Systematic Cholecystectomy During Cytoreductive Surgery Plus HIPEC: A Critical Analysis of an Empirical Tradition.

BACKGROUND: Some procedures performed during cytoreductive surgery (CRS) and hyperthermic intraoperative intraperitoneal chemotherapy (HIPEC) are based on empirical data. One of these procedures is systematic cholecystectomy. This study aimed to perform a critical analysis of the need for systematic cholecystectomy during CRS+HIPEC of patients with peritoneal carcinomatosis using long-term follow-up data. METHODS: Patients with peritoneal surface malignancies who were candidates for CRS+HIPEC and underwent surgery between January 2008 and December 2022 were analyzed. For patients with gallbladder involvement due to the disease or for patients whose preoperative study showed the presence of cholelithiasis, cholecystectomy was performed as part of the surgery, which was avoided for the remaining patients. All postoperative adverse events that occurred in the first 90 days were recorded, and clinical records focused on the development of biliary pathology during the follow-up period were studied. RESULTS: The results from a consecutive series of 443 patients with peritoneal surface malignancies who underwent surgery between January 2008 and December 2022 were analyzed. The average age of the cohort was 50 years. The median follow-up period for the cohort was 41 months (range, 12-180 months), with a disease-free survival of 17 months. For 373 of the patients, CRS+HIPEC was completed without an associated cholecystectomy, and in 16 of them, the appearance of cholelithiasis was detected during the follow-up period. Only two patients in the series showed complications derived from gallstones and required a delayed cholecystectomy. CONCLUSIONS: Although cholecystectomy is a safe procedure in the context of CRS+HIPEC, it is not risk free, and its routine performance may be unnecessary.

Cáncer incidental de vesícula biliar tras Colecistectomía Laparoscópica / Incidental gallbladder cancer after Laparoscopy Cholecystectomy

El carcinoma de vesícula biliar es una entidad poco frecuente. El diagnóstico precoz de esta neoplasia es difícil, ya que sus síntomas son muy inespecíficos y muchas veces estes se realiza de manera tardía cuando el enfermo posee una enfermedad avanzada y solo para mitigar los síntomas. Con el crecimiento exponencial en el número de colecistectomías laparoscópicas en las últimas décadas, se ha generado un aumento en la detección de neoplasias incidentales, permitiendo ofrecer tratamiento curativo en un gran grupo de pacientes. Se evaluaron todas las colecistectomías realizadas durante julio de 2019 a diciembre de 2022 en el Hospital Nacional de Clínicas, Córdoba, Argentina. La evaluación patológica de todas las muestras quirúrgicas reveló una incidencia de 0,83% de adenocarcinoma insospechado en colecistectomías realizadas. 66% de los pacientes con neoplasias insospechadas pertenecían al sexo femenino

Gallbladder carcinoma is a rare entity. Early diagnosis of this neoplasia is difficult, since its symptoms are very unspecific and often this is done late when the patient has an advanced disease and only to mitigate symptoms. With the exponential growth in the number of laparoscopic cholecystectomies in recent decades, there has been an increase in the detection of incidental neoplasms, allowing offering curative treatment in a large group of patients. All cholecystectomies performed during July 2019 to December 2022 were evaluated at the National Hospital of Clinics, Córdoba, Argentina. Pathological evaluation of all surgical samples revealed an incidence of 0.83% of unsuspected adenocarcinoma in cholecystectomies performed. 66% of patients with unsuspected neoplasms were female

Cholelithiasis and cholecystitis in children and adolescents: Does this increasing diagnosis require a common guideline for pediatricians and pediatric surgeons?

BACKGROUND: In contrast to adults, for whom guidelines on the cholelithiasis treatment exist, there is no consistent treatment of pediatric patients with cholelithiasis throughout national and international departments, most probably due to the lack of evidence-based studies. METHODS: We evaluated the German management of pediatric cholelithiasis in a dual approach. Firstly, a retrospective, inter-divisional study was established, comparing diagnostics and therapy of patients of the pediatric surgery department with the management of patients aged < 25 years of the visceral surgery department in our institution over the past ten years. Secondarily, a nation-wide online survey was implemented through the German Society of Pediatric Surgery. RESULTS: Management of pediatric patients with cholelithiasis was primarily performed by pediatricians in the retrospective analysis (p < 0.001). Pediatric complicated cholelithiasis was not managed acutely in the majority of cases with a median time between diagnosis and surgery of 22 days (range 4 days-8 months vs. 3 days in visceral surgery subgroup (range 0 days-10 months), p = 0.003). However, the outcome remained comparable. The hospital's own results triggered a nation-wide survey with a response rate of 38%. Primary pediatric medical management of patients was confirmed by 36 respondents (71%). In case of acute cholecystitis, 22% of participants perform a cholecystectomy within 24 h after diagnosis. Open questions revealed that complicated cholelithiasis is managed individually. CONCLUSIONS: The management of pediatric cholelithiasis differs between various hospitals and between pediatricians and pediatric surgeons. Evidence-based large-scale population studies as well as a common guideline may represent very important tools for treating this increasing diagnosis.

Frail geriatric patients with acute calculous cholecystitis: Operative versus nonoperative management?

INTRODUCTION: Nonoperative management of acute calculous cholecystitis (ACC) in the frail geriatric population is underexplored. The aim of our study was to examine long-term outcomes of frail geriatric patients with ACC treated with cholecystectomy compared with initial nonoperative management. METHODS: We conducted a 2017 analysis of the Nationwide Readmissions Database and included frail geriatric (≥65 years) patients with ACC. Frailty was assessed using the five-factor modified frailty index. Patients were stratified into those undergoing cholecystectomy at index admission (operative management [OP]) versus those managed with nonoperative intervention (nonoperative management [NOP]). The NOP group was further subdivided into those who received antibiotics only and those who received percutaneous drainage. Primary outcomes were procedure-related complications in the OP group and 6-month failure of NOP (readmission with cholecystitis). Secondary outcomes were mortality and overall hospital length of stay. RESULTS: A total of 53,412 geriatric patients with ACC were identified, 51.0% of whom were frail: 16,791 (61.6%) in OP group and 10,472 (38.4%) in NOP group (3,256 had percutaneous drainage, 7,216 received antibiotics only). Patients were comparable in age (76 ± 7 vs. 77 ± 8 years; p = 0.082) and modified frailty index (0.47 vs. 0.48; p = 0.132). Procedure-related complications in the OP group were 9.3%, and 6-month failure of NOP was 18.9%. Median time to failure of NOP management was 36 days (range, 12-78 days). Mortality was higher in the frail NOP group (5.2 vs. 3.2%; p < 0.001). The NOP group had more days of hospitalization (8 [4-15] vs. 5 [3-10]; p < 0.001). Both receiving antibiotics only (odds ratio, 1.6 [1.3-2.0]; p < 0.001) and receiving percutaneous drainage (odds ratio, 1.9 [1.7-2.2]; p < 0.001) were independently associated with increased mortality. CONCLUSION: One in five patients failed NOP and subsequently had complicated hospital stays. Nonoperative management of frail elderly ACC patients may be associated with significant morbidity and mortality. LEVEL OF EVIDENCE: Therapeutic, level IV.

Antibiotic selection based on microbiology and resistance profiles of bile from gallbladder of patients with acute cholecystitis.

With the progression of acute cholecystitis, antimicrobial therapy becomes important for infection control. Current antibiotic recommendations were mostly based on reports of patients with acute cholangitis whose bile specimens were sampled from the biliary tract. However, as most infections of acute cholecystitis are limited to the gallbladder, direct sampling from the site increases the probability of identifying the causative pathogen. We investigated 321 positive bile cultures from 931 patients with acute cholecystitis who underwent laparoscopic cholecystectomy between January 2003 and December 2017. The frequency of enterococci declined (P = 0.041), whereas that of Enterobacteriales (P = 0.005), particularly Escherichia (P = 0.008), increased over time. The incidence of ciprofloxacin-resistant Enterobacteriales showed a significant increasing trend (P = 0.031). Vancomycin-resistant E.faecium, carbapenem-resistant Enterobacteriales, and extended-spectrum beta-lactamase-producing Enterobacteriales were recently observed. In grade I and II acute cholecystitis, there were no significant differences in perioperative outcomes in patients with and without early appropriate antimicrobial therapy. In conclusion, the changing incidence of frequently isolated microorganisms and their antibiotic resistance over time would be considered before selecting antibiotics for the treatment of acute cholecystitis. Surgery might be a crucial component of infection control in grade I and II acute cholecystitis.

Gallbladder Disorders: A Comprehensive Review.

Gallbladder disorders encompass a wide breadth of diseases that vary in severity. We present a comprehensive review of literature for the clinical presentation, pathophysiology, diagnostic evaluation, and management of cholelithiasis-related disease, acute acalculous cholecystitis, functional gallbladder disorder, gallbladder polyps, gallbladder hydrops, porcelain gallbladder, and gallbladder cancer.

Etiology-Based Decision-Making Protocol for Pediatric Cholelithiasis.

OBJECTIVES: We reviewed hospital records of pediatric cholelithiasis to develop an etiology-based decision-making protocol. METHODS: This retrospective study was conducted on consecutive pediatric cholelithiasis patients from July, 2014 to June, 2019 in a tertiary care center. Pediatric cholelithiasis was classified according to etiology, and the outcome of medical/surgical treatment was noted. RESULTS: Data of 354 pediatric patients were analyzed. Commonest (56.2%) etiology was idiopathic; followed by ceftriaxone pseudo-lithiasis (26.8%). Pigment stones were associated with the highest rate of complications. Non-hemolytic stones had a lower complication rate and a high rate of resolution with medical therapy. CONCLUSIONS: Hemolytic and symptomatic stones warrant an early cholecystectomy, whereas asymptomatic idiopathic stones, ceftriaxone stones, and TPN-induced stones are candidates for medical therapy under close observation.

Ceftriaxone-associated biliary pseudolithiasis in children: do we know enough?

Ceftriaxone is an antibiotic agent frequently used in paediatric hospital practice for the treatment of severe bacterial infections. The use of this agent can result in cholelithiasis and/or biliary sludge, more commonly in children than in adults. This systematic review was aimed at analysing available literature concerning ceftriaxone-associated biliary pseudolithiasis in paediatric patients, with a special emphasis on the clinical aspects. A literature analysis was performed using Medline and Embase electronic databases (articles published in English up to December 2019), with the search terms and combinations as follows:'ceftriaxone', 'cholelithiasis', 'biliary sludge' 'gallstones' 'neonates' 'children' 'clinical aspects' 'management'. Several case reports, case series and prospective/retrospective studies have documented a relationship between ceftriaxone treatment and biliary pseudolithiasis in the paediatric population, even though literature data regarding neonates and infants are scarce. Ceftriaxone-associated biliary pseudolithiasis is dose-dependent and usually asymptomatic but, sometimes, it may present with abdominal pain, nausea and emesis. Abdominal ultrasonography should be performed when this complication is suspected. Generally, ceftriaxone-associated cholelithiasis resolves over a variable period of time (days to months) after cessation of therapy. Therefore, a conservative approach to this condition is advocated, but a prolonged follow-up may be necessary. A personalized assessment of factors predisposing to ceftriaxone-associated biliary pseudolithiasis before prescribing the drug can allow to minimize the risk of developing it, with significant advantages in terms of human and economic costs.

Symptomatic cholelithiasis patients have an increased risk of pancreatic cancer: A population-based study.

BACKGROUND AND AIM: Pancreatic cancer is a fatal disease; currently, the risk factor survey is not suitable for sporadic pancreatic cancer, which has neither family history nor the genetic analysis data. The aim of the present study was to evaluate the roles of cholelithiasis and cholelithiasis treatments on pancreatic cancer risk. METHODS: Symptomatic adult patients with an index admission of cholelithiasis were selected from one million random samples obtained between January 2005 and December 2009. The control group was matched with a 1:1 ratio for sex, age, chronic pancreatitis, and pancreatic cystic disease. Subsequent pancreatic cancer, which we defined as pancreatic cancer that occurred ≥ 6 months later, and total pancreatic cancer events were calculated in the cholelithiasis and control groups. The cholelithiasis group was further divided into endoscopic sphincterotomy/endoscopic papillary balloon dilatation, cholecystectomy, endoscopic sphincterotomy/endoscopic papillary balloon dilatation and cholecystectomy, and no-intervention groups for evaluation. RESULTS: The cholelithiasis group and the matched control group included 8265 adults. The cholelithiasis group contained 86 cases of diagnosed pancreatic cancer, and the control group contained 8 cases (P < 0.001). The incidence rate ratio (IRR) of subsequent pancreatic cancer was significantly higher in the cholelithiasis group than in the control group (IRR: 5.28, P < 0.001). The IRR of subsequent pancreatic cancer was higher in the no-intervention group comparing with cholecystectomy group (IRR = 3.21, P = 0.039) but was similar in other management subgroups. CONCLUSION: Symptomatic cholelithiasis is a risk factor for pancreatic cancer; the risk is similar regardless of the intervention chosen for cholelithiasis.

National survey on the treatment of cholelitiasis in Spain during the initial period of the COVID-19 pandemic. / Encuesta nacional sobre el tratamiento de la colelitiasis en España durante la fase inicial de la pandemia por COVID-19.

INTRODUCTION: The extraordinary impact of COVID-19 pandemic on Spanish hospitals has led to a redistribution of resources for the treatment of these patients, with a decreased capacity of care for other common diseases. The aim of the present study is to analyse how this situation has affected the treatment of cholecystitis and cholelythiasis. METHODS: It is a descriptive national study after online voluntary distribution of a specific questionnaire with Google Drive™ to members of the Spanish Association of Surgeons (AEC). RESULTS: We received 153 answers (one per hospital). Elective cholecystectomies have been cancelled in 96.7% of centres. Conservative treatment for acute cholecystitis has been selected in 90% (previously 18%), and if operated, 95% have been performed laparoscopically. Globally, only 49% perform preoperative diagnostic tests for SARS-CoV-2, and 58.5% recognize there have been cases confirmed postoperatively after other surgeries, with worse surgical outcomes in 54%. CONCLUSIONS: This survey shows that most of the Spanish centers are following the surgical societies suggestions during the pandemic. However, some data requires to be taken into account for the next phase of the pandemic.

Bedside Biliary Drainage without Fluoroscopy for Critically Ill Patients.

BACKGROUND: Bedside biliary drainage by endoscopic retrograde cholangiopancreatography (ERCP) without fluoroscopy for critically ill patients in the intensive care unit (ICU) remains challenging for endoscopists. The present study was to evaluate the efficacy and safety of radiation-free ERCP for these patients. METHODS: Consecutive ICU patients with severe pancreaticobiliary disorders who underwent bedside radiation-free ERCP were retrospectively analyzed. RESULTS: Radiation-free ERCP was performed in 80 patients with acute physiology and chronic health evaluation (APACHE II) score of 24.1 ± 6.2. Cannulation was achieved in 75 (93.75%) patients. Biliary drainage was successfully conducted in 74 (92.5%) patients, including 54 (67.5%) and 20 (25.0%) cases of endoscopic retrograde biliary drainage (ERBD) and endoscopic nasobiliary drainage (ENBD), respectively. Adverse event (mild post-ERCP pancreatitis (PEP)) occurred only in 1 case. The 30-day mortality rate of these patients was 36.25% (29/80) and was much more higher in patients with ERBD in contrast to that of patients with ENBD, 40.7% (22/54) vs. 20% (4/20), OR = 2.750, 95%CI = 0.810 - 9.3405, P = 0.110. The APACHE II score in nonsurvivors was significantly higher than survivors, 27.6 ± 4.3 versus 22.2 ± 6.3, P = 0.009. The APACHE II score > 22 was an independent risk factor for mortality, 50% versus 10.7%, 95%CI = 2.148 - 31.569, P = 0.002. CONCLUSIONS: Radiation-free ERCP guided bedside biliary drainage is effective and safe for critically ill patients, and ENBD may be an optimal procedure due to a low mortality in these patients.

Bouveret's Syndrome - Case Series with Therapeutic Implications.

Bouveret's syndrome is a rare complication resulting from gallstone disease. Both surgical and endoscopical procedures are performed, with the disease to be seen as strictly interdisciplinary. There are no well-established recommendations for this condition. In this paper, we want to describe our experience from 6 cases in 3 Swiss hospitals from 2015 to 2017 with emphasis on the endoscopic technique of electrohydraulic lithotripsy followed by balloon dilatation and propose a treatment algorithm.

Management of Hepatolithiasis: Review of the Literature.

PURPOSE OF REVIEW: Hepatolithiasis is a disease characterized by intrahepatic stone formation. In this article, we review the features of this disease and explore the established and emerging treatment modalities. RECENT FINDINGS: Recent reports show an increasing prevalence of hepatolithiasis, likely owed to increased immigration and shifts in the Western diet. New pharmacotherapy options are limited and are often only supportive. Endoscopic intervention still cruxes on removal of impacted stones, though new techniques such as bile duct exploratory lithotomy and lithotripsy continue to advance management. Although hepatectomy of the effected portion of the liver offers definitive therapy, alternative less invasive modalities such as combined endoscopic/interventional radiology modalities have been utilized in select patients. Additionally, liver transplant serves as an option for otherwise incurable hepatolithiasis with coexisting liver dysfunction. Multiple emerging pharmacologic and procedural interventions may provide novel treatment for hepatolithiasis. While definitive therapy remains resection of affected liver segments, these modalities offer hope for less invasive approaches in the future.

Beta-Thalassemia Intermedia: A Single Thalassemia Center Experience from Northeastern Iraq.

OBJECTIVE: To determine the molecular characterization and disease-associated complications of beta-thalassemia intermedia (ß-TI) patients in Sulaymaniyah province, northeastern Iraq. METHODS: A total of 159 ß-TI) patients in Sulaymaniyah province, northeastern Iraq. ß-TI) patients in Sulaymaniyah province, northeastern Iraq. RESULTS: Nineteen different ß-globin gene mutations arranged in 37 various genotypes were determined. The most frequent were IVS-II-I (G>A) (47.2%), followed by IVS-I-6 (T>C) (23.3%) and IVS-I-110 (G>A) (5%). Among disease-related morbidities documented, bone disease amounted to 53% (facial deformity and osteoporosis), followed by endocrinopathies 17.6% (growth retardation and subclinical hypothyroidism), cholelithiasis 13.8%, pulmonary hypertension 11.3%, and abnormal liver function test 7.5%, whereas venous thrombosis, extramedullary hemopoiesis, and leg ulcer were less frequently observed. Age ≥ 35 and female sex were risk factors for cholelithiasis, while age was an independent risk for hypothyroidism and female sex was associated with increased risk for osteoporosis. Mean serum ferritin of ≥1000 µg/L was associated with an increased risk of osteoporosis, whereas chelation therapy was protective for a multitude of other complications. Transfusion, on the other hand, increased the risk of osteoporosis, yet it was protective for cholelithiasis and hypothyroidism. Moreover, splenectomy was protective for cholelithiasis, although it was an independent risk for hypothyroidism. Finally, hydroxyurea was associated with an increased risk of osteoporosis, while it was protective for cholelithiasis. Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.ß-TI) patients in Sulaymaniyah province, northeastern Iraq. µg/L was associated with an increased risk of osteoporosis, whereas chelation therapy was protective for a multitude of other complications. Transfusion, on the other hand, increased the risk of osteoporosis, yet it was protective for cholelithiasis and hypothyroidism. Moreover, splenectomy was protective for cholelithiasis, although it was an independent risk for hypothyroidism. Finally, hydroxyurea was associated with an increased risk of osteoporosis, while it was protective for cholelithiasis. Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.Discussion and Conclusion. ß +-thalassemia mutation had contributed to 41.25 of families with a less severe ß-thalassemia phenotype in the northeastern part of Iraq, justifying the need to investigate the contribution of genetic modifiers in ameliorating disease severity. In addition, the substantial number of ß-TI patients developed disease-related morbidities, which necessitates the need for more appropriate clinical management with earlier intervention.ß-TI) patients in Sulaymaniyah province, northeastern Iraq. ß-TI) patients in Sulaymaniyah province, northeastern Iraq.

New management of hepatolithiasis: Can surgery be avoided? (with video).

BACKGROUND: The presence of hepatolithiasis (HL) is prevalent in eastern countries. It is a clinical entity which is rarely reported in non-surgical series because the standard treatment is the surgical option. Currently, treatment has evolved, with the use of endoscopic techniques being increased and the number of hepatectomies being decreased. SpyGlass™ is a small-calibre endoscopic direct cholangiopancreatoscopy developed to explore and perform procedures in the bile and pancreatic ducts. Single-operator peroral cholangioscopy (POC) is an endoscopic technique useful for treating difficult bile duct stones. AIMS: To assess the usefulness, efficacy, and safety of POC with the SpyGlass™ system in patients with HL. PRIMARY OBJECTIVES: to achieve technical success of the procedure and clinical success of patients with HL. STUDY DESIGN AND PATIENTS: Retrospective, single-centre cohort study of patients with HL from April 2012 to August 2018. SpyGlass™ was chosen in symptomatic patients referred from the surgery unit as the first-line procedure. To perform electrohydraulic lithotripsy (EHL), we used a Northgate Autolith IEHL generator with a 0.66-mm biliary probe. RESULTS: We performed a total of 13 procedures in 7 patients with HL. The mean age was 46 years (range 35-65) and 3/7 of patients were female. We achieved technical success in 5/7 cases (71.4%) and clinical success in 4/7 cases (57%). DISCUSSION: SpyGlass™ is safe and effective in the treatment of HL. With these results, we confirm the need for management of patients with HL in a multidisciplinary team. When the endoscopic approach is the option, this procedure must be performed by experts in advanced endoscopy.

Percutaneous Biliary Endoscopy for Stones.

Intraductal biliary stones can result in significant acute and long-term complications. When patients' anatomy precludes more traditional management, the interventional radiologist may be called upon to provide well-established techniques for percutaneous biliary drainage and stone removal. This can be particularly challenging when the patient has excessively mobile, impacted, large or multiple stones. Percutaneous biliary endoscopy with adjunct interventional techniques can successfully treat these patients avoiding the patient dreaded "tube for life" scenario. Direct percutaneous visualization of the biliary tree can also diagnose and provide symptomatic relief for stone-mimicking pathologic conditions such as biliary tumors. This article will review the role, technique, and considerations for percutaneous biliary endoscopy and adjunct interventions in patients with isolated and complex, biliary stone disease and stone-mimicking pathologies.

Percutaneous transhepatic Laser lithotripsy for intrahepatic cholelithiasis.

PURPOSE: To report the technical success, complications and long-term outcomes of patients with intrahepatic cholelithiasis not amenable to endoscopic retrograde cholangiopancreatography (ERCP) who were treated with percutaneous transhepatic biliary Laser lithotripsy (PTBLL). PATIENTS AND METHODS: A retrospective review of 12 patients who underwent PTBLL for intrahepatic cholelithiasis was performed. There were 5 men and 7 women with a mean age of 46±18 (SD) years (range: 23-75 years). PTBLL was performed when stone clearance using ERCP had previously failed or was not appropriate due to unfavourable anatomy or the presence of multiple stones or a large stone. Percutaneous biliary access into intrahepatic ducts was obtained if not already present and lithotripsy was performed using a HolmiumYAG 2100nM Laser. Patients files were analyzed to determine the technical success, complications and long-term outcomes. RESULTS: Eleven patients (11/12; 92%) had a history of previous hepatobiliary surgery and nine (9/12; 75%) had multiple stones confirmed on preprocedure imaging. A 100% success rate in fragmenting the target stone(s) was achieved and 11/12 patients (92%) had successful first pass extraction of target stone fragments. Two patients (2/12; 17%) required repeat lithotripsy. One patient (1/12; 8%) experienced a major complication in the form of cholangitis. Of patients with long-term follow-up, 4/10 (40%) had recurrence of intrahepatic calculi with a mean time interval of 31 months (range 3-84 months). CONCLUSION: For patients with intrahepatic biliary calculi not amenable to ERCP, PTBLL provides an effective and safe alternate treatment.

Low-Phospholipid Associated Cholelithiasis (LPAC) syndrome: A synthetic review.

Low-Phospholipid Associated Cholelithiasis (LPAC) is a genetic disease responsible for the development of intrahepatic lithiasis. It is associated with a mutation of the ABCB4 gene which codes for protein MDR3, a biliary carrier. As a nosological entity, it is defined by presence of two of the three following criteria: age less than 40 years at onset of biliary symptoms, recurrence of biliary symptoms after cholecystectomy, and intrahepatic hyperechogenic foci detected by ultrasound. While the majority of clinical forms are simple, there also exist complicated forms, involving extended intrahepatic lithiasis and its consequences: lithiasis migration, acute cholangitis, intrahepatic abscess. Chronic evolution can lead to secondary sclerosing cholangitis or secondary biliary cirrhosis. In unusual cases, degeneration into cholangiocarcinoma may occur. Treatment is built around ursodeoxycholic acid, which yields dissolution of biliary calculi. Complicated forms may call for interventional, radiological, endoscopic or surgical treatment. This synthetic review illustrates and summarizes the different aspects of this entity, from simple gallbladder lithiasis to cholangiocarcinoma, as well as secondary biliary cirrhosis requiring liver transplant, on the basis of clinical cases and the iconography of patients treated in our ward.