Crigler-Najjar syndrome (CNS) and Gilbert syndrome (GS; OMIM: 143500) are rare autosomal recessive diseases that cause unconjugated hyperbilirubinemia due to decreased UGT1A1 enzyme activity. Crigler-Najjar syndrome type 2 (CNS2; OMIM: 606785) increases the risk of gallbladder stone formation and cholecystitis, while GS seldom causes health issues. We found a 28-year-old male patient with recurring right upper abdomen pain who experienced persistent jaundice from birth. CNS2 with gallbladder stones and cholecystitis was diagnosed after genetic testing revealed rare double homozygous mutations A(TA)₇TAA (rs3064744) and P229Q (rs35350960) in the UGT1A1 gene. After pedigree investigation, we found that the patient's parents with modestly increased bilirubin had compound heterozygous mutations A(TA)₇TAA and P229Q, which were GS. Bioinformatics analysis showed that A(TA)₇TAA is in the TATA-box region of the gene UGT1A1 promoter, affecting gene transcriptional initiation, whereas P229Q modifies protein three-dimensional structure and may be harmful. In this pedigree, double homozygous mutations have a more severe phenotype than compound heterozygous mutations. Inherited causes of hyperbilirubinemia should be suspected after ruling out biliary obstruction, and early bilirubin reduction (< 103 µmol/L (6 mg/dL)) may reduce the risk of complications like cholecystitis in CNS2 patients, though further studies with longer follow-up are needed to confirm this observation.
Humans ; Male ; Glucuronosyltransferase/genetics* ; Adult ; Crigler-Najjar Syndrome/complications* ; Cholecystitis/etiology* ; Homozygote ; Mutation ; Pedigree

BACKGROUND

Gastric outlet obstruction (GOO) results from intrinsic and extrinsic obstruction of the pyloric channel or the duodenum. Here we present a rare case of GOO attributed to dense adhesions between the gallbladder and duodenum secondary to chronic cholecystitis with choledococystoduodenal fistula formation. Previous reports identified elderly females with comorbidities as a predisposing factor; however, our patient was an immunocompetent adult male.

A 43-year-old male with no comorbidities consulted for recurrent epigastric pain, vomiting and weight loss. On contrast enhanced abdominal CT scan, a lamellated cholelithiasis with pneumobilia and an irregular thickening at the proximal duodenum with subsequent GOO was identified. A choledococystoduodenal fistula was considered. Exploratory laparotomy revealed extensive fibrosis and cholecystitis with dense adhesions to surrounding structures. Dissection revealed a gallstone impacted in and adherent to the wall of the gallbladder and a fistula opening into the duodenum. However, there was no definite evidence of impacted gallstone in the duodenum. The dense adhesions secondary to chronic cholecystitis caused duodenal narrowing and subsequent GOO. He eventually underwent antrectomy, pancreatic sparing, total duodenectomy, cholecystectomy, with loop gastrojejunostomy, cholecystojejunostomy and pancreaticojejunostomy. Biopsy specimens taken were negative for malignancy. He was discharged subsequently. However, he was readmitted after five months due to acute abdomen secondary to small bowel rupture, likely from a marginal ulcer.

This case highlights that preoperative and intraoperative differential diagnosis of GOO is a challenge. Chronic calculous cholecystitis through severe inflammation can present as a rare cause of GOO. Optimal treasaFtment plan should take into consideration the underlying etiology of the GOO.

Gangrenous cholecystitis is a kind of acute cholecystitis, whose course of disease progresses rapidly, early diagnosis is difficult and mortality is high, and clinicians are prone to misdiagnosis and missed diagnosis in clinical work.However, gangrenous cholecystitis has been ignored in various guidelines.This paper systematically summarized the pathogenesis, pathological manifestations, epidemiology, clinical diagnosis and treatment of gangrenous cholecystitis, hoping to provide a complete and clear diagnosis and treatment process for clinicians.
Cholecystectomy ; Cholecystitis/surgery* ; Cholecystitis, Acute/surgery* ; Gangrene/surgery* ; Humans

Endobiliary radiofrequency ablation (RFA) is a procedure performed widely to induce locoregional tumor control by the transfer of thermal energy to the lesion and subsequent tumor necrosis. A 72-year-old male with a prior history of acute calculous cholangitis and perforated cholecystitis was admitted to the Kyungpook National University Hospital complaining of fever and nausea. He had an indwelling percutaneous transhepatic gallbladder drainage (PTGBD) catheter from the previous episode of perforated cholecystitis. An abdominal CT scan showed marked dilation of both the intrahepatic and extrahepatic bile ducts. Common bile duct cancer was confirmed histologically after an endobiliary biopsy. A surgical resection was considered to be the initial treatment option. During open surgery, multiple metastatic nodules were present in the small bowel mesentery and anterior abdominal wall. Resection of the tumor was not feasible, so endobiliary RFA was performed prior to biliary stenting. Cholecystectomy was required for the removal of the PTGBD catheter, but the surgical procedure could not be performed due to a cystic ductal invasion of the tumor. Instead, chemical ablation of the gallbladder (GB) with pure ethanol was performed to breakdown the GB mucosa. Palliative treatment for a biliary obstruction was achieved successfully using these procedures. In addition, a PTGBD catheter was removed successfully without significant side effects. As a result, an improvement in the patient's quality of life was accomplished.
Abdominal Wall ; Aged ; Bile Ducts, Extrahepatic ; Biopsy ; Catheter Ablation ; Catheters ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Cholecystectomy ; Cholecystitis ; Common Bile Duct ; Cystic Duct ; Drainage ; Ethanol ; Fever ; Gallbladder ; Gyeongsangbuk-do ; Humans ; Male ; Mesentery ; Mucous Membrane ; Nausea ; Necrosis ; Palliative Care ; Quality of Life ; Stents ; Tomography, X-Ray Computed

A 64-year-old man was treated with sunitinib as a first-line therapy for metastatic renal cell carcinoma. He was given oral sunitinib in cycles of 50 mg once daily for 2 weeks followed by a week off. During the 5th week of treatment right upper quadrant pain developed, but this resolved spontaneously during the 6th week (off treatment). However, on the 8th week of treatment, he was admitted to hospital because the acute right upper quadrant pain recurred with nausea, vomiting, and fever. Acute acalculous cholecystitis was then diagnosed by ultrasonography and CT. In addition, his laboratory findings indicated disseminated intravascular coagulation. Accordingly, sunitinib therapy was discontinued and broad-spectrum antibiotics initiated. He subsequently recovered after emergent percutaneous cholecystostomy. His Naranjo Adverse Drug Reaction Probability Scale score was 7, indicaing a probable association of the event with sunitinib. Suspicion of sunitinib-related acute cholecystitis is required, because, although uncommon, it can be life-threatening.
Acalculous Cholecystitis ; Anti-Bacterial Agents ; Carcinoma, Renal Cell ; Cholecystitis, Acute ; Cholecystostomy ; Disseminated Intravascular Coagulation ; Drug-Related Side Effects and Adverse Reactions ; Fever ; Humans ; Middle Aged ; Nausea ; Ultrasonography ; Vomiting

BACKGROUND/AIMS: Gallstone disease (GSD) is a common gastrointestinal disorder. Clinical epidemiological studies revealed that alcohol consumption has a preventive effect on the development of GSD. This study aimed to evaluate the relative risks of drinking for GSD development and investigate the dose-response relationships. METHODS: A systematic search of the MEDLINE, EMBASE, and Cochrane Library databases for studies published up to 2018 was performed. All studies that satisfied the following eligibility criteria were included: patients with GSD with or without cholecystitis; and cohort or case-control studies investigating the association between alcohol consumption and GSD development. RESULTS: Sixteen case-control studies including 24,401 gallstone cases and 76,185 controls, and eight cohort studies with 14,693 GSD cases among 2,432,471 person-years were enrolled. Alcohol consumption presented a decreased overall risk of GSD (pooled relative ratio [RR], 0.84; 95% confidence interval [CI], 0.79 to 0.89; p=0.02). Subgroup analyses according to drinking levels indicated a gradual risk reduction for GSD compared to nondrinkers (light: RR, 0.96; 95% CI, 0.94 to 0.99; p=0.75; moderate: RR, 0.80; 95% CI, 0.75 to 0.85; p=0.27; high: RR, 0.66; 95% CI, 0.56 to 0.79; p < 0.01). A nonlinear risk reduction was observed in a dose-response meta-analysis of all the studies (n=14, p < 0.01 for nonlinearity). CONCLUSIONS: In this systematic review with meta-analysis, alcohol consumption could decrease the risk of GSD, and the dose-response analysis revealed a dose-dependent linear risk reduction and a weakened linear trend between alcohol consumption levels less than and greater than 28 g/day.
Alcohol Drinking* ; Case-Control Studies* ; Cholecystitis ; Cohort Studies* ; Drinking ; Epidemiologic Studies ; Gallstones* ; Humans ; Risk Reduction Behavior

PURPOSE: The aim of this study was to evaluate whether the comet tail artifact on ultrasonography can be used to reliably diagnose benign gallbladder diseases. METHODS: This retrospective study reviewed the clinical findings, imaging findings, preoperative ultrasonographic diagnoses, and pathological diagnoses of 150 patients with comet tail artifacts who underwent laparoscopic cholecystectomy with pathologic confirmation. The extent of the involved lesion was classified as localized or diffuse, depending on the degree of involvement and the anatomical section of the gallbladder that was involved. This study evaluated the differences in clinical and imaging findings among pathologic diagnoses. RESULTS: All gallbladder lesions exhibiting the comet tail artifact on ultrasound examination were confirmed as benign gallbladder diseases after cholecystectomy, including 71 cases of adenomyomatosis (47.3%), 74 cases of chronic cholecystitis (49.3%), two cases of xanthogranulomatous cholecystitis (1.3%), and three cases of cholesterolosis (2.0%); there were two cases of coexistent chronic cholecystitis and low-grade dysplasia. There were no statistically significant differences in any of the clinical and ultrasonographic findings, with the exception of gallstones (P=0.007), among the four diseases. There were no significant differences in the average length, thickness, or number of comet tail artifacts among the four diagnoses. No malignancies were detected in any of the 150 thickened gallbladder lesions. CONCLUSION: The ultrasonographic finding of the comet tail artifact in patients with thickened gallbladder lesions is associated with the presence of benign gallbladder diseases, and can be considered a reliable sign of benign gallbladder disease.
Artifacts ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Diagnosis ; Gallbladder Diseases ; Gallbladder ; Gallstones ; Humans ; Retrospective Studies ; Tail ; Ultrasonography

The da Vinci surgical system released its new pure single-port platform, the da Vinci SP, offering improvements and refinements for established robotic single-site procedures. Herein, we present the first case of robotic single-site cholecystectomy using the da Vinci SP system (RSPC) demonstrating its safety and technical feasibility. A 59-year-old female with chronic calculus cholecystitis was admitted for elective RSPC. Docking time took 6 minutes. The patient underwent successful RSPC with a total operation time of 89 minutes. There was no significant intraoperative event. The patient had unremarkable postoperative course. Multijoint instruments, simple docking process, and third-arm functionality are among the RSPC's advantages. Absence of the port for an assistant surgeon can be a hindrance in performing more complicated surgeries. The present case suggests that RSPC is safe and feasible. The promising features and potential application of da Vinci SP in hepatobiliary and pancreas surgery need further study.
Calculi ; Cholecystectomy ; Cholecystitis ; Female ; Humans ; Middle Aged ; Pancreas

PURPOSE: The aims of this case series study were to review the 10 patients who were diagnosed with left-sided gallbladder and analyze their anatomic variations in the bile duct, portal vein, and hepatic vessels. METHODS: In this case series study, 10 patients with left-sided gallbladder were retrospectively analyzed at 2 tertiary referral centers between April 2004 and May 2019. RESULTS: Mean age was 61.1 years; there were 7 women and 3 men. Ten patients underwent laparoscopic cholecystectomy for acute cholecystitis or symptomatic gallbladder stone. The mean operation time was 77.2 minutes. Three ports were used in laparoscopic cholecystectomy procedures. The mean postoperative hospital stay was 3.5 days, and there were no cases of surgery-related morbidity. Two patients had type 1 bile duct and 3 had type 3 bile duct (2 type 3B and 1 type 3A). The right posterior portal vein as the first branch of the main portal vein was observed in all patients. Segment IV branches of the left portal vein crossing over to the segment VIII territory were observed in 7 of the 10 patients. CONCLUSION: Although left-sided gallbladder is a very rare disease, it is possible to diagnose it preoperatively and perform laparoscopic cholecystectomy safely by adjusting port position. The common important features of left-sided gallbladder include distribution of the left portal vein crossing over to the right side of the liver and increased size of the left portal vein. These variations may have important clinical implications in the management of hepatic resection including donor hepatectomy.
Anatomic Variation ; Bile Ducts ; Cholecystectomy, Laparoscopic ; Cholecystitis, Acute ; Crossing Over, Genetic ; Female ; Gallbladder ; Hepatectomy ; Humans ; Length of Stay ; Liver ; Male ; Portal Vein ; Rare Diseases ; Retrospective Studies ; Tertiary Care Centers ; Tissue Donors

BACKGROUND/AIMS: It is often difficult to manage acute cholecystitis after metal stent (MS) placement in unresectable malignant biliary strictures. The aim of this study was to evaluate the feasibility of endoscopic ultrasonography-guided gallbladder drainage (EUS-GBD) for acute cholecystitis. METHODS: The clinical outcomes of 10 patients who underwent EUS-GBD for acute cholecystitis after MS placement between January 2011 and August 2018 were retrospectively evaluated. The procedural outcomes of percutaneous transhepatic gallbladder drainage (PTGBD) with tube placement (n=11 cases) and aspiration (PTGBA) (n=27 cases) during the study period were evaluated as a reference. RESULTS: The technical success and clinical effectiveness rates of EUS-GBD were 90% (9/10) and 89% (8/9), respectively. Severe bile leakage that required surgical treatment occurred in one case. Acute cholecystitis recurred after stent dislocation in 38% (3/8) of the cases. Both PTGBD and PTGBA were technically successful in all cases without severe adverse events and clinically effective in 91% and 63% of the cases, respectively. CONCLUSIONS: EUS-GBD after MS placement was a feasible option for treating acute cholecystitis. However, it was a rescue technique following the established percutaneous intervention in the current setting because of the immature technical methodology, including dedicated devices, which need further development.
Bile ; Cholecystitis, Acute ; Constriction, Pathologic ; Dislocations ; Drainage ; Gallbladder ; Humans ; Retrospective Studies ; Stents ; Treatment Outcome