Search Results (10)

A 2-year-old male neutered domestic shorthair cat was presented with a 12-month history of anorexia, weight loss, vomiting, polyuria, and polydipsia. Physical examination revealed a responsive but debilitated cat with icteric mucous membranes and a low body-condition score. Laboratory tests showed mild macrocytic anemia, elevated liver enzymes, hyperproteinemia with hypoalbuminemia, hyperbilirubinaemia, and a decreased vitamin B12 level. Abdominal ultrasound revealed marked dilation of the common bile duct (CBD) and gallbladder, consistent with suspected extrahepatic biliary tract obstruction, as well as secondary pancreatic and intrahepatic duct distension. Cytological and bacteriological analyses confirmed bacterial cholangiohepatitis due to a mixed infection with Escherichia coli and Peptostreptococcus canis. Despite initial conservative management, the cat’s clinical condition remained unchanged, prompting exploratory laparotomy. Intraoperative findings confirmed the ultrasonographic suspicions and revealed severe pancreatitis. Based these findings, a cholecystectomy, choledochoenterostomy, and placement of a cholecystostomy tube as well as an abdominal drain and jejunal feeding tube were performed. Postoperatively, the cat initially showed improvement but subsequently developed signs of sepsis requiring intensive care. Although temporary stabilization was achieved, the cat died suddenly ten days after surgery due to unknown causes. This case report highlights the diagnostic and therapeutic challenges associated with bacterial cholangiohepatitis secondary to biliary obstruction in cats and documents the first clinical application of choledochoenterostomy—a surgical procedure previously only described hypothetically in this species. Full article

Background/Objectives: Ultrasonography is a diagnostic modality characterized by high sensitivity in detecting hepato-biliary pathology, particularly intrahepatic biliary duct dilation. This study compares the sonographic findings obtained by a radiographer/sonographer in a Basic Emergency Service (BES) using Point-of-Care Ultrasonography (POCUS) in Portugal with the sonographic findings from the same patients acquired by radiologists at a referral hospital (RH) for suspected intrahepatic biliary dilatation. Methods: Nineteen patients presenting with right upper quadrant (RUQ) pain and suspected abdominal pathology underwent sonographic screening using POCUS in the BES. Subsequently, the same patients were referred to the RH, where a radiologist performed a comprehensive ultrasound. Both examinations were compared to determine whether the findings obtained in the BES were confirmed by radiologists in the RH. Results: Cholestasis, cholangitis, lithiasis, pancreatitis, peri-ampullary lithiasis, and neoplasms were observed in association with intrahepatic biliary dilation in this study sample. All six variables showed a strong association between the BES and RH findings (Cramer’s V > 0.6; p < 0.006). A strong kappa measure of agreement between the radiographer and radiologist findings was obtained in “cholelithiasis/sludge/gallbladder acute sonographic changes” (k = 0.802; p = 0.000). A moderate kappa value was obtained for the variable “abdominal free fluid”, (k = 0.706; p = 0.001). Conclusions: In this study, all patients referred from the BES to the RH required hospitalization for treatment and additional imaging exams. Although pre-hospital screening ultrasound is not intended for definitive diagnoses, the early detection of intrahepatic biliary tract dilatation through screening sonography played a significant role in the clinical referral of patients, with a sensitivity of 94% and specificity of 75%. Full article

Background/Objectives: Intrahepatic cholangiocarcinoma (ICC) is subclassified into small and large duct types. Although these subclassifications may help determine the appropriate treatment strategy, subclassification diagnosis currently depends on postoperative pathological examinations. This study aimed to establish a nomogram to predict ICC subclassifications. Methods: This study included 126 patients with ICC who underwent liver resection. The participants were divided into small and large duct-type ICC groups. A nomogram to predict large duct-type ICC was developed using four diagnostic imaging findings: rim-type enhancement in the early phase, an absence of tumor enhancement in the early phase, the presence of peripheral biliary dilatation due to tumor invasion, the presence of penetrating Glisson’s vessels in the tumor, and two laboratory test results: serum gamma-glutamyl transpeptidase and carbohydrate antigen 19-9 levels. Nomogram performance was also assessed. Moreover, the bootstrap method and calibration plots were used to assess nomogram validity. Results: Seventy and fifty-six patients were pathologically diagnosed with small and large duct-type ICCs, respectively. The area under the curve of the established nomogram was 0.93 and remained 0.91 after Harrell’s bias correction. The sensitivity and specificity of the nomogram developed using the Youden index were higher than those of any of the characteristic imaging findings. Calibration plots demonstrated a strong association between the nomogram and the actual data. Conclusions: We developed a novel preoperative nomogram to predict large duct-type ICC. This nomogram can be clinically useful for predicting the subclassifications of ICCs and may contribute to the establishment of a more appropriate treatment strategy for ICC. Full article

Autosomal recessive polycystic kidney disease (ARPKD) is often associated with hepatobiliary disease in the form of hepatic fibrosis and/or Caroli disease. Combined liver–kidney transplantation (CLKT) is a transplant modality of choice in children with both end-stage renal disease (ESRD) and severe hepatic disease. However, there is no consensus on whether children with ARPKD-associated ESRD without severe hepatic disease can be treated with isolated kidney transplantation (KT) without the need for CLKT. We retrospectively studied the efficacy of isolated KT in children with ARPKD without severe hepatic disease, and followed the course of hepatic disease post KT. This is a single-center study of three children with ARPKD and ESRD who underwent isolated KT. None of them had severe hepatic disease at the time of KT. All children were clinically diagnosed with ARPKD in the immediate postnatal period. All had hepatic fibrosis of varying degrees and two had intrahepatic biliary duct (IHBD) dilatation. None had gastrointestinal (GI) bleed, portal hypertension or cholangitis. Two children had preemptive KT. Pre-transplant unilateral or bilateral native nephrectomy were performed for two children, and one underwent unilateral native nephrectomy at the time of KT. The median creatinine clearance at a median post-KT follow-up of 24 months was 60.3 mL/min/1.73 m². The two-year graft and patient survival were both 100%. Post KT, all three patients continued to demonstrate evidence of hepatic fibrosis and IHBD on sonogram; however, none of them were either evaluated for or required liver transplantation given normal synthetic liver function and absence of portal hypertension or other severe hepatobiliary disease. There were no adverse events observed such as cholangitis, GI bleed, or multiorgan failure. Hence, an excellent short-term graft and patient survival was demonstrated in this study of children with ARPKD and mild to moderate hepatic disease who received isolated KT. Long-term follow-up and larger studies are important to assess the efficacy of isolated KT in this subset of children with ARPKD. Full article

Introduction. Pancreatic ductal adenocarcinoma (PDA) is associated with poor prognosis and 98% loss-of-life expectancy. 80% of patients with PDA are unfit for radical surgery. In those cases, emphasis is set on management of cancer-related symptoms, among which obstructive jaundice is most common. Endoscopic ultrasound-guided biliary drainage (EUS-BD) emerges as a valid alternative to the well-accepted methods for treatment of biliary obstruction. Patient Selection. Five consecutive patients with unresectable pancreatic malignancy, were subjected to EUS-BD, particularly EUS-guided choledochoduodenostomy (EUS-CDS). Ethics. Oral and written informed consent was obtained in all cases prior procedure. Technique. EUS-guided puncture of the common bile duct was performed, followed by advancement of a guidewire to the intrahepatic bile ducts. After dilation of the fistulous tract with a cystotome, a fully covered self-expandable metal stent was inserted below the hepatic confluence and extending at least 3 cm in the duodenum. Technical and clinical success was achieved in four patients without adverse events. In one patient procedure failed due to dislocation of the guidewire, with consequent biliary leakage requiring urgent surgery. Recovery was uneventful with no further clinical sequelae and there was no mortality associated with procedure. Discussion. Introduced in 2001, EUS-guided biliary drainage has become an accepted option for treatment of obstructive jaundice. According to recent guidelines published by European Society of Gastrointestinal Endoscopy (ESGE) in 2022, EUS-CDS is a preferred modality to percutaneous transhepatic biliary drainage (PTBD) and surgery in patients with failed ERCP, with comparable efficiency and better safety profile, which is supported by our experience with the procedure. Conclusions. Our case series suggests that EUS-CDS is an excellent option for palliative management of malignant distal biliary obstruction, emphasizes on the importance of adequate technique and experience for the technical success, and urges the need for future research on establishing the best choice for guidewire and dilation device. Full article

Introduction: Portal cavernoma cholangiopathy (PCC)’ refers to abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension. Although there is data on clinical and imaging aspects of PCC, the description of liver pathology has been strikingly deficient. The purpose of this study was to examine the histopathological characteristics of PCC. Patients and Methods: A retrospective study of patients clinically diagnosed with extrahepatic portal vein obstruction (EHPVO) with portal cavernoma cholangiopathy, was conducted. Vascular anatomy was characterized by computerized tomographic angiography, and endoscopic retrograde cholangiography (ERC) and magnetic resonance cholangiography (MRC) were used to characterize the biliary anatomy. Histological features were analyzed by two hepatopathologists in a blinded manner, with mutual discussion to resolve any discrepancies. Results: A total of 50 patients with portal cavernoma cholangiopathy were included in the study. The mean age of the patients was 26.2 ± 11.6 years. Radiologically, bilobar intrahepatic biliary dilatation was seen in 98% with common bile duct abnormality in 100% of patients, along with extrinsic ductal impressions in 77 % of cases. Liver tests were deranged total bilirubin 1.5 mg/dL (IQR 0.8–2.4) and alkaline phosphatase 109.5 IU/L (IQR 70–193). Histologically; dilated multiple portal venous channels (72%), hepatic artery thickening (70%). The presence of aberrant vascular channels around portal tracts (54%), elastosis of portal veins (50%), and bile ductular reaction in (44%) were the other prominent findings. A 12% of cases show focal thin bridges. Advanced fibrosis was not seen in any of the cases. One-fourth of the cases showed concomitant minimal to mild hepatocyte steatosis. Conclusions: Histologically, intrahepatic portal vein and portal tract abnormalities were noted in cases with portal cavernoma cholangiopathy, associated with mild derangement of liver tests. Full article

Background and Objectives: Choledocal cyst is a rare congenital disease of the biliary tree defined by dilatation of the extrahepatic and/or intrahepatic biliary ducts. Untreated, it leads to complications such as cholangitis, stone formation and malignant degeneration. The standard treatment for choledocal cyst is complete excision and subsequent biliary reconstruction via hepaticojejunostomy or hepatiocoduodenostomy. Materials and Methods: We report our experience with 16 pediatric cases of choledocal cyst over a 10-year period. Results: The predominant symptoms were nausea and jaundice, both at 62.5% (n = 10), followed by abdominal pain at 56.3% (n = 9). Ultrasonography was the diagnostic method used in all patients. Computed tomography was used in 75% (n = 12) and magnetic resonance imaging in 25% (n = 4) of cases. Age at the time of intervention ranged from 2 months to 17 years with a mean of 4 years and 5 months. The open approach was used in nine patients and the laparoscopic approach was used in seven patients, with one conversion to open surgery. Complete excision of the choledocal cyst was performed in 15 cases (93.7%), and partial excision with mucosectomy was performed in one case (6.2%). Eight patients (50%) underwent hepaticoduodenostomy and eight (50%) underwent hepaticojejunostomy, out of which one was attempted laparoscopically but was converted. We had a postoperative complication rate of 12.5% (n = 2) represented by anastomotic leak and pancreatitis. Conclusions: From our experience with these cases, we concluded that a wide hepaticoduodenostomy constitutes a favorable choice over the traditional hepaticojejunostomy, being more physiological and less time consuming. Full article

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary hepatic malignancy, with mass-forming growth pattern being the most common. The typical imaging appearance of mass-forming ICC (mICC) consists of irregular ring enhancement in the arterial phase followed by the progressive central enhancement on portal venous and delayed phases. However, atypical imaging presentation in the form of hypervascular mICC might also be seen, which can be attributed to distinct pathological characteristics. Ancillary imaging features such as lobular shape, capsular retraction, segmental biliary dilatation, and vascular encasement favor the diagnosis of mICC. Nevertheless, these radiological findings may also be present in certain benign conditions such as focal confluent fibrosis, sclerosing hemangioma, organizing hepatic abscess, or the pseudosolid form of hydatid disease. In addition, a few malignant lesions including primary liver lymphoma, hemangioendothelioma, solitary hypovascular liver metastases, and atypical forms of hepatocellular carcinoma (HCC), such as scirrhous HCC, infiltrative HCC, and poorly differentiated HCC, may also pose a diagnostic dilemma by simulating mICC in imaging studies. Diffusion-weighted imaging and the use of hepatobiliary contrast agents might be helpful for differential diagnosis in certain cases. The aim of this manuscript is to provide a comprehensive overview of mICC imaging features and to describe useful tips for differential diagnosis with its potential mimickers. Full article

The concomitant occurrence of duodenal atresia (DA) and a choledochal cyst (CC) has rarely been reported. Knowledge of both the presentation and management of this rare co-occurrence is imperative in avoiding potential complications and sequelae, such as biliary metaplasia. Herein we describe a female infant born at 32 weeks gestational age who was diagnosed with duodenal atresia and annular pancreas postnatally, who had subsequent findings of malrotation and a choledochal cyst, as seen from contrast imaging. Uncomplicated repair of the DA and obstruction was performed at 4 days of life. She re-presented 2 years later with non-bloody, nonbilious emesis and was found to have elevated amylase, lipase and liver enzymes. Imaging revealed dilated intra-hepatic ducts, a distended gallbladder and a large choledochal cyst. She underwent a cholecystostomy tube placement followed by a definitive choledochal cyst excision with immediate improvement following surgery and full resolution of symptoms before discharge. Full article

Isolated dilation of common bile duct (CBD) with normal sized pancreatic duct and without identifiable stones or mass lesion (unexplained) is frequently encountered by computed tomography/magnetic resonance imaging. We studied the final diagnoses in these patients and tried to elucidate factors that can predict a malignant etiology. This is a retrospective analysis of prospective database from a University based clinical practice (2002- 2008). We included 107 consecutive patients who underwent endoscopic ultrasound (EUS) for evaluation of isolated and unexplained CBD dilation noted on contrast computed tomography scans. EUS examination was performed using a radial echoendoscope followed by a linear echoechoendoscope, if a focal mass lesion was identified. Fine-needle aspirates were assessed immediately by an attending cytopathologist. Main outcome measurements included i) prevalence of neoplasms, CBD stones and chronic pancreatitis and ii) performance characteristics of EUS/EUS-fine needle aspiration (EUS-FNA). A malignant neoplasm was found in 16 patients (14.9%) of the study subjects, all with obstructive jaundice (ObJ). Six patients had CBD stones; three with ObJ and three with abnormal liver function tests. EUS findings suggestive of chronic pancreatitis were identified in 27 patients. EUSFNA had 97.3% accuracy (94.1% in subset with ObJ) with a sensitivity of 81.2% and specificity of 100% for diagnosing malignancy. Presence of ObJ and older patient age were only significant predictors of malignancy in our cohort. Amongst patients with isolated and unexplained dilation of CBD, the risk of malignancy is significantly higher in older patients presenting with ObJ. EUS-FNA can diagnose malignancy in these patients with high accuracy besides identifying other potential etiologies including missed CBD stones and chronic pancreatitis. Full article