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EUS diagnosis of asymptomatic type III choledochal cyst

Open AccessPublished:December 10, 2020DOI:https://doi.org/10.1016/j.vgie.2020.10.015
      Choledochal cysts occur in approximately 0.007% of live births in the United States, compared with 0.1% in Asia.
      • Hoilat G.J.
      • John S.
      Choledochal cyst.
      • Lobeck I.N.
      • Dupree P.
      • Falcone Jr., R.A.
      • et al.
      The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature.
      • Antaki F.
      • Tringali A.
      • Deprez P.
      • et al.
      A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video).
      There are 6 types of choledochal cysts, with type I being the most common (Fig. 1). A type III cyst, accounting for 1% to 5% of biliary cysts, involves dilation of the intraduodenal part of the distal common bile duct; these cysts are further subclassified into type IIIA, in which both the bile and pancreatic ducts terminate in the cyst, and type IIIB, in which an intra-ampullary or intraduodenal diverticulum is formed.
      • Hoilat G.J.
      • John S.
      Choledochal cyst.
      • Lobeck I.N.
      • Dupree P.
      • Falcone Jr., R.A.
      • et al.
      The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature.
      • Antaki F.
      • Tringali A.
      • Deprez P.
      • et al.
      A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video).
      In a 40-year systematic review, a total of 325 patients were reported between 1975 and 2015, with more cases diagnosed in adulthood.
      • Lobeck I.N.
      • Dupree P.
      • Falcone Jr., R.A.
      • et al.
      The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature.
      Although many can be found incidentally, symptoms include abdominal pain, nausea, vomiting, pancreatitis, jaundice, and cholangitis.
      • Hoilat G.J.
      • John S.
      Choledochal cyst.
      • Lobeck I.N.
      • Dupree P.
      • Falcone Jr., R.A.
      • et al.
      The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature.
      • Antaki F.
      • Tringali A.
      • Deprez P.
      • et al.
      A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video).
      • Lipsett P.A.
      • Pitt H.A.
      • Colombani P.M.
      • et al.
      Choledochal cyst disease. A changing pattern of presentation.
      Malignancy risk is thought to be lower in type III cysts compared with type I and IV.
      • Hoilat G.J.
      • John S.
      Choledochal cyst.
      • Lobeck I.N.
      • Dupree P.
      • Falcone Jr., R.A.
      • et al.
      The presentation and management of choledochocele (type III choledochal cyst): a 40-year systematic review of the literature.
      • Antaki F.
      • Tringali A.
      • Deprez P.
      • et al.
      A case series of symptomatic intraluminal duodenal duplication cysts: presentation, endoscopic therapy, and long-term outcome (with video).
      • Lipsett P.A.
      • Pitt H.A.
      • Colombani P.M.
      • et al.
      Choledochal cyst disease. A changing pattern of presentation.
      Treatment with sphincterotomy, endoscopic, or surgical resection is indicated in symptomatic type III cysts.
      • Chatila R.
      • Andersen D.K.
      • Topazian M.
      Endoscopic resection of a choledochocele.
      ,
      • Alonso-Lej F.
      • Rever Jr., W.B.
      • Pessagno D.J.
      Congenital choledochal cyst, with a report of 2 and analysis of 94, cases.
      We present a case of EUS-guided diagnosis of a type IIIA choledochal cyst.
      Figure thumbnail gr1
      Figure 1Classification of choledochal cysts. GB: gallbladder; RHD: right hepatic duct, LHC: left hepatic duct; CHD: common hepatic duct; CBD: common bile duct; CD: cystic duct.
      An 81-year-old woman with a medical history of diabetes mellitus type II, hypertension, hyperlipidemia, GERD, and irritable bowel syndrome presented to the outpatient gastroenterology clinic for symptoms of abdominal cramping and constipation. She had a history of mixed-type irritable bowel syndrome but reported worsening constipation with abdominal cramping localized to the lower abdomen. She underwent a colonoscopy that was remarkable for severe diverticulosis in the sigmoid.
      An abdominal CT scan showed cystic dilation of the distal common bile duct measuring 1.7 cm and extending into the lumen of the descending duodenum (Fig. 2). The common bile duct was 9 mm, and the pancreatic duct was unremarkable. Liver function tests were unremarkable. The patient was referred for an EUS examination. A bulging of the ampullary region was noted, with papilla located inferiorly on this bulge (Fig. 3). On EUS examination, an anechoic cystic dilation of the intraduodenal segment of the bile duct was seen with normal caliber of bile duct (Fig. 4). There were no signs of endosonographic or endoscopic abnormalities in the ampulla. The proximal bile duct appeared normal. The pancreas and pancreatic duct also appeared normal.
      Figure thumbnail gr2
      Figure 2Coronal view of a CT scan of the abdomen and pelvis. A, Arrow showing type III choledochal cyst. B, Arrow showing type III choledochal cyst and common bile duct.
      Figure thumbnail gr3
      Figure 3A, Duodenoscopy view of type III choledochal cyst. B, Evidence of ampullary orifice.
      Figure thumbnail gr4
      Figure 4EUS examination demonstrating type III choledochal cyst (yellow arrow). A, Postbulbar curvilinear endosonographic view. B, Bulbar curvilinear endosonographic view (yellow arrow).
      Although duodenal duplication cysts can be misdiagnosed as type III choledochal cysts, duplication cysts often appear to be multilayered with epithelial lining and muscularis proporia that are continuous with the duodenum. They often have an echogenic inner layer with a hypoechoic surrounding layer on EUS.
      • Liu R.
      • Adler D.G.
      Duplication cysts: diagnosis, management, and the role of endoscopic ultrasound.
      This was not present in this patient, thus confirming the diagnosis of type III choledochal cyst. Given the patient’s advanced age, normal liver biochemistry, and absence of clinical symptoms, no endoscopic or surgical intervention was pursued. A 6-month follow-up is planned (Video 1, available online at www.VideoGIE.org).

      Disclosure

      All authors disclosed no financial relationships.

      Supplementary data

      References

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