Uncommon Presentation of Xanthogranulomatous Cholecystitis in a True Gallbladder Diverticulum: A Case Report and Literature Review.

BACKGROUND The gallbladder develops from the hepatic diverticulum during the fourth week of gestation, which also give rise to the liver, extrahepatic biliary ducts, and ventral part of the pancreas. Infrequently, the gallbladder has malformation or disruption in embryogenesis, leading to congenital anomalies. There are various congenital anomalies that can arise in the gallbladder. True or congenital diverticulum of the gallbladder is a rare entity that accounts for only 0.06% of gallbladder congenital anomalies and 0.0008% of cholecystectomies at the Mayo Clinic. CASE REPORT Herein, we report a rare case of a 38-year-old woman who presented to Jubail General Hospital's surgery clinic with right upper-quadrant (RUQ) pain associated with vomiting after meals for 1 month. Laparoscopic cholecystectomy was done and gallbladder tissue was sent to histopathology. Gross examination revealed an outpouching mucosa within the wall that was proven to consist of muscularis and serosa layers under light microscope. Interestingly, xanthogranulomatous inflammation was confined to the diverticulum, unlike the chronic inflammation involving the remaining gallbladder. Based on the above findings, the diagnosis of congenital diverticulum with xanthogranulomatous cholecystitis was made. CONCLUSIONS Gallbladders associated with a true diverticulum are uncommonly found to be buried in the liver, leading to surgical difficulties during cholecystectomy. Therefore, background knowledge of occasional anomalies plays a crucial role in guiding the surgeon to choose the optimal method of management. We also discuss the associated complications that accompany these anomalies, such as non-specific prolonged ailments, acalculous cholecystitis, cholecystitis and cholelithiasis, recurrent cholangitis, and carcinoma of the gallbladder.

Perforated duodenal ulcer in a dog with gallbladder agenesis.

Background: Duodenal ulceration (DU) in dogs derives from different causes but has never previously been related to gallbladder agenesis (GA). GA is a rare congenital disorder in dogs and is considered a predisposing factor for DU in humans. Case Description: A 5-month-old intact female Maltese was presented for acute vomiting and diarrhea. Abdominal ultrasound suggested duodenal perforation and absence of the gallbladder. Exploratory laparotomy was performed to treat the perforation and confirmed GA. Hepatic ductal plate malformation (DPM) was histologically diagnosed in liver biopsy, but no signs of liver dysfunction were detected by blood work at first admission. Two months later, the dog developed signs of portal hypertension and medical treatment was started. However, the clinical condition gradually worsened until liver failure and the dog was euthanized 8 months after surgery. Necropsy confirmed hepatic abnormalities. Conclusion: This report describes a case of DU associated with GA and DPM in a dog. As in humans, GA may represent a hepatobiliary disease predisposing to gastroduodenal ulcerations.

Duplication of gallbladder: a rare anomaly of biliary tract- A case report.

Incomplete duplication of gall bladder or vesica fellea divisa is one of the rare anomalies of gallbladder. Until now, 25 cases have been reported; of which four were proceeded with laparoscopic cholecystectomy. In our case, we diagnosed this nadir anomaly laparoscopically, posing a technical challenge since no radiological clue was observed beforehand. Successful laparoscopic resection of duplicated gall bladders was performed followed by Magnetic Resonance CholangioPancreaticography.

Duplication of the gallbladder in a patient with right colon cancer. A case report and review of the literature review.

AIM: Duplication of the gallbladder is a very rare congenital variant, showing an incidence of about 1 in 4000 in the population, occurring twice as often in women than in men. In the literature only a few cases of prenatal diagnosis are reported. The knowledge of the existence of this anatomical variable is very important in order to avoid complications and iatrogenic damage during interventional and surgical procedures involving the biliary tract or adjacent organs. CASE REPORT: A 79-year-old patient was admitted to our Hospital in May 2021 presenting abdominal pain. During hospitalization, a 5cm adenocarcinoma of the ascending colon was found. During surgery the known accessory gallbladder was found strongly adhering to the proximal transverse colon. The challenging viscerolysis maneuvers caused a lesion on one of the gallbladders, so we decided to proceed with cholecystectomy of both gallbladders. RESULTS AND DISCUSSION: Duplication of the gallbladder is a rare congenital anatomical variant and requires special attention to the biliary and arterial anatomy in order to avoid iatrogenic damage. This variant can complicate surgical treatment for complications that need urgent treatment, such as cholecystitis. Currently the technique of choice for the evaluation of the biliary tree is magnetic resonance cholangiography. Laparoscopic cholecystectomy is the treatment of choice. CONCLUSION: Surgeons should be aware of the different forms of presentation of gallbladder pathologies, also the ones that are not "the standard" of routine. Detailed preoperative study is essential to avoid a missed diagnosis. KEY WORDS: Gallbladder, Mininvasive surgery, Variant, Anatomical.

Anomalies of the fetal gallbladder: pre-and postnatal correlations.

The gallbladder is not part of the standard survey of a fetus during obstetrical ultrasound. Yet, some anomalies can be detected. Most are clinically benign, but some are features of or associated with more serious complications. Biliary atresia constitutes the diagnosis with the worst prognosis. Its in utero diagnosis is difficult. Still, markers do exist and should be searched for. Knowledge of the normal appearances, variants and abnormalities of the gallbladder contributes to proper management. Any suspicion should lead to meticulous postnatal workup. Pre- or postnatal magnetic resonance imaging can provide additional information in select cases.

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.

Duplicated gallbladder: an incidental anatomical variation in a patient with symptomatic cholelithiasis.

Congenital malformations of the biliary tract represent a relatively rare entity with which surgeons, radiologists and clinicians are not adequately familiarized. We present a rare case of gallbladder duplication in a 40-year-old female, with the accessory cystic duct entering the left hepatic duct, which depicts the fifth reported case in the international bibliography. Our case illustrates the importance of detailed knowledge of anatomical malformations of the biliary tree, serving the purpose of a preoperative diagnosis of symptomatic cholelithiasis. It is also of paramount importance to take under consideration biliary tract malformations to avoid inadvertent complications such as biliary duct injuries in case of laparoscopic cholecystectomy.

A Single Gallbladder Fold Creates a Unique Visual and Temporal Pattern of Radiotracer Accumulation on Hepatobiliary Scintigraphy.

ABSTRACT: Duplication of the gallbladder represents a variant of anomalous biliary anatomy that is rarely encountered on hepatobiliary scintigraphy. We describe the case of 76-year-old man with a gallbladder fold mimicking duplication of the gallbladder or an associated choledochal cyst on scintigraphy. Correlative imaging with CT and ultrasound helped elucidate the true anatomy. This case demonstrates the type of difficult scintigraphic patterns that can arise with variant anatomy and the necessary role of cross-sectional, anatomic imaging in unraveling such situations.

Non-visualisation of fetal gallbladder in a Chinese cohort.

INTRODUCTION: Non-visualisation of fetal gallbladder (NVFGB) is associated with chromosomal abnormalities, biliary atresia, cystic fibrosis, and gallbladder agenesis in Caucasian fetuses. We investigated the outcomes of fetuses with NVFGB in a Chinese cohort. METHODS: This retrospective analysis included cases of NVFGB among Chinese pregnant women at five public fetal medicine clinics in Hong Kong from 2012 to 2019. We compared the incidences of subsequent gallbladder visualisation, chromosomal abnormalities, biliary atresia, cystic fibrosis, and gallbladder agenesis between cases of isolated NVFGB and cases of non-isolated NVFGB. RESULTS: Among 19 cases of NVFGB detected at a median gestational age of 21.3 weeks (interquartile range, 20.0-22.3 weeks), 10 (52.6%) were isolated and nine (47.4%) were non-isolated. Eleven (58.0%) cases had transient non-visualisation, four (21.0%) had gallbladder agenesis, three (15.8%) had chromosomal abnormalities (trisomy 18, trisomy 21, and 22q11.2 microduplication), one (5.2%) had biliary atresia, and none had cystic fibrosis. The incidence of serious conditions was significantly higher in the non-isolated group than in the isolated group (44.4% vs 0%; P=0.029); all three cases with chromosomal abnormalities and the only case of biliary atresia were in the non-isolated group, while all four cases with gallbladder agenesis were in the isolated group. The incidences of transient non-visualisation were similar (55.6% vs 60.0%; P=1.000). CONCLUSION: Isolated NVFGB is often transient or related to gallbladder agenesis. While investigations for chromosomal abnormalities and biliary atresia are reasonable in cases of NVFGB, testing for cystic fibrosis may be unnecessary in Chinese fetuses unless the NVFGB is associated with consistent ultrasound features, significant family history, or consanguinity.

Laparoscopic cholecystectomy for left-sided gallbladder.

PURPOSE: Left-sided gallbladder (LSGB) is a rare congenital anomaly in the gallbladder, which is defined as a gallbladder located on the left side of the falciform ligament without situs inversus. We retrospectively analyzed 13 patients diagnosed with LSGB in a single center to confirm the safety of laparoscopic cholecystectomy (LC) and reviewed the anatomical implications in those patients. METHODS: Of the 4910 patients who underwent LC for the treatment of gallbladder disease between August 2007 and December 2019, 13 (0.26%) were diagnosed as having LSGB. We retrospectively analyzed these 13 patients for general characteristics, perioperative outcomes, and other variations through the perioperative imaging workups. RESULTS: All patients underwent LC for gallbladder disease. In all cases, the gallbladder was located on the left side of the falciform ligament. The operation was successfully performed with standard four-trocar technique, confirming "critical view of safety (CVS)" as usual without two cases (15.4%). In one case, which had an intraoperative complication and needed choledochojejunostomy because of common bile duct injury, there was an associated variation with early common bile duct bifurcation. The other patient underwent an open conversion technique because of severe fibrosis in the Calot's triangle. Furthermore, on postoperative computed tomography, abnormal intrahepatic portal venous branching was found in all cases. CONCLUSIONS: Although LSGB is usually encountered by chance during surgery, it can be successfully managed through LC with CVS. However, surgeons who find LSGB have to make efforts to be aware of the high risk of bile duct injury and possibility of associated anomalies.

Complete enclosure of gall bladder inside the lesser omentum - a rare anomaly.

Gall bladder is known for many congenital anomalies such as duplication, intrahepatic position, floating position etc. Most of its anomalies can be detected in ultrasound examination and radiography. We report an extremely rare congenital anomaly of gall bladder as observed in a male cadaver during dissection classes. The gall bladder was totally enclosed in the right free margin of the lesser omentum, in front of the usual contents of the right free margin of lesser omentum. The size, shape and blood supply of the gall bladder were normal. Though this anomaly might not cause any functional disturbances, it might result in complications during laparoscopic cholecystectomy.

Postnatal outcomes of fetuses with isolated gallbladder anomalies: be aware of biliary atresia.

OBJECTIVES: To analyze the normal development of fetal gallbladder (GB) and evaluate the postnatal outcomes of fetuses with isolated GB anomalies without other structural abnormalities by ultrasound. METHODS: This was a retrospective study. We collected normal cases during 2016 between 15 and 40 gestational weeks and measured the length and width of GB to establish a normal reference range of GB. Using the 10th and 90th percentiles of the normal reference range, isolated cases of enlarged or small GB without other structural anomalies were selected between January 2017 and December 2019. Simultaneously, cases of echogenic material in the GB were included. Fetuses with non-visualization of the fetal gallbladder (NVFGB) were collected between January 2010 and December 2019. Postnatal outcomes of these cases were followed up and analyzed. RESULTS: There were 670 cases in the normal group and a linear correlation was found between the dimensions of GB and the gestational age, including the length (r = 0.69; p < .05; y = 0.97 + 0.70 × gestational age) and width (r = 0.48; p < .05; y = 1.65 + 0.12 × gestational age). Sixty-two cases with isolated GB anomalies were collected, including nine cases of enlarged GB, four cases of small GB, three cases of echogenic material in the GB, and 46 cases of NVFGB. Sixty fetuses (96.8%, 60/62) had good outcomes, except that two neonates (4%, 2/50) in small GB and NVFGB groups were both born with BA. In the NVFGB group, 97.8% (45/46) were with good outcomes, in which gallbladders were finally visualized in 37 (80.4%, 37/46) cases and GB agenesis was postnatally diagnosed in eight (17.4%, 8/46) patients by ultrasound. CONCLUSION: The postnatal outcomes of most fetuses (96.8%) with isolated GB anomalies such as enlarged GB, echogenic material in the GB were favorable. When it comes to cases of small GB or NVFGB in prenatal ultrasound diagnosis, we should alert biliary atresia in prenatal counseling.

Multiseptate gallbladder: A case report and literature review.

RATIONALE: Multiseptate gallbladder (MSG) is a rare congenital gallbladder anomaly. Between 1963 and June 2021, only 56 cases were reported. There is currently no treatment guideline for pediatric or adult cases of MSG. PATIENT CONCERNS: A 14-year-old woman visited our out-patient clinic in September 2020 for epigastric pain that last for 6 months. Honeycomb appearance of the gallbladder was noted under ultrasonography. DIAGNOSIS: The patient was diagnosed with MSG. The diagnosis was confirmed through computed tomography and magnetic resonance cholangiopancreatography. INTERVENTIONS: Cholecystectomy was performed. OUTCOMES: Epigastric pain showed limited improvement after the surgery. Since she was diagnosed with gastritis at the same time, a proton-pump inhibitor was prescribed. Epigastric pain was eventually resolved. LESSONS: MSG cases can undergo cholecystectomy and show good recovery without complications. However, concomitant treatment may be required to resolve in the presence of other symptoms such as epigastric pain.

Variación anatómica de la vía biliar tipo F de Blumgart / Anatomical variation of Blumgart´s F type bile duct / Variacão anatômica do ducto biliar do tipo F de Blumgart

Se presenta el caso de una paciente de 40 años portadora de litiasis biliar sintomática que se operó de coordinación por abordaje laparoscópico

Mutations and variants of ONECUT1 in diabetes.

Genes involved in distinct diabetes types suggest shared disease mechanisms. Here we show that One Cut Homeobox 1 (ONECUT1) mutations cause monogenic recessive syndromic diabetes in two unrelated patients, characterized by intrauterine growth retardation, pancreas hypoplasia and gallbladder agenesis/hypoplasia, and early-onset diabetes in heterozygous relatives. Heterozygous carriers of rare coding variants of ONECUT1 define a distinctive subgroup of diabetic patients with early-onset, nonautoimmune diabetes, who respond well to diabetes treatment. In addition, common regulatory ONECUT1 variants are associated with multifactorial type 2 diabetes. Directed differentiation of human pluripotent stem cells revealed that loss of ONECUT1 impairs pancreatic progenitor formation and a subsequent endocrine program. Loss of ONECUT1 altered transcription factor binding and enhancer activity and NKX2.2/NKX6.1 expression in pancreatic progenitor cells. Collectively, we demonstrate that ONECUT1 controls a transcriptional and epigenetic machinery regulating endocrine development, involved in a spectrum of diabetes, encompassing monogenic (recessive and dominant) as well as multifactorial inheritance. Our findings highlight the broad contribution of ONECUT1 in diabetes pathogenesis, marking an important step toward precision diabetes medicine.

Hepatic hilum-type II choledochal cyst masquerading as gallbladder duplication.

Choledochal cyst and gallbladder duplication are rare congenital anomalies. They typically are surgical problems of infancy or childhood but rarely may present in adults also. Despite high resolution imaging, the differentiation of type II choledochal cyst from gallbladder duplication often causes the diagnostic dilemma; which may result in high risk for intraoperative iatrogenic injury. Operative management of choledochal cyst is the definite treatment because of its malignant potential. A type II choledochal cyst arising from the hepatic hilum presenting as gallbladder duplication on imaging has not been reported earlier in the literature and here we present a case report of the same which was managed successfully.