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Published on: February 5, 2021
Diagnosis and management of congenital type D esophageal atresia
Cuizhu Feng1,2, Long Li1, Yanxia Zhang1
1Department of Pediatric Surgery, Capital Institution of Pediatrics, Beijing, People's Republic of China.
Insights
Type D esophageal atresia often presents with vague symptoms, leading to frequent misdiagnosis as type C. Early detection and appropriate surgical repair are crucial for favorable outcomes, especially in patients without severe comorbidities.
Area of Science:
- Pediatric Surgery
- Congenital Malformations
- Gastrointestinal Surgery
Background:
- Type D esophageal atresia (EA), characterized by a proximal and distal fistula, presents diagnostic challenges.
- Its rarity and lack of specific clinical manifestations contribute to delayed or incorrect diagnoses.
Purpose of the Study:
- To describe current clinical practices and outcomes for Type D EA.
- To highlight the impact of misdiagnosis on patient management and reoperation rates.
Main Methods:
- Retrospective analysis of 10 patients with Type D EA treated between January 2017 and May 2022.
- Review of diagnostic methods, surgical approaches (thoracoscopic and open), and patient outcomes.
Main Results:
- 70% of patients were initially misdiagnosed as Type C EA.
- Neonatal repairs involved thoracoscopic ligation and anastomosis, with variable detection of proximal fistulas.
- Non-neonatal repairs utilized both thoracic and cervical approaches.
- Nine out of ten patients achieved a cure; one death was attributed to severe congenital heart disease.
Conclusions:
- Type D EA requires high clinical suspicion due to non-specific symptoms.
- Misdiagnosis as Type C EA is a significant factor leading to unplanned reoperations.
- Patients without severe associated anomalies demonstrate a good prognosis following appropriate surgical intervention.
Abstract:
This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed with type D esophageal atresia and underwent esophageal atresia and tracheoesophageal fistula repair in the Capital Institute of Pediatrics and Beijing Children's Hospital from January 2017 to May 2022. Ten patients include three newborns and seven non-newborns. Seven (70%) cases were misdiagnosed as type C esophageal atresia before the first operation. Three neonatal children underwent thoracoscopic distal tracheoesophageal fistula ligation and esophageal anastomosis: the proximal tracheoesophageal fistula was simultaneously repaired with thoracoscopy in one of these children, and the proximal tracheoesophageal fistula was not detected under thoracoscopy in the other two children. Among the seven non-neonatal children, one underwent repair of the proximal tracheoesophageal fistula through the chest and the other six underwent repair through the neck. Nine patients were cured, and one died of complications of severe congenital heart disease. Type D esophageal atresia lacks specific clinical manifestations. Misdiagnosis as type C esophageal atresia is the main cause of an unplanned reoperation. Patients without severe malformations have a good prognosis.
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