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Type VI Choledochal Cyst Diagnosed Intraoperatively



















            Figs 1A and B: Laparoscopy showing choledochal cyst of the cystic duct with a narrow outlet of the cystic duct into CHD. (A) An acute angulation
            of the cystic duct with CHD; (B) The squaring of the cystic duct

                                                               of surgery of nonmalignant choledochal cyst requires complete
                                                               excision of the cystic wall.
                                                                  The diagnosis of CDC type VI requires high suspicion.
                                                               Abdominal ultrasound is a good initial screening tool to identify
                                                               any cystic lesion. The abnormality of the cystic duct can be localized
                                                               with ultrasound by tracing its connection to the gallbladder.
                                                               However, an operator-dependent property of ultrasound may fail
                                                               to delineate the biliary origin of the cyst. The normal diameter of
                                                               the cystic duct varies from 1 to 5 mm. A CDC of the cystic duct
                                                               is defined as the diameter of the cystic duct of more than 5 mm
                                                               without any evidence of biliary obstruction. Any nonvascular dilated
                                                               cystic structure near the porta hepatis should be evaluated for
                                                               its relationship with the CBD, cystic duct, and gallbladder and its
                                                               connection with the biliary tract. 4
                                                                  CT scan helps in accessing hepatobiliary and pancreatic
                                                               anatomy and evaluation of possible malignancy but failed
                                                               to show pancreaticobiliary maljunction. Magnetic resonance
            Fig. 2: Postoperative MRCP showing normal biliary system  cholangiopancreatography appears superior to CT scan for defining
                                                               pancreaticobiliary maljunction. The gold standard investigation
                                                       2
            1983, where the patient presents with acute cholangitis.  It was   for the diagnosis of choledochal cyst is cholangiography.
            Serradel et al. in 1991, who recommended the incorporation of   Cholangiography is effective in demonstrating the anatomy of the
            cystic dilation of the cystic duct as a CDC type VI in the Todani   biliary tree, stone, obstruction, and pancreaticobiliary maljunction.
                      3
            classification.  In the review of literature, there are less than 50   The only disadvantage of cholangiography is that it is an invasive
            cases reported to date. In most of the case reports, the cystic duct   method. 7
            cyst presented as cholelithiasis and was identified intraoperatively.  Typical radiological features of CDC type VI are acute angulation
               A patient with a CDC VI can present to the hospital at any age.   of cystic duct and common hepatic duct junction with a distinct
            However, no sex predilection for cystic duct cyst was found in   plane, squaring and dilatation of the cystic duct, a normal or wide
            the literature of 10 cases over a period of 2 years, as reported by   outlet of the cystic duct into CHD, and associated pancreaticobiliary
                                                                         4
            Maheshwari in 2012. 4                              maljunction.  In our patient intraoperative findings showed acute
               Most of these were detected intraoperatively or occasionally on   angulation of cystic duct and common hepatic duct junction with
            imaging for evaluation of biliary symptoms mostly epigastric and/  a distinct plane, squaring, and dilatation of the cystic duct, which
            or right hypochondrium pain. It is also reported as associated with   were similar to radiological findings and confirmed the diagnosis
            complicated biliary disease, e.g., common hepatic duct obstruction   of type VI CDC.
            due to the mass effect of cyst or inflammation from cholangitis,   Treatment of cystic duct cyst includes complete excision of
            biliary pancreatitis, and rarely reported biliary cancer. 4–6  cystic duct cyst with cholecystectomy. For the cystic duct cyst with
               Choledochal cyst is an established risk factor of biliary cancer   a narrow outlet of the cystic duct into the common hepatic duct,
            and reported incidence varies and depend at age of diagnosis, the   complete excision of the cystic duct cyst with cholecystectomy
            incidence of cancer is 0.7% in the patient under 10 years of age,   can suffice. It can be accomplished laparoscopically by clipping the
            6–8% in the patient’s second decade, and 14.3% after 20 years of age   cyst just adjacent to the opening in the CHD, as done in our case.
                               7
            and as high as up to 50%.  Choledochal cyst, though rarely, is also   Due to the presence of anatomical difficulty and associated biliary
            associated with carcinoma gall bladder, periampullary carcinoma,   anomalies, which are seen in most reported cases in the literature,
            and pancreatic carcinoma. These carcinomas should be excluded   laparoscopic cholecystectomy can be done with a low threshold
            before attempting any surgical procedure. The basic principle   for conversion to open cholecystectomy. 5




            256   World Journal of Laparoscopic Surgery, Volume 15 Issue 3 (September–December 2022)
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