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

Magnetic Resonance CholangioPancreatography Signs for the Diagnosis of Caroli Disease

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

Study Overview

• Status: Completed
• Conditions: Magnetic Resonance Imaging; Caroli Disease; Cholangiopancreatography
• Intervention / Treatment: Diagnostic test: Magnetic Resonance CholangioPancreatography

Detailed Description

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.

• Study Type: Observational
• Enrollment (Actual): 61

Contacts and Locations

Study Locations

• France
  • Le Kremlin-Bicêtre, France, 94804
    • Maite Lewin

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: ADULT; OLDER_ADULT; CHILD
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients with illness or Caroli syndrome

Description

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

Study Plan

How is the study designed?

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: LEWIN MAITE, MD, PhD, Paul Brousse Hospital

Study record dates

Study Major Dates

• Study Start (ACTUAL): March 18, 2020
• Primary Completion (ACTUAL): September 1, 2020
• Study Completion (ACTUAL): December 1, 2020

Study Registration Dates

• First Submitted: June 4, 2019
• First Submitted That Met QC Criteria: July 2, 2019
• First Posted (ACTUAL): July 5, 2019

Study Record Updates

• Last Update Posted (ACTUAL): November 16, 2021
• Last Update Submitted That Met QC Criteria: November 15, 2021
• Last Verified: November 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Digestive System Diseases; Congenital Abnormalities; Genetic Diseases, Inborn; Biliary Tract Diseases; Abnormalities, Multiple; Bile Duct Diseases; Ciliopathies; Digestive System Abnormalities; Choledochal Cyst; Caroli Disease
• Other Study ID Numbers: APHP190470

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No