Zacharias J, Genc O, Goldstraw P (2004) Congenital tracheoesophageal fistulas presenting in adults: presentation of two cases and a synopsis of the literature. [link.springer.com]

The clinical presentation of an H type fistula is different than the other fistulae because it does not share the common presenting signs associated with atretic esophagus. [pedclerk.uchicago.edu]

Isolated tracheoesophageal fistula (TEF) (H-type fistula) Recurrent pneumonia may be present, with a widespread pneumonia pattern. Fistula delineation can be difficult. Excessive air may be present in the esophagus. [thehealthscience.com]

If there is a TOF present, saliva gastric secretions can pass directly to the bronchial tree. H-type fistulae usually present later in infancy as there is no 'blind end' to the oesophagus and the child is able to feed. [patient.info]

Therefore, the aim of present study is to evaluate postoperative outcomes of patients treated for Gross type B-D and E EA, on regards of their cervical surgery. [moh-it.pure.elsevier.com]

• Surgical Procedure

  Sometimes, a narrowed esophagus can be widened with a surgical procedure. This procedure may need to be repeated more than one time in the child's lifetime. [chw.org]

  We conclude that, despite an apparently less severe esophageal malformation, patients with isolated TEF present with impaired esophageal motility before any surgical procedure. [journals.lww.com]

  Several different surgical procedures have been created to treat long-gap esophageal atresia. [rarediseases.org]

• Developmental Delay

  The median age at surgery was 8 days for the TEF group vs. 1 day for the EA group (p CONCLUSION: Developmental delay and a high rate of morbidity were found in both groups. More complex surgery increased perioperative morbidity in cases of EA. [ncbi.nlm.nih.gov]

  Children with neurologic impairment (OR 4.65), developmental delays (OR 4.62), aspiration lung disease (OR 3.22), and enteral feeding (OR 2.03) were more likely to have silent aspiration. [frontiersin.org]

• Aspiration

  Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. [ncbi.nlm.nih.gov]

  Department of Pediatric Surgery, CHU Caen, Avenue Cote de Nacre, 14033 Caen cedex 9, France; aurore.haffreingue{at}gmail.com Statistics from Altmetric.com Tracheoesophageal Fistula Diagnosis Children A 4-year-old boy was referred with recurrent pneumonia and aspiration [adc.bmj.com]

  Feeding exacerbates these symptoms, causes regurgitation, and may precipitate aspiration. Aspiration of gastric contents via a distal fistula causes more damaging pneumonitis than aspiration of pharyngeal secretions from the blind upper pouch. [easypediatrics.com]

  Diagnosing Aspiration during Swallowing Oropharyngeal dysphagia with resultant aspiration can be diagnosed by several different diagnostic tests. [frontiersin.org]

  Diagnosis of an H-type fistula can be significantly delayed because the only symptoms may be frequent episodes of pneumonia due to recurrent aspiration. [pedclerk.uchicago.edu]

• Cough

  RESULTS: The clinical picture consisted in a coughing bout and cyanosis after each feeding. Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. [ncbi.nlm.nih.gov]

  They often present later with milder coughing or choking with feeds or reflux. Normal upper GI’s may miss isolated TEF. Diagnosis is therefore made with either a “pullback” esophagram using a feeding tube, or rigid bronchoscopy. [surgery4children.com]

  Tracheomalacia can lead to a 'TOF cough' (a harsh barking cough). Recurrent chest infections can lead to bronchiectasis and irreversible lung damage. Respiratory morbidity tends to improve as the child ages. [patient.info]

  The neonate with EA typically has frothing and bubbling at the mouth and nose after birth as well as episodes of coughing, cyanosis, and respiratory distress. Feeding exacerbates these symptoms, causes regurgitation, and may precipitate aspiration. [easypediatrics.com]

  When fed, these infants swallow normally but begin to cough and struggle as the fluid returns through the nose and mouth. [en.wikipedia.org]

• Eructation

  293 746 310 References 341 747 342 Diffuse Esophageal Spasm By G WANTRAPPEN and J HELLEMANs With 3 Figures 355 Related Conditions 361 Gastroesophageal Sphincter 375 750 377 Related Syndromes 390 Myasthenia Gravis 398 Extrapyramidal Disturbances 404 Eructation [books.google.com]

• Bilious Vomiting

  However, the high GGTP levels, even if much lower than in a bilious vomiting pattern, were not be comprehensible. We also excluded the presence of an AMP inhibitor in the EA-AF. [nature.com]

• Sweating

  Clinical examination, complete blood count and sweat test were normal. A tracheo-oesophageal fistula (TEF) was suspected as a differential diagnosis. Barium swallow was unremarkable. [adc.bmj.com]

  Sweat test was normal. Viral studies continued to be negative. At 14 weeks of age, following a two week period of respiratory stability, the infant had thorascopic repair of his esophageal atresia. He was extubated at 48 hours post-operatively. [springerplus.springeropen.com]

• Flushing

  Despite replacement and flushing of his Repogle suction tube, he remained tachypneic for several days but gradually returned to baseline. [springerplus.springeropen.com]

• Suggestibility

  CONCLUSION: Although a rare malformation, tracheoesophageal fistula should be suggested as a diagnosis when respiratory symptoms occur during feeding starting during the neonatal period. Copyright 2012 Elsevier Masson SAS. All rights reserved. [ncbi.nlm.nih.gov]

  The presence of a dilated blind-ending esophageal pouch on a sonogram is suggestive of EA. [thehealthscience.com]

  However, further investigation has suggested that this process does not occur in humans. 1, 3 Kluth and Fiegel 3 suggest that the formation of the esophagus and trachea is the result of “a system of folds” in the cranial and caudal ends of the tracheoesophageal [radiologykey.com]

  This finding suggests that the esophageal dysmotility known to affect patients with EA and TEF is probably primary in origin rather than secondary to mechanical surgical trauma. [journals.lww.com]

  In the setting of early-onset respiratory distress, the inability to pass a nasogastric or orogastric tube in the newborn is suggestive of esophageal atresia. Maternal polyhydramnios may alert the physician to EA. [easypediatrics.com]

• Excitement

  It is exciting to think what the future may hold with regard to treatment of complex cases or comorbidities, and the development of minimally invasive therapies. [neoreviews.aappublications.org]

Preoperative Planning In a term baby, EA or EA/TEF is rarely an emergency and there is usually adequate time to complete the preoperative workup and plan the operative approach. [basicmedicalkey.com]

[…] aortic arch that should be identified on preoperative echo or ultrasound since esophageal repair is performed on the side opposite the arch. 1, 11, 12 Treatment/Follow-up: Surgical repair is typically undertaken within 1 to 2 days, after the clinical workup [radiologykey.com]

In both patients, esophageal manometry was part of the diagnostic workup. Information was gathered from the medical charts: demographic, medical history, symptomatology before HREM. [journals.lww.com]

• Atelectasis

  Thoracic and abdominal imaging showed aspiration pneumonia, atelectasis, and gas within the colon. Gastrointestinal opacification demonstrated the fistula in 2 cases. Tracheoscopy visualized the tracheoesophageal fistula in the other 2 cases. [ncbi.nlm.nih.gov]

  Chest radiographs were performed during this time: two were interpreted as being normal, one showed minor right upper lobe opacities or atelectasis, and one showed minor right lower lobe atelectasis which resolved on subsequent radiograph. [springerplus.springeropen.com]

  Pneumonitis (inflammation of the lungs) and atelectasis (collapse of part of one or both lungs) can develop quickly in these babies. [chw.org]

  Aspiration pneumonia (especially in the right upper lobe) and patchy atelectasis are frequently present. [thehealthscience.com]

  Gastrostomy for gastric decompression is reserved for use in patients with significant pneumonia or atelectasis, to prevent reflux of gastric contents through the fistula and into the trachea. [aafp.org]

Karnak I, Şenocak M, Hiçsönmez A, Büyükpamukçu N (1997) The diagnosis and treatment of H-type tracheoesophageal fistula. J Pediatr Surg 32(12):1670–1674 PubMed CrossRef Google Scholar 12. [link.springer.com]

Treatment was surgical and consisted in the section-ligation of the tracheoesophageal fistula with pleural interposition in all cases. [ncbi.nlm.nih.gov]

Not included in classification by Ladd 4% Treatment [ edit ] Treatments for the condition vary depending on its severity. [en.wikipedia.org]

Standard Therapies Treatment Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

Although EA surgical treatment is well established, the outcome of EA with proximal fistula (type B and D EA) or isolated H-type fistula (type E EA) is poorly explored. [moh-it.pure.elsevier.com]

Furthermore, evolution in medical and surgical management has vastly improved the prognosis for these lesions since the first reported survivor nearly 80 years ago. [neoreviews.aappublications.org]

Prognosis: Survival is primarily dependent on gestation at delivery and the presence of other anomalies. [fetalmedicine.org]

What is my baby's long-term prognosis? The prognosis for survival for an isolated TEF/EA is excellent. An associated anomaly can alter that prognosis. [chw.org]

The prognosis in these fetuses depends on the associated abnormalities, the birth weight and whether the neonate will develop pneumonia secondary to aspiration. References: Hertzberg BS. [fetalsono.com]

Prognosis This depends on associated anomalies and their complications. Overall survival now exceeds 90% in dedicated centres. [ 14 ] Associated congenital heart defects and low birth weight can affect survival. [patient.info]

Its etiology is obscure. Diagnosis is difficult but must be made early. PURPOSE: To study the clinical, radiological, and evolutionary sights of this malformation. [ncbi.nlm.nih.gov]

Although the absolute etiology of these lesions is yet to be determined, much has been learned from embryologic studies and syndromic associations. [neoreviews.aappublications.org]

Definition A tracheo-esophageal fistula (TEF) is a congenital defect that is thought to be a result of a foregut malformation of uncertain etiology around the 4th to 5th week of gestation age. [openanesthesia.org]

Embryology It remains controversial as to the exact etiology for the development of EA/TEF. Around the fourth week of gestation, the foregut begins to differentiate into a dorsal esophageal part and a ventral respiratory segment. [basicmedicalkey.com]

We recommend in all cases of esophageal atresia that a respiratory deterioration be fully investigated, with consideration of the possibility of a tracheoesophageal fistula as the etiology. [springerplus.springeropen.com]

An international collaborative study of the epidemiology of esophageal atresia or stenosis. Reprod Toxicol 1993 ;7: 405 – 21. Google Scholar Crossref Medline ISI 9. Spitz, L, Kiely, EM, Morecroft, JA, Drake, DP. [doi.org]

Epidemiology: The condition is rare, occurring in roughly one infant for every 4,000 births in the USA. [eapsa.org]

The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group. Arch Dis Child 1993; 68:743. GeneReviews [Internet]. Pagon RA, Bird TD, Dolan CR, et al., editors. [pedclerk.uchicago.edu]

Epidemiology The estimated incidence is 1 in 3,000 births. [ 2 ] Finland has a comparatively high figure of 1 in 2,500 births. [patient.info]

Epidemiology EA is usually a sporadic occurrence. The incidence of EA/TEF is about 1 per 3,500 births. There appears to be a slight male predominance of 1.3. [basicmedicalkey.com]

Belching 414 References 421 References 427 751 430 Benign Tumors and Cysts of the Esophagus By G WANTRAPPEN and J PRINGOT With 431 Intraluminal Tumors 443 Pseudosarcoma and Carcinosarcoma 485 Chapter 5 493 Clinical Features 504 Endoscopy 514 References 521 Pathophysiology [books.google.com]

The aim of this study was to find a pathophysiological explanation for this dissociated biochemical pattern observed in EA. [nature.com]

Pathophysiology TE fistulas occur due to abnormal septation of the caudal foregut during the fourth and fifth weeks of embryonic development. [pedclerk.uchicago.edu]

[…] deeply disorganized in these young patients who had no other reasons (no esophagitis, no congenital stenosis, no abnormal LES relaxation, and no surgical insult) to explain the dysmotility, we believe that these findings provide important elements in the pathophysiology [journals.lww.com]

This suggests that distal esophageal dysmotility, rather than pooling over the anastomosis, may be a bigger contributor to feeding difficulties in many children and supports imaging to understand the pathophysiology of dysphagia first before dilation [frontiersin.org]

Initially, maintaining a patent airway and preventing aspiration of secretions are paramount. [easypediatrics.com]

The EA / TEF is a condition that endangers life, so affected babies usually require surgery to correct the defect in order to allow food and prevent lung damage from repeated exposure of esophageal fluids. [ivami.com]

GERD may need to be treated with medication, or in severe cases, with surgery to prevent the acid from doing damage to the esophagus or lungs. [eapsa.org]

Suctioning of the blind ending oesophagus is necessary to prevent aspiration and protect the airway. This should continue until surgery is performed. Prophylactic antibiotics may also be needed. [patient.info]

Paediatr Anaesth: 2012, 22(3);268-74 M R Salem, A Y Wong, Y H Lin, H V Firor, E J Bennett Prevention of gastric distention during anesthesia for newborns with tracheoesophageal fistulas. Anesthesiology: 1973, 38(1);82-3 [openanesthesia.org]