Intraoperative Diagnosis of Gallbladder Agenesis

Gallbladder agenesis is a congenital anomaly that often presents with symptoms of biliary colic. Due to the rarity of this condition, it is often difficult to diagnose pre-operatively. Here we present a case of a 33-yo female with a 6-month history of right upper quadrant abdominal pain and associated nausea. With false-positive imaging findings of cholelithiasis on ultrasound examination, an incidental intraoperative diagnosis of gallbladder agenesis was made during a routine elective laparoscopic cholecystectomy. This finding was confirmed with postoperative magnetic resonance cholangiopancreatography. The primary aim in reporting this case is to further promote awareness of this rare condition out of concern for increased risk of iatrogenic operative injury in the setting of a condition where conservative management is recommended.

P-BN16 Gallbladder agenesis in a gentleman presenting with biliary sepsis: a case report and review of literature

Background Gallbladder agenesis (GBA) is a well-recognised, yet rare embryological malformation, that is thought to stem from a failure of the gallbladder and cystic duct to bud from the common bile duct in the 5th week of gestation. The anomaly has an estimated incidence of 10-65 per 100 000 and is often an incidental finding. A quarter of those affected are symptomatic, often presenting in a similar manner to cholecystitis or choledocholithiasis. There is a lack of awareness and guidance on its investigation and management, making GBA a diagnostic challenge, which often leads to unnecessary high-risk surgical exploration. Methods A 64-year-old man presented with right upper quadrant abdominal pain, fevers, jaundice and persistent vomiting. On examination he was confused, visibly jaundiced and septic with deranged liver function tests. An initial ultrasound scan of the abdomen revealed dilated common bile ducts, with no evidence of choledocholithiasis, however, a gallbladder could not be identified. Magnetic resonance cholangiopancreatography confirmed a ‘post-cholecystectomy’ picture, despite no history of abdominal surgery. This led us to the diagnosis of gallbladder agenesis. Results There are no dedicated guidelines regarding the investigation and management of GBA. Efforts have been made to stratify the diagnostic imaging of GBA. Malde et al suggest that if the gallbladder is not visualised on USS, the next most appropriate investigations in order of accuracy are MRCP, CT and ERCP, respectively. Interestingly, they further suggest that if results of imaging remain inconclusive, they should be repeated again once the acute phase of the illness or symptoms have resolved. Inadvertently, this suggestion was applied in our case, as the patient underwent a repeat MRCP following the resolution of his acute symptoms. In our case, MRCP allowed an effective final diagnosis, avoiding unnecessary investigations and exploratory surgery. It also provided a detailed anatomical picture, excluding the possibility of an ectopic gallbladder. Conclusions The present case accentuates the importance of non-invasive imaging such as MRCP in appropriately diagnosing this phenomenon and avoiding unnecessary operative exploration.

EP.TH.665Where’s the gallbladder? A rare case of gallbladder agenesis with cystic duct stone

Aims Gallbladder agenesis is a rare congenital anatomical malformation with an incidence of 0.007-0.0027%. Although usually asymptomatic, clinically it may present similarly to biliary colic. There may be increased risk of de novo biliary duct stone formation due to biliary dyskinesia, subsequent duct dilatation and biliary stasis. We present a case of incidental gallbladder agenesis identified during intended laparoscopic cholecystectomy. Methods A 68 year-old female with no significant comorbidities presented with a 16 hour history of colicky right upper quadrant pain and vomiting. She had been awaiting cholecystectomy for increasingly symptomatic gallstones, most recently visualised within a contracted gallbladder on ultrasound two months prior. Recurrently obstructive LFT’s warranted urgent magnetic resonance cholangiopancreatography (MRCP), which identified a poorly-visualised contracted gallbladder and a 10mm common bile duct without filling defect. At index laparoscopic cholecystectomy, a very small thick-walled structure resembling the gallbladder was identified and safely excised in retrograde; a stone was seen within. Results Macroscopic examination revealed a 27 x 9 x 11mm piece of dark brown tissue containing soft orange material. Microscopic histological examination confirmed this to be cystic duct focally lined by mucinous epithelium with chronic inflammation. Findings were consistent with gallbladder agenesis. Conclusions Although rare, it is important for surgeons to maintain an index of suspicion for gallbladder agenesis, particularly if repeated imaging demonstrates a poorly-visualised or ‘contracted’ gallbladder despite adequate fasting. Furthermore, as in this case, the presence of ‘gallbladder’ stones radiologically does not preclude gallbladder agenesis. Anticipation of this malformation may aid interpretation of biliary anatomy, if incidentally found intra-operatively.

Hepatic encephalopathy in adult dog secondary to cirrhosis due to congenital biliary agenesis: a case report

Gallbladder agenesis is a congenital malformation that is considered extremely rare in dogs. The disease can course asymptomatically or with clinical signs, usually non-specific and including vomiting, anorexia, diarrhea, ascites, and lethargy. The objective of this report was to describe the clinical and anatomopathological aspects of a dog with hepatic encephalopathy secondary to gallbladder agenesis. This condition can be diagnosed during surgery or imaging examinations; however, it is often an incidental finding. In the biochemical examinations, a decrease in alanine aminotransferase and an increase in alkaline phosphatase and hypoalbuminemia were observed. During the necropsy, hepatomegaly was observed with absence of the gallbladder, congestion, cerebral edema, lipiduria, and pulmonary edema. Microscopically, there was intense fibrosis and inflammation in the liver due to chronic cholangiohepatitis (cirrhosis of the liver). The consequence of this lesion secondary to gallbladder agenesis was hepatic encephalopathy. Chronic liver failure exposes the cerebral cortex to toxins that are not metabolized by the liver, such as ammonia, mercaptans, short-chain fatty acids, scatols, indols, and aromatic amino acids. These toxins cause reversible damage to the brain, which results in neurological disorders. In this report, the dog had no clinical neurological signs, and the diagnosis of this condition was observed histologically. Dogs with gallbladder agenesis usually have clinical and pathological findings of hepatobiliary lesions such as cholestasis, cholangiohepatitis, and, in severe cases, hepatic encephalopathy, which are necessary to differentiate from other diseases that affect the hepatobiliary system, such as cholelithiasis, neoplasms, and chronic hepatitis.

Congenital gallbladder agenesis in a 9-month-old Bull Terrier

Congenital gallbladder agenesis is an extremely rare disorder, which has, to the best of our knowledge, only been reported in seventeen dogs (mainly in Japan). In almost all of these cases, gallbladder agenesis or hypoplasia was detected in small dogs. In this report, we present a case of gallbladder agenesis in a 9-month-old intact female Bull Terrier. The clinical signs included diarrhoea, sporadic vomiting, apathy and decreased appetite. The serum biochemistry revealed an increased liver enzyme activity, an increased concentration of serum bile acids and mild hyperbilirubinaemia. A diagnostic laparotomy demonstrated the lack of a gallbladder and dilation of the common bile duct, which was misinterpreted as the gallbladder in the ultrasonographic examination. The histological examination of the liver revealed degenerative changes in the hepatocytes with glycogen accumulation and some necrotic hepatocytes. The therapy included a low protein diet, fluids, silymarin and ursodeoxycholic acid. After nine weeks of therapy, the dog was in good condition, the diarrhoea and vomiting ceased, and the liver function parameters, such as the AST and GLDH activities, and the concentration of bile acids had decreased to reference intervals.

Gallbladder agenesis diagnosed intraoperatively complicated by common hepatic duct injury

Gallbladder agenesis is a rare congenital anomaly among biliary abnormalities, and its preoperative diagnosis has been considered very difficult. Gallbladder agenesis becomes symptomatic in almost 23% of cases mostly with symptoms mimicking biliary colic, which is poorly understood. Initial workup for suspected gallbladder diseases, such as abdominal ultrasound, can be misleading or inconclusive. Often it is misinterpreted as other diseases leading to unnecessary surgery. We reported a case of a 31-year-old Bahraini male presenting with recurrent symptoms, suggesting biliary colic. Abdominal ultrasound showed False-positive contracted gallbladder. The patient was then taken to the operative theater for laparoscopic cholecystectomy. Intraoperatively, the gallbladder could not be seen, and eventually the decision was made to converted to open surgery which was complicated by common hepatic duct injury. Gallbladder agenesis was confirmed postoperatively by magnetic resonance cholangiopancreatography (MRCP).