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Sonographic visualization of the Y configuration sign in the diagnosis of a duodenal duplication cyst


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Sonographic visualization of the Y configuration sign in the diagnosis of a duodenal duplication cyst

Denise A Castro1, Achire N Mbanwi2, Sean Nurmsoo3, Donald A Soboleski4

1The Hospital for Sick Children, Toronto, ON; 2Queen’s University, Kingston, ON; 3Dalhousie University, Halifax, ON; 4Kingston General Hospital, Kingston, ON



• To demonstrate the sonographic Y configuration sign as a specific finding for the preoperative diagnosis of an enteric duplication cyst 
• To highlight the challenges involved in accurately diagnosing enteric duplication cysts using the sonographic double wall sign or gut signature sign
Patient Presentation
• A 2-day old Caucasian boy was admitted to hospital with non-bilious projectile vomiting, decreased urine output and jaundice. Initial sonography at an outside institute revealed a RUQ cystic elongated mass with adjacent dilated common bile duct (Fig1-arrows,1-CBD). The patient was referred to the local tertiary care hospital where initial sonogram displayed a dilated duct with apparent adjacent fluid filled duodenum (Fig.2-arrows) with no cystic mass. The patient clinically improved with bilirubin levels returning to normal and a cessation of vomiting and was discharged home . Follow-up dedicated sonography of the region performed 1 month later displayed both the ‘double-wall’ and the ‘split wall’ or ‘Y’ sign (Fig 3a/b arrows).
• GI study (Fig.4) demonstrates effacement on duodenal loop by the  mass. Given the location of the cyst spanning ampulla the infant was transferred to a second institution for surgery. Repeat sonography  again raised concern of the cyst potentially reflecting a choledochal cyst  variant as opposed to a duodenal duplication.
• MR imaging (Fig 5a- T2 TSE FS; 5b- MIP-MRCP ) better delineated course of  CBD(arrows) and the cystic mass (labelled 1)  without visualization of duodenal wall stratification. Further investigation with ERCP was also consistent with a duodenal duplication cyst. Patient subsequently underwent successful surgery with a diagnosis of duodenal duplication cyst.
Case Review
• Enteric duplication cysts are rare congenital anomalies that normally present in the first year of life with signs and symptoms of obstruction, palpable abdominal mass, hemorrhage and pancreatitis; malignant transformation is rare
• Histology reveals an inner mucosal/submucosal layer with outer smooth muscle layer which on sonography results in the double wall sign which has been used to support the diagnosis
       •inner hyperechoic rim: mucosa-submucosa
       •outer hypoechoic rim: muscularis propria
• Multiple mimics of the double wall or muscular rim sign have been documented in the literature including torted ovarian cyst, Meckel’s diverticulum,  mesenteric cyst complicated by past haemorrhage and cystic teratoma where the peripheral distribution of fat resulted in an apparent hyperechoic inner wall layer
• Cases have also illustrated a false negative double wall sign due to a duplication cyst being complicated by infection, inflammation and/or necrosis. A non distended cyst related to communication/collapse with native bowel lumen or obscuration of wall margins due to overlying bowel gas also can result in lack of visualization of a double wall
• ‘Y ‘ or ‘split’ hypoechoic configuration sign on sonography is the result of splitting of a shared muscularis propria layer between the duplication cyst and native bowel (Fig 6 a/b- previously published
Teaching Points
• The double wall sign is not specific for the diagnosis of a duplication cyst
• The Y configuration sign has not been described with other entities to date and may be specific to the diagnosis of a duplication cyst
• Lack of identification of the double wall sign does not exclude an enteric duplication cyst
• This case highlights the challenges often encountered in correctly diagnosing these abdominal lesions and re-presents in vivo demonstration of the Y configuration sign in the diagnosis of a duodenal duplication cyst
• Macpherson RI. Gastrointestinal tract duplications: clinical, pathologic, etiologic, and radiologic considerations. Radiographics. 1993;13(5):1063-80
• Sanguesa Nebot C, Llorens Salvador R, Carazo Palacios E, Pico Aliaga S, Ibanez Pradas V. Enteric duplication cysts in children: varied presentations, varied imaging findings. Insights Imaging. 2018;11(10):018-0660
• Barr LL, Hayden CK, Jr., Stansberry SD, Swischuk LE. Enteric duplication cysts in children: are their ultrasonographic wall characteristics diagnostic? Pediatr Radiol. 1990;20(5):326-8
• Kumar D, Ramanathan S, Haider E, Khanna M, Otero C. Education and Imaging. Gastroenterology: Revisiting the forgotten sign: Five layered gut signature and Y configuration in enteric duplication cysts on high resolution ultrasound. J Gastroenterol Hepatol. 2015;30(7):1111
• Cheng G, Soboleski D, Daneman A, Poenaru D, Hurlbut D. Sonographic pitfalls in the diagnosis of enteric duplication cysts. AJR Am J Roentgenol. 2005;184(2):521-5


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