Hasan Ashraf, MSIII Pediatric Surgery

What are duplication cysts?

Duplication cysts are generally benign and rare

anomalies that arise during embryonic development Coined by Ladd in 1937 which included 3 characteristics: (1) ) the epithelial lining represents some portion of the alimentary tract, (2) a welldeveloped coat of smooth muscle is present, and (3) the duplications were attached to some portion of the GI tract Two broad categories: (1) those that are adjacent to the lumen and (2) those that are tubular and communicated directly with the lumen

Epidemiology
Observed in 1/4500 autopsies
Predominantly in white males Small intestine is most frequent site involved (44%),

followed by colonic (15%), though thoracic, gastric, and rectal duplications have been observed

Etiology
Exact etiology unknown; several theories have been proposed:
(1) the initial developmental abnormality occurs during the gastrulation stage and results in a split notochord with persistence of an endomesenchymal tract (2) partial twinning theory (3) Intrauterine environmental during a vascular accident

Symptoms and Complications of Duplication Cysts

Usually asymptomatic in small intestine; depends on

size and location of the duplication Esophageal duplication cysts frequently cause symptoms unlike intestinal duplications: dysphagia, respiratory symptoms, and abdominal pain Duodenal duplications may contain ectopic gastric mucosa which predisposes to ulceration Other symptoms include hematemesis and pancreatitis Case reports have described malignant transformation

Diagnosis of Duplication Cysts

Usually found incidentally on

endoscopy or radiologic imaging Most commonly diagnosed by CT or MRI since they are infrequently seen endoluminally Endoscopic appearance: appear as a bulge with normal overlying mucosa or as a diverticulum

 Endosonographic findings:

anechoic homogenous lesions with regular margins arising from the submucosa or extrinsic to the GI wall. Endosonography is beneficial in discriminating a duplication cyst from a solid mass EUS guided FNA can be used for esophageal duplications though this is not necessary and has complications

Management
May be expectant
Evaluation before surgery based on blood supply Resection in asymptomatic cases for most GI

duplications recommended in recent years based on possibility of malignant transformation and other complications Duodenal and complex tubular colonic duplications are exceptions Resection in symptomatic cases via surgery or endoscopy

References
Banner K, Helft S, et al, An unusual cause of dysphagia in a young woman: esophageal duplication cyst, Gastrointest Endosc. 2008;68(4):793 Coit DG, Mies C, Adenocarcinoma arising within a gastric duplication cyst, J Surg Oncol. 1993;50(4):274 Chiu AS, et al, Enteric Duplication Cyst of the Pancreas Associated with Chronic Pancreatitis and Pancreatic Cancer, J Gastrointest Surg. 2012;23(93):343 Faigel DO, Burgke A, Ginsberg GG, et al, The role of endoscopic ultrasound in the evaluation and managemen of foregut duplications, Gastrointest Endosc. 1997;45(1):99 Geller A, Wang KK, et al, Diagnosis of foregut duplication cysts by endoscopic ultrasonography, Gastroenterology. 1995;109(3):838 Van Dam J, Rice TW, Sivak MV Jr, Endoscopic ultrasonography and endoscopically guided needle aspiration for the diagnosis of upper gastrointestinal tract foregut cysts, Am J Gastroenterol. 1992;87(6):762 Woolfolk GM, McClave SA, Use of endoscopic ultrasound to guide diagnosis and endoscopic management of a large gastric duplication cyst, Gastrointest Endosc. 1998;47(1):76