Endoscopic treatment of periampullary duodenal duplication cysts in children: Four case reports and review of the literature

Abstract

Background: Duodenal duplications are rare congenital anomalies of the gastrointestinal tract. As the periampullary variant is much rarer, literature is scant and only few authors have reported their experience in diagnosis and treatment, particularly with operative endoscopy.

Case sumary: To report our experience with the endoscopic treatment in a series of children with periampullary duodenal duplication cysts, focusing on the importance of obtaining an accurate preoperative anatomic assessment of the malformations. The pediatric periampullary duodenal duplication cyst literature is reviewed. We conducted a systematic review according to the PRISMA guidelines. The PubMed database was searched for original studies on "duodenal duplication", "periampullary duplication" or "endoscopic management" published since 1990, involving patients younger than 18 years of age. Eligible study designs were case report, case series and reviews. We analyzed the data and reported the results in table and text. Fifteen eligible articles met the inclusion criteria with 16 patients, and analysis was extended to our additional 4 cases. Median age at diagnosis was 13.5 years. Endoscopic treatment was performed in 10 (50%) patients, with only 2 registered complications.

Conclusion: Periampullary duodenal duplication cysts in pediatric patients are very rare. Our experience suggests that an accurate preoperative assessment is critical. In the presence of sludge or stones inside the duplication, endoscopic retrograde cholangio-pancreatography is mandatory to demonstrate a communication with the biliary tree. Endoscopic treatment resulted in a safe, minimally invasive and effective treatment. In periampullary duodenal duplication cyst endoscopically treated children, long-term follow-up is still necessary considering the potential malignant transformation at the duplication site.

Keywords: Case report; Double wall sign; Duodenal duplication; Endoscopic treatment; Endoscopic ultrasound; Periampullary duodenal duplication cyst.

Figure 1

Magnetic resonance imaging on HASTE T2 w sequence. A: Homogeneously hyperintense cyst located within the duodenum, which was partially occluded (arrow); B: On 3D cholangiographic reconstruction, intrahepatic bile ducts were normal, cystic duct was dilated with tortuous course and common hepatic duct presented saccular dilation. Common bile duct had a caliber at the upper limits of the normal range with a regular course and was in communication with periampullary duodenal duplication cysts.

Figure 2

Magnetic resonance imaging of case 2. A: Round homogeneously hyperintense lesion at the level of uncinate process of the pancreas determined a major compression on the second portion of duodenum (arrow); B: At cholangiographic reconstruction, the intra- and extrahepatic biliary tree and pancreatic ductal system were normal.

Figure 3

Magnetic resonance imaging of case 3. A: An oval heterogeneously hyperintense lesion containing multiple stones and located in the second part of the duodenum; B: Cholangiographic reconstruction showed normal gallbladder and intra- and extrahepatic bile ducts.

Figure 4

Magnetic resonance imaging showed periampullary duodenal duplication cysts filled with stones.

Figure 5

Magnetic resonance imaging. A: Oval mass is located below the gallbladder and lateral to the common bile duct and pancreatic duct, adjacent to the pancreatic head. The cyst was filled with fluid and multiple stones; B: Cholangiographic reconstruction showed normal gallbladder and intra- and extrahepatic bile ducts.

Figure 6

Endoscopic ultrasound. The probe is inside the duodenum, and the common wall separates the duodenum and the duodenal duplication.

Figure 7

Endoscopic retrograde cholangio-pancreatography. After distal papillotomy, contrast filled the periampullary duodenal duplication cysts.

Figure 8

Cyst marsupialization was performed with subsequent extraction of biliary microstones.

Figure 9

Magnetic resonance cholangiopancreatography performed 3 months after the endoscopic treatment did not show periampullary duodenal duplication cysts.

Figure 10

Magnetic resonance cholangiopancreatography performed after 9 mo endoscopic treatment did not show periampullary duodenal duplication cysts.

Figure 11

PRISMA 2009 flow diagram.