Atrophic Gallbladder Research Articles

Gallbladder Agenesis Discovered During Surgery: A Case Report

Agenesis of the gallbladder is a rare congenital anomaly. The aim of this study is to explore, through the case we present, the epidemiological aspects of this anomaly as well as the specificities of diagnosis and therapeutic management. The patient is a 35-year-old individual with no significant medical history, who presented with hepatic colic and an ultrasound showing a sclerotic and atrophic gallbladder. A laparoscopic cholecystectomy was indicated. Intraoperatively, the gallbladder was not visualized, even after examining various ectopic sites for the gallbladder.

Radial nerve palsy caused by a rapidly growing intramuscular hematoma in an infant with biliary atresia: a case report

BackgroundBiliary atresia (BA) is a rare cause of persistent jaundice in infants that can result in vitamin K malabsorption and vitamin K deficiency bleeding (VKDB). We present an infant with BA who developed a rapidly growing intramuscular hematoma in her upper arm after a vaccination which caused a radial nerve palsy.Case presentationAn 82-day-old girl was referred to our hospital because of a rapidly growing left upper arm mass. She had received three doses of oral vitamin K before age 1 month. At age 66 days, she received a pneumococcal vaccination in her left upper arm. On presentation, she showed no left wrist or finger extension. Blood examination revealed direct hyperbilirubinemia, liver dysfunction, and coagulation abnormalities, indicating obstructive jaundice. Magnetic resonance imaging showed a hematoma in the left triceps brachii. Abdominal ultrasonography revealed an atrophic gallbladder and the triangular cord sign anterior to the portal vein bifurcation. BA was confirmed on cholangiography. VKDB resulting from BA in conjunction with vaccination in the left upper arm were considered the cause of the hematoma. The hematoma was considered the cause of her radial nerve palsy. Although she underwent Kasai hepatic portoenterostomy at age 82 days, the obstructive jaundice did not sufficiently improve. She then underwent living-related liver transplantation at age 8 months. The wrist drop was still present at age 1 year despite hematoma resolution.ConclusionsDelayed detection of BA and inadequate prevention of VKDB can result in permanent peripheral neuropathy.

Gallstoneileus: A Rare Cause of Smallbowel Obstruction

Biliary ileus is a rare complication of gallstones secondary to a bilio-digestive fistula with passage of a macro calculus in the small intestine and which may be responsible for intestinal obstruction. Its misleading symptomatology often leads to diagnostic delay which worsens the prognosis. Computed tomography is the main imaging modality for diagnosis of gallstone ileus. We report the case of a 63-year-old patient presenting vomiting with diffuse abdominal pain and inability to pass gas or stool evolving for 3 days. Abdominal computed tomography revealed moderate aerobilia, an atrophic gallbladder with air bubbles associated with small bowel obstruction due to a jejunal calculus. Laparotomy revealed the presence of a cholecystoduodenal fistula, a sclero-atrophic gallbladder with the presence of a stone obstructing the lumen in the jejunum; an enterolithotomy was performed with a cholecystectomy and closure of the fistula path in a single operative step; the postoperative outcome werefavorable.

A Rare Presentation of Subhepatic Caecum and Appendix with Atrophic Gall Bladder in a Cadaver

A case of subhepatic, mobile caecum and appendix was found in a 65-year-old male cadaver. The appendix was attached to the right wall of caecum, in addition, a small and atrophic gall bladder, only 1.5 cm in length, was noted in the fossa for gall bladder of liver, connected to the right colic flexure by a cystocolic peritoneal band. The case was found during the routine dissection for the undergraduate medical students. This kind of finding has not been reported in any previous literature associated with subhepatic caecum. This unusual position of appendix might cause atypical sign and symptoms during episode of acute appendicitis. This might affect the gall bladder, located in close vicinity, leading to inflammation, atrophy and adhesion to the adjacent right colic flexure. This type of case might cause diagnostic and management challenges for the clinicians and complicated disease conditions in the patients.

Intraoperative indocyanine green fluorescence cholangiography can rule out biliary atresia: A preliminary report

The prognosis of BA is known to be poor if definitive surgery is performed too late. Therefore, excluding BA as a diagnosis at an early stage is crucial. Conventional cholangiography requiring cannulation through the gallbladder may be unnecessarily invasive for patients, especially when ruling out BA. Therefore, a less invasive alternative such as indocyanine green (ICG) cholangiography, which does not require cannulation, should be established. In this study, we focused on excluding BA and confirmed the usefulness of intravenous ICG fluorescence cholangiography. To the best of our knowledge, this is the first preliminary study to report the use of intravenous ICG cholangiography for BA exclusion. The study participants were patients who underwent liver biopsy and intraoperative cholangiography after they were suspected to have BA, between 2013 and 2022. ICG fluorescence cholangiography was performed on all patients who provided informed consent. During the study period, 88 patients underwent a laparoscopic liver biopsy and cholangiography. Among them, 65 (74%) were diagnosed with BA and underwent a subsequent laparoscopic Kasai portoenterostomy. BA was ruled out intraoperatively in 23 patients. Of the 23 patients in whom BA was ruled out, 14 underwent ICG cholangiography, 11 had gallbladder (GB) fluorescence, and 9 had both GB and common bile duct (CBD) fluorescence. Conventional cholangiography was very difficult in 2 of 23 cases: in 1 case, cannulation of the atrophic gallbladder was impossible, and cholecystectomy was indicated after multiple attempts; in 1 case, upstream cholangiography was not possible. In both cases, ICG fluorescence cholangiography successfully imaged the CBD and the GB. In conclusion, intravenous ICG fluorescence cholangiography might be a useful and less invasive diagnostic procedure that can rule out BA in infants.

Ultrasonography is useful in differentiating between cystic biliary atresia and choledochal cyst.

With the advent of ultrasonography (US), perinatal diagnosis of biliary cystic malformation (BCM) has become more prevalent. BCM includes cystic biliary atresia (CBA) and choledochal cyst (CC). Both share similar ultrasonographic features and clinical manifestations, and yet the postnatal management is very different. This study was to assess whether CBA can be distinguished from CC by US. We retrospectively studied the clinical data of 98 BCM patients aged less than 130days between January 2013 and November 2015. The patients were divided into the CBA group and the CC group based on intraoperative cholangiography. The ultrasonographic features in each group were then analyzed. Thirty-one children had CBA (type I: 24, type III: 7) and 67 children had CC (all: type I). The cyst volume did not show a significant change in the CBA group while the cyst volume increased postnatally in the CC group. Preoperative cyst volume can be used as a sensitive indicator to distinguish choledochal cyst from cystic biliary atresia. Gallbladder size in the two groups differed significantly (P = 0.034); 23 of 31 patients in the CBA group had atrophic gallbladder, whereas none of the patients in the CC group had atrophic gallbladder (P < 0.001). The triangular cord sign was detected in ten patients in the CBA group and in none of the patients in the CC group (P < 0.001). Serial prenatal and postnatal ultrasonographic studies may help differentiate CBA from CC in the majority of BCM, facilitating prenatal counseling and postnatal management.

Unexpected Gallbladder Cancer during or after Laparoscopic Cholecystectomy: Risk Factors and Experience of Diagnosis and Treatment of 22 Cases

This study aims to provide some experience in diagnosis and treatment of unexpected gallbladder cancer (UGBC) and find the major risk factors. Retrospective data were collected and analyzed on 22 patients who were diagnosed with UGBC during or after laparoscopic cholecystectomy from January 2013 to January 2018 at our hospital. Average age of the patients was (60.2 ± 12.8) years (range, 42-83 years). Among them, there were 6 men and 16 women. Gallbladder stones, atrophic gallbladder, uneven thickened wall of the gallbladder, and choledocholithiasis were found to be the major risk factors. Eight patients (36.4%) were diagnosed intraoperatively. Seven cases (31.8%) were at the T1 stage; of these, three were treated with laparoscopic cholecystectomy; two were converted to cholecystectomy; and two underwent cholecystectomy, lymph node dissection, and liver resection. Eight (36.4%) T2 patients, five (22.7%) T3 patients, and one T4 patient had radical cholecystectomy. Partial cholecystectomy and cholecystotomy were carried out in another T4 patient. T1 patients did not receive chemotherapy or radiotherapy. Eleven had chemotherapy and four received chemoradiotherapy. The follow-up period ranged from six months to five years. The one-year survival rate for T1 to T4 patients was 100 per cent, 75 per cent, 40 per cent, and 0 per cent, respectively. A high index of clinical suspicion of UGBC is needed if one patient suffered from both gallbladder stones and choledocholithiasis with atrophic gallbladder or uneven thickened wall of the gallbladder preoperatively. To avoid more UGBC and reoperation, imaging examinations combined with tumor marker tests and intraoperative histopathologic examination are highly recommended.

Mirizzi syndrome complicated by common hepatic duct fistula and left hepatic atrophy: a case report.

BackgroundMirizzi syndrome is a rare complication of chronic cholecystitis, usually caused by gallstones impacted in the cystic duct or the neck of the gallbladder. Mirizzi syndrome results in compression of the hepatic duct or fistula formation between the gallbladder and common bile duct (or hepatic duct, right hepatic duct, or even mutative right posterior hepatic duct). Clinical features include abdominal pain, fever, and obstructive jaundice. Severe inflammation and adhesion at Calot’s triangle are potentially very dangerous for patients with Mirizzi syndrome undergoing cholecystectomy.Case presentation: We report the case of a 68-year-old Asian woman who presented with abdominal pain and jaundice. She had a medical history of gallstones, but no fever. Magnetic resonance cholangiopancreatography revealed cholecystitis, cholelithiasis, common hepatic duct stones, and ascites. Findings at surgery included a porcelainized, atrophic gallbladder that was full of gallstones, fistula formation between the gallbladder and common hepatic duct, and left hepatic atrophy. The prominent feature was the left hepatic atrophy, but stones were not visible pre-operatively in the left liver by radiologic examination.ConclusionsThis patient exhibited what can be considered a special type II of Mirizzi syndrome with a fistula of the common hepatic duct as well as left hepatic atrophy.

Toll-like receptor 7 agonist induces hypoplasia of the biliary system in a neonatal mouse model.

Viral infections and innate immunity signaling, especially Toll-like receptor 7 (TLR7) have been implicated in the pathogenesis of biliary atresia (BA). Administration of rhesus rotavirus-type A to newborn Balb/c mice produces inflammatory obstruction of bile ducts, which resembles human BA. However, whether activation of TLR7 signaling plays a role in neonatal hepatobiliary injury remains to be investigated. TLR7 agonist, imiquimod (R837), was intraperitoneally administered to Balb/c mice within 24hours of birth and then every other day. Morphological and histological injuries of liver and gallbladder were examined at 2weeks. Hepatic messenger RNA expression of TLR7 signaling was studied. Terminal deoxynucleotidyl transferase 2'-deoxyuridine 5'-triphosphate nick end labeling staining was used to delineate hepatobiliary apoptosis upon TLR7 stimulation. TLR7 agonist, imiquimod, induced hypoplasia of the biliary system of neonatal Balb/c mice both in atrophic gallbladder and in paucity of intrahepatic bile ducts. There was significantly higher hepatic expression of TLR7 and downstream innate immunity-mediated interferon regulatory factor 7, interferon-α, and tumor necrosis factor-α. In addition, terminal deoxynucleotidyl transferase 2'-deoxyuridine 5'-triphosphate nick end labeling-positive cells in the liver were increased after injections of TLR7 agonist. The results demonstrate that TLR7 activation may trigger innate immunity pathways and induce apoptosis and hypoplasia of neonatal biliary trees in Balb/c mice. The novel findings give an implication of pathogenesis of infantile cholestasis, such as BA.

Individualized nomogram improves diagnostic accuracy of stage I-II gallbladder cancer in chronic cholecystitis patients with gallbladder wall thickening

Early diagnosis of gallbladder cancer (GBC) can remarkably improve the prognosis of patients. This study aimed to develop a nomogram for individualized diagnosis of stage I-II GBC in chronic cholecystitis patients with gallbladder wall thickening. The nomogram was developed using logistic regression analyses based on a retrospective cohort consisting of 89 consecutive patients with stage I-II GBC and 1240 patients with gallbladder wall thickening treated at one biliary surgery center in Shanghai between January 2009 and December 2011. The accuracy of the nomogram was validated by discrimination, calibration and a prospective cohort treated at another center between January 2012 and December 2014 (n=928). Factors included in the nomogram were advanced age, hazardous alcohol consumption, long-standing diagnosed gallstones, atrophic gallbladder, gallbladder wall calcification, intraluminal polypoid lesion, higher wall thickness ratio and mucosal line disruption. The nomogram had concordance indices of 0.889 and 0.856 for the two cohorts, respectively. Internal and external calibration curves fitted well. The area under the receiver-operating characteristic curves of the nomogram was higher than that of multidetector row computed tomography in diagnosis of stage I-II GBC (P<0.001). The proposed nomogram improves individualized diagnosis of stage I-II GBC in chronic cholecystitis patients with gallbladder wall thickening, especially for those the imaging features alone do not allow to confirm the diagnosis.

Preoperative Prediction of Laparoscopic Subtotal Cholecystectomy for Severe Cholecystitis

Introduction: Laparoscopic cholecystectomy for cholecystitis with severe inflammation and/or fibrosis in Calot's triangle is associated with bile duct injury and a high conversion rate. However, some studies have reported the usefulness of laparoscopic subtotal cholecystectomy (LSC) in such condition, although laparoscopic experience and skills are demanded. In this study, we displayed our LSC, and developed a predictive score for LSC to avoid risky dissection on Calot's and conversion to open. Methods: Our LSC consists of transection at the gallbladder neck followed by closure of the remnant gallbladder stump, without dissection of Calot's. Between January 2004 and December 2013, 110 patients underwent LSC due to severe inflammation or fibrosis in Calot's triangle, and 1466 underwent conventional laparoscopic cholecystectomy (LC). Their clinical records were analyzed for their effect on the ratio of LSC to LC (LSC rates) by multivariate analysis, and a constant and coefficients for these independent variables were calculated and formed the predictive score. Results: All of 110 LSC were completed without conversion to open procedure. Postoperative complications were recorded in 11 of 110 patients (10.0%), of which all were treated conservatively. Bile duct injury and mortality were not observed in LSC series. Among the clinical characteristics of all patients, significant association with LSC rates were found in 5 variables, that is, preoperative CRP elevation (Symbol5), wall thickened Gallbladder, atrophic Gallbladder, pericholecystic abscess, and structure of extra hepatic bile duct. Mean of the predictive score in LSC was 8.2, and ideal cut-off point for score was 8; specificity and sensitivity toward LSC was 76.0% and 77.0%, respectively.SymbolConclusion: LSC for severe cholecystitis is safe and feasible. An experienced surgeon should operate on the patients who have the predictive score over 8, making a decision to LSC appropriately.

Mirizzi syndrome with an unusual type of biliobiliary fistula-a case report.

Gallstone obstruction of the cystic duct, resulting in chronic cholecystitis and pressure necrosis leads to the formation of biliobiliary fistula (BBF). We herein reported a case of Mirizzi syndrome (MS) with an unusual type of BBF (Corlette type I) that was successfully managed by a staged treatment strategy. The patient was diagnosed with a solitary gallstone, marked atrophy of the gallbladder, and BBF and underwent mucosal incineration of the atrophic gallbladder and simple closure, followed by extirpation of gallbladder. Although an optimal treatment strategy has not yet been established for MS with BBF because of its rarity and anatomical variations in fistulas, the current treatment strategy may be applicable. In conclusion, clinicians need to carefully diagnose and evaluate chronic cholecystitis in MS with BBF and adopt an optimal treatment strategy to avoid the complication associated with this disease.

Fumarase Deficiency Associated With Noninflammatory Biliary Atresia

Fumarase Deficiency Associated With Noninflammatory Biliary Atresia

Laparoscopic cholecystocholangiography for diagnosis of prolonged jaundice in infants, experience of 144 cases

Cholangiography is often crucial for establishing the definitive diagnosis in infants with prolonged jaundice. Here, we describe our protocol of using the two-hole laparoscopic technique and discuss its benefits. 144 consecutive patients with suspected biliary atresia were included in this retrospective study. A 5-mm umbilical port is introduced for a 30 degrees laparoscope. An additional 5-mm trocar was inserted at right subcostal incision. A liver biopsy was performed first if needed. The fundus of gallbladder was then exteriorized through the incision of the working port after pneumoperitoneum was released and a catheter is inserted into the gallbladder for cholangiography. The average duration of operation was 34 min (range 20-55 min). Laparoscopic cholangiography failed in 21 cases (14.6%) where atrophic gallbladder was found and BA was confirmed by subsequent laparotomy. For the remaining 123 cases, biliary atresia were diagnosed in 88 (71.5%), biliary hypoplasia in 14 (11.4%), and cholestasis in 21 (17.1%), respectively. There was no bleeding or any other complications intraoperatively. The technique of laparoscopic cholecystocholangiography is simple, safe and efficient. It can provide an accurate diagnosis. Furthermore, for patients without biliary atresia, unnecessary laparotomy can be avoided.

Laparoscopic Cholecystocholangiography as an Effective Alternative Exploratory Laparotomy for the Differentiation of Biliary Atresia

Exploratory laparotomy with cholecystocholangiography is often performed for the definitive diagnosis of biliary atresia (BA) when radiological and biochemical studies are inconclusive. Laparoscopic cholecystocholangiography (LCC) has recently been introduced as an alternative procedure to avoid unnecessary laparotomy. We discuss the advantages and indications of LCC based on our experience of performing this diagnostic procedure in four infants with prolonged direct hyperbilirubinemia. We performed LCC in four infants by direct percutaneous puncture of the gallbladder by inserting a cholangiocatheter into the gallbladder or cystic duct. The decision to perform LCC was based on ultrasonography and computed tomography findings of an atrophic gallbladder with a patent lumen, although analysis of duodenal juice and hepatobiliary scintigraphy showed no sign of the excretion of bile into the small intestine. In three infants, LCC did not show the entire biliary system, and laparotomy was necessary to confirm the diagnosis of BA. However, in the other patient LCC showed a normal biliary system and BA was excluded. Laparoscopic cholecystocholangiography may be useful for the differential diagnosis of BA in patients with a patent gallbladder when imaging and biochemical findings are inconclusive. Furthermore, unnecessary exploratory laparotomy may be avoided in patients without BA.

A Case of Endoscopically Collected Cholelith with Cholecystogastric Fistula

A 74-year-old man, who had a history of choledocho-lelithiasis with obstructive jaundice treated by endoscopic papillary balloon-dilation method, admitted to our hospital, because of treatment of diabetes mellitus. Abdominal CT showed cholelithiasis and wall thickening of the gallbladder. We suspected gallbladder cancer, and performed endoscopic retrograde cholangiography (ERC) . When we inserted endoscope, cholelith was found in the antrum of the stomach, and ERC visualized common bile duct (CBD) , atrophic gallbladder and also the stomach. We found fistula on the greater curvature of the antrum, and injected contrast agents under balloon-occlusion, therefore atrophic gallbladder and CBD was seen, and made confirmation as cholecystogastric fistula. Then we collected cholelith endoscopically. Surgical operation was performed with a diagnosis of chronic cholecystitis. Cholecystogastric fistula is very rare among the spontaneous internal biliary fistulae. It is difficult to make a preoperative diagnosis of cholecystogastric fistula, but in our case, we timely performed an examination of ERC, therefore we found cholelith and fistula in the stomach, and were able to confirm as cholecystogastric fistula.

A CASE OF CONGENITAL ABSENCE OF THE GALLBLADDER ASSOCIATED WITH CHOLEDOCHOLITHIASIS

We encountered a case of congenital absence of the gallbladder associated with choledocholithiasis. A 70-year-old man was seen at another hospital because of jaundice and right hypochondralgia. Abdominal ultrasonography revealed an obstructive jaundice due to choledocolithiasis. He was referred to the hospital. On admission we inserted PTCD to reduce jaundice. But the gallbladder and cystic duct were not visualized by ultrasonography, computed tomography and cholangiography. Laboratory data on admission showed a high value of serum CA19-9 (68, 920U/ml). After improvement of jaundice, serum CA19-9 value was down (357U/ml) and there was no malignant cell in the bile. Laparotomy was performed under a diagnosis of severe atrophic gallbladder with choledocolithiasis or a congenital absence of the gallbladder. The dilated common bile duct and a stone in the duct were found. But the gallbladder and cystic duct were absent. Therefore this case was suspected as congenital absence of the gallbladder. A choledocholithotomy was carried out. Congenital absence of the gallbladder is a rate entiry and there have been only 108 cases including ours in Japan. A possible diagnosis of congenital absence of the gallbladder must be also considered in patients with a nonvisualized gallbladder by some examinations.

The internal biliary fistula--reappraisal of incidence, type, diagnosis and management of 33 consecutive cases.

To reevaluate the current features of spontaneous internal biliary fistulas, we reviewed 1,929 consecutive patients who had been treated for biliary tract diseases during the recent 12-year period. Thirty-three patients had internal biliary fistulas and the incidence was 1.9%. Of 33 patients, 20 were women and 13 were men with the average age 63 years, and their mean duration of illness was 4 years. A total of 37 fistulas were found and the most common type was choledochoduodenal (62%), followed by cholecystoduodenal (19%), cholecystocholedochal (11%) and cholecystocolonic (8%) fistulas. Internal biliary fistulas of thirty-one patients were caused by biliary stones and those of two patients by malignant tumors. All of the 17 bile samples examined were bacteria positive and the majority of calculi were brown pigment stones. All of the choledochoduodenal fistulas were correctly diagnosed by endoscopic retrograde cholangiography. In 14 patients with cholecystoenteric or cholecystocholedochal fistulas, direct evidence of the internal fistula was obtained only in 7 patients (50%) preoperatively. Pneumobilia, a small atrophic gallbladder adherent to the neighboring organs and a history of spontaneous disappearance of jaundice in elderly patients may indicate the presence of a cholecystoentric fistula. Since the preoperative diagnostic rate for internal biliary fistula involving the gallbladder is still low, care is necessary before and at the time of surgery especially during laparoscopic cholecystectomy for elderly patients with cholelithiasis.