Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.
Alagille Syndrome ; Bile ; Bile Canaliculi ; Cholelithiasis ; Cholestasis* ; Diagnosis ; Diagnosis, Differential ; Hepatitis ; Hepatolenticular Degeneration ; Humans ; Jaundice ; Pruritus

Agenesis of the right lobe of the liver is an extremely rare anomaly of the liver, and few cases are reported in the literature. Most of the patients with this anomaly are accompanied by additional anormalies such as retrohepatic or suprahepatic gallbladder and other biliary tract diseases, including cholelithiasis, carcinoma of the gallbladder and portal hypertension. The diagnosis of this rare anatomical variant was established by ultrasonography and computed tomography. The radiological findings, clinical presentation, and differential diagnosis are reviewed.
Biliary Tract Diseases ; Cholelithiasis ; Diagnosis ; Diagnosis, Differential ; Gallbladder ; Humans ; Hypertension, Portal ; Liver* ; Ultrasonography

A 24-year-old lady with recurrent upper abdominal pain, underwent surgery for cholelithiasis based on imaging diagnosis by ultrasound scanning. At laparotomy, the gall bladder could not be found either in its normal or ectopic locations. The diagnosis of agenesis of the gall bladder was confirmed by operative cholangiography. Duplication cysts of the hepatic flexure were discovered in the position normally occupied by the gall bladder. The stony hard faeces in the cysts were probably interpreted as gallstones on ultrasound. This rare condition is discussed and the importance of intraoperative cholangiography is stressed.
Adult ; Cholelithiasis ; diagnosis ; Colonic Diseases ; complications ; pathology ; Cysts ; complications ; pathology ; Diagnosis, Differential ; Female ; Gallbladder ; abnormalities ; Humans

PURPOSE: To assess the accuracy of MR cholangiography(MRC) in the diagnosis of cholelithiasis, and to determine interobserver agreement. MATERIALS AND METHODS: Between March and September 1999, 43 consecutive patients with biliary obstruction [24 men and 19 women aged 25 -85 (mean, 58) years] underwent MRC using the single-shot fast spin-echo technique. Heavily T2-weighted source images(axial and coronal) 3 -5 mm thick and 12 projection images with 15- degree rotation and 5-cm thickness were obtained. All images were reviewed blindly and indepen-dently by two radiologists specialized in the interpretation of abdominal imaging information. Choledocholithiasis was evaluated in eight segments of the intrahepatic duct(IHD), extrahepatic duct(EHD) and gall bladder lumen. Final diagnosis was established on the basis of operative (n=31) and other radiological (n=12) findings. The sensitivity, specificity and accuracy of the MRC findings were assessed, and using kappa measurement (cross-table analysis,SPSS Windows for 8.0), interobserver agreement was determined. RESULTS: Thirty of the 43 patients, had choledocholithiasis (IHD stones in 7 cases, EHD stones in 15, and GB stones in 18). For radiologist 1, sensitivity, specificity and accuracy were 86%, 100% and 98%, respectively, in the diagnosis of IHD stones; 100%, 89% and 93%, respectively, in the diagnosis of EHD stones; and 81%, 96% and 91%, respectively, in the diagnosis of GB stones. For radiologist 2, the corresponding figures were 86%, 94% and 93% (1HD stones); 87%, 89% and 88% (EHD stones); and 81%, 86% and 84% (GB stones). Interobserver agreement for the diagnosis of choledocholithiasis was excellent in all cases. The kappa mesurement was 0.91 for 1HD stones, 0.77 for EHD stones, and 0.70 for GB stones. CONCLUSION: MRC is an excellent imaging modality for the diagnosis of choledocholithiasis, and interobserver agreement was also excellent.
Cholangiography* ; Choledocholithiasis ; Cholelithiasis* ; Diagnosis* ; Female ; Humans ; Male ; Sensitivity and Specificity ; Urinary Bladder

Various complications following laparoscopic cholecystectomy have been reported. We describe a case of intraabdominal abscess formation which was developed two months after the inadvertently spilling of gallstones laparoscopic cholecystectomy in a patient with acute cholelithiasis. The condition was initially found on computed tomography and the diagnosis was confirmed with ultrasound. Although this is a rare complication of laparoscopic cholecystectomy, the spilling of gallstones should be recognized as a potential source of intra-abdominal abscess formation even in a patient presenting months after laparoscopic cholecystectomy. We suggest that routine use of the specimen retrieval bag is highly recommended especially for beginners of laparoscopic cholecystectomy during their initial learning period.
Abdominal Abscess ; Abscess* ; Cholecystectomy, Laparoscopic* ; Cholelithiasis ; Diagnosis ; Gallstones* ; Humans ; Learning ; Postoperative Complications ; Ultrasonography

A small-cell carcinoma of the gallbladder is an uncommon cancer with high malignancy potential. It is usually found in elderly women and is associated with cholelithiasis. These tumors show an aggres sive clinical course and death usually occurs within a few months after diagnosis. The diagnosis of a small-cell carcinoma should be based on cell morphology, histologic pattern, and immunohistochemical study. Correct diagnosis is important not only because these tumors give rise to endocrine syndrome but also because the chemotherapeutic approach to a small-cell carcinoma differs from that for an adeno carcinoma. We experienced a case of a small-cell carcinoma of the gallbladder in a 73-year-old woman with acute cholecystitis syndrome. She was treatment with a palliative cholecystectomy and chemotherapy with short-term excellent response. The literature on the subject is reviewed.
Aged ; Carcinoma, Small Cell* ; Cholecystectomy ; Cholecystitis, Acute ; Cholelithiasis ; Diagnosis ; Drug Therapy ; Female ; Gallbladder* ; Humans

Congenital absence of the gallbladder is a rare anomaly and an interesting subject. The overall incidence of gallbladder agenesis is said to approximately 0.035% to 0.065%. It is extremely difficult to make the correct diagnosis of gallbladder agenesis preoperatively in symptomatic patients because its clinical and radiological features are like those of cholecystitis, cholelithiasis, or choledocholithiasis in patients with anatomically normal biliary tracts. Gallbladder agenesis is suspected when the surgeon or the pathologist has failed to identify the gallbladder in its usual position but ectopic location or necrosis of the gallbladder from any causes should be ruled out. Recently, two cases of gallbladder agenesis were encountered in adult patients. We summarize our experience and give a brief review of the literature.
Adult ; Biliary Tract ; Cholecystitis ; Choledocholithiasis ; Cholelithiasis ; Diagnosis ; Gallbladder* ; Humans ; Incidence ; Necrosis

Small cell carcinoma of the gallbladder (GB) is a very rare tumor, found usually in elderly women and associated with cholelithiasis. It carries a grave prognosis, metastasizing early and causing death shortly after diagnosis. We experienced a case of small cell carcinoma of the GB with metastasis to the liver and adjacent lymph nodes in a 67-year-old woman. The patient underwent palliative resection and histologically, the tumor was made up of sheets of uniform small round cells with hyperchromatic nuclei, inconspicuous nucleoli and scant cytoplasm, consistent with small cell carcinoma of the GB.
Aged ; Carcinoma, Small Cell* ; Cholelithiasis ; Cytoplasm ; Diagnosis ; Female ; Gallbladder* ; Humans ; Liver ; Lymph Nodes ; Neoplasm Metastasis ; Prognosis

We have reported a case of Mirizzi syndrome preoperatively diagnosed using MR cholangiopancreatography. MRCP and T2-weighted image using a single-shot fast spin-echo sequence accurately depicted all components of Mirizzi syndrome, including impacted stone in the neck of the gallbladder compressing the common hepatic duct and wall-thickening of the gallbladder without any evidence of malignancy. The combination of MRCP and T2-weighted image can be counted on to replace conventional modalities of diagnosing Mirizzi syndrome without any loss of diagnostic accuracy.
Bile Duct Diseases/etiology* ; Bile Duct Diseases/diagnosis ; Case Report ; Cholangiopancreatography, Endoscopic Retrograde ; Cholelithiasis/diagnosis ; Cholelithiasis/complications* ; Constriction, Pathologic/etiology ; Gallbladder/pathology ; Hepatic Duct, Common* ; Human ; Magnetic Resonance Imaging* ; Male ; Middle Age ; Syndrome

BACKGROUNDS/AIMS: Gallbladder perforation is a rare but potentially fatal disease. We herein present our clinical experience in diagnosis and management of 32 cases of gallbladder perforation. METHODS: This retrospective study was conducted with inclusion of all cases of gallbladder perforation that presented to our hospital from January 2012 to November 2014. Cases of traumatic gallbladder perforation and patients younger than 12 years of age were excluded from this study. RESULTS: This study included 32 patients (13 males and 19 females). The mean age of patients was 55.9 years. Gallbladder perforation was most common in the 5th and 6th decade of life. The mean age of patients with type I, II, and III gallbladder perforation was 57.0 years, 57.6 years, and 49.8 years, respectively. The most common site of perforation was the fundus, followed by the body and Hartmann's pouch (24 : 5 : 2). Most of the type I gallbladder perforations were diagnosed intraoperatively, type II gallbladder perforations were diagnosed by enhanced abdominal computed tomography, and type III gallbladder perforations were diagnosed during laparoscopic cholecystectomy converted to open cholecystectomy for cholelithiasis. Mortality was highest in patients with type I gallbladder perforation. The mean hospital stay was 10.1 days, 6.4 days, and 9.2 days in patients with type I, II, and III gallbladder perforation, respectively. The histopathologic analysis in 28 patients who were operated on showed acute cholecystitis in 19 cases, acute-on-chronic cholecystitis in 4 cases, chronic cholecystitis in 4 cases, and mucinous adenocarcinoma of the gallbladder in a single case. CONCLUSIONS: Gallbladder perforation represents a special diagnostic and surgical challenge. Appropriate classification and management are essential.
Adenocarcinoma, Mucinous ; Cholecystectomy ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Cholecystitis, Acute ; Cholelithiasis ; Classification ; Diagnosis ; Gallbladder* ; Humans ; Length of Stay ; Male ; Mortality ; Retrospective Studies