What is the recommended treatment approach for a pediatric patient presenting with a duodenal duplication cyst?

Duodenal Duplication Cyst: Recommended Treatment Approach

Surgical resection is the definitive treatment for duodenal duplication cysts in pediatric patients, with complete excision preferred to prevent complications including pancreatitis, bleeding, obstruction, and rare malignant transformation. 1, 2

Diagnostic Confirmation

• Endoscopic ultrasound (EUS) is the gold standard for diagnosis, demonstrating an anechoic, smooth, spherical or tubular structure with a well-defined wall that does not communicate with the gastrointestinal lumen 1, 3, 4
• If the cyst is adjacent to the pancreas, EUS-guided fine needle aspiration may be necessary to exclude pancreatic pseudocyst or pancreatic cystic neoplasm 1, 4
• Abdominal radiographs showing a "double bubble" or "triple bubble" sign with absent distal gas suggest duodenal obstruction, which may be caused by a duplication cyst in neonates 1
• Cross-sectional imaging (CT or MRI) with 3D reconstruction can delineate anatomical relationships to the biliary and pancreatic ducts, which is critical for surgical planning 2

Clinical Presentation Requiring Intervention

• 67% of pediatric patients present with abdominal pain, and 43% develop pancreatitis as a complication 5
• Other presentations include gastric outlet obstruction, gastrointestinal bleeding (melena or hematemesis), nausea, vomiting, and weight loss 6, 2, 7, 8
• The median interval between initial presentation and definitive diagnosis is 17 months (range: 2 months to 12 years), highlighting the importance of early recognition 5
• Duplication cysts can enlarge over time, resulting in mass effect, rupture, or bleeding because they generally do not communicate with the gastrointestinal lumen 1

Treatment Algorithm

Primary Treatment: Complete Surgical Resection

• Complete surgical excision is the ideal treatment to eliminate the risk of recurrent symptoms, complications, and rare malignant transformation 2
• Enucleation or cyst excision is the preferred surgical approach when there is no communication with the biliary or pancreatic ducts, allowing total resection while minimizing morbidity 2
• For cysts with complex anatomy near the ampulla of Vater, duodenotomy with careful dissection is performed, with intraoperative localization of the papilla using methylene blue injection through the cystic duct 2
• Pancreaticoduodenectomy should be reserved as an ultimate option only when complete resection cannot be achieved by less invasive means, given its high morbidity, mortality, and poor quality of life 2

Alternative Treatment: Endoscopic Marsupialization

• Endoscopic marsupialization is increasingly reported as a minimally invasive option for symptomatic duodenal duplication cysts, establishing communication between the cyst cavity and duodenal lumen for continuous drainage 6
• Techniques include deroofing with diathermic snare or fenestration with biopsy forceps followed by expansion with a sphincterotome 6
• However, endoscopic approaches do not provide complete resection and leave residual tissue with potential for malignant degeneration, which has been reported in three cases in the literature 2
• Endoscopic treatment may be considered in adults with significant comorbidities or when surgical risk is prohibitive, but surgical resection remains superior for definitive cure in pediatric patients 6, 2

Surgical Approach for Specific Locations

• For cysts in the duodenal bulb or second portion: Direct excision or marsupialization using a GIA stapler can be performed 8
• For cysts near the ampulla (within 5 cm): Careful dissection is required to preserve the biliary and pancreatic ducts; preoperative 3D reconstruction helps identify any communication (present in 29% of cases) 2
• For cysts in the third or fourth portion of the duodenum: Marsupialization on the duodenum may be technically feasible with favorable outcomes 8

Critical Pitfalls to Avoid

• Failing to distinguish duodenal duplication cysts from pancreatic pseudocysts, pancreatic cystic neoplasms, choledochoceles (Todani type III), or other cystic lesions can lead to inappropriate management 4, 2
• Delayed diagnosis is common (median 17 months from presentation to treatment), which increases the risk of life-threatening complications including recurrent pancreatitis, hemorrhage, and obstruction 5
• Biopsy or FNA should be avoided for mediastinal esophageal duplication cysts due to risk of mediastinitis, but may be necessary for duodenal cysts adjacent to the pancreas 3, 4
• Incomplete resection or marsupialization leaves residual tissue with potential for malignant transformation, which has been documented in the literature 2

Postoperative Considerations

• Early postoperative bleeding is a recognized complication, occurring in up to 25% of cases after endoscopic or surgical intervention, typically within the first 48 hours 6, 2
• No routine surveillance is required after complete surgical resection of benign duplication cysts, as they have a benign natural history when fully excised 3
• Outcomes are favorable in the majority of patients after appropriate treatment, with resolution of symptoms and no recurrence when complete excision is achieved 6, 2, 8