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.

No abstract available.
Adult ; Biopsy ; Cholecystitis/diagnostic imaging/*etiology/therapy ; Churg-Strauss Syndrome/*complications/diagnosis/drug therapy ; Diagnosis, Differential ; Female ; Gallstones/diagnostic imaging/*etiology/therapy ; Glucocorticoids/therapeutic use ; Humans ; Lymphadenopathy/diagnostic imaging/*etiology/therapy ; Magnetic Resonance Imaging ; Mediastinum ; Methylprednisolone/therapeutic use ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome

PURPOSE: Several studies have reported that laparoscopic cholecystectomy with percutaneous transhepatic gallbladder drainage (PTGBD) is associated with a reduced duration of surgery and a lower rate of conversion to open laparotomy compared with laparoscopic cholecystectomy without PTGBD and delayed laparoscopic cholecystectomy after conservative therapy. However, these results are contradictory. This retrospective study investigated the safety and usefulness of laparoscopic cholecystectomy combined with pre-operative PTGBD in patients with acute cholecystitis. METHODS: The clinicopathologic data and surgical outcomes of 101 patients who underwent laparoscopic cholecystectomy between January 2010 and September 2015 were reviewed retrospectively. RESULTS: Patients in the PTGBD group vs. the non-PTGBD group were significantly older (mean age: 65.47±12.2 vs. 56.32±13.7; p=0.001). Underlying diseases were also significantly more common in the PTGBD group (75.4% vs. 45.5%; p=0.002). There were no significant differences between the two groups in terms of operative time, blood loss, rate of open conversion, postoperative oral intake, and postoperative hospital stay. Total hospital day was significantly longer in the PTGBD group (11.14±7.22 vs. 6.23±5.17; p=0.049). There was no significant difference in the postoperative complications between the two groups, and all patients in this study lived. CONCLUSION: This study suggested that satisfactory results can be achieved with selective preoperative PTGBD in older and sicker patients with acute cholecystitis.
Cholecystectomy, Laparoscopic* ; Cholecystitis, Acute* ; Drainage* ; Gallbladder* ; Humans ; Laparotomy ; Length of Stay ; Operative Time ; Postoperative Complications ; Retrospective Studies

Cholangitis and cholecystitis are intra-abdominal infections that show poor prognosis upon progression to sepsis and multiorgan failure. Administration of antibiotics with high antimicrobial susceptibility and removal of infected bile at the initial treatment are important. After undergoing ERCP for diagnostic purposes, a 58-year-old man developed acute cholangitis and cholecystitis accompanied by rhabdomyolysis, multi-organ failure, and severe sepsis. Broad-spectrum antibiotics with bedside endoscopic nasobiliary drainage were administered, but clinical symptoms did not improve. Therefore, bedside EUS-guided transgastric gallbladder aspiration and lavage was performed, resulting in successful treatment of the patient. We report the above described case along with a discussion of relevant literature.
Cholangiopancreatography, Endoscopic Retrograde ; Cholecystitis, Acute/complications/*diagnosis/diagnostic imaging ; Drainage ; Duodenoscopy ; Endosonography ; Escherichia coli/isolation & purification ; Humans ; Male ; Middle Aged ; Multiple Organ Failure/pathology ; Rhabdomyolysis/complications/diagnosis ; Sepsis/diagnosis/etiology/microbiology ; Therapeutic Irrigation ; Tomography, X-Ray Computed

PURPOSE: Extremely elderly patients who present with complicated gallstone disease are less likely to undergo definitive treatment. The use of laparoscopic cholecystectomy (LC) in older patients is complicated by comorbid conditions that are concomitant with advanced age and may increase postoperative complications and the frequency of conversion to open surgery. We aimed to evaluate the results of LC in patients (older than 80 years). METHODS: We retrospectively analyzed 302 patients who underwent LC for acute cholecystitis between January 2011 and December 2013. Total patients were divided into three groups: group 1 patients were younger than 65 years, group 2 patients were between 65 and 79 years, and group 3 patients were older than 80 years. Patient characteristics were compared between the different groups. RESULTS: The conversion rate was significantly higher in group 3 compared to that in the other groups. Hematoma in gallbladder fossa and intraoperative bleeding were higher in group 3, the difference was not significant. Wound infection was not different between the three groups. Operating time and postoperative hospital stay were significantly higher in group 3 compared to those in the other groups. There was no reported bile leakage and operative mortality. Preoperative percutaneous transhepatic gallbladder drainage and endoscopic retrograde cholangiopancreatography were performed more frequently in group 3 than in the other groups. CONCLUSION: LC is safe and feasible. It should be the gold-standard approach for extremely elderly patients with acute cholecystitis.
Aged* ; Bile ; Cholangiopancreatography, Endoscopic Retrograde ; Cholecystectomy, Laparoscopic* ; Cholecystitis, Acute ; Conversion to Open Surgery ; Drainage ; Gallbladder ; Gallstones ; Hematoma ; Hemorrhage ; Humans ; Length of Stay ; Mortality ; Postoperative Complications ; Retrospective Studies ; Treatment Outcome ; Wound Infection

Acute cholecystitis is a common cause of right upper quadrant pain in patients presenting at the emergency department. Early diagnosis and recognition of associated complications, though challenging, are essential for timely management. Imaging studies, including ultrasonography, computed tomography and magnetic resonance imaging, are increasingly utilised for the evaluation of suspected cases of cholecystitis. These investigations help in diagnosis, identification of complications and surgical planning. Imaging features of acute cholecystitis have been described in the literature and are variable, depending on the stage of inflammation. This article discusses the spectrum of cholecystitis-associated complications and their imaging manifestations. We also suggest a checklist for the prompt and accurate identification of complications in acute cholecystitis.
Abdominal Pain ; diagnosis ; Adult ; Aged ; Cholecystitis ; complications ; diagnostic imaging ; Cholecystitis, Acute ; complications ; diagnostic imaging ; Diagnosis, Differential ; Emergency Medicine ; methods ; Emergency Service, Hospital ; Female ; Humans ; Inflammation ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed ; Ultrasonography

BACKGROUND/AIMS: Several studies have shown the usefulness of endoscopic nasogallbladder drainage (ENGBD) in patients with acute cholecystitis. However, the procedure is difficult, and factors that affect technical success have not yet been clarified. We conducted a prospective study to evaluate the technical feasibility, efficacy, and predictive factors for the technical success of ENGBD in patients with acute cholecystitis. METHODS: All patients with moderate or severe acute cholecystitis who were enrolled underwent ENGBD between April 2009 and April 2011. Patients with surgically altered anatomy or pancreatobiliary malignancies were excluded. The primary outcomes included technical success, clinical success, and complications. Factors that could affect the technical success were also examined. RESULTS: Of the 27 patients who underwent ENGBD during the study period, technical success was achieved in 21 (78%) and clinical improvement was achieved in 20 (95%). Early complications were encountered in four patients (15%). Gallbladder wall thickness (odds ratio [OR], 1.64; 95% confidence interval [CI], 1.08 to 2.47) and age (OR, 1.16; 95% CI, 1.00 to 1.35) were effective predictors of technical failure. CONCLUSIONS: ENGBD was effective in resolving acute cholecystitis; however, this modality was technically challenging and had a limited success rate. Because of technical difficulties, ENGBD should be reserved for limited indications.
Age Factors ; Aged ; Aged, 80 and over ; Cholecystitis, Acute/*surgery ; Drainage/*methods ; Endoscopy, Gastrointestinal/*methods ; Feasibility Studies ; Female ; Gallbladder/pathology/*surgery ; Humans ; Male ; Middle Aged ; Odds Ratio ; Postoperative Complications/epidemiology ; Prospective Studies ; Sphincterotomy, Endoscopic ; Treatment Outcome

BACKGROUND/AIMS: Several studies have shown the usefulness of endoscopic nasogallbladder drainage (ENGBD) in patients with acute cholecystitis. However, the procedure is difficult, and factors that affect technical success have not yet been clarified. We conducted a prospective study to evaluate the technical feasibility, efficacy, and predictive factors for the technical success of ENGBD in patients with acute cholecystitis. METHODS: All patients with moderate or severe acute cholecystitis who were enrolled underwent ENGBD between April 2009 and April 2011. Patients with surgically altered anatomy or pancreatobiliary malignancies were excluded. The primary outcomes included technical success, clinical success, and complications. Factors that could affect the technical success were also examined. RESULTS: Of the 27 patients who underwent ENGBD during the study period, technical success was achieved in 21 (78%) and clinical improvement was achieved in 20 (95%). Early complications were encountered in four patients (15%). Gallbladder wall thickness (odds ratio [OR], 1.64; 95% confidence interval [CI], 1.08 to 2.47) and age (OR, 1.16; 95% CI, 1.00 to 1.35) were effective predictors of technical failure. CONCLUSIONS: ENGBD was effective in resolving acute cholecystitis; however, this modality was technically challenging and had a limited success rate. Because of technical difficulties, ENGBD should be reserved for limited indications.
Age Factors ; Aged ; Aged, 80 and over ; Cholecystitis, Acute/*surgery ; Drainage/*methods ; Endoscopy, Gastrointestinal/*methods ; Feasibility Studies ; Female ; Gallbladder/pathology/*surgery ; Humans ; Male ; Middle Aged ; Odds Ratio ; Postoperative Complications/epidemiology ; Prospective Studies ; Sphincterotomy, Endoscopic ; Treatment Outcome

BACKGROUND/AIMS: Laparoscopic cholecystectomy is the standard treatment for acute cholecystitis. Percutaneous cholecystostomy is an alternative treatment to resolve acute inflammation in patients with severe comorbidities. The purpose of this study is to determine the optimal timing of laparoscopic cholecystectomy after percutaneous cholecystostomy for the patients with acute cholecystitis. METHODS: This retrospective study was conducted in patients who underwent cholecystectomy after percutaneous cholecystostomy from January 2010 through November 2014. Seventy-four patients were included in this study. The patients were divided into two groups by the operation timing. Group I patients underwent cholecystectomy within 10 days after percutaneous cholecystostomy (n=30) and group II patients underwent cholecystectomy at more than 10 days after percutaneous cholecystostomy (n=44). RESULTS: There was no significant difference between groups in conversion rate to open surgery, operation time, perioperative complications rate, and days of hospital stay after operation. However, complications related to cholecystostomy such as catheter dislodgement occurred significantly more often in group II than group I (group I:group II=0%:18.2%; p=0.013). CONCLUSIONS: Timing of laparoscopic cholecystectomy after percutaneous cholecystostomy did not influence postoperative outcomes. However, late surgery caused more complications related to cholecystostomy than early surgery. Therefore, early laparoscopic cholecystectomy should be considered over late surgery after percutaneous cholecystostomy insertion.
Aged ; Aged, 80 and over ; Cholecystectomy, Laparoscopic/adverse effects ; Cholecystitis, Acute/*diagnosis/surgery ; Cholecystostomy ; Female ; Humans ; Length of Stay ; Male ; Middle Aged ; Postoperative Complications ; Retrospective Studies