Diagnosis of Caroli’s Disease: a Rare Case Report

Bangladesh Medical Journal Pub Date : 2024-01-02 DOI:10.3329/bmj.v51i3.70139

Lubana Akram, R. Alam, Md Rukunuzzaman

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Abstract

Caroli’s disease is a rare disease caused by Ductal Plate malformation. Patients may present with history of intermittent abdominal pain, pruritus and/ or symptoms of cholangitis. Cholangitis, liver cirrhosis, and cholangiocarcinoma are potential complications. It is rarely diagnosed in childhood. A six (06) years old was presented with intermittent abdominal pain, fever, pruritus, and hepatomegaly at the Department of Paediatric Gastroenterology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. After evaluating history, signs/ symptoms, physical examination, biochemical, ultrasonographic and endoscopic !ndings; this case was provisionally diagnosed as a compensated chronic liver disease with portal hypertension due to cause of cholestasis. Finally magnetic resonance cholangiopancreatography (MRCP) was done for further evaluation and the diagnostic !ndings of MRCP was suggestive of Caroli’s disease. After consultation the MRCP report with Pediatric Surgery Department of BSMMU the patient was advised for liver transplantation due to multifocal involvement of liver. Lastly patient was discharged with supportive management. Bangladesh Med J. 2022 Sept; 51(3): 43-46

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卡洛里氏病的诊断：罕见病例报告

卡洛里氏病是一种由导管板畸形引起的罕见疾病。患者可能会出现间歇性腹痛、瘙痒和/或胆管炎症状。胆管炎、肝硬化和胆管癌是潜在的并发症。这种疾病很少在儿童时期确诊。孟加拉国达卡班加班杜-谢赫-穆吉布医科大学（BSMMU）儿科消化内科接诊了一名因间歇性腹痛、发热、瘙痒和肝肿大而就诊的六（06）岁儿童。在对病史、体征/症状、体格检查、生化检查、超声波检查和内窥镜检查结果进行评估后，该病例被初步诊断为因胆汁淤积引起的代偿性慢性肝病伴门脉高压症。最后做了磁共振胰胆管造影术（MRCP）以进一步评估，MRCP 的诊断结果提示为卡洛里氏病。BSMMU小儿外科部门对MRCP报告进行会诊后，建议患者进行肝脏移植手术，因为肝脏多灶性受累。最后，患者在接受支持性治疗后出院：43-46

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Bangladesh Medical Journal

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