Study of New Imaging Criteria for the Diagnosis of Caroli's Disease

Study Overview
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Brief Summary
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The Caroli disease is a very rare pathology that can be revealed early in childhood or in adulthood, whose diagnosis is based on Magnetic Resonance CholangioPancreatography, which shows the communication of these malformations with the rest of the biliary tree and allows to eliminate biliary stenosis. The radiologist has a central role in the diagnostic orientation between malformative intra-hepatic bile duct dilatation and obstructive benign or malignant intra-hepatic bile duct dilatation dilatation. However, imaging of Caroli disease is polymorphous and therefore subject to misinterpretation. The benefit of this research is to reduce diagnostic errors by highlighting imaging criteria specific to the Caroli disease

Detailed Description
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The Magnetic Resonance CholangioPancreatography will have been performed in all patients using different Magnetic Resonance Imaging systems (Gyroscan Intera, Philips Medical Systems, Best in the Netherlands, Magnetom Avanto, Siemens Medical Solutions, and Signa Hdxt, General Electric Medical Systems) with a free-breathing three-dimensional high-spatial-resolution Fast Spin Echo sequence and/or a breath-hold two-dimensional single-shot sequence. Magnetic Resonance CholangioPancreatography may also include a Fast Spin Echo weighted T2-weighted sequence with fat-spectral saturation, a T1-weighted breath-suppressed gradient echo pulse sequence and a chemical shift sequence in phase and opposite phase, in the axial plane, and a weighted three-dimensional T1 saturated fat sequence after intravenous administration of gadolinium chelate. Images will be analyzed in consensus by two abdominal radiologists on a Picture Archiving and Communication System station and compared with the pathological results.

Official Title
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Magnetic Resonance CholangioPancreatography Signs for the Diagnosis of Caroli Disease

Conditions
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Caroli Disease, Magnetic Resonance Imaging, Cholangiopancreatography

Intervention / Treatment
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* Diagnostic Test: Magnetic Resonance CholangioPancreatography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: were patients identified in the database of the hospitals who underwent a diagnosis of Caroli disease on an imaging report with bile duct dilatation limited to the intrahepatic biliary tree and an available Magnetic Resonance CholangioPancreatography on a Picture Archiving and Communication System. Exclusion Criteria: obstructive benign or malignant proximal biliary stricture on Magnetic Resonance CholangioPancreatography prior surgical interventions for hepatobiliary disease except cholecystectomy
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Diagnostic Test: Magnetic Resonance CholangioPancreatography|The imaging data will be collected from the hospital records and image archiving system of the radiology departments of the University Hospitals|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Intra-hepatic bile duct dilatation | study of intra-hepatic bile duct shape (qualitative measurement) | 2 years | 
| Intra-hepatic bile duct dilatation | intra-hepatic bile duct localisation (qualitative measurement) | 2 years | 
| Intra-hepatic bile duct dilatation | biliary calculi (qualitative measurement) | 2 years | 
| Intra-hepatic bile duct dilatation | dot sign (qualitative measurement) | 2 years | 
| Intra-hepatic bile duct dilatation | liver abnormalities (qualitative measurement) | 2 years | 
| Intra-hepatic bile duct dilatation | signs of portal hypertension (qualitative measurement) | 2 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Pathological examination | Absence or presence of tumors | 2 years | 
| Pathological examination | Absence or presence of calculi | 2 years | 
| Biology | bilirubin level | 2 years | 
| Biology | Alkaline phosphatase level | 2 years | 
| Biology | Creatinine level | 2 years |