Kenji Sasaki, Department of Internal Medicine, Shiogama City Hospital, 7-1, Kouzumachi, Shiogama, Miyagi, 985-0054, Japan,
Tel: +81-22-364-5521; Fax: +81-22-364-5529; E-mail:
Keywords: Agenesis of the gallbladder; Hypogenesis of the cystic duct; Peroral cholangioscopy; ERCP
This report presents a case of such an anomaly successfully treated with EST and choledocholithotomy under the preoperative diagnosis of CAGB with the hypogenetic cystic duct complicated with choledocholithiasis inducing acute cholangitis and pancreatitis, which was definitely confirmed by peroral cholangioscopy without resorting to exploration through the non-natural orifices.
Abdominal X-ray showed no niveaux or free air. Only the dilated extrahepatic bile duct was barely depicted through her extremely thick adipose tissue by US. CT showed a round, solitary, calcified-surfaced stone in the intrapancreatic bile duct with the dilated upstream and increased density in the adipose tissue around the swollen pancreatic head (Figure 1). The main pancreatic duct was not dilated and there was no peripancreatic fluid. No entopic or ectopic gallbladder was detectable by US, CT, or MRCP.
Laboratory data showed conjugated type-dominant hyperbilirubinemia (total bilirubin 3.6 mg/dl, normal 0.2-1.1, direct bilirubin 2.5 mg/dl, normal 0.0-0.4), moderate liver dysfunction (AST 677, normal 10-38, ALT 555, normal 5-40, Al-P 1,025, normal 104-338, γ-GTP 561, normal 8-45, LDH 714 IU/l, normal 100-230), moderately elevated serum amylase (506 IU/l, normal 40-130), lipase (370 IU/l, normal 13-49), and CRP (4.9 mg/dl, normal 0.0-0.3), and moderate leukocytosis (WBC 15,400, normal 3,500-9,500).
Esophagogastroduodenoscopy showed the patent-orificed papilla of Vater. ERC demonstrated a stone measuring 10.0 x 18.6 mm in diameter in the dilated, distal bile duct measuring 15.7 mm in maximum diameter and a thin, short, rudimentary, blind duct measuring 4.0 mm in width and 17.0 mm in length originating from the mid extrahepatic bile duct and running cephalad parallel to it (Figure 2). No entopic or intrahepatic gallbladder was detected (Figure 3). Lack of previous history suggestive of biliary diseases and too short a duration of the present symptoms excluded the possibility of the scleroatrophic gallbladder. The gallbladder in the aberrant positions was also ruled out by the above mentioned examinations. Under the preoperative diagnosis of CAGB with the hypogenetic cystic duct complicated with choledocholithiasis inducing acute cholangititis and pancreatitis, EST was performed and a stone was successfully retrieved (Figure 4), which resulted in immediate improvement of her general status and laboratory data. Subsequent peroral cholangioscopy clearly showed the duct to be a crescent-folded cul-de-sac arising from and running cephalad parallel with the common hepatic duct (Figures 5a-5c), undoubtedly revealing it to be the hypogenetic cystic duct, the vestige of the abortive primordium which should have developed into the full-fledged cystic duct and gallbladder. No other abnormalities were detected in the papillary orifice through the bifurcation of the hepatic ducts. Thus the preoperative diagnosis was definitely proven accurate on the basis of the endoscopic characteristics satisfying Frey et al.’s two diagnostic criteria . She has kept an uneventful post-operative course for two years.
The definite proof of CAGB necessitates elimination of the conditions which could be mistaken for the anomaly, such as the ectopic or scleroatrophic gallbladder . Cases of the gallbladder located in the aberrant positions have been described, such as in the retroperitoneum , suprahepatic space , falciform ligament , liver  and anterior abdominal wall . Some of them were detected by CT [13, 14] and the others are considered clearly detectable by it [15-17]. No structures reminiscent of the gallbladder were revealed at such locations by any diagnostic modalities, so that the possibility of the ectopic gallbladder is excluded in this case.
The present case, unlike Frey et al.'s case four , had no previous history suggestive of hepatobiliary diseases and complained of no severe biliary symptoms only for too short a period to suffer from necrosis of the gallbladder. It is also inconceivable, even if an intense inflammatory reaction had occurred in the region of the gallbladder, that the organ and the major part of the cystic duct proximal to it went scleroatrophic, leaving the rest of the duct patent and that the cholecystolith migrated to the choledochus to become a large stone to trigger acute cholangitis and pancreatitis for the first time after a long dormant interval. It is more reasonable to consider the stone to have been formed in the choledochus de novo rather than in the illusory gallbladder. Sufficient ground is, therefore, lacking to suspect the present case to be the scleroatrophic gallbladder.
Frey et al.  proposed the two diagnostic criteria to prove the cases of CAGB: the operative findings confirmed by cholangiography and visualization of the biliary tract by the operative dissection from the duodenum to the bifurcation of the hepatic ducts . Based on the criteria, some authors stressed the need of thorough surgical exploration , exploratory laparotomy with intraoperative cholangiography , or laparoscopy combined US . In the present case, CT and MRCP failed to depict the entopic or ectopic gallbladder and ERC demonstrated no entopic or intrahepatic gallbladder but the dilated extrahepatic bile duct with an incompletely developed blind duct in the mid bile duct, which appeared to be the rudimentary cystic duct. These findings and awareness of the anomaly entity led to the preoperative diagnosis of CAGB with the hypogenetic cystic duct.
A case of CAGB with the absence of the cystic duct was reported to have been diagnosed by ERCP, in which the symptoms
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