A case series of prenatal hepatic hilar cyst in the presence of a gallbladder - navigating the dilemma between biliary atresia and choledochal cyst.

BACKGROUND: Prenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed "hepatic hilar cysts". CASE PRESENTATION: A hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm. CONCLUSIONS: The spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types.

Morphological changes in the biliary mucosa in pediatric patients with congenital biliary dilatation are more influenced by the duration of amylase exposure than by amylase levels in the gallbladder.

PURPOSE: We investigated the relationship between bile amylase (AMY) levels and biliary epithelial changes in pancreaticobiliary maljunction (PBM), a congenital anomaly characterized by pancreaticobiliary reflux due to duct fusion outside the duodenal wall. METHODS: We enrolled 43 children with congenital biliary dilatation (CBD) of Todani types Ia, Ic, and IVa who underwent surgery at the Hokkaido Medical Center for Child Health and Rehabilitation between November 2007 and June 2023. We defined total AMY exposure in bile as bile AMY levels multiplied by the patient's age (months), representing amount of estimated AMY exposure until surgery. We retrospectively investigated the relationships between bile AMY levels and clinicopathological findings. RESULTS: All patients exhibited hyperplasia in the gallbladder and bile duct epithelium, with dysplasia observed in 13 cases, but no carcinoma. Exposure to bile AMY ≥ 662,400 IU/L × months was an independent risk factor for dysplasia. CONCLUSION: The amount of estimated AMY exposure in bile rather than AMY levels in the bile is an independent risk factor for dysplasia in the biliary mucosa.

A case of H-type double gallbladder anomaly: A case report.

RATIONALE: Biliary system anomalies, such as duplicated gallbladders, are rare congenital conditions that present significant diagnostic challenges. Dr. Boyden's classification system, especially the H-type anomaly, offers vital insight into these variations. Failure to detect these anomalies preoperatively can increase the risk of surgical complications, making early identification crucial for surgical planning. PATIENT CONCERN: A 42-year-old male, asymptomatic, was incidentally found to have a gallbladder mass during routine imaging. An upper abdominal magnetic resonance imaging showed gallbladder wall thickening, gallstones, and a liver lesion. Despite the absence of symptoms, a laparoscopic cholecystectomy revealed an atrophied gallbladder with a cystic duct cyst, which was identified as an H-type double gallbladder anomaly. The surgery was completed without complications, and pathology confirmed the presence of gallstones and inflammation. DIAGNOSES: The patient was diagnosed with a duplicated gallbladder, classified as an H-type anomaly, following laparoscopic cholecystectomy. Preoperative imaging identified gallbladder wall thickening and gallstones, and further investigation during surgery confirmed the congenital anomaly. INTERVENTIONS: The patient underwent laparoscopic cholecystectomy for the removal of the gallbladder, and during the procedure, an H-type double gallbladder anomaly was discovered. The surgery proceeded without incident, ensuring the complete excision of the gallbladders. OUTCOMES: The case highlights the diagnostic difficulty of identifying duplicated gallbladders and the importance of advanced imaging techniques in detecting atypical anatomical variations. The successful laparoscopic removal of both gallbladders illustrates the current capabilities of minimally invasive surgery. Postoperative recovery was uneventful, and the pathology confirmed gallstones and inflammation. LESSONS: This case emphasizes the importance of recognizing biliary anomalies such as duplicated gallbladders to avoid complications during surgery. Preoperative identification, aided by imaging, and careful surgical planning are key to managing these rare conditions. The case contributes to the growing body of knowledge about biliary system anomalies and reinforces the need for comprehensive management strategies to ensure optimal patient outcomes.

A 42-Year-Old Woman with Recurrent Pancreatitis Associated with Gallstones and Phrygian Cap Gallbladder.

BACKGROUND Gallbladder anomalies are rare congenital defects with an incidence rate of approximately 2% in the general population. Phrygian cap gallbladder is a common anatomical variant in which the fundus of the gallbladder folds on itself. Gallstone impaction is rare, and it can be associated with acute pancreatitis. This report describes a 42-year-old woman with recurrent pancreatitis associated with gallstones and Phrygian cap gallbladder. CASE REPORT We report the case of a 42-year-old woman with acute biliary pancreatitis and a history of repeated hospitalizations for episodes of pancreatitis. A preoperative MRI was conducted, which revealed the presence of a Phrygian cap gallbladder that had not been previously reported in imaging studies. The patient underwent cholecystectomy surgery with a laparo-endoscopic approach (rendezvous technique). No intra- or postoperative complications occurred. CONCLUSIONS We report a case of acute biliary pancreatitis caused by stone migration and describe the anatomical variant of the Phrygian cap gallbladder with its clinical implications. The literature contains very few reports of cholecystitis or pancreatitis in patients with a gallbladder anomaly. Continuous reporting of anatomical variations of the gallbladder and biliary tract improves clinical knowledge, and knowledge of gallbladder anomalies is crucial to avoid injury to the biliary tract during laparoscopic cholecystectomy. This case emphasizes the importance of accurate preoperative evaluation to prevent serious surgical complications.

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.

Agenesis of the gallbladder: A multicenter case series and review of the bibliography. / Agenesia de la vesícula biliar: una serie de casos multicéntrica y revisión de la literatura.

Gallbladder agenesis is a rare condition in pediatrics that is usually asymptomatic and represents a diagnostic challenge for physicians seeing these cases for the first time. Some patients may, however, present with symptoms that mimic other diseases of the bile ducts, and many of them undergo surgery due to such suspicion. Still, a timely diagnosis of gallbladder agenesis allows for medical treatment that is often sufficient to resolve the patient's problem. Although it is a benign condition, patients often present with other associated, more serious malformations and should be actively studied for a timely referral to other specialists. Here we describe our experience with the diagnosis and treatment of these patients and a brief review of the bibliography. We hope it will be helpful for physicians facing similar cases.

La agenesia de la vesícula biliar es una entidad rara en pediatría con una evolución normalmente silente, y representa un desafío diagnóstico para el médico que enfrenta estos casos por primera vez. Algunos pacientes pueden, sin embargo, presentar síntomas que simulan otras patologías del árbol biliar, y muchos de ellos son operados ante esta sospecha. Sin embargo, el diagnóstico oportuno de esta entidad permite llevar a cabo un tratamiento médico que muchas veces es suficiente para resolver el problema del paciente. Si bien es una condición benigna, los pacientes suelen presentar otras malformaciones asociadas que son más graves en naturaleza y que deben investigarse activamente para poder derivarlos a los especialistas de manera oportuna. Presentamos nuestra experiencia en el diagnóstico y tratamiento de estos pacientes, así como una breve revisión de la literatura. Esperamos que sea de utilidad para el médico que encuentre un caso similar.

Challenges, Preoperative Assessment, and Surgical Strategies for the Left-Sided Gallbladder.

Left-sided gallbladder positioning, or sinistroposition, is a rare anatomical variation that poses challenges during surgical intervention due to associated vascular and biliary anomalies. While existing literature suggests an incidence of approximately 0.04-1.1%, it remains an underreported phenomenon that falls well outside the realm of "expected" anatomical variation and are rarely identified on preoperative imaging. Here, we present a case of acute cholecystitis in a patient with unexpected left-sided gallbladder, highlighting the associated challenges and outlining both preoperative and intraoperative strategies for managing this rare but consequential anatomical variant. In this case, a 49-year-old woman with a prior history of bilateral ovarian cysts presented with clinical, laboratory, and imaging findings consistent with acute cholecystitis. She underwent laparoscopic cholecystectomy and was found to have a severely inflamed left-sided gallbladder that was obscured by omentum. Her gallbladder was found in the midline immediately beneath the falciform ligament, with most of the gallbladder body and fundus attached to liver segment III, situated to the left of the midline. An additional left-sided mid-abdominal port was required to enhance retraction, and an intraoperative cholangiogram (IOC) was performed given the elevated risk of structural injury. This case underscores the heightened intraoperative risk associated with deviations in vascular and biliary anatomy and provides recommendations for intraoperative adaptations to mitigate these risks.

Acute cholecystitis with double gall bladder diaphragm.

Gall bladder diaphragm is a rare congenital malformation of the gall bladder, entailing its segmentation in several chambers. Functioning as a non-return valve, it interferes with drainage, leading to gallstone formation and cholecystitis. We are reporting a rare case of acute cholecystitis with a double vesicular diaphragm.

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.

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.

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.

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.

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.

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.