Tracheoesophageal Fistula & Esophageal Atresia
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Questions and Answers

What is the most common type of esophageal atresia with a tracheoesophageal fistula?

  • Type D
  • Type B
  • Type C (correct)
  • Type A

    • What may result from acidic stomach contents refluxing through a tracheoesophageal fistula?

  • Esophageal rupture
  • Aspiration pneumonia (correct)
  • Intestinal obstruction
  • Cyanosis

    • What is one of the presentations likely observed during pregnancy for a neonate with tracheoesophageal fistula?

  • Gestational diabetes
  • Oligohydramnios
  • Low birth weight
  • Polyhydramnios (correct)

    • Which genetic abnormality is associated with esophageal atresia and tracheoesophageal fistula?

    <p>Trisomy 21 (Down syndrome)</p> Signup and view all the answers

    What should be the initial management for a neonate presenting with a tracheoesophageal fistula?

    <p>NPO status and hydration</p> Signup and view all the answers

    Which of the following is a classic symptom associated with esophageal atresia?

    <p>Excessive pharyngeal secretions and drooling</p> Signup and view all the answers

    What is one of the main goals during intraoperative management of a neonate undergoing repair of tracheoesophageal fistula?

    <p>Minimize inspiratory pressure for adequate gas exchange</p> Signup and view all the answers

    What is the potential consequence of failing to adequately ventilate during surgery for tracheoesophageal fistula?

    <p>Need for emergency occlusion or ligation of the fistula</p> Signup and view all the answers

    What anatomical development issue can lead to esophageal atresia and a tracheoesophageal fistula?

    <p>Deviated formation of the tracheoesophageal septum</p> Signup and view all the answers

    Which of the following statements about the VACTERL association is true?

    <p>It includes at least two of the listed anomalies.</p> Signup and view all the answers

    Which fluid replacement should be considered for insensible losses during surgery?

    <p>D10/0.2 NS</p> Signup and view all the answers

    During prenatal ultrasound, what finding may suggest esophageal atresia?

    <p>Absence of a fluid-filled stomach bubble</p> Signup and view all the answers

    What should be monitored in a neonate undergoing surgery for a tracheoesophageal fistula?

    <p>Temperature, SpO2, and blood pressure</p> Signup and view all the answers

    What is the primary goal when positioning the endotracheal tube (ETT) during intubation?

    <p>To ensure the ETT is distal to the carina and proximal to the fistula</p> Signup and view all the answers

    Which of the following approaches is commonly used to minimize the danger of an anastomotic leak during surgical procedures?

    <p>Extrapleural approach</p> Signup and view all the answers

    What complication is associated with congenital diaphragmatic hernia due to failure of pleuroperitoneal canal closure?

    <p>Pulmonary vascular hypoplasia</p> Signup and view all the answers

    What should be the focus of preoperative care for a patient with pyloric stenosis?

    <p>Correction of hypovolemia and dehydration</p> Signup and view all the answers

    Which statement about the management of respiratory compensation during metabolic alkalosis is accurate?

    <p>Hypoventilation and apnoea may lead to respiratory acidosis</p> Signup and view all the answers

    What is the occurrence rate of congenital diaphragmatic hernia in live births?

    <p>1 in 3000</p> Signup and view all the answers

    What is a common intraoperative problem associated with intubation in patients with a fistula?

    <p>ETT kinking</p> Signup and view all the answers

    What is a significant postoperative consideration for infants with tracheomalacia?

    <p>They are at risk for aspiration pneumonia.</p> Signup and view all the answers

    Which surgical technique involves splitting pyloric muscle fibers without cutting the mucosa?

    <p>Pyloromyotomy (Ramstedt)</p> Signup and view all the answers

    What is a common manifestation of congenital diaphragmatic hernia at birth?

    <p>Scaphoid abdomen with barrel chest</p> Signup and view all the answers

    In the case of metabolic alkalosis due to pyloric stenosis, which electrolyte imbalance commonly occurs?

    <p>Hypokalemia</p> Signup and view all the answers

    What is a risk associated with using positive pressure ventilation (PPV) in infants with a fistula?

    <p>Potential barotrauma and ETT malposition</p> Signup and view all the answers

    When considering postoperative care, which condition in an infant is most critical to monitor following repair of a tracheoesophageal fistula?

    <p>Aspiration pneumonia</p> Signup and view all the answers

    Which fluid replacement strategy is recommended for infants with 5-10% dehydration?

    <p>Maintenance fluid of NS at 75ml/kg/day</p> Signup and view all the answers

    Study Notes

    Tracheoesophageal Fistula & Esophageal Atresia

    • Incidence: 1:3000 live births.
    • Tracheoesophageal fistula allows inhaled air to bypass the lungs and enter the stomach, leading to hypoventilation and gastric distension.
    • Risk of acidic stomach content refluxing into the trachea through the fistula, causing aspiration pneumonitis.
    • Esophageal atresia results in pooling of secretions from the hypopharynx in the proximal portion, causing drooling, coughing and choking during feeding.
    • Gross classification:
      • Type A: EA without fistula (7.7%)
      • Type B: EA with proximal fistula (0.8%)
      • Type C: EA with distal fistula (86%) - most common
      • Type D: EA with proximal and distal fistula (0.7%)
      • Type E: Tracheoesophageal fistula without atresia (4.2%)
    • Associated anomalies: VACTERL association (50% of TEF/EA), involving three or more of the following:
      • Vertebral defects (10%)
      • Anal atresia (14%)
      • Cardiac anomalies (29%)
      • Tracheo-Esophageal fistula
      • Renal dysplasia, hydronephrosis, ureteral malformation, hypospadias
      • Limb defects (radial aplasia)
    • Other associated anomalies include malrotation of the midgut and duodenal atresia.
    • Genetic abnormalities: Trisomy 13 (Patau), 18 (Edward), 21 (Down), CHARGE syndrome, Feingold syndrome, Pallister-Hall syndrome, Anophthalmia-esophageal-genital (AEG) syndrome.
    • Presentations:
      • Polyhydramnios during pregnancy.
      • Failure to pass an orogastric tube.
      • Excessive pharyngeal secretions and drooling.
      • 3Cs: choking, coughing, cyanosis during first feeding attempt.
      • Pneumonia or pneumonitis may be present.
      • Absence of a fluid-filled stomach bubble on prenatal ultrasound.

    Pre-operative Evaluation & Preparation

    • Okamoto et al. Classified neonates into 4 groups:
      • Group 1: Weight >2 kg, no major cardiac disease, survival 100%.
      • Group 2: Weight <2 kg, major cardiac disease, survival 72%.
      • Group 3: Weight <1.5 kg, no major cardiac disease, survival 70%.
      • Group 4: Weight <1 kg, major cardiac disease, survival 28%.
    • NBM status.
    • Warm Isolette (incubator) with head elevated 30 degrees.
    • Replogle tube in the upper pouch with intermittent lower pressure suction applied to one lumen.
    • The second lumen entrains air to prevent obstruction.
    • Hydration and electrolyte balance: acid-base balance, electrolytes, glucose.
    • Treat pneumonia with antibiotics.
    • Intubation is not routinely performed to minimize gastric distension from positive pressure ventilation unless severe pulmonary disease is present.

    Timing of Surgery

    • Very early primary repair – standard of care.
    • If severe pneumonia or respiratory distress, intubation may be necessary.
    • HFOV can optimize ventilation with minimal peak inspiratory pressure while minimizing gastric distension.
    • If insufficient ventilation, gastrostomy may allow drainage of gastric fluid and prevent gastric distension.
    • Emergency occlusion or ligation of the fistula may be necessary if adequate ventilation is unsuccessful.

    Intra-operative Management

    • Monitoring: temperature, SpO2, ECG, EtCO2, BP.
    • IV Access: arterial line, 2 peripheral lines, central line.
    • Replogle suction tube should be suctioned before induction.
    • Dextrose-containing solution at a controlled rate to avoid hypoglycemia (e.g., D10/0.2 NS).
    • Insensible losses (3-4 ml/kg/hr) should be replaced with an isotonic solution.
    • Urine output should be 1 ml/kg/hr.

    Intra-operative Management: Transport & Temperature

    • Transport to the operating room in a warm isolette.
    • Surgical irrigation, IV crystalloid and blood products should be warmed.
    • Forced-air convective warming system.
    • Appropriate size Heat and Moisture Exchanger (HME).
    • Neonates are prone to hypothermia due to limited subcutaneous fat.
    • Compensate with brown fat non-shivering thermogenesis.

    Induction of Anaesthesia

    • Goal: adequate gas exchange with the lowest inspiratory pressure needed to inflate the lungs, minimize atelectasis, and minimize gas flow through the fistula.
    • Intravenous induction with rapid sequence intubation to minimize face mask ventilation.
    • Options include deep inhalational induction, gentle positive pressure ventilation and intubation.
    • Gentle assisted or controlled ventilation is preferred.
    • Keep low peak pressure and high rate.
    • Correct positioning of the endotracheal tube:
      • Tip of the ETT distal to the fistula, yet proximal to the carina.
      • Presence of tracheomalacia and a second fistula.
      • Occlusion of the fistula by a 3.5 Fogarty occlusion catheter, allowing normal positive pressure ventilation.
      • Keep the tip of the ETT above the fistula with low ventilation pressure until the fistula is ligated to minimize risk of ETT malposition.
    • Role of bronchoscopy.

    Surgical Procedure

    • Depends on the distance between the upper and lower portions of the esophagus.
    • Options:
      • Primary anastomosis and ligation of the fistula.
      • Feeding gastrostomy and cervical esophagostomy until definitive surgery later.
    • Thoracotomy approach:
      • Left lateral position with the right side up.
      • Extrapleural approach minimizes the danger of anastomotic leak.
    • Thoracoscopic surgery:
      • Arterial line is required due to significant gradient between EtCO2 and PaCO2.
      • Lung isolation is not required.
      • Sudden desaturation requiring high FiO2 and sudden increase in EtCO2 may occur if one-lung ventilation is required.

    Intra-operative Problems

    • Common intraoperative problems:
      • Hypoventilation: hypercarbia, atelectasis, desaturation.
      • Gastric distension requiring gastric decompression.
      • ETT kinking or displacement to endobronchial/fistula.
      • Blood and secretions can block the ETT.

    Post-operative Care

    • Analgesia:
      • Fentanyl 1-2 mcg/kg.
      • Caudal epidural catheter threaded up to the thoracic region.
    • Keep intubated in the NICU.
    • If aspiration pneumonia, lung disease from prematurity, low birth weight, prematurity, receiving narcotics for pain, or risk of tracheomalacia (deficiency of tracheal cartilage at the level of the fistula) are present, care should be taken to limit inspiratory pressure to protect the repair.

    Complications

    • Anastomotic leak (15%) requiring immediate exploration.
    • Esophageal dysmotility due to vagal nerve injury.
    • GERD.
    • Esophageal stricture requiring repeated dilatations.
    • Tracheomalacia.

    Congenital Diaphragmatic Hernia

    • Herniation of abdominal viscera into the thorax.
    • Failure of the pleuroperitoneal canal to close.
    • Prevents normal lung development, resulting in lung hypoplasia.
    • Airway maldevelopment: reduced number of alveoli, low lung compliance, reduced ventilation, and oxygenation.
    • Pulmonary vascular hypoplasia: pulmonary hypertension, right-to-left shunt via PDA, and hypoxemia.

    Diaphragmatic Hernia: Incidence & Associated Anomalies

    • High mortality (30-50%).
    • Incidence: 1:3000 live births.
    • Male:Female (1:2).
    • 90% posterolateral (Bochdalek): 85% left-sided.
    • 2% anterior (Morgagni).
    • Other: anteromedial, paraoesophageal, eventrations.
    • Associated anomalies:
      • Cardiovascular system: VSD, ASD, TOF, COA (13-23%).
      • Central nervous system: spina bifida, hydrocephalus (28%).
      • Gastrointestinal tract: malrotation and atresia (20%).
      • Genitourinary tract: hypospadias (15%).

    Diaphragmatic Hernia: Presentations & Diagnosis

    • Respiratory distress.
    • Cyanosis.
    • Apparent dextrocardia.
    • Scaphoid abdomen with barrel chest.
    • Diminished air entry on the affected side.
    • Bowel sounds in the chest.
    • Diagnosis on prenatal ultrasound.
      • Lung to head circumference.
      • Diameter of proximal pulmonary artery indexed to the descending aorta.
      • Intrathoracic position of the liver.

    Diaphragmatic Hernia: Types & Pre-operative Care

    • Types:
      • Minor herniation: asymptomatic at birth, incidental finding or late presentation.
      • Major herniation: respiratory distress at birth requiring mechanical ventilation, hypoxia, pulmonary hypertension, right-to-left shunt via PDA.
    • Pre-operative care:
      • Delay surgery and focus on medical stabilization (hemodynamic and respiratory support), which improves outcome.
      • If the neonate cannot be stabilized, proceed with surgery with HFOV, ECMO, or nitric oxide.
      • Initial resuscitation: intubation if respiratory distress to improve oxygenation.
      • Orogastric tube to decompress the stomach.
      • Avoid positive pressure ventilation by facemask.
      • Ventilate with a high rate and low pressure to avoid barotrauma.
      • Correct hypothermia, acidosis, and hypoglycemia.

    General Assessment of Dehydration

    • Alert, restless, thirsty: Mild dehydration.
    • Lethargic, restless, thirsty: Moderate dehydration.
    • Drowsy, sweaty, cold: Severe dehydration.

    Signs & Symptoms of Dehydration

    • Pulse:
      • Normal: Mild dehydration.
      • Increased, weak: Moderate dehydration.
      • Rapid, feeble: Severe dehydration.
    • Anterior fontanel:
      • Normal: Mild dehydration.
      • Sunken: Moderate dehydration.
      • Markedly depressed: Severe dehydration.
    • Skin turgor:
      • Normal: Mild dehydration.
      • Reduced: Moderate dehydration.
      • Markedly reduced: Severe dehydration.
    • Eye:
      • Normal: Mild dehydration.
      • Sunken, dry: Moderate dehydration.
      • Markedly sunken, very dry: Severe dehydration.
    • Mucous membrane:
      • Normal: Mild dehydration.
      • Dry: Moderate dehydration.
      • Very dry: Severe dehydration.
    • Capillary refill:
      • Normal: Mild dehydration.
      • 2 seconds: Moderate dehydration.
    • Blood pressure:
      • Normal: Mild dehydration.
      • Normal/low: Moderate dehydration.
      • Low/very low: Severe dehydration
    • Respiration:
      • Normal: Mild dehydration.
      • Deep: Moderate dehydration.
      • Deep/rapid: Severe dehydration.
    • Urine output:
      • Adequate: Mild dehydration.
      • Decreased : Moderate dehydration.
      • Oliguria/anuria: Severe dehydration.

    Pyloric Stenosis: Metabolic Derangement & Adverse Effects

    • Metabolic derangement:
      • Vomiting: loss of Na+, K+, Cl-, H+.
      • Renal compensation for metabolic alkalosis: large HCO3 load exceeds absorptive threshold, leading to HCO3- excretion with Na+ and alkaline urine.
      • Persistent vomiting: hypovolemia, RAAS activation, body conserves Na+ and excretes K+, H+ with HCO3-, resulting in paradoxical aciduria and worsening hypokalemia, further worsening metabolic alkalosis.
      • Respiratory compensation for metabolic alkalosis: hypoventilation and apnea, leading to respiratory acidosis (insufficient, as hypoxic drive will be triggered).
      • Severe dehydration: hypovolemic shock with impaired renal and hepatic function, leading to metabolic acidosis and oliguria, and respiratory alkalosis.
    • Adverse effects of metabolic alkalosis:
      • Shifting of oxygen dissociation curve to the left, unloading less oxygen at tissue level. More important in newborns as they still have fetal hemoglobin with a low p50 value.
      • Respiratory compensation with hypoventilation leads to atelectasis and apnea.
      • Decreases ionized calcium.
      • Increases potential for seizure.
      • Severe cases can be life-threatening.

    Pyloric Stenosis: Severity

    • Severity indicators (pH, pCO2, CO2, pO2, Na, K, Cl, HCO3) should be monitored closely.

    Pyloric Stenosis Pre-operative Management

    • Correct hypovolemia/dehydration.
    • Correct metabolic derangement.
    • Prevention of aspiration due to the risk of regurgitation.

    Fluid Management for Pyloric Stenosis

    • Maintenance fluid:
      • First 48 hours of life: 75 ml/kg/day or 3 ml/kg/hr.
      • 2 days - 1 month old: 150 ml/kg/day or 6 ml/kg/hr.
      • 1 month old onward (up to 10 kg): 100 ml/kg/day or 4 ml/kg/hr.
    • Replenishment of losses:
      • Evaporative, 3rd space loss, from lungs (3-5 ml/kg/hr).

    Electrolyte Imbalance

    • Maintenance electrolytes:
      • Sodium: 3-5 mmol/kg/day.
      • Potassium: 2-3 mmol/kg/day.
      • Chloride: 1-3 mmol/kg/day.

    Timing of Surgery for Pyloric Stenosis

    • Not an emergency surgery.
    • Hemodynamically stable.
    • Well hydrated with good urine output.
    • Biochemical parameters:
      • pH>7.30,
      • Cl->90,
      • K+>3.0,
      • HCO3>18

    Pyloromyotomy (Ramstedt) Surgical Procedure

    • Pyloric muscle fibers split without cutting the mucosa.
    • Open vs laparoscopic vs endoscopic approaches.

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    Description

    This quiz explores the complexities of tracheoesophageal fistula and esophageal atresia, including incidence, classifications, and associated anomalies. Understand the clinical implications and risk factors involved with these congenital conditions.

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