Cacchi-ricci and Caroli: Both Rare but Frequently Associated

Aims: Caroli and Cacchi Ricci are two rare diseases but frequently associated. We report the case of a young girl who presented these exceptional entities.

Results: We report the case of a 14-year-old girl with no particular pathological history, who presented to the emergency room with rectal bleeding that had been evolving for 1 months. The association of Cacchi Ricci and Caroli diseases was diagnosed on imaging (abdominal ultrasound and computed tomogrphy, bili-MRI), following an assessment carried out in the face of signs of portal hypertension and gastrointestinal bleeding.

Conclusion: Although both rare, Cacchi Ricci disease and Caroli syndrome are frequently associated. This association is to be watched all the more since it increases overall morbidity and mortality and often requires consideration of double hepatorenal transplantation as the only curative therapeutic alternative.