in 3500 live births. Anorectal malformation is found to be associated with 14% of TEF. Esophageal atresia with TEF is a congenital anomaly which classically presents as excessive frothing from the mouth and respiratory distress. Rarely gastric position of the feeding tube in a case of TEF can be obtained delaying the diagnosis of TEF. We had an uncommon situation where a nasogastric tube reached the stomach through the trachea and tracheo-esophageal fistula, leading to misdiagnosis in a case of esophageal atresia with tracheoesophageal fistula. By using a stiff rubber catheter instead of a soft feeding tube for the diagnosis of esophageal atresia and TEF, such situation can be avoided.
doi:10.12998/wjcc.v2.i7.309
PMCID: PMC4097162  PMID: 25032210
Esophageal atresia; Tracheoesophageal fistula; Nasogastric tube; Red rubber catheter; Misdiagnosis
5.  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
6.  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
7.  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
8.  Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography 
Tracheoesophageal fistula (TEF) or bronchoesophageal fistula may be congenital, inflammatory, neoplastic, or secondary to trauma. Congenital TEF or bron-choesophageal fistula is usually associated with eso-phageal atresia and is readily diagnosed in infancy. But if it is not associated with esophageal atresia, it may persist until adulthood. Some theories have been proposed to explain this delay in diagnosis. We present a case of a 70-year-old man with congenital TEF. The TEF was successfully diagnosed by multidetector-row CT esophagography.
doi:10.3748/wjg.v12.i9.1476
PMCID: PMC4124334  PMID: 16552825
Tracheoesophageal fistula; Congenital; Virtual endoscopy; Computed tomography; Esophagography
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.  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
11.  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
12.  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
13.  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
14.  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
15.  Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant 
Case Reports in Medicine  2014;2014:738981.
Anastomotic stricture (AS) and recurrent tracheoesophageal fistula (TEF) are two complications of surgical repair of esophageal atresia (EA). Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.
doi:10.1155/2014/738981
PMCID: PMC4279268  PMID: 25580132
16.  Management of patients with combined tracheoesophageal fistula, esophageal atresia, and duodenal atresia 
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.
doi:10.1016/j.ijscr.2013.09.016
PMCID: PMC4275966  PMID: 25460495
Esophageal atresia; Tracheo-esophageal fistula; Duodenal atresia; Surgical management
17.  Expanding the BP1-BP2 15q11.2 Microdeletion Phenotype: Tracheoesophageal Fistula and Congenital Cataracts 
Case Reports in Genetics  2013;2013:801094.
The proximal q arm of chromosome 15 contains breakpoint regions BP1–BP5 with the classic deletion of BP1–BP3 best known to be associated with Prader-Willi and Angelman syndromes. The region is approximately 500 kb and microdeletions within the BP1-BP2 region have been reported in patients with developmental delay, behavioral abnormalities, and motor apraxia as well as dysmorphic features including hypertelorism, cleft or narrow palate, ear abnormalities, and recurrent upper airway infections. We report two patients with unique, never-before-reported 15q11.2 BP1-2 microdeletion syndrome findings, one with proximal esophageal atresia and distal tracheoesophageal fistula (type C) and one with congenital cataracts. Cataracts have been described in Prader-Willi syndrome but we could not find any description of cataracts in Angelman syndrome. Esophageal atresia and tracheoesophageal fistula have not been reported to our knowledge in either syndrome. A chance exists that both cases are sporadic birth defects; however, the findings of the concomitant microdeletion cannot be overlooked as a possible cause. Based on our review of the literature and the presentation of our patients, we recommend that esophageal atresia and distal tracheoesophageal fistula as well as congenital cataracts be included in the phenotypic spectrum of 15q11.2 BP1-2 microdeletion syndrome.
doi:10.1155/2013/801094
PMCID: PMC3707229  PMID: 23864968
18.  Role of Multislice Computed Tomography and Local Contrast in the Diagnosis and Characterization of Choanal Atresia 
Objective. To illustrate the role of multislice computed tomography and local contrast instillation in the diagnosis and characterization of choanal atresia. To review the common associated radiological findings. Methods. We analyzed 9 pediatric patients (5 males and 4 females) with suspected choanal atresia by multislice computed tomography. We recorded the type of atresia plate and other congenital malformations of the skull. Results. Multislice computed tomography with local contrast installed delineated the posterior choanae. Three patients had unilateral mixed membranous and bony atresia. Three patients had unilateral pure bony atresia. Only 1 of 7 patients have bilateral bony atresia. It also showed other congenital anomalies in the head region. One patient is with an ear abnormality. One patient had congenital nasal pyriform aperture stenosis. One of these patients had several congenital abnormalities, including cardiac and renal deformities and a hypoplastic lateral semicircular canal. Of the 6 patients diagnosed to have choanal atresia, 1 patient had esophageal atresia and a tracheoesophageal fistula. The remaining patients had no other CHARGE syndrome lesions. Conclusions. Local Contrast medium with the application of the low-dose technique helps to delineate the cause of the nasal obstruction avoiding a high radiation dose to the child.
doi:10.1155/2011/280763
PMCID: PMC3134835  PMID: 21772853
19.  Platysma myocutaneous flap interposition in surgical management of large acquired post-traumatic tracheoesophageal fistula: A case report 
INTRODUCTION
Acquired post-traumatic tracheoesophageal fistula (TEF) is an uncommon entity requiring early diagnosis. Among the many strategies in surgical management, we report a case successfully treated with a single-stage tracheal resection and esophageal repair with platysma myocutaneous interposition flap.
PRESENTATION OF CASE
A 24-year-old man had a motor vehicle accident with head injury and cerebral contusion who required mechanical ventilation support. Three weeks later, he developed hypersecretion, and recurrent episodes of aspiration pneumonia. The chest computed tomography, esophagogastroduodenoscopy, and bronchoscopy revealed a large TEF diameter of 3 cm at 4.5 cm from carina. Single-stage tracheal resection with primary end-to-end anastomosis and esophageal repair with platysma myocutaneous interposition flap was performed. A contrast esophagography was done on post-operative day 7 and revealed no leakage. He was discharged on post-operative day 10. Esophagogastroduodenoscopy at 1 month revealed patient esophageal lumen. At present he is doing well without any evidence of complications such as esophageal stricture or fistula.
DISCUSSION
There are many choices of myocutaneous muscle flaps in trachea and esophageal closure or reinforcement. The platysma myocutaneous flap interposition is simple with the advantage of reduced bulkiness. Concern on the vascular supply is that flap should be elevated with the deep adipofascial tissue under the platysma to ensure that the flap survival is not threatened.
CONCLUSION
The treatment of acquired TEF with platysma myocutaneous flap is an alternative procedure for a large uncomplicated TEF as it is effective, technically ease, minimal donor site defect and yields good surgical results.
doi:10.1016/j.ijscr.2014.03.017
PMCID: PMC4008854  PMID: 24727740
Tracheoesophageal fistula; Tracheal reconstruction; Esophageal repair; Interposition flap; Platysma flap
20.  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
21.  Current Application of Thoracoscopy in Children 
Background
The safety and efficacy of thoracoscopy for thoracic lesions and conditions in children is evolving. Our experience with thoracoscopy has expanded in recent years. Therefore, we reviewed our most recent 7-year experience to examine the current applications for thoracoscopy in children.
Methods
A retrospective review of all patients undergoing a thoracoscopic operation at Children’s Mercy Hospital (Kansas City, MO) between January 1, 2000, and June 18, 2007, was performed. Data points reviewed included patient demographics, type of operation, final diagnosis, complications, and recovery.
Results
During the study period, 230 children underwent 231 thoracoscopic procedures. The mean age was 9.6 ± 6.1 years with a mean weight of 36.6 ± 24.1 kg. Fifty percent of the patients were male. The thoracoscopic approach was used for decortication and debridement for empyema in 79 patients, wedge resection for lung lesions in 37, exposure for correction of scoliosis in 26, excision or biopsy of an extrapulmonary mass in 26, operation for spontaneous pneumothorax in 25, lung biopsy for a diffuse parenchymal process in 15, lobectomy in 9, repair of esophageal atresia with a tracheoesophageal fistula (EA-TEF) in 8, clearance of the pleural space for hemothorax or effusion in 3, diagnosis for trauma in 1, and repair of bronchopleural fistula in 1. Conversion was required in 3 patients, all of whom were undergoing a lobectomy. Two of these were right upper lobectomies and the other was a left lower lobectomy with severe infection and inflammation.
The mean time of chest tube drainage (excluding scoliosis and EA-TEF patients) was 2.9 ± 2.0 days. There were no major intraoperative thoracoscopic complications. A correct diagnosis was rendered in all patients undergoing a biopsy. One patient required a second thoracoscopic biopsy to better define a mediastinal mass. Two patients developed postoperative atelectasis after scoliosis procedures. One patient had a small persistent pneumothorax after a bleb resection for a spontaneous pneumothorax. This subsequently resolved.
Conclusions
In pediatric patients with thoracic pathology, thoracoscopy is highly effective for attaining the goal of the operation, with a low rate of conversion and complications.
doi:10.1089/lap.2007.0154
PMCID: PMC3082471  PMID: 18266592
22.  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
23.  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
24.  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
25.  Comparative Study of Esophageal Stent and Feeding Gastrostomy/Jejunostomy for Tracheoesophageal Fistula Caused by Esophageal Squamous Cell Carcinoma 
PLoS ONE  2012;7(8):e42766.
Background
A malignant tracheoesophageal/bronchoesophageal fistula (TEF) is a life-threatening complication of esophageal squamous cell carcinoma. A feeding gastrostomy/jejunostomy had been the most common treatment method for patients with TEF before the era of stenting. The aim of this retrospective study is to compare the prognosis of esophageal squamous cell carcinoma patients with TEF treated with an esophageal metallic stent to those treated with a feeding gastrostomy/jejunostomy.
Methods
We retrospectively reviewed a total of 1011 patients with esophageal squamous cell carcinoma between 1996 and 2011 at Kaohsiung Chang Gung Memorial Hospital, and 86 patients with TEF (8.5%) were identified. The overall survival and other clinical data were compared between 30 patients treated with an esophageal metallic stent and 35 patients treated with a feeding gastrostomy/jejunostomy.
Results
Among the 65 patients receiving either an esophageal metallic stent or a feeding gastrostomy/jejunostomy, univariate analysis showed that treatment modality with an esophageal metallic stent (P = 0.007) and radiotherapy treatment after fistula diagnosis (P = 0.04) were predictive of superior overall survival. In the multivariate comparison, treatment modality with an esophageal metallic stent (P = 0.026, odds ratio: 1.859) represented the independent predictive factor of superior overall survival. There were no significant differences between groups in mean decrease in serum albumin or mean body weight loss. Compared to the feeding gastrostomy/jejunostomy group, a significantly higher proportion of patients in the stenting group (53% versus 14%, P = 0.001) were able to receive chemotherapy within 30 days after fistula diagnosis, indicating better infection control in the stenting group.
Conclusions
Compared with a feeding gastrostomy/jejunostomy, an esophageal metallic stent significantly improves overall survival in patients with malignant TEF in our retrospective analysis. Esophageal metallic stent placement may be considered the first-line of treatment for patients with malignant TEF.
doi:10.1371/journal.pone.0042766
PMCID: PMC3418295  PMID: 22912737

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