ESOPHAGEAL ATRESIA/TRACHEO ESOPHAGEAL FISTULA (EA/TEF)MANAGEMENT

What are the classification of TEF?

Once baby with TEF is born - what are the priorities ?

1. 1. Care of excessive secretions:
   Place Replogle suction tube to the proximal end of the esophageal pouch to low intermittent suction at -15 to -35 cm H2O ( -11 to -25 mmHg).

2. 2. Position the infant in a semi-prone (“recovery”) position to facilitate drainage of excessive secretions and to minimize risk of gastroesophageal reflux and aspiration.

3. 3. If the neonate requires ventilation, try to avoid Mask PPV or CPAP (to reduce air in the stomach, as most of these neonates have a fistula)

4. A neonate in respiratory distress should be intubated While theoretically, it is ideal to place the tip of the ETT below the level of the fistula, in reality, the position of the fistula is rarely known prior to surgery, and so care should be taken to not place the tip of the ETT too low.

If TEF was not diagnosed in utero, what are some findings that will hint at the diagnosis?

• History of polyhydramnios
  
  Presence of excessive oropharyngeal secretions with or without respiratory difficulty at delivery
  
  

How would TEF/EA be diagnosed ?

• - The diagnosis can be confirmed by the inability to pass a nasogastric tube into the gastric cavity
  
  - An early chest and abdominal xray to confirm the NG tip in the pouch of the esophagus and to determine the presence of bowel gas indicative of a distal fistula.
  ( to determine the length of the upper pouch and presence or absence of bowel gas.)

• - After delivery, episodes of cyanosis and choking during feeding are suspicious of a fistula
  - Rigid bronchoscopy may be indicated to determine the anatomy of TEF in selected cases.

• - Complete physical exam with careful exclusion of other anomalies.
  - Surgical consultation.
  - Discussion regarding timing and preparation for transport to ACH NICU. If an H type defect is suspected, an upper GI contrast should be planned

What are some PREOPERATIVE NICU CARE for TEF?

• - NPO with a Replogle tube on low intermittent suction (-15 – 35 cm H2O)
  -(The Replogle should be flushed and assessed for function q4-6h.)
  - Insert single lumen PICC line.
  - The infant should be nursed with the head of the bed up 45 degrees, to reduce the risk of aspiration
  - Adequate perfusion (blood pressure, pulse volume, tachycardia, lactate). Adequate ventilatory support if clinically indicated (pH, pCO2, respiratory rate, FiO2 required, SpO2)
  - This may be indicated if the neonate has an associated anomaly of imperforate anus which might predispose the neonate to abdominal distension if needing CPAP for respiratory suppor

• Infection (temperature instability, hypotension, thrombocytopenia, neutropenia)
  5. Maintain euglycemia. - Investigations for associated anomalies, such as vertebral or renal, should be ordered. 7. Draw preoperative laboratory studies as clinically indicated:
  -CBC and differential
  -Electrolyte panel
  -Blood gas
  - Coagulation profile (PTT, INR)
  -. Type/cross match 8. Echocardiography to detect significant coexistent congenital heart disease and to determine on which side the aortic arch is located

What about post operation? What would you look out for as bedside and NNP?

Keep infant NPO.
• The Replogle or NG tube as placed by the surgeon should be labeled as a critical NG and maintained open to straight drainage.
(If NG losses are greater than 5mL/kg over 6 hours, the loss greater than 5mL/kg should be replaced 1:1 with D5W or D10W, (depending on the infant’s serum glucose) and 0.2% NaCl and 20mmol/L KCl over the following 6 hours. (ie. If NG losses are 8mL/kg over 6 hrs, then 3mL/kg would be replaced.)

Vital signs assessment, including temperature and blood pressure (invasive or noninvasive):
• q15 mins x4,
then • q30 mins x2,
then • q1hx4, then
• as per unit routine.

Continuous oxygen saturation monitoring.
Continuous transcutaneous or end-tidal CO2 monitoring.
Ventilatory support as clinically indicated.

The endotracheal tube should be labeled as a critical airway.
Re-intubation should be avoided in the post-operative period as the potential hyperextension of the neck required for airway visualization may create excessive strain on the anastomosis.

Ventilator support will be maintained as clinically needed and extubation will be done once the infant breathing pattern is regular and capable of maintaining proper gas exchange as in any other clinical scenario. The use of CPAP post-extubation will be discussed with surgery, but its use should be discouraged if it is considered a risk for proper anastomosis healing.


Fluid and electrolyte balance (strict intake and output should be monitored).

Follow blood gases as ordered.

Common laboratory studies include:
• CBC • Blood culture • Electrolyte panel • Blood gas • Coagulation studies as necessary

Infection prevention measures:
Perform dressing changes as ordered and PRN using aseptic technique.
Administer antibiotics as ordered (usually for first 48 hours post-op) unless otherwise indicated.

Blood cultures should be drawn prior to start of antibiotics and followed for results.
Ampicillin and Gentamicin are recommended, and the duration of treatment is variable.
Antibiotics should be continued for at least 48 hours to confirm blood cultures are negative but may be continued until the anastomosis is confirmed to have no leak.

What are some adverse signs and symptoms s associated with complications of TEF

Postoperative anatomical leakage (16-25%); or pneumothorax, which is at highest risk of occurring between the first and the fifth postoperative days with a mean of two days

Conservative management: • Chest tube drainage (range 5-20 days).
Document and monitor chest tube set, suction and drainage.

Chest Tubes. • Monitor chest tube drainage for changes in type and color. Drainage should be clear amber with small amounts of blood initially. Report any changes, particularly white frothy drainage (may be saliva) or green colored drainage (may be bile).

• Holding of the baby with a chest tube in situ is permitted following consultation with the medical team and a physician to nurse communication in SCM.

• Nil per os (NPO) and initiation of total parenteral nutritional support. • Administration of broad-spectrum antibiotics: ampicillin /aminoglycoside for initial empyema/infection.

Analysis of pleural fluid for bacteria as well as for determining chylothorax, or anastomosis leakage (lymphocyte count, amylase).

• If suggestive of infection (increased neutrophils, organisms on gram stain), consider Escherichia coli, klebseilla, acinetobacter baumannii, pseudomonas aeroginosa, enterobacter cloacae, staphylococcus aureus, streptococcus viridans as possible pathogens .

Dopamine and/or dobutamine may be used to support blood pressure. 15.1. Aim for a mean blood pressure of greater than 45 mmHg in a term baby (Systolic BP >60 mmHg) A fluid bolus with normal saline may be beneficial as the maneuver of first choice to support intravascular volume.
Aim to keep hematocrit greater than 0.30 during the acute phase or while on assisted respiratory support.
Genetics review-Conduct a detailed clinical assessment for associated anomalies.

Consider blood for chromosomal analysis if associated anomalies are found.
Request genetics consultation as needed.
Diligent skin assessment and care at gastrostomy tube site (if delayed anastomosis was done): Assess the area q6h to ensure it is clean and dry from leakage of stomach contents.

Apply Aquaphor as needed to skin and then apply drain sponges between the skin and G-Tube button

Maintain integrity of any other wound sites such as ostomies according to the appropriate protocols.
Anti-reflux management: These infants are at high risk of acid reflux due to compromise of distal esophageal motility. Acid reflux increases the risk of stricture formation and oral feeding aversion

An H2 antagonist should be initiated postoperatively. Once the infant is taking enteral feedings, consideration should be given to giving a proton pump inhibitor (PPI).

Developmental care: These infants tend to be very irritable due to potentially long pre-operative wait time and physical irritation from the indwelling suction. Care should be targeted at normalizing neurodevelopment and limiting pain and irritation from the repogle suction tube.

Strategies should include: 1. Slow gentle care and handling.
2. Provision of deep touchable boundaries or bundling depending on other concomitant monitoring that is required.
3. Provision of Oral Immune Therapy as ordered by the neonatologist.
4. Older infants may respond well to distraction such as music and toys.
5. Skin to skin (STS) care or holding as much as possible.
6. Frequent opportunity for non-nutritive sucking (NNS).
7. Family support, including teaching and explanation of upcoming surgery and procedures.
8. Social Work/psychology referral
9. Lactation support, including