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[Duplication of the digestive tract in children. Apropos of 12 cases]
T Scheye1, G Vanneuville, P Dechelotte
1Service de Chirurgie Pédiatrique, Hôtel-Dieu, Clermont-Ferrand.
Insights
Gastrointestinal duplications are rare congenital malformations. While diagnosis can be challenging, surgical intervention is the only effective treatment for these anomalies.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Congenital Malformations
Background:
- Gastrointestinal duplications are rare congenital anomalies characterized by two muscular layers with myenteric cells.
- These duplications are typically found on the mesenteric border and share a common blood supply with the adjacent gastrointestinal tract.
Observation:
- Duplications can manifest as cystic or tubular structures located anywhere along the alimentary tract.
- Clinical presentations are highly variable, depending on the duplication's site, and can include abdominal masses, mediastinal compression, or intestinal obstruction, bleeding, and perforation.
- While diagnosis can be aided by preoperative investigations, these malformations are often discovered incidentally during surgery.
Findings:
- A review of 12 cases highlights the diverse clinical manifestations and variable presentation ages, predominantly within the first year of life.
- The pathogenesis involves early fetal development, influenced by various mechanisms.
- Surgical treatment is the definitive management, with smaller cystic lesions being more amenable to resection than extensive duplications.
Implications:
- Early and accurate diagnosis of gastrointestinal duplications is crucial for timely surgical planning.
- Understanding the variable presentations aids clinicians in suspecting this rare condition.
- While surgical management is effective, the complexity of extensive duplications poses surgical challenges, necessitating specialized expertise.
Abstract:
Duplications of the gastro intestinal tract are characterised (Ladd and Gross) by the following histological features two muscular layers with myenteric cells inside. Cystic or tubular, they are always situated on the mesenteric border of the gastro intestinal tract with a common blood supply. The pathogenesis of duplication affects early fetal development via various mechanisms. Located anywhere along the alimentary tract as in our review of 12 cases, clinical manifestations vary according to the site. Abdominal cystic mass, mediastinal compression or intestinal obstruction bleeding or perforation are possible symptoms, but the presentation is very variable. The age of presentation is usually within the first year of life. The diagnosis can be made preoperatively by several investigations but the malformation is frequently discovered during surgery. Surgery is the only treatment for these duplications, but smaller cystic lesions are easily treated. However surgery of extensive duplications remains difficult.