Tracheoesophageal Fistula Research Articles

Staged repair of esophageal atresia, tracheoesophageal fistula, and duodenal atresia in a newborn

Staged repair of esophageal atresia, tracheoesophageal fistula, and duodenal atresia in a newborn

Internal mammary artery perforator flap in the management of tracheoesophageal fistulae.

A pathological communication between the trachea and oesophagus - a tracheoesophageal fistula - may be congenital or acquired, benign or malignant, necessitating a multidisciplinary approach. Conservative attempts at closure of this abnormal connection are ineffective; the interposition of healthy vascular tissue offers the least chance of recurrence. Outcomes of an islanded fasciocutaneous internal mammary artery perforator flap applied for tracheoesophageal fistula management were assessed in four radiated patients with laryngeal carcinoma using retrospective records. Four male patients, with an average age of 60.75 years, underwent tracheoesophageal fistula closure between September 2017 and February 2021. A left-sided second internal mammary artery perforator flap was used in all cases, with an average dimension of 10.5 × 4.5 cm. There were no complications of tracheoesophageal leak, flap issues or donor site morbidity on follow up. Recent advances in angiosomal territory mapping and microvascular dissection techniques, combined with an understanding of tracheoesophageal fistula pathology, have changed management perspectives in these difficult-to-treat patients.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. To investigate diagnostic and treatment strategies for this rare condition. We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Characterization of risks and pathogenesis of respiratory diseases caused by rural atmospheric PM2.5

Forty-six percent of the world's population resides in rural areas, the majority of whom belong to vulnerable groups. They mainly use cheap solid fuels for cooking and heating, which release a large amount of PM2.5 and cause adverse effects to human health. PM2.5 exhibits urban-rural differences in its health risk to the respiratory system. However, the majority of research on this issue has focused on respiratory diseases induced by atmospheric PM2.5 in urban areas, while rural areas have been ignored for a long time, especially the pathogenesis of respiratory diseases. This is not helpful for promoting environmental equity to aid vulnerable groups under PM2.5 pollution. Thus, this study focuses on rural atmospheric PM2.5 in terms of its chemical components, toxicological effects, respiratory disease types, and pathogenesis, represented by PM2.5 from rural areas in the Sichuan Basin, China (Rural SC-PM2.5). In this study, organic carbon is the most significant component of Rural SC-PM2.5. Rural SC-PM2.5 significantly induces cytotoxicity, oxidative stress, and inflammatory response. Based on multiomics, bioinformatics, and molecular biology, Rural SC-PM2.5 inhibits ribonucleotide reductase regulatory subunit M2 (RRM2) to disrupt the cell cycle, impede DNA replication, and ultimately inhibit lung cell proliferation. Furthermore, this study supplements and supports the epidemic investigation. Through an analysis of the transcriptome and human disease database, it is found that Rural SC-PM2.5 may mainly involve pulmonary hypertension, sarcoidosis, and interstitial lung diseases; in particular, congenital diseases may be ignored by epidemiological surveys in rural areas, including tracheoesophageal fistula, submucous cleft of the hard palate, and congenital hypoplasia of the lung. This study contributes to a greater scientific understanding of the health risks posed by rural PM2.5, elucidates the pathogenesis of respiratory diseases, clarifies the types of respiratory diseases, and promotes environmental equity.

A Case of Neonatal Diabetes and Multiple Congenital Anomalies: Variant of Mitchell-riley/Martinez-frias Syndrome

Neonatal diabetes along with congenital anomalies like duodenal atresia, tracheoesophageal fistula, intra uterine growth retardation, extrahepatic biliary obstruction and hypoplasia of pancreas is a very rare occurrence with evidence linking it to RFX6 gene mutation. The presence in newborn babies necessitates a detailed genetic evaluation and multidisciplinary management. Here, we present a preterm baby with persistent neonatal hyperglycemia and gastrointestinal anomalies similar to cases associated with Mitchel-riley/Martinez-frias syndrome.

Severe Respiratory Compromise in a Neonate with Tracheo-oesophageal Fistula and Dual Atresia-Gastric Perforation is to Blame for it

Severe Respiratory Compromise in a Neonate with Tracheo-oesophageal Fistula and Dual Atresia-Gastric Perforation is to Blame for it

Comparison of percutaneous single-stage dilatational tracheostomy and surgical tracheostomy in critically ill patients: A randomized controlled trial.

This study was done to compare single stage percutaneous dilation tracheostomy (PDT) and open surgical tracheostomy (ST) in critically ill patients. A randomized controlled study was conducted on 60 critically ill patients admitted in the intensive care unit (ICU). The patients were randomized into ST or PDT group with 30 in each group. The duration of procedure and associated perioperative/postoperative complications were noted and compared. A total of 60 critically ill patients were included with 30 each in both groups. Compared to ST, PDT had significantly lesser mean duration of procedure (5 ± 1.64 vs. 21.33 ± 4.77 min, P < 0.0001) and comparable incidence of complications (3.33% vs. 20%, P = 0.103), which included 5-10 ml of bleeding (0% vs. 13.33%), cardiac arrest (0% vs. 3.33%), atrial fibrillation (3.33% vs. 0%), and tracheoesophageal fistula (0% vs. 3.33%). PDT performed in the ICU is a quick, safe, and reliable procedure with comparable complications to ST.

Comparative Analysis of Two National Surveys on Esophageal Atresia With or Without Tracheoesophageal Fistula: A Retrospective Study by the Korean Association of Pediatric Surgeons

Comparative Analysis of Two National Surveys on Esophageal Atresia With or Without Tracheoesophageal Fistula: A Retrospective Study by the Korean Association of Pediatric Surgeons

Vacterl Association with Mayer-Rokitansky-Kuster-Hauser Syndrome, Description of a Case

Vacterl association is a complex dysfunction of congenital malformaciones that implies several structures. Is typically defined by the presence of at least three of the following congenital malformations: vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. Etiology has only been identified in some patients due to sporadic nature and her high degree of clinical heterogeneity. We report a girl with VACTERL association, which co-occurrence with unusual anomalies of the branchial arch: Mayer-Rokitansky-Küster-Hause syndrome (MRKHS). Vaginal agenesis, is a clinical malformations due to severe dysfunctions in the development of Muller conduits in feminine patient, with absence of vagina and uterus, but with functionals ovaries. Therapeutic management is typically multidisciplinary and centers around surgical correction of specific congenital anomalies in the immediate postnatal period, followed by long-term follow-up for postoperative complications and sequelae of congenital malformations. If optimal surgical correction is achievable, the prognosis may be relatively positive, although some patients will remain affected throughout life. This presentation constitutes a new modality of presentation of polyformative syndromes that need of experience and medical investigation for patients with complex malformation to improve a quality of life.

A case of anaplastic thyroid carcinoma suffering from tracheoesophageal fistula and wound healing disorder of the gastrostomy site during lenvatinib therapy

A case of anaplastic thyroid carcinoma suffering from tracheoesophageal fistula and wound healing disorder of the gastrostomy site during lenvatinib therapy

Glycopyrrolate as an Adjunct in the Management of Anastomotic Leak Following Repair of Esophageal Atresia: A Clinicoradiological Perspective.

Anastomotic leak after primary repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a well-known complication and can represent a challenging clinical scenario. The present study aimed to evaluate the role of glycopyrrolate as an adjunct in the treatment of anastomotic leak after primary repair of EA Vogt type 3b. A retrospective study was carried out in our tertiary care teaching institute from January 2015 to December 2022. Neonates with EA with distal TEF with primary repair who had developed anastomotic leak, managed by the author(s), were studied. The study included patients with major, minor, and radiological leaks. Glycopyrrolate was administered in the dose of 4 μg/kg 8 hourly. The outcomes of the study were either resolution or progression of the leak. There were 21 patients who were managed with glycopyrrolate in addition to the classical treatment of the anastomotic leak following repair of EA with distal TEF. The male: female ratio was 1:1.1. All the cases had anastomotic leaks with either clinically detectable in the chest tube (15) or radiological leak (6). The leaks were detected early in patients with major leak (mean = 3.2 ± 0.84 days) compared to minor leak (mean =4.9 ± 1.29 days). Radiological leaks were detected in all the neonates on postoperative day 7. In five patients with major leak, there was a negligible reduction in the amount of chest tube output, and were subjected to diversion procedures. There were a total of three deaths out of five in this group. In 10 patients with minor leak, there was complete resolution of anastomotic leak in eight patients (80%); there was one patient each with mortality and diversion procedure. The patients with a radiological leak (6) did not show any deterioration, and they were fed 1-5 days after the esophagogram. Glycopyrrolate may be an advantageous postoperative adjunct in the management of minor and radiological leak after tracheoesophageal repair.

Long-gap esophageal atresia: is native esophagus preservation always possible?

Esophageal atresia (EA) is a congenital defect that causes esophageal discontinuity, often with an associated tracheo-esophageal fistula (TEF) in 70%-90% of cases. When the distance between esophageal ends precludes primary anastomosis, it results in long gap esophageal atresia (LGEA), complicating the surgical management. This study retrospectively reviewed LGEA cases from the past decade, treated with the goal of preserving the native esophagus, comparing surgical techniques and outcomes with current literature. The data of patients treated for LGEA between 2013 and 2024 were collected from medical charts, focusing on patients treated with the preservation of their native esophagus. Ten patients were enrolled for this study. All of them had a gap between the esophageal ends equal to or greater than three vertebral bodies. Four patients (40%) underwent a delayed primary anastomosis (DPA) procedure, while the remaining six (60%) underwent a traction staged repair. All patients were treated with open surgery. The follow-up period extended from 3 months to 10 years. Preserving the native esophagus in patients with LGEA is a challenging but feasible goal, with delayed primary anastomosis and traction techniques playing key roles. We advocate for the preservation of the native esophagus as the preferred approach for ensuring a high quality of life for patients, as it helps to avoid severe long-term complications associated with esophageal substitution.

Esophageal atresia with tracheoesophageal fistula: two case reports

BackgroundThe incidence of esophageal atresia with tracheoesophageal fistula is 1 out of 3000–5000 live births. Its incidence in lower middle income countries is not known. The infants usually present with excessive secretions or choking while feeding and are at risk for aspiration. The outcome of these infants in lower middle income countries is not encouraging due to delays in referral, sepsis at presentation requiring preoperative stabilization, postoperative complications such as anastomosis leaks, pneumonia, and pneumothorax.Case presentationWe present two African babies who were term infants at age 2 days (male) and 5 days (female) with diagnosis of esophageal atresia and tracheoesophageal fistula. The 5-day-old infant required preoperative stabilization due to sepsis and delayed surgery with a poor postoperative outcome. The 2-day-old infant was preoperatively stable and had a good postoperative outcome. The challenges faced in management of these two cases have been highlighted.ConclusionOutcome of infants with esophageal atresia and tracheoesophageal fistula in lower middle income countries is not encouraging due to delays in referral and poor postoperative healing attributed to sepsis and recurrent pneumothorax. Timely referral, preoperative condition of the infant, and timely management has shown to be a contributory factor for an improved outcome.

Gastrointestinal mucosal damages caused by ingestion of corrosive substances: A case study of hydrochloric acid and sodium hydroxide.

Ingestion of corrosive substances is most common in children, while in adults causes more severe damages. Massive ingestion of corrosive substances results in severe damage to the gastrointestinal tract and oropharynx if not treated properly. Corrosive substances with pH<2 or >12 can result in severe esophageal damage with either colliquative (alkaline) or coagulative (acidic) necrosis and, at the same time various gastrointestinal injuries could lead to late post-corrosive complications. The aim of the case study was to report the gastrointestinal mucosal damages due to hydrochloric acid (HCl) and sodium hydroxide (NaOH) ingestion. A 55-year-old male patient was presented to the emergency room with a chief complaint of vomiting an hour before admission. Continuous vomiting with a volume of approximately 10-20 cc per vomit. The vomit was initially bluish and turned in to blackish brown over time. Other complaints included nausea, rapid breathing, heartburn, and burning mouth and throat, and had weakness and dizziness. The patient accidentally drank floor cleaning liquid containing HCl. The patient was diagnosed with hematemesis due to ulceration of esophageal, gastric, and duodenal mucosa induced by HCl. Tracheoesophageal fistula developed later in the patient as a long-term complication. Another a 22-year-old male patient was presented to the emergency room with chief complaints of nausea and vomiting an hour before admission. Headache and slight tightness were also experienced. The patient mouth felt burned pain in the solar plexus and frothy saliva. An hour earlier, the patient attempted suicide by drinking two bottles of floor cleaning liquid due to economic problems. The patient was diagnosed with erosive mucosal esophagogastroduodenum induced by NaOH. These cases highlight that intoxication with corrosive substances can complicate damage to the gastrointestinal mucosal and damage features depend on the type of substance concentration and quantity of the corrosive substance.

3D Silicone Prosthesis in the Management of a Large and Late Tracheoesophageal Fistula Following Primary Puncture in a Laryngectomee

3D Silicone Prosthesis in the Management of a Large and Late Tracheoesophageal Fistula Following Primary Puncture in a Laryngectomee

A novel genotype-phenotype between persistent-cloaca-related VACTERL and mutations of 8p23 and 12q23.1.

The mechanism underlying anorectal malformations (ARMs)-related VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, and renal and limb abnormalities) remains unclear. Copy number variation (CNV) contributed to VACTERL pathogenicity. Here, we report a novel CNV in 8p23 and 12q23.1 identified in a case of ARMs-related VACTERL association. This 12-year-old girl presented a cloaca (urethra, vagina, and rectum opening together and sharing a single tube length), an isolated kidney, and a perpetuation of the left superior vena cava at birth. Her intelligence, growth, and development were slightly lower than those of normal children of the same age. Array comparative genomic hybridization revealed a 9.6-Mb deletion in 8p23.1-23.3 and a 0.52-Mb duplication in 12q23.1 in her genome. Furthermore, we reviewed the cases involving CNVs in patients with VACTERL, 8p23 deletion, and 12q23.1 duplication, and our case was the first displaying ARMs-related VACTERL association with CNV in 8p23 and 12q23.1. These findings enriched our understanding between VACTERL association and the mutations of 8p23 deletion and 12q23.1 duplication. IMPACT: This is a novel case of a Chinese girl with anorectal malformations (ARMs)-related VACTERL with an 8p23.1-23.3 deletion and 12q23.1 duplication. Cloaca malformation is presented with novel copy number variation in 8p23.1-23.3 deletion and 12q23.1 duplication.

Case report of radiotherapy combined with anlotinib and immunotherapy for a patient with esophageal cancer and esophageal fistula

BackgroundEsophageal cancer (EC) is a frequent gastrointestinal malignancy. The most common types of EC pathology worldwide are esophageal squamous cell carcinoma (ESCC) and esophageal adenocarcinoma (EAC). Although surgical resection is still the main treatment modality for EC, most patients are already lost to surgery at the time of presentation due to the late stage. In recent years, the development of radiation therapy (RT) combined with targeted therapy (TT) and immunization therapy (IT) has brought more options for the treatment of EC. During radiation therapy, the radiation therapy area is very close to the trachea and esophagus, so radiation therapy may cause damage to the tissues of the trachea and esophagus, which is also known as a tracheoesophageal fistula (TF). We present the case of an EC patient who developed TF during radiation therapy and gradually improved after a combination of anlotinib and immunotherapy. MethodsThe patient was diagnosed with poorly differentiated ESCC by pathological biopsy and treated with “lobaplatin + Tegafur Gimeracil Oteracil Porassium Capsule” for 5 cycles. ResultsCT scan of the chest showed progression after treatment. During RT, the patient developed radiotherapy-related adverse effects, which were relieved by symptomatic support therapy. At the end of RT, the patient developed TF, but we chose to let the patient continue his radiation treatment plan with the anti-angiogenic drug “anlotinib.” ConclusionAfter radiation therapy, the patient continued to be treated with anlotinib and immunotherapy with camrelizumab, and the patient's lesion improved.

Autonomous and non-cell autonomous role of cilia in structural birth defects in mice.

Ciliopathies are associated with wide spectrum of structural birth defects (SBDs), indicating important roles for cilia in development. Here, we provide novel insights into the temporospatial requirement for cilia in SBDs arising from deficiency in Ift140, an intraflagellar transport (IFT) protein regulating ciliogenesis. Ift140-deficient mice exhibit cilia defects accompanied by wide spectrum of SBDs including macrostomia (craniofacial defects), exencephaly, body wall defects, tracheoesophageal fistula (TEF), randomized heart looping, congenital heart defects (CHDs), lung hypoplasia, renal anomalies, and polydactyly. Tamoxifen inducible CAGGCre-ER deletion of a floxed Ift140 allele between E5.5 to 9.5 revealed early requirement for Ift140 in left-right heart looping regulation, mid to late requirement for cardiac outflow septation and alignment, and late requirement for craniofacial development and body wall closure. Surprisingly, CHD were not observed with 4 Cre drivers targeting different lineages essential for heart development, but craniofacial defects and omphalocele were observed with Wnt1-Cre targeting neural crest and Tbx18-Cre targeting epicardial lineage and rostral sclerotome through which trunk neural crest cells migrate. These findings revealed cell autonomous role of cilia in cranial/trunk neural crest-mediated craniofacial and body wall closure defects, while non-cell autonomous multi-lineage interactions underlie CHD pathogenesis, revealing unexpected developmental complexity for CHD associated with ciliopathies.

Commentary on Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center

Commentary on Endoscopic Chemocauterization with Trichloroacetic Acid for Congenital or Recurrent Tracheoesophageal Fistula in Children with Esophageal Atresia: Experience from a Tertiary Center

Expert group recommendation on inhaled mucoactive drugs in pediatric respiratory diseases: an Indian perspective.

Currently, there are no guidelines or consensus statements about the usage of inhaled mucoactive drugs in pediatric respiratory disease conditions from an Indian perspective. To develop a practical consensus document to help pediatricians in clinical decision-making when choosing an appropriate mucoactive drug for the management of specific respiratory disease conditions. A committee of nine experts with significant experience in pediatric respiratory disease conditions and a microbiological expert constituted the panel. An electronic search of the PubMed/MEDLINE, Cochrane Library, Scopus, and Embase databases was undertaken to identify relevant articles. Various combinations of keywords such as inhaled, nebulized, mucoactive, mucolytic, mucokinetic, expectorants, mucoregulators, mucociliary clearance, respiratory disorders, pediatric, cystic fibrosis (CF), non-CF bronchiectasis, acute wheezing, asthma, primary ciliary dyskinesia (PCD), critically ill, mechanical ventilation, tracheomalacia, tracheobronchomalacia, esophageal atresia (EA), tracheoesophageal fistula (TEF), acute bronchiolitis, sputum induction, guideline, and management were used. Twelve questions were drafted for discussion. A roundtable meeting of experts was conducted to arrive at a consensus. The level of evidence and class of recommendation were weighed and graded. Inhaled mucoactive drugs (hypertonic saline, dry powder mannitol, and dornase alfa) can enhance mucociliary clearance in children with CF. Experts opined that hypertonic saline could be beneficial in non-CF bronchiectasis, acute bronchiolitis, and PCD. The current state of evidence is inadequate to support the use of inhaled mucoactive drugs in asthma, acute wheezing, tracheomalacia, tracheobronchomalacia, and EA with TEF.