Esophagus, Esophageal Atresia, TEF Flashcards
(122 cards)
What is the best surgical approach to repair the most common type of TEF?
A. Left thoracotomy
B. Right thoracotomy
C. Left cervicotomy
D. Right cervicotomy
E. Midline laparotomy
ANSWER: B
Five types of TEFs exist.
Type C, esophageal atresia with a distal TEF, is the most common type accounting for 80%–90% of all TEFs.
Radiographs will show a coiled orogastric tube in the proximal esophagus and air within the stomach.
The best access to the esophagus for repair of this fistula is through a right thoracotomy.
As part of the preoperative workup, an echocardiogram should be obtained to evaluate for congenital cardiac anomalies as well as a right-sided aortic arch.
If identified, repair can be performed through a left-sided thoracotomy.
Type A (5%–10%) is pure atresia without a fistulous connection. Radiographs will also show a coiled orogastric tube in the proximal esophagus, but there is no air within the stomach.
Type E (4%), or H type, is a TEF without atresia. This often presents in the first few days of life with repeated choking with attempted feeds and cyanotic events as the baby aspirates into the lungs. However, this can be missed in the newborn period and may present later in life with recurrent aspirations or pneumonias. These fistulas are located at the thoracic inlet, and a right cervicotomy allows for the best access for fistula ligation.
The final two types of TEF, type B atresia with both a proximal and distal TEF and type D atresia with a proximal TEF, are both extremely rare.
The surgical approach should be through a right thoracotomy.
Which of the following is a complication after the repair of a TEF?
A. Anastomotic leak
B. Esophageal stricture
C. Recurrent fistula
D. Gastroesophageal reflux
E. All of the above
ANSWER: E
COMMENTS: All of the options are potential complications following the repair of a TEF.
The early complications include anastomotic leak, esophageal stricture, and recurrent fistula, while late complications include gastroesophageal reflux disease (GERD) and tracheomalacia.
Esophageal stricture is the most common early complication and may occur in up to 80% of cases.
This complication is managed with serial esophageal dilations.
If the stricture fails to respond, resection of the anastomosis with a new anastomosis is warranted.
Anastomotic leak is another early complication, occurring in about 15% of repairs.
The majority of these leaks are not clinically significant and may be managed with continuous drainage and parenteral nutrition until the leak has sealed.
Large leaks within the first few days of repair are generally the result of a technical error or ischemia of the anastomosis; surgical revision is advised to avoid development of a tension pneumothorax and mediastinitis.
GERD is by far the most common late complication and is thought to be related to shortening of the intraabdominal portion of the esophagus.
An 8-h-old newborn has mild respiratory distress and excessive drooling. An abdominal radiograph shows a complete lack of air in the GI tract. What is the most likely diagnosis?
A. Bilateral choanal atresia
B. Pyloric atresia
C. Duodenal atresia
D. Esophageal atresia with a distal TEF
E. Esophageal atresia without a TEF
ANSWER:
E
How common is esophageal atresia (EA) with or without tracheoesophageal fistula (TEF)?
The worldwide prevalence of EA is estimated at 2–3 per 10,000 births, and 70–90% are associated with TEF.
Spontaneous intrauterine fetal demise occurs in ~3% of cases.
A 3000-g infant is born with esophageal atresia and a distal TEF. If the infant does not exhibit respiratory distress and associated anomalies are not present, which of the following is the preferred treatment?
A. Gastrostomy, cervical esophagostomy, and delayed repair
B. Gastrostomy, sump tube drainage of the proximal pouch, and delayed repair
C. Fistula ligation and delayed esophageal repair
D. Division of the fistula with primary esophageal anastomosis
E. Primary repair with colonic interposition
ANSWER:
D
COMMENTS: The timing and type of surgical intervention for esophageal atresia and TEF depend on the maturity of the infant and associated cardiorespiratory problems or other congenital anomalies.
Medically stable infants weighing more than 2500 g are treated by primary repair with fistula division, closure of its tracheal end, and end-to-end anastomosis of the esophageal segments.
Unstable infants with respiratory issues are treated by gastrostomy and sump drainage of the blind proximal pouch until they are ready for repair.
A distal TEF often results in the loss of ventilatory pressure or retrograde aspiration of gastric contents into the lungs, further exacerbating respiratory compromise.
Therefore some infants may benefit from primary fistula ligation without esophageal repair, with or without gastrostomy placement.
The final esophageal repair is performed after cardiorespiratory recovery.
The VACTERL association most commonly includes which of the following?
A. Ankylosis
B. Imperforate anus
C. Eye deformities
D. Congenital cystic lung malformation
E. Choanal atresia
ANSWER:
B
COMMENTS: Neonates have a gasless GI tract at birth. They start to swallow soon after birth, and air reaches the colon within 6 to 12 h.
In pure esophageal atresia without an associated fistula, swallowed air has no access to the GI tract, and so abdominal films show a gasless abdomen.
Esophageal atresia is also suggested when an infant drools excessively because of an esophageal obstruction or spits up during attempted feedings.
When an orogastric tube is passed in an infant with esophageal atresia, a chest radiograph shows the tube coiled in a blind pouch in the chest.
About 85%–90% of patients with a tracheoesophageal malformation have a blind proximal pouch with a distal TEF, also known as TEF type C.
Respiratory symptoms are secondary to aspiration from the esophageal pouch or retrograde reflux of gastric contents through the fistula into the lungs.
In esophageal atresia with a TEF, the inspired air reaches the stomach and small bowel through the TEF.
Contrast-enhanced studies and a bronchoscopy may be useful in select cases to confirm the diagnosis and demonstrate the location of the fistula.
Recognition of the anatomy of the anomaly is important for establishing appropriate initial treatment and definitive repair.
Air fills the stomach but fails to pass into the duodenum and small bowel in neonates with pyloric atresia, a rare congenital anomaly.
Radiographic studies show extreme distention of the stomach with air-fluid levels.
Choanal atresia is narrowing or blockage of the posterior nasal airway by soft or boney tissue.
It is a congenital condition that is due to the failure of the recanalization of the nasal airway during embryologic development.
Neonates, being obligatory nasal breathers, have major respiratory problems when born with bilateral choanal atresia but do not have difficulty swallowing air.
The VACTERL association refers to a group of commonly associated congenital defects: vertebral anomalies, imperforate anus, cardiac defects, tracheoesophageal fistula, radial and renal malformations, and limb defects.
What is the etiology of EA?
EA is a congenital anomaly that results from abnormal embryonic development of the foregut. The exact mechanisms are unknown, yet the process likely involves a combination of genetic, environmental, and biomechanical factors.
Which genetic syndromes and other congenital anomalies are associated with EA/TEF?
The most common congenital anomalies associated with EA/TEF involve the Vertebral, Anorectal, Cardiac, TEF, Renal and Limb abnormality (VACTERL) spectrum of disorders (Table 5.1).
Nearly 70% of infants with EA will have at least one other associated anomaly, most commonly cardiac (~35%), with many patients having multiple.
Associated chromosomal abnormalities include trisomies 18 and 21, as well as CHARGE syndrome.
What are the relationships between prematurity, low birth weight, and EA/TEF?
Up to one-third of patients with EA are born prematurely, likely due to associated polyhydramnios.
Low birth weight infants with EA have a higher risk of mortality than their normal weight counterparts, and often surgical repair is delayed until infants reach 1,500–2,000 g to lower the risk of operative morbidity.
What is the mortality associated with EA/TEF?
Prior to the first successful EA/TEF repair in 1941, mortality of this disorder was 100%.
Survival has steadily increased over time and is currently>90%.
Mortality is primarily related to the associated comorbidities of congenital heart disease and prematurity.
How is EA classified?
The most widely used classification system for EA/TEF is the Gross classification, which includes types A–E.
The most common malformation is Gross Type C, which involves EA associated with a distal TEF.
Gross Type E has no atresia but instead an isolated, or “H-type” TEF.
Other congenital anomalies associated with esophageal atresia and their respective incidences?
Associated anomaly and incidence (%):
Cardiac 35%
Renal 23%
Vertebral 22%
Anorectal 20%
Limb 14%
Chromosomal 11%
How is EA diagnosed?
Prenatal diagnosis of EA is about 20% in patients with EA/TEF and over 50% in patients with isolated EA.
Prenatal imaging findings that are indicative of EA include polyhydramnios, a small or absent stomach bubble, and a dilated upper esophageal pouch (“pouch sign”).
The sensitivity and specificity of prenatal diagnosis are significantly improved with MRI over ultrasound.
After birth, infants with EA commonly present with early feeding intolerance and increased oral secretions that can lead to respiratory compromise.
The diagnosis of EA is confirmed with failed attempts at orogastric tube placement followed by an abdominal x-ray demonstrating coiling of the enteric tube within the proximal esophageal pouch.
The presence of an associated distal TEF is indicated by the finding of gas within the stomach and/or intestine.
What further clinical workup is recommended following the diagnosis of EA/TEF?
Further workup is aimed at determining EA type, evaluating for the presence of a TEF, estimating esophageal gap length (distance between the proximal and distal esophageal pouches), and assessing for other anomalies (VACTERL).
An x-ray of the chest and abdomen can diagnose a TEF if intestinal gas is present, estimate the length of the proximal esophageal pouch by placing gentle downward traction on the oral-esophageal (OE) tube, and identify vertebral anomalies.
Preoperative laryngotracheobronchoscopy is used to assess for coexisting laryngeal clefts and to identify the location of a TEF.
Occasionally pre-operative fluoroscopy is utilized to diagnose EA and determine EA type and gap length.
Echocardiogram is essential prior to EA repair to identify congenital heart disease and to determine the side of the aortic arch.
Approximately 5% of EA/TEF patients will have a right sided aortic arch, and this may alter the decision regarding which side the surgical repair should be performed on.
Lastly, renal ultrasound and anorectal exam are performed to evaluate for renal anomalies and anorectal malformations.
How is long gap atresia defined and what is its impact on management?
The term “gap length” refers to the distance between the proximal and distal esophageal pouches, and primary EA repair can be difficult or impossible to achieve if the gap is long.
There is no consensus on the specific definition of long gap EA, however it is often described by the number of vertebral bodies present between the two ends of esophagus (typically 2–5), the gap length in centimeters (typically 2–5 cm), or based on a surgeon’s intraoperative gestalt.
How can one assess esophageal gap length preoperatively?
Gap length can be evaluated preoperatively by plain film or fluoroscopy.
The length of the proximal pouch is estimated by placing gentle downward traction on the OE tube while an x-ray is taken.
In patients with Type C or D EA/TEF, the esophageal length can be estimated by using the carina on x-ray as a landmark for the proximal extent of the distal pouch.
Therefore, esophageal gap length can be predicted by the distance between the distal extent of the proximal pouch and the carina on x-ray.
In patients with Type A or B EA that have a gastrostomy tube in place, fluoroscopy can be utilized to determine the length of the distal pouch either via contrast administration alone or by inserting a guidewire, endoscope, or metal probe retrograde through the g-tube site and advancing into the distal pouch.
What are the important aspects of preoperative management in the setting of EA/TEF?
Immediately following a diagnosis of EA, the patient should be positioned in reverse-Trendelenburg position and an OE tube placed to continuous suction with its tip located just above the distal end of the proximal esophageal pouch.
Adequate decompression of the proximal pouch is essential to prevent aspiration and further respiratory compromise.
Respiratory status should be carefully monitored as intubation and mechanical ventilation may be necessary.
Non-invasive positive pressure strategies should be avoided as they can result in significant gastric distention when a TEF is present.
If severe gastric distention occurs and impedes ventilation, emergent percutaneous decompression of the stomach using a large-bore needle can be lifesaving.
Other options for management of severe gastric distention include placing a gastrostomy tube, advancing the endotracheal tube past the fistula in intubated patients, and finally ligating and dividing the TEF if all else fails.
When should initial TEF ligation be considered prior to definitive EA
repair?
TEF ligation may be necessary prior to definitive EA repair if the fistula is causing physiologic compromise and EA repair is delayed due to patient size, long gap length, or other reasons.
How do outcomes of thoracoscopic versus standard thoracotomy approaches compare for EA/TEF repair?
A recent meta-analysis comparing open and thoracoscopic approaches to esophagoesophagostomy for EA/TEF repair found no differences in outcomes including anastomotic leak rate, esophageal stricture rate, pulmonary complications, time to first oral feeding, or blood loss.
Thoracoscopic repair was found to decrease post- operative ventilation time and length of stay, though operative times were significantly longer compared to open repair.
What are surgical options for long gap EA if primary esophagoesophagostomy is not possible?
The least invasive option is placement of a gastrostomy tube followed by delayed primary repair, generally after waiting a period of ~12 weeks.
This theoretically allows the esophageal pouches to lengthen from somatic growth as well as a combination of pooled oral secretions (proximal pouch) and gastroesophageal reflux (distal pouch).
Other options for esophageal lengthening include circular or spiral myotomy, the Kimura extrathoracic lengthening procedure, and the Foker staged suture-traction method.
Finally, esophageal replacement with a stomach, colon, or small bowel interposition graft may be required if esophageal preservation is not feasible.
What postoperative complications are most common following EA/TEF repair?
Over 60% of patients undergoing EA/TEF repair will have a post-operative complication, with the most common being anastomotic stricture (>40%).
Other common complications include anastomotic leaks (~20%), recurrent fistulas (~5%), and vocal cord paralysis (~5%).
Recent research has found associations between the use of transanastomotic esophageal tubes with increased rates of anastomotic stricture, as well as placement of prosthetic material between the esophageal and tracheal suture lines with increased rates of anastomotic leak.
What long-term comorbidities are associated with EA following repair?
Dysphagia, esophageal dysmotility, gastroesophageal reflux (GERD) and respiratory conditions such as wheezing and recurrent infections are common problems that persist following EA/TEF repair and must be managed over a lifetime.
Are there guidelines for the management of GERD and subsequent screening for Barrett esophagus and malignancy later in life?
Due to the nearly 4-fold increased risk of Barrett esophagus identified in adults with repaired congenital EA and the relative lack of reported GERD symptoms in this population, current guidelines recommend performing lifelong endoscopic surveillance (with multistaged biopsies) starting before the age of 15 years.
Further, fundoplication should be considered in the setting of recurrent esophageal stricture, respiratory complications, or medically refractory GERD.
What is known about the quality of life (QOL) for individuals who were born with EA?
Though there is relatively little data regarding QOL in older patients born with EA, overall adult survivors report their health-related QOL to be equivalent to that of the general population.