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Congenital TEF can arise due to failed fusion of the tracheoesophageal ridges after the fourth week of embryological development. [4] A fistula, from the Latin meaning 'a pipe', is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-esophageal fistula it runs between the trachea and the esophagus.
Congenital esophageal atresia (EA) represents a failure of the esophagus to develop as a continuous passage. Instead, it ends as a blind pouch. Tracheoesophageal fistula (TEF) represents an abnormal opening between the trachea and esophagus. EA and TEF can occur separately or together. EA and TEF are diagnosed in the ICU at birth and treated ...
T - Tracheoesophageal fistula; E - Esophageal atresia; R - Renal (Kidney) and/or radial anomalies; L - Limb defects; Although it was not conclusive whether VACTERL should be defined by at least two or three component defects, [4] it is typically defined by the presence of at least three of the above congenital malformations. [5]
This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision. Generally, diseases outlined within the ICD-10 codes Q35-Q45 within Chapter XVII: Congenital malformations, deformations and chromosomal abnormalities should be included in this category.
Twenty to 27% of individuals with a laryngeal cleft also have a tracheoesophageal fistula and approximately 6% of individuals with a fistula also have a cleft. [4] Other congenital anomalies commonly associated with laryngeal cleft are gastro-oesophageal reflux, tracheobronchomalacia, congenital heart defect, dextrocardia and situs inversus. [5]
Developmental abnormalities of the tracheoesophageal septum can lead to a tracheoesophageal fistula. [6] This may be caused by certain mutations of genes involved in its development. [4] Other theories for the origin of tracheoesophageal fistula have been suggested. [7] [8] A laryngotracheal cleft is a related abnormality. [9]
Incidence of congenital malformations associated with tracheal agenesis. Results were obtained from a total of 32 individual case studies. [11]The classic in-utero symptoms of tracheal agenesis are an absence of the trachea leading to congenital high airway obstruction syndrome, [12] [13] lung distention, polyhydramnios, heart malformations, heart displacement and hydrops fetalis.
An imperforate anus or anorectal malformations (ARMs) are birth defects in which the rectum is malformed. ARMs are a spectrum of different congenital anomalies which vary from fairly minor lesions to complex anomalies. [1] The cause of ARMs is unknown; the genetic basis of these anomalies is very complex because of their anatomical variability.