Delay in Diagnosis of Caroli’s Disease: About a First Case in Internal Medicine at Donka University Hospital

Caroli disease is a rare congenital condition characterized by multifocal segmental dilatation of the intrahepatic bile ducts, described in 1958 by Jacques Caroli due to a genetic mutation. It is a cause of chronic cholestasis and intrahepatic lithiasis in children and young adults. We report the case of Caroli’s disease diagnosed in a 36-year-old patient. The onset of symptoms was gradual and began three months prior (January 10, 2023), marked by the onset of jaundice, persistent fever, early postprandial vomiting without any apparent triggering factors, associated with mild epigastric pain without radiation that subsided when vomiting stopped, and prostration. She consulted a health facility in Nongo on January 15, 2023, where a diagnosis of severe malaria was made based on the jaundice, fever, and vomiting. She was treated with artesunate 60 mg, sodium chloride 0.9%, and vogalene 10 mg. After a period of remission, on January 19, 2023, she experienced a fixed, moderate pain in her right hypochondrium without radiation, and non-bloody, watery diarrhea. On January 19, 2023, she was using self-medication based on tramadol 50 mg and amoxicillin 1000mg. Given the persistence of the aforementioned symptoms after an unsuccessful course of treatment since January 10, 2023, she was referred to Internal Medicine on April 20, 2023, hence her hospitalization, admitted for watery diarrhea without mucus or blood without tenesmus or straining more than 4 times at a time, early postprandial food vomiting, jaundice, fixed pain in the right hypochondrium of moderate intensity without triggering or relieving factors, fever, permanent dizziness, not diabetic, nor hypertensive, with a history of pulmonary TB on microscopy (？) in 2016 treated and declared cured. The patient’s general condition was preserved, with normal skin and conjunctiva. Scleral jaundice was noted, but there was no venous circulation, ascites, lower limb edema, or splenomegaly. Physical examination revealed tenderness in the right hypochondrium and epigastrium without a palpable mass, and a normal cardiorespiratory function. Laboratory tests showed cholestasis (Total Bilirubin: 2.3N and Conjugated Bilirubin: 2.3N, Alkaline Phosphatase: 1.4N, GGT: 1.8N), cytolysis (ALT: 3.4N and AST: 4.4N), and viral markers: HBsAg (？), total Anti-HBc (？), Anti-HCV (？). Abdominal and pelvic ultrasound revealed heterogeneous hepatomegaly due to the presence of a biliary cyst in the right lobe and segmental dilation of the intrahepatic bile ducts. She received 0.9% saline, 500 mg