Esophageal atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) is a congenital Congenital Chorioretinitis anomaly in which the upper esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy is separated from the lower esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy and ends in a blind pouch. The condition may be isolated or associated with tracheoesophageal fistula Fistula Abnormal communication most commonly seen between two internal organs, or between an internal organ and the surface of the body. Anal Fistula, which is an abnormal connection between the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy and esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy. The most common congenital Congenital Chorioretinitis tracheoesophageal abnormality is a combination of esophageal atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) and distal tracheoesophageal fistula Fistula Abnormal communication most commonly seen between two internal organs, or between an internal organ and the surface of the body. Anal Fistula. The defect manifests early after birth as an inability to feed, regurgitation Regurgitation Gastroesophageal Reflux Disease (GERD), choking, and aspiration, and requires surgical correction. Survival is good, but long-term complications are common.
Last updated: 8 Jan, 2021
Tracheoesophageal fistula Fistula Abnormal communication most commonly seen between two internal organs, or between an internal organ and the surface of the body. Anal Fistula (TEF) and esophageal atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) (EA) are congenital Congenital Chorioretinitis malformations of the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy and lower respiratory tract.
A newborn Newborn An infant during the first 28 days after birth. Physical Examination of the Newborn with radial atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) of the right arm Arm The arm, or “upper arm” in common usage, is the region of the upper limb that extends from the shoulder to the elbow joint and connects inferiorly to the forearm through the cubital fossa. It is divided into 2 fascial compartments (anterior and posterior). Arm: Anatomy: This birth deformation is a VACTERL association VACTERL Association VACTERL association is a rare disorder that affects multiple body systems in fetal development. There is no clear genetic cause or inheritance pattern for the development of this disorder. The acronym VACTERL stands for its characteristic anomalies: Vertebral abnormalities, Anal atresia, Cardiac defects, Tracheoesophageal abnormalities, Renal anomalies, and Limb abnormalities. VACTERL Association disorder.
Image: “Radial atresia Atresia Hypoplastic Left Heart Syndrome (HLHS)” by CDC/ Dr. James W. Hanson. License: Public DomainTypes | Description | Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency |
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Type A | EA without TEF | Approximately 8% of cases |
Type B | EA with TEF to the proximal esophageal segment | Approximately 1% of cases |
Type C | EA with TEF to the distal esophageal segment | Approximately 84% of cases |
Type D | EA with TEF to the proximal and distal esophageal segments | Approximately 3% of cases |
Type E | H-type TEF without atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) | Approximately 4% of cases |
Types of EA with TEF (gross classification): From top left, images in order of incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency: type C (84%), type A (8%), type E (4%); bottom row: type D and type B (least common).
Image by Lecturio.Esophageal atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) and TEF type C (most common type): EA is accompanied by a TEF to the distal esophageal segment.
Image: “Esophageal Atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) TYPE C” by CDC. License: Public DomainChest radiograph of a neonate Neonate An infant during the first 28 days after birth. Physical Examination of the Newborn with contrast shows a blind-ending upper end of the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy (black arrow) and a gas-filled stomach Stomach The stomach is a muscular sac in the upper left portion of the abdomen that plays a critical role in digestion. The stomach develops from the foregut and connects the esophagus with the duodenum. Structurally, the stomach is C-shaped and forms a greater and lesser curvature and is divided grossly into regions: the cardia, fundus, body, and pylorus. Stomach: Anatomy (white arrow).
Image: “Chest radiograph” by Department of Radiology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India. License: CC BY 2.0Chest radiograph of a neonate Neonate An infant during the first 28 days after birth. Physical Examination of the Newborn with contrast shows the blind-ending upper end of the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy (long white arrow), contrast in the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy (black arrow), the lower end of the esophagus Esophagus The esophagus is a muscular tube-shaped organ of around 25 centimeters in length that connects the pharynx to the stomach. The organ extends from approximately the 6th cervical vertebra to the 11th thoracic vertebra and can be divided grossly into 3 parts: the cervical part, the thoracic part, and the abdominal part. Esophagus: Anatomy communicating with the trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy (black arrowhead), and contrast in the stomach Stomach The stomach is a muscular sac in the upper left portion of the abdomen that plays a critical role in digestion. The stomach develops from the foregut and connects the esophagus with the duodenum. Structurally, the stomach is C-shaped and forms a greater and lesser curvature and is divided grossly into regions: the cardia, fundus, body, and pylorus. Stomach: Anatomy (thick white arrow).
Image: “Chest radiograph” by Department of Radiology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India. License: CC BY 2.0Supportive:
Surgery (definitive):