scholarly journals Caroli Disease

2007 ◽  
Vol 82 (2) ◽  
pp. 208
Author(s):  
Nicola Mumoli ◽  
Marco Cei
Keyword(s):  
Caroli Disease

scholarly journals Imaging manifestations of Caroli disease with autosomal recessive polycystic kidney disease: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiuzhen Yao ◽  
Weiqun Ao ◽  
Jianhua Fang ◽  
Guoqun Mao ◽  
Chuanghua Chen ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Magnetic Resonance ◽  
Kidney Disease ◽  
Portal Vein ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney ◽  
Left Liver Lobe ◽  
Caroli Disease ◽  
T1 And T2

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.

Nephronophthisis 13: implications of its association with Caroli disease and altered intracellular localization of WDR19 in the kidney

2015 ◽  
Vol 30 (9) ◽  
pp. 1451-1458 ◽  
Author(s):  
Jiwon M. Lee ◽  
Yo Han Ahn ◽  
Hee Gyung Kang ◽  
II Soo Ha ◽  
Kyoungbun Lee ◽  
...  
Keyword(s):  
Intracellular Localization ◽  
Caroli Disease

Caroli Disease Associated With Vein of Galen Malformation in a Male Child

PEDIATRICS ◽  
2014 ◽  
Vol 134 (1) ◽  
pp. e284-e288 ◽  
Author(s):  
D. Grieb ◽  
A. Feldkamp ◽  
T. Lang ◽  
M. Melter ◽  
C. Stroszczynski ◽  
...  
Keyword(s):  
Male Child ◽  
Vein Of Galen ◽  
Vein Of Galen Malformation ◽  
Caroli Disease

scholarly journals Large-duct cholangiopathies: aetiology, diagnosis and treatment

2019 ◽  
Vol 10 (3) ◽  
pp. 284-291 ◽  
Author(s):  
Shyam Menon ◽  
Andrew Holt
Keyword(s):  
Bile Flow ◽  
Biliary Tree ◽  
Biliary Cirrhosis ◽  
Caroli Disease ◽  
End Stage Liver Disease ◽  
Recurrent Pyogenic Cholangitis ◽  
Chronic Cholestasis ◽  
End Stage ◽  
Extrahepatic Biliary Tree ◽  
Portal Biliopathy

Cholangiopathies describe a group of conditions affecting the intrahepatic and extrahepatic biliary tree. Impairment to bile flow and chronic cholestasis cause biliary inflammation, which leads to more permanent damage such as destruction of the small bile ducts (ductopaenia) and biliary cirrhosis. Most cholangiopathies are progressive and cause end-stage liver disease unless the physical obstruction to biliary flow can be reversed. This review considers large-duct cholangiopathies, such as primary sclerosing cholangitis, ischaemic cholangiopathy, portal biliopathy, recurrent pyogenic cholangitis and Caroli disease.

Radionuclide hepatobiliary imaging in congenital biliary tract ectasia (Caroli disease)

1982 ◽  
Vol 12 (3) ◽  
pp. 111-114 ◽  
Author(s):  
J. R. Sty ◽  
A. M. Hubbard ◽  
R. J. Starshak
Keyword(s):  
Biliary Tract ◽  
Hepatobiliary Imaging ◽  
Caroli Disease

scholarly journals Diffuse Oligosymptomatic Caroli’s Disease: Case Report

2020 ◽  
Vol 31 (2) ◽  
pp. 107-110
Author(s):  
Lemfadli Y ◽  
Bouchrit S ◽  
Ait Errami A ◽  
Oubaha S ◽  
Samlani Z ◽  
...  
Keyword(s):  
Case Report ◽  
Female Patient ◽  
Bile Ducts ◽  
Clinical Case ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Caroli Disease ◽  
Intrahepatic Bile Ducts ◽  
Disease Case

This article describes a case of Caroli’s disease in a 53-year-old female patient who complained nonspecificabdominal pain without cholestasis or cholangitis. Ultrasound and hepatic magnetic resonanceimaging showed segmental saccular dilations connected to intrahepatic bile ducts without hepaticfibrosis. This clinical case shows the possibility of having oligosymptomatic forms in the diffuse formsof Caroli disease, therefore the interest to consider this diagnosis in case of non-specific abdominalsigns and to request a hepatic ultrasound. Bangladesh J Medicine July 2020; 31(2) : 107-110

Complications in Caroli Disease

2013 ◽  
Vol 108 ◽  
pp. S350-S351 ◽  
Author(s):  
Jennifer Horsley-Silva ◽  
Katherine Duello ◽  
Michael Phillips
Keyword(s):  
Caroli Disease

Caroli disease in magnetic resonance imagining

2013 ◽  
Vol 26 (2) ◽  
pp. 152-154
Author(s):  
Franciszek Burdan ◽  
◽  
Agnieszka Mocarska ◽  
Lidia Kołodziej ◽  
Marzena Janczarek ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Caroli Disease ◽  
Magnetic Resonance Imagining

Massive left lobe primary hepatolithiasis with extremely high CA 19.9

2013 ◽  
Vol 19 (S4) ◽  
pp. 37-38
Author(s):  
A. Murinello ◽  
P. Guedes ◽  
A.M. Carvalho ◽  
J.S. Coelho ◽  
B.B. Leite ◽  
...  
Keyword(s):  
Bile Duct ◽  
Epigastric Pain ◽  
Acute Cholangitis ◽  
Intrahepatic Bile Duct ◽  
Left Lobe ◽  
Left Hepatectomy ◽  
Caroli Disease ◽  
Acute Calculous Cholecystitis ◽  
Ultrasound Study ◽  
Calculous Cholecystitis

Hepatolithiasis (HL) or intrahepatic calculi is an uncommon condition in Western countries, with a prevalence under 1%. It is much more frequent in East Asia, reaching 20% in China and Taiwan, up to 50% of which show associated cholelithiasis. The authors report a European patient with primary HL of the left hepatic lobe, 12 years after cholecystectomy for acute calculous cholecystitis, now successfully treated after left lobe hepatectomy.A 63-year-old caucasian man with a past medical history of hypertension, coronary artery disease, dyslipidaemia and urgent cholecystectomy 12 years ago (due to an acute calculous cholecystitis without ultrasonographic or perioperative evidence of bile duct dilation or choledocholithiasis, and with no cholangitis ever since) was now admitted to our ward following a 2-day course of fever (39ºC), epigastric pain, nausea, vomiting and tender right upper abdominal quadrant.Bloodwork showed mild leukocytosis with neutrophilia and low platelets; elevated C-reactive protein, γGT, alkaline phosphatase and LDH; normal AST, ALT, bilirubin and amylase. Negative HBV, HCV and HIV serologies. CA19.9 was notably high (7500 U/mL), 200x above normal range (N≤37). A diagnosis of acute cholangitis was assumed on clinical and laboratory basis, despite the absence of jaundice. The patient began therapy with iv ceftriaxone and improved clearly over the next few days. Raised CA19.9 in the setting of acute cholangitis, however, forced us into further study directed at the possibility of underlying biliary or pancreatic malignancy.Abdominal ultrasound disclosed multiple calcifications in the left lobe. CT displayed several left lobe intrahepatic bile duct dilations but no calcifications, thus suggesting intrahepatic cholesterol stones and cholangitis due to an obstacle before the hilum. MR-cholangiography was performed, showing marked dilation of the left intrahepatic bile duct and moderate dilation in the extrahepatic portion of the common bile duct (CBD). Neither exam showed any sign of cancer. Surgery was the therapeutic approach, and the patient underwent a left hepatectomy.Intra-operative ultrasound study unveiled severe dilation of the left intrahepatic bile duct, which was filled with gallstones, but no choledocholithiasis and no tumour whatsoever. Both the surgical procedure and the post-operative period were uneventful.Macroscopic liver section showed multiple dilated bile ductules containing fragile yellowish-green gallstones (Fig.1). Microscopy revealed cystic dilations of the left intrahepatic biliary tree packed with intraluminal gallstones (Fig.2), fibrosis and moderate chronic inflammatory infiltrate with lymphoid aggregates (Fig.3). Some areas of the epithelium displayed erosion and reactive changes. No biliary neoplasm was found.Immunocytochemistry for angiogenesis or lymphocyte membrane markers are not available in our Hospital. Soon after surgery, CA19.9 values decreased abruptly and just 4 months after the left hepatectomy they were back within reference range.Our working hypotheses were primary hepatolithiasis (HL), secondary HL and Caroli disease.Secondary HL, due to stone migration from the CBD, was discarded because calculi in the CBD were absent in the cholecystectomy 12 years ago and in ultrasonographic studies both then and now. Caroli disease, a congenital condition characterised by intrahepatic bile duct dilation, was contradicted by the ultrasonography in 2000 showing normal bile duct dimension. Excluding these two entities made primary idiopathic HL our final diagnosis.The patient is now asymptomatic, having returned to his normal life with no limitations.This report showcases a rare entity known as primary hepatolithiasis, usually causing recurrent cholangitis in older patients of Asian descent but seldom seen in Europe. The steep increase in CA19.9 related to cholangitis, although previously reported, is also very uncommon. Another interesting aspect is the conspicuous inexistence, over 12 years, of recurrent episodes of acute cholangitis (a traditional finding in hepatolithiasis). These three features help to compose this most peculiar case.

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