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This case report describes a 23-year-old male who was incidentally diagnosed with Caroli disease while investigating symptoms of recurrent upper quadrant pain and fever. Imagining studies, including magnetic resonance cholangiography revealed the “central dot sign,” which is pathognomonic for this condition, along with cystic dilatations of the intrahepatic bile ducts and the presence of biliary stones. The diagnosis was confirmed and treatment with ursodeoxycholic acid led to a notable reduction in symptoms. This case underscores the need for further research and understanding to develop more effective diagnostic and treatment strategies. Regular follow-up remains essential for managing disease progression and preventing complications.

We present a rare and first case of Caroli Disease (CD) in a child who presents with abdominal distension, regurgitation of feeds, passage of greenish stools and fever, typical ultrasonographic and abdominal and x-ray findings. The purpose of presenting this case report is to highlight the distinctive manifestation of Caroli disease and to provide a concise report of this rare disease with the hope that such information will help identify patients earlier in the course of their illness. This will add to medical literature by helping clinicians to know that this anomaly is treatable in a good setting and if detected early.

Abstract Introduction Caroli disease is multifocal segmental dilatation of the large intrahepatic bile ducts that connect to the main duct. It is considered a rare disease with an incidence rate of 1 in 1,000,000 births. There are two types of Caroli: the first type is the simple type, Caroli disease, which includes only cystic dilatation of the intrahepatic bile ducts. The second is called Caroli syndrome, which consists of Caroli disease and congenital hepatic fibrosis and might lead to portal hypertension leading to esophageal varices and splenomegaly. Atrial septal defect is one of the most common congenital heart diseases, occurring when the connection between the left and the right atriums fails to close. Polydactyly is one of the most common congenital malformations of the hands and feet. It manifests in excess fingers on the hands or toes. Case presentation A 6-year-old Arab girl presented to the hospital with abdominal pain for the last month with abdominal enlargement. The patient was already diagnosed with Caroli disease and polydactyly (six fingers on each limb) when she was born. Investigations including complete blood count, blood smear, bone marrow biopsy, esophagoscopy, abdominal ultrasound, and computed tomography scan showed splenomegaly associated with hypersplenism, fourth-grade non-bleeding varices, intrahepatic cystic formations in the left and right lobes, and an atrial septal defect with a left-to-right shunt. The patient was scheduled for a splenectomy after she was vaccinated with the appropriate vaccines. After follow-up for a week in the hospital, complete blood count showed an improvement. A month after that, the patient had liver abscesses and biliary fistula that were treated appropriately and her symptoms resolved. Conclusion The association of liver diseases, polydactyly, and congenital heart diseases is extremely rare and was only documented few times in the literature. However, to our knowledge, atrial septal defect has never been part of this combination before. The family history also makes this case unique and strongly suggests genetic etiology.

Caroli disease is a rare congenital disorder of segmental cystic dilatations of the intrahepatic biliary ducts that maintain communication with the rest of the biliary tree. Its typical clinical presentation is recurrent episodes of cholangitis. The diagnosis is usually made using abdominal imaging modalities. We present a patient with Caroli disease showing atypical presentation of acute cholangitis with ambiguous labs and initial negative imaging findings, which was later diagnosed by [18F]-fluorodeoxyglucose positron emission tomography/computed tomography and confirmed by magnetic resonance imaging and tissue pathology. The use of such imaging modalities in moments of doubt or clinical suspicion provide the patient with accurate diagnosis, proper management, and better clinical outcome, thus obviating the need for further invasive investigations.

Introduction: Caroli disease is a rare congenital disorder causing nonobstructive segmental dilatation of intrahepatic bile ducts. Caroli disease can affect the liver diffusely or be confined to a single segment or lobe, although localized Caroli disease is extremely uncommon. We present a rare case of an 8-year-old child with incidentally found Caroli disease limited to two hepatic segments who was treated with partial hepatectomy, as well as a review of the literature regarding the presentation, diagnosis, and management of Caroli disease in children and adults. Case Presentation: Our patient is an 8-year-old male with an incidentally found multiloculated cystic liver mass referred to our institution for further management. Due to its heterogeneous nature and lack of evidence of direct communication with the biliary system, our differential diagnosis included hepatic teratoma, mesenchymal hamartoma, and undifferentiated embryonal sarcoma. A decision was made to resect the lesion both for definitive diagnosis and to mitigate the risk of malignant transformation of this lesion. At the time of surgery, the mass was observed to occupy portions of liver segments 6 and 7, necessitating a formal right hepatectomy and cholecystectomy. The patient tolerated the procedure well, was extubated in the operating room, and recovered in the ICU in stable condition without further complications and was seen in clinic for follow-up without further postoperative concerns. Conclusion: Though rare, Caroli disease can present as an asymptomatic focal heterogeneous mass in children. Pediatric patients with segmental Caroli disease can be treated with partial hepatectomy safely.

Contradictions over the meaning of adoration (adoratio) in Theodulf of Orléans' Opus Caroli regis contra synodum have been used to minimize the role of mistranslation in the late eighth‐century Greek–Latin dispute over images. This study, however, scrutinizes the contested meaning of adoration in the original manuscript to expose tensions among Charlemagne's key theological advisers. These fissures reveal the text's collaborative production and purpose: neither the fully independent work of a lone scholar, nor intended for publication to an extensive audience, but a project to stimulate debate amidst the king's counsellors. [ABSTRACT FROM AUTHOR]

This study gives the first data on the body and beak morphometric characteristics, age, and genetic structure of neon flying squid, a rarely caught cephalopod in the Adriatic Sea. We identified specimens as recently resurrected Ommastrephes caroli species using two mitochondrial markers, 16S ribosomal RNA gene and cytochrome c oxidase subunit I gene. Overall, 23 juveniles (3 females, 3 males, and 17 unsexed), with a dorsal mantle range of 65–152 mm, were caught in September 2020 in the waters of the Korčula Channel, island of Palagruža, and island of Jabuka, thus providing the most abundant sample of this species in the Mediterranean waters. The length–weight relationship showed an isometric growth. The results of the beak/length regressions suggest hood length is a useful characteristic for biomass estimation studies, as it showed a good linear fit to the dorsal mantle length. Statolith growth increments were easily visible and statolith microstructure analysis was successfully used to determine the age of 22 individuals. The estimated age ranged from 36 to 64 days (mean = 48 days). The back-calculation analysis showed that the squid hatched during July and August 2020, indicating that O. caroli spawns during the warmer, summertime period. Considering the size and age of the caught individuals, the Adriatic Sea could represent a potential feeding ground for this species. The genetic structure analyses indicate the existence of separate Atlantic and Mediterranean/Adriatic subclusters; however, this warrants further investigation.

Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension.

The WDR19 gene has been reported to be involved in nephronophthisis-related ciliopathies such as isolated nephronophthisis 13 (NPHP13), Sensenbrenner syndrome, Jeune syndrome, Senior-Loken syndrome, Caroli disease, retinitis pigmentosa and Asthenoteratospermia. In the present study, we provided the detailed clinical characteristics and genetic analysis of a patient with four variants in WDR19 and TG, reviewed a comprehensive mutation analysis in the WDR19-related ciliopathies, discussed the relationship between genotype and phenotype, and compared the allele frequencies (AFs) of WDR19 variants depending on the ethnic background. We used whole-exome sequencing (WES) combined with bioinformatics analysis to investigate the genetic variants of a 3-year-old boy with common features of WDR19-associated NPHP13 and Caroli disease, bilateral central blindness, refractory epilepsy, and elevated thyroid stimulating hormone. A novel splice-donor variant, c.98+1G > C, and a recurrent missense variant, c.3533G > A, were identified in the WDR19 gene. We used effective mRNA analysis to verify the effects on pre-mRNA processing and to assess the pathogenicity of the splice-site variant. The patient also harbored compound heterozygous variants of the TG gene (c.4889A > G, c.274+2T > G). Of note, using a review of an in-house database, we identified four additional likely pathogenic WDR19 variants and estimated the overall AF of WDR19 mutations to be 0.0025 in the southern Chinese population. Our findings have expanded the allelic spectrum of mutations in the WDR19 gene and broadened the clinical phenotype spectrum of WDR19-related ciliopathies. The results have also provided new insights into the clinical heterogeneity of the disorder, which would be useful in accurate genetic counseling for affected individuals and carrier screening in a general population.

Elytroatra Daccordi, status novus, and description of E. caroli, species nova, from Bolivia with brief notes on some related neotropical genera of Chrysomelinae Doryphorini (Coleoptera: Chrysomelidae). Elytroatra Daccordi 2008 is raised to genus level from its original status as subgenus of Proseicela Chevrolat 1836. The new status is proposed in consideration of the wide clypeus, almost smooth, the supra-orbital area strongly raised, with the eyes placed at its vertex so that the head appears notably transversally widened, absence of metathoracic wings, elytral shape elongate with a narrow apical angle (nearly 60°), for the presence of a wide, continuous, bulgy elytral lateral callus, narrow and elongated femora and particularly narrow and elongated tibiae. Type species of the genus is Elytrosphaera melas Jolivet 1950. Elytroatra caroli sp. n. is here described from the same biogeographic region of the Bolivian Yungas. and is the second known species of the genus. Elytroatra caroli sp. n. can be separated at first glance from its relative E. melas for its thoroughly shining black coloration, absence of two symmetrical pores at sides of pronotal disc, the shape of aedeagus with the apex narrower and elongated and shorter and less arcuate tegmen. [Article in Italian]

Abstract Background Caroli disease (CD, OMIM #600643) is a rare autosomal recessive disorder characterized by polycystic segmental dilatation of the intrahepatic bile ducts and extreme variability in age of onset and clinical manifestations. When congenital hepatic fibrosis is associated with the polycystic dilatation of the biliary tract, the condition is referred as Caroli syndrome. The disease is thought to be caused by pathogenic variants in the PKHD1 gene (OMIM *606702). Method We report the clinical, biochemical, and molecular characterization of three patients with a clinical suspicion of CS belonging to two different families. The genetic screening was performed using a target custom panel and sequencing was performed on Illumina platform. Results Genetic analysis revealed the presence of rare variants in the PKHD1 gene of the analyzed patients. In the first case, and his younger sister, two pathogenic variants (c.2702A>C and c.4870C>T) were found to be associated with a hepatic phenotype at clinical onset, followed by renal disease probably age‐related; while in the second case, one pathogenic variant (c.5879C>G) and a complex allele with uncertain clinical significance [c.3407A>G; c.8345G>C; c.8606C>A] were found to be associated with a severe hepatic phenotype. Conclusion The identification of the genetic causes of the disease and their relationship with the clinical phenotype could have a favorable impact on clinical management and complication prevention.

Background: If a young patient presents with fever, abdominal pain, jaundice and significant imaging abnormalities, especially dilation of the biliary system, it is usually due to obstruction from stones or strictures. However, on very rare occasions, it can be due to complications of congenital cystic dilatation of the biliary system, known as Caroli disease. We present such a patient and discuss the differential diagnosis and implications for long-term management. Case Report: A 14-year-old boy presented to the Emergency Department with a sudden onset of high-grade fever and abdominal pain for 2 weeks, accompanied by vomiting of blood. The patient had no relevant medical history. He was malnourished and had moderate pallor, jaundice, and right upper quadrant pain. Imaging revealed cystic dilatation of intrahepatic ducts and a central dot sign. There were no features suggesting advanced liver disease otherwise, and no tumors or cysts in the kidneys. A diagnosis of Caroli disease was made. The symptoms were ascribed to acute cholangitis and improved with antibiotics. He was discharged home 1 week later. No further blood loss was observed. Conclusions: This case study describes a patient with ascending cholangitis, a complication of Caroli disease. This diagnosis should be considered in the differential diagnosis when a child or young adult presents with features of cholangitis, abnormal biliary imaging, and/or upper gastrointestinal bleeding, or portal hypertension. No prior cases of this disease have been encountered, documented, or published in Kenya. This case can increase awareness among primary care clinicians, including pediatricians. [ABSTRACT FROM AUTHOR]

A Síndrome de Caroli é uma entidade incomum caracterizada pela presença de dilatações císticas da árvore biliar intra-hepática e fibrose hepática congênita. Pode comportar-se como condição pré-maligna, evoluindo em alguns casos para colangiocarcinoma. Relatamos um caso diagnosticado em paciente do sexo masculino de 37 anos de idade, complicado por carcinomatose peritoneal de sítio desconhecido e revisamos a literatura.

Background: Liver resection is the treatment of choice for patients with localised Caroli disease. While liver resection was traditionally performed as open procedure, this case series aims to evaluate the safety and efficacy of minimally invasive, laparoscopic liver surgery in these patients. Methods: A systematic review of electronic case files of patients seen between April 2015 and December 2017 at the Department of Surgery, Charité University Hospital Berlin, was conducted. Patients with Caroli disease in whom laparoscopic liver resection had been performed were identified and analysed in this single-centre case series. Results: Seven patients who underwent laparoscopic liver surgery for Caroli syndrome were identified and presented with a median age of 49 (range = 44–66) years, of which four (57%) were female. Preoperatively, six patients were classified as the American Society of Anaesthesiologists (ASA) 2 and one patient as ASA 3. Two operations were performed as single-incision laparoscopic surgery, whereas the others were done as multi-incision laparoscopic surgery. One patient required a conversion to an open procedure. The length of operation varied between patients, ranging from 128 to 758 min (median = 355). The length of stay in the intensive care unit ranged from 0 to 2 days. Two patients presented with post-operative complications (Clavien–Dindo Grade ≥3a), whereas no patient died. In histopathological analysis, all patients demonstrated characteristic findings of Caroli disease and no cholangiocarcinoma was found. Conclusion: These results indicate that minimally invasive, laparoscopic liver surgery is a safe and efficacious treatment option for patients with Caroli disease who require liver resection.

Abstract Background Both Caroli disease (CD) and autosomal recessive polycystic kidney disease (ARPKD) are autosomal recessive disorders, which are more commonly found in infants and children, for whom surviving to adulthood is rare. Early diagnosis and intervention can improve the survival rate to some extent. This study adopted the case of a 26-year-old pregnant woman to explore the clinical and imaging manifestations and progress of CD concomitant with ARPKD to enable a better understanding of the disease. Case presentation A 26-year-old pregnant woman was admitted to our hospital for more than 2 months following the discovery of pancytopenia and increased creatinine. Ultrasonography detected an enlarged left liver lobe, widened hepatic portal vein, splenomegaly, and dilated splenic vein. In addition, both kidneys were obviously enlarged and sonolucent areas of varying sizes were visible, but color Doppler flow imaging revealed no abnormal blood flow signals. The gestational age was approximately 25 weeks, which was consistent with the actual fetal age. Polyhydramnios was detected but no other abnormalities were identified. Magnetic resonance imaging revealed that the liver was plump, and polycystic liver disease was observed near the top of the diaphragm. The T1 and T2 weighted images were the low and high signals, respectively. The bile duct was slightly dilated; the portal vein was widened; and the spleen volume was enlarged. Moreover, the volume of both kidneys had increased to an abnormal shape, with multiple, long, roundish T1 and T2 abnormal signals being observed. Magnetic resonance cholangiopancreatography revealed that intrahepatic cystic lesions were connected with intrahepatic bile ducts. The patient underwent a genetic testing, the result showed she carried two heterozygous mutations in PKHD1. The patient was finally diagnosed with CD with concomitant ARPKD. The baby underwent a genetic test three months after birth, the result showed that the patient carried one heterozygous mutations in PKHD1, which indicated the baby was a PKHD1 carrier. Conclusions This case demonstrates that imaging examinations are of great significance for the diagnosis and evaluation of CD with concomitant ARPKD.

ABSTRACT: Caroli syndrome is characterized by a combination of intrahepatic biliary ductal ectasia and congenital ductal fibrosis due to the failure of involution of ductal plates and large intrahepatic ducts. This study aims to report Caroli syndrome in a dog, with emphasis on clinical characteristics, complementary examinations, and diagnostic approach. A 1-year-old mixed-breed intact male dog was presented with a 7-day history of acute vomiting and anorexia. Clinical evaluation revealed severe jaundice, abdominal pain, dehydration, lethargy, and abdominal distension. Ultrasonography revealed hepatic cysts filled with fluid. Exploratory laparotomy was performed to clarify the imaging findings; however, the dog died shortly after surgery. Postmortem examination revealed that the liver was reduced in size and had multiple interconnected and distended saccular dilations. The bile ducts were markedly dilated and replaced a large part of the liver parenchyma. Multiple small cysts were also observed in the kidneys. Microscopically, the hepatic lesions were characterized by extensive bridging fibrosis associated with proliferating and ectatic bile ducts contiguous with the biliary tree. Marked fibrosis and small cysts were observed in the kidneys. Therefore, a final diagnosis of Caroli syndrome was made. While uncommon, this syndrome should be considered as a differential diagnosis for young dogs with biliary cysts and hepatic fibrosis.

Abstract Background Caroli syndrome (CS) is a rare congenital disorder without pathognomonic clinical symptoms or laboratory findings; therefore, the diagnosis is often delayed. The objective of this study was to investigate the diagnostic delay and associated risk factors in CS patients. Methods This was a retrospective analysis of 16 CS patients admitted to a single tertiary medical center on mainland China. The diagnostic timelines of CS patients were reviewed to demonstrate the initial findings of CS at diagnosis, the risk factors associated with diagnostic delay, and potential clues leading to early diagnosis. Results The median diagnostic delay was 1.75 years (range: 1 month to 29 years, interquartile range: 6.2 years) in 16 enrolled CS patients. Sex, age, and initial symptoms were not associated with diagnostic delay. 87.5% of CS patients were diagnosed by imaging, and the accuracies of ultrasonography, computed tomography (CT), and magnetic resonance cholangiopancreatography were 25, 69.2, and 83.3%, respectively. The median diagnostic delays for patients with or without CT performed at the first hospital visited according to physician and radiologist suspicion of the diagnosis were 7.4 months and 6 years, respectively (p = 0.021). Hepatic cysts with splenomegaly were detected by ultrasound in over half of CS patients. Conclusions The majority of CS patients were not diagnosed until complications of portal hypertension had already developed. Recognition and early suspicion of the disease were important factors influencing diagnostic delay of CS. Hepatic cysts plus splenomegaly detected by US might raise the clinical suspicion to include CS in the differential diagnosis.

In einem Hochendemiegebiet der alveolären Echinokokkose (AE), einer Zoonose verursacht durch den Fuchsbandwurm Echinococcus multilocularis wurde in Südwestdeutschland ein Feldhase (Lepus europaeus) während der offiziellen Jagd erlegt. Bei der amtlichen Fleischuntersuchung zeigten sich makroskopisch für eine AE suspekte, subkapsulär im Leberparenchym liegende, zystenähnliche Veränderungen von ca. 1–3 cm Größe. Auch im Rahmen einer an der Hasenleber durchgeführten Ultraschall- und MRT-Untersuchung ergab sich bildmorphologisch der hochgradige Verdacht auf eine AE. Um die Struktur des Inhaltes der Zysten nicht zu zerstören, wurde auf ihre Eröffnung verzichtet. Die histologische und immunhistochemische Untersuchung durch eine Referenzpathologie für Echinokokkose konnte die Verdachtsdiagnose der AE oder die Differentialdiagnose einer Zystizerkose, verursacht durch T. crassiceps oder T. pisiformis, nicht bestätigen. Die histopathologischen Befunde sprachen für das Vorliegen eines Cholangiofibroms (Von-Meyenburg-Komplex). Dieser Befund wurde anhand der Bildmorphologie des Ultraschalls und der MRT als Caroli-Krankheit eingeordnet. Der vorliegende Fallbericht zeigt, dass als Differentialdiagnose einer AE oder einer Zystizerkose auch an ein seltenes Cholangiofibrom mit der makroskopisch-/bildmorphologischen Ausprägung einer Caroli-Krankheit gedacht werden sollte.

Introducción: la enfermedad de Caroli es un trastorno congénito poco usual, el cual cursa con dilatación segmentaria multifocal de los conductos biliares intrahepáticos. Fue descrita por Jacques Caroli en 1958 como una dilatación sacular, segmentada o fusiforme de los ductos biliares intrahepáticos. Objetivo: caracterizar a la población que ha padecido la enfermedad de Caroli. Metodología: Revisión sistemática de la literatura. Resultados: se analizaron 66 artículos, el grupo de edad más afectado es los menores de 10 años, en el que se evidenció una mayor prevalencia en varones, y se encontró asociado con otras comorbilidades como el riñón poliquístico (20 %). La manifestación más frecuente fue la hepatomegalia (44,7 %), seguido de la fiebre (42,4 %) y el dolor abdominal en el hipocondrio derecho (41,2 %). Como método diagnóstico más utilizado se encontraba la resonancia magnética (73,8 %); dentro de los hallazgos predominó la dilatación intrahepática (76,5 %) y el manejo más empleado fue la antibioticoterapia para tratar las recurrencias por colangitis. Conclusión: la enfermedad de Caroli tiene una baja prevalencia, se presenta con mayor frecuencia en el continente americano, afecta principalmente a la primera década de la vida, tiene predilección por el sexo masculino y se caracteriza por una dilatación de los conductos intrahepáticos que puede afectar a otros órganos como los riñones, lo que produce quistes renales.

Molecular identifications based on two mitochondrial markers (cytochrome c oxidase subunit I -COI- and 16S ribosomal RNA gene -16S-) have been implemented to confirm the morphological identification of eight specimens collected in the Central western Mediterranean. Molecular data show they belonged to a recently resurrected species of the genus Ommastrephes, i.e., O. caroli, known to be distributed in the Atlantic Ocean and Mediterranean Sea. Despite this, molecular analyses of COI sequences evidenced the presence of potential genetic differentiation between Mediterranean and Atlantic samples, highlighting the need for further studies, with more individuals to investigate the connectivity between individuals living in the two areas. Furthermore, morphological, biometric and reproductive features here reported, could be useful in evaluating possible distinctive biological features between the Mediterranean and Atlantic individuals. Female mature size was larger than the male. The relationships obtained between the beak measurements and body sizes (DML; TW) were better described by a power model. Asynchronous oocytes development with relatively small oocytes (0.05–1.10 mm) and a protracted intermittent spawning with active feeding were observed. This study also reported for the specie O. caroli the first data on the potential fecundity estimated (840061 oocytes), the oviducal load (90000 ripe oocytes) as well as the number of seminal receptacles and the size and morphology of the spermatangia found in the buccal mass of all mated females. Even if on a low sample size, beaks and eye lenses were used for the first time in O. caroli for age estimation. The statistically significant relationship found between increments counted in eye lenses and beaks highlighted the reliability of the lenses to estimate age in O. caroli, even if further studies will be needed for its validation. Assuming a daily increment for both structures, a mean life span of about 12–13 months was estimated for both sexes, which is consistent with the sexual maturity condition observed in all the samples and the semelparity known for cephalopods coleoids.

The incidence of Caroli disease is estimated to be 1 case per 1 million people; as a result, there are very few available clinical observations. At the moment, the treatment of Caroli disease is limited to symptomatic therapy and the prevention of complications, as well as to corrective surgery and liver transplantation.Aim. The aim of this observation is to describe one case of a rare congenital liver disease – Caroli disease – characterised by a segmented non-obstructive fibrocystic dilation of the intrahepatic bile ducts.Key findings. A 21-year-old woman was hospitalised with the signs of a systemic inflammatory reaction, hepatosplenomegaly, jaundice, portal hypertension, hepatocellular insufficiency, as well as with the manifestations of cytolytic and cholestatic syndromes. At the age of 8, she was diagnosed with a cyst of the bile ducts, which was treated with cystoenteroanastomosis. At the age of 20, in connection with high portal hypertension, portocaval shunting was performed and a mesenteric-caval anastomosis was applied. During the present hospitalisation, an expansion of the intrahepatic bile ducts was revealed by ultrasound and MSCT of the abdominal cavity with contrast, which made it possible to diagnose Caroli disease.Conclusion. A case of Caroli disease is described, which resulted in continuously recurrent cholangitis and biliary cirrhosis. This state required liver transplantation. Caroli disease should be included in differential diagnosis in patients suffering from the fever of unknown origin and cholestasis syndrome. An early diagnosis of Caroli disease is important for preventing complications, improving the quality of life and increasing the life expectancy of patients suffering from this rare disease.

Caroli syndrome is characterized by a combination of intrahepatic biliary ductal ectasia and congenital ductal fibrosis due to the failure of involution of ductal plates and large intrahepatic ducts. This study aims to report Caroli syndrome in a dog, with emphasis on clinical characteristics, complementary examinations, and diagnostic approach. A 1-year-old mixed-breed intact male dog was presented with a 7-day history of acute vomiting and anorexia. Clinical evaluation revealed severe jaundice, abdominal pain, dehydration, lethargy, and abdominal distension. Ultrasonography revealed hepatic cysts filled with fluid. Exploratory laparotomy was performed to clarify the imaging findings; however, the dog died shortly after surgery. Postmortem examination revealed that the liver was reduced in size and had multiple interconnected and distended saccular dilations. The bile ducts were markedly dilated and replaced a large part of the liver parenchyma. Multiple small cysts were also observed in the kidneys. Microscopically, the hepatic lesions were characterized by extensive bridging fibrosis associated with proliferating and ectatic bile ducts contiguous with the biliary tree. Marked fibrosis and small cysts were observed in the kidneys. Therefore, a final diagnosis of Caroli syndrome was made. While uncommon, this syndrome should be considered as a differential diagnosis for young dogs with biliary cysts and hepatic fibrosis. [ABSTRACT FROM AUTHOR]