Surgical Management of Newborns with Combined TEF, EA, and Duodenal Obstruction

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Surgical Management of Newborns with Combined Tracheoesophageal Fistula, Esophageal Atresia, and Duodenal Obstruction

The coexistence of tracheoesophageal fistula (TEF), esophageal atresia (EA), and duodenal obstruction represents a rare and complex congenital anomaly requiring meticulous surgical planning. This combination, often associated with other VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) malformations, poses significant challenges in neonatal care. The incidence of TEF/EA is approximately 1/3,000 live births, while duodenal obstruction occurs in 1/7,000 live births, primarily due to duodenal atresia (DA) or annular pancreas (AP). Approximately 3–6% of EA cases are complicated by duodenal obstruction, leading to heightened morbidity and mortality. This article synthesizes clinical experiences from four infants treated for combined TEF/EA and duodenal obstruction, highlighting diagnostic strategies, surgical approaches, and long-term outcomes.

Clinical Presentation and Diagnostic Evaluation

Prenatal ultrasound findings such as polyhydramnios, dilated gastric bubbles, or the “double bubble” sign often raise suspicion for upper gastrointestinal anomalies. Postnatal confirmation involves radiographic imaging and clinical assessments:

  1. Esophageal Atresia: Failure to pass a nasogastric tube (NGT) into the stomach, with radiography revealing the NGT coiled in the proximal esophageal pouch (Figure 1A, 1C–1E).
  2. Duodenal Obstruction: Abdominal X-rays showing gastric distension with absent distal bowel gas (Figure 1B–1D). Associated cardiac anomalies, such as patent ductus arteriosus (PDA) or atrial septal defects, are common.

In this series, all four infants exhibited polyhydramnios prenatally. Three cases were diagnosed with DA or AP prenatally or immediately postnatally, while one case had delayed diagnosis of DA nine days after EA/TEF repair.

Surgical Strategies and Techniques

The management dilemma lies in prioritizing repairs and determining whether to perform staged or synchronous procedures. Traditional approaches advocate gastrostomy for gastric decompression followed by staged repairs. However, advancements in neonatal anesthesia and surgical techniques enable simultaneous correction of multiple anomalies.

Case-Specific Management

  1. Patient 1: Staged repair due to delayed DA diagnosis.

    • First Surgery: Right posterolateral thoracotomy with extrapleural TEF ligation and EA repair using “diamond-shape” anastomosis (Figure 2E–2H).
    • Second Surgery: Exploratory laparotomy with duodenoduodenostomy 17 days later.
    • Outcome: Full oral feeding achieved by day 27; no complications at 20-month follow-up (Figure 1F).

  2. Patients 2–4: Synchronous repair of EA/TEF and duodenal obstruction.

    • Procedure: Thoracotomy for TEF ligation and EA anastomosis, followed immediately by laparotomy for duodenoduodenostomy (DA) or bypass (AP).
    • Technical Highlights:
      • Extrapleural approach minimized pulmonary complications.
      • “Diamond-shape” anastomosis involved transverse incision of the proximal esophageal pouch and longitudinal incision of the distal pouch, creating a wide lumen for suturing (Figure 2E–2H).
      • No intraoperative gastrostomy; nasojejunal tubes facilitated early enteral feeding.
    • Outcomes: Full oral feeds by days 14–20; patent anastomoses confirmed radiographically (Figure 1G–1I).

Key Surgical Considerations

1. Timing of Intervention

  • Emergency Repairs: Performed within 24 hours of birth to prevent aspiration pneumonia and gastric rupture.
  • Synchronous vs. Staged Repairs:
    • Synchronous repairs (Patients 2–4) reduced cumulative operative stress and hospital stays (average: 25 days vs. 28 days for staged repair).
    • Staged repairs (Patient 1) were necessitated by delayed diagnosis but still achieved favorable outcomes.

2. Omission of Gastrostomy

Contrary to historical practices, gastrostomy was avoided in all cases. Decompression via NGT and early nasojejunal feeding minimized anastomotic strain, with no instances of leakage or stricture.

3. Anastomotic Technique

The “diamond-shape” anastomosis, characterized by opposing transverse and longitudinal incisions, provided a tension-free, wide-lumen connection. This technique likely contributed to the absence of strictures in all patients.

Postoperative Care and Outcomes

Feeding Protocols

  • Enteral Feeding Initiation: Ranged from day 8 to day 22, depending on surgical approach.
  • Transition to Oral Feeds: Gradual replacement of tube feeds with oral feeds over 7–14 days.

Complications

  • Patient 1: Pneumonia and hyperbilirubinemia, managed conservatively.
  • Patient 2: Neonatal hyperbilirubinemia and left ventricular hemorrhage, resolved without intervention.
  • No anastomotic leaks, strictures, or mortality observed.

Long-Term Follow-Up

At 6–26 months, all patients demonstrated normal growth, patent anastomoses (Figure 1F–1I), and absence of gastroesophageal reflux or respiratory issues.

Discussion

Challenges in Diagnosis and Timing

Delayed diagnosis of duodenal obstruction post-EA repair (Patient 1) underscores the importance of comprehensive postnatal imaging. Upper gastrointestinal contrast studies are critical for identifying concurrent anomalies.

Advantages of Synchronous Repair

  • Reduced Hospital Stay: Synchronous repairs shortened hospitalization by 3–7 days compared to staged approaches.
  • Anesthetic Efficiency: Single anesthesia exposure minimized risks in critically ill neonates.
  • Physiological Continuity: Simultaneous restoration of esophageal and duodenal continuity promoted earlier enteral feeding and gut maturation.

Role of Minimally Invasive Techniques

While thoracoscopic and laparoscopic approaches were not utilized in this series, their potential for reducing surgical trauma warrants exploration. Future studies may evaluate hybrid techniques for multi-level obstructions.

Conclusion

Synchronous repair of TEF/EA and duodenal obstruction without gastrostomy is a safe and effective strategy in hemodynamically stable neonates. The “diamond-shape” anastomosis and early enteral feeding protocols optimize outcomes, reducing morbidity and hospital stays. This approach challenges traditional staged interventions, offering a paradigm shift in managing complex congenital gastrointestinal anomalies.

doi.org/10.1097/CM9.0000000000000102

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